History

Thursday, March 10, 2011 10:22 PM CST

Was my post last week with the question wondering what this year would hold prophetic?? If the year is anything like today or like the past few weeks have been with providers im not sure I want to go fwd! (kidding of course but seriously, ugh!!)

I make this comment really because 2 of my providers in the last month have commented how little time they have and that they dont neccessarily have the time to be effective members of my team - ie one has said (and I dont think it was to be mean it was just being honest which is soemthing I really appreciate alot) that she doesnt have the time to really be a point person but that she would try and the other made the comment that she could really only focus on one area not on multiple areas related to my care and that this would only get worse and she would have even less time soon. Then today while seeing my GC which i'll talk about the appt in a bit she told me that she is passing my care on to a different GC (due to re-organizing of the metabolic clinic and my GC not being a part of that clinic better overall flow) which is really crappy - as much as it sometimes has been really up and down with this GC she has been a part of my care since the beginning (05') and we pretty much agree that we butt heads at times only bc we are equally stubborn, committed and passionate about finding answers and about care. We've always eventually been able to agree to disagree or come around to one or the others side in time and we've made it work. I for one really appreciated her and would give just about anything to keep her on my team. I cant express the disapointment and discouragement I feel about this - change is always (ALWAYS) hard but this probably even more so bc she's so familiar with my care and really the only provider I have besides my PM dr who really takes the time to listen and is ok with not always agreeing. it makes it even worse that the new GC will be a male GC (which I have no problem with male drs actually) but in some areas females are much easier to talk to simply bc they tend to get the female stuff, listen and patience for figuring things out.. I just have this incredibly empty feeling right now and like I wrote in a summary to another person maybe my ability to rationalize this will return and hopefully soon and i'll figure out why this may be good but for now I just am feeling sad about it. Who knew in relation to ones healthcare you'd become attached to some providers - I dont even think in some cases it's attached it's more the being comfortable w/a provider, knowing how they work, and working fairly well together and being able to effectively communicate bc your not uncomfortable. Comfort is definitely a good thing especially in care where everything changes so much and these providers are really an integral part of your life simply due to the nature of the disease and the nature of the treatment you receive through them. I just pray to God this works out better than how I feel about it now. =/ Im sure it will but like anything it's never very easy!

Here essentially is a summary of the appt which was long but very useful and I think w/the GC's help I have been able to identify quite a few things I need to work on related to who to see and what to get done as well as possible things to look at.

I had talked in the past w/my PM dr (pain mngmt) about the blood sugar and blood pressure issues and she in turn had talked to my endocrine dr. So my GC is going to call my Insur CM and ask about ordering a blood pressure and blood sugar monitors and what she needs to do to get these approved. I then would have to use these daily for some time in conjunction with symptoms and keeptrack in the H/A log.

Other recommendations GC had where to do the sleep study and then follow up with Pulmonary to see what their opinion of the breathing issues is – ie are we missing the ‘elephant’ in the room by focusing on Cardiac or cardio-pulmonary causes to this symptoms. Otherwise if Pulmonary had no recommendations then pulmonary and cardiology perhaps work together. I am to use an xopenex inhaler nightly to see if these may improve the breathing symptoms as this was a reccomendation my cardiologist had previously given. My cardiologist nurse is also talking about re-doing the ECHO sooner than may though she is talking to my cardiologist and will get back to me.

I talked to her about maybe finding a new PCP and after talking about it and back and forth she agreed that a new PCP is probably a good idea - not bc my current one isnt good but bc she isnt available/have the time to help manage the care team really and decisions. – we talked about the role my former PCP played and this really brought it into perspective for the two of us what has been missing – ie someone who keeps up on the MPS literature and who is engaged with providers guiding the care and decision making. NOT that my current PCP isnt extremely nice and quite lovely really just that she herself has kind of identified she doesnt feel she really has the time to adequately manage my care and has given me the option to choose a different PCP if I ever wanted. I hate to do it bc I like the staff at this clinic to and am still somewhat undecided but feel like maybe I need to switch? Regardless I probably would interview a PCP who one of my providers gave me the name of and go from there talk to my current PCP about it all again.

Probably the most surprising (besides the shock and dismay of my GC not being my GC anymore =( ) was that my GC had also talked to the 3 infusion nurses and agreed that it probably is time to be evaluated for a Port a Cath/access device and get an opinion on this. – ie does the access team think the benefits out weight the small but very real risks and also will talk to Cardiologist to get his opinion since Ports directly impact cardiac issues if they become problematic. **(update 3/14 --I talked to FMLH last fri and Interventional Radiology does these there so I have an appt this coming fri to go over the different types/risks/pros and see what they think. We dont think it will be a problem just have to see first and get all the eggs in the basket first)***

She also talked to a dietician in her clinic about the headache log/food log and having the dietician come talk to me during infusion clinic about potential ways to improve diet despite the nausea and lack of appetite. Also advised I should talk to the nutritionist at the health store I go to about her suggestions. (this is where I get supplements I take) given I really have a pretty terrible day to day food intake. Sort of funny she brought this up as it is something I have thought of before but there has been so much going on both medical wise and in other ways that I just havent done anything with the thought. Great minds think alike?! :)

One of the other things she recommended talking to my Physical Therapist to see if he had any recommendations to try and improve sleep – given I sleep propped up and do have a good bed (pillow top) but could the being propped up be causing more pressure on hips thus part of the reason for waking up.. and another thing was various types of juices she knows of which we will try since it cant hurt and dont seem to have the side effect to them like orange juice and some really sugary juices do for GI issues.

Pain Mngmt appt today was fine nothing new really just refills and much talk about the budget bill in Madison of which I feel absolutely sick over (this week has been a week to make my stomach roll as far as emotions go :( ). All I can say is people ough to wake up and stop making it about themselves and realize it IS about everyone and how this WILL impact everyone in a negative way except the top 1Ugh just absolute ugh and disgust.

I will end on the note of being happy it is Lent and knowing the knowledge my savior lives and so thus may I!

Erica

1 Peter 1:3 ESV / 5 helpful votes
Blessed be the God and Father of our Lord Jesus Christ! According to his great mercy, he has caused us to be born again to a living hope through the resurrection of Jesus Christ from the dead,

Saturday, March 5, 2011 11:23 PM CST

28 years ago today.... 2 new lives came into this world... Happy Birthday to my dear twin brother!

Just was laying in bed tonight thinking of the past and of the future and couldnt help but wonder what this coming year will hold and even more so what will 5 years, 10 years or even longer from now hold? Worry a little over things like what would happen if I lost my ability to be independent which is the 1 thing that keeps me going, the ability to get up and do as I please each day on my own. Thinking of and wondering if I ever lost my parents (I am sorry for the morbid thoughts sometimes you cant help but think about these things) who would I have to really be there? Sure I have my siblings but would they really be willing to be there for the rough parts and take time for things unplanned? Again you cant help but think of these things. Then I was thinking about my neices and nephews growing up and while I know each will always be a part of my life and I know I have a long while before they are on their own I cant help but think of what a big part of my life they each are. I already see less of my older nephew Quinn due to goings on between his Mom and my brothers/disagreements and miss him terribly and I think about Zander and his 'zander-isms' and how i'll miss these when he gets older and grows out of the stage he is in. I'll miss the 'Auntie Erica's and wanting to come stay and well I guess for now since thankfull this is years away i'll jsut keep praying these kiddos are always a part of my life as much as possible. Since I wont have kids of my own they are the closest to it and fill a huge void and spoiling them while also teaching them is the best and most wonderful gift in the world! I think maybe ive been thinking of this a little lately because there has been so much upheaval with the uncertainties around the WI public employees budget and what will happen with my parents insurane thus my insurance and worrying about that innevitably at times leads to other things even though they are really not to connected. I pray the insurance stays the same as I have such disarray with my providers and many providers being to busy to really want to be an active part or willing to take on issues and with the case manager I have through my insur. now she has been a sounding board as far as some decisions go and in some cases helping me to figure out if I should change some things. She has tried to coordinate most of the core providers to get them communicating on a plan and overall been good when everything else sometimes seems very just messy.

This week I meet with genetics - not on monday after infusion (which really frustrated me since these providers constantly talk about "coordinating care to try and make it better and easier" so instead I see them thurs - even though we know at every 6 month appt that we'll fup again in 6 months. VERY frustrating - my infusion nurse said something ot my GC last week and the GC said something like 'ooh she can reschedule for next month if she wants' - really?? because you dont know im here every monday week in and week out and that I request to be seen on mondays after/during infusion for time sake since I drive from an hour away 1 way?? I am sure this appt thurs will be nice and tense for many reasons beyond this. I do plan to ask them to try and be more involved and for their help bc I have so many health issues going on and really only my PM dr trying to help - everyone else says basically they just dont have the time. =/

I saw neurologist last tues - which was slightly unusual appt - she wants me to retry a medication I was formerly on (midodrine) which I have to run past my Cardiologist due to the cardio-pulmonary breathing issues, lasix and long acting calcium channel blocker. This would be for the headaches. She also told me (second dr in several weeks at this particular hospital) that she doesnt have the time to manage my MRI/spine issues which was unusual I thought since she did this in the past and that clearly is a neurology issue - this to will likely end up falling back on my PM drs lap which seems unfair. I often wonder lately do I just need to find new drs - NOT that I dont like a couple of these drs - I DO like my Neurologist and 1 of my other drs who said as much about the time thing but if they dont have the time to manage my care should I be seeing them? Should I find another specialist? One of my drs did give me a reccomendation of a dr she sees for another non-neurology dr but I need to think about it more and talk to a few people including my PM dr and maybe genetics though that I am unsure about. This dr like the dr she would replace has alot of experience with chronic illness/patients and especially autonomic dysfunction which I have alot of issues with.. Sometimes in these kinds of decisions even when the answer is seemingly staring me right in the eye I still donr really know what is right..

I do have the hip injection scheduled for 3/18 and Ortho for 3/26/a fri.

I will update after the upcoming appts which also includes PMnR and PM dr.
God Bless and many wishes for sweet dreams and care free days,
Love,

Erica

Friday, February 18, 2011 10:09 PM CST

What a week is all I can asy - I dont often feel comflicted on my life and care but the past few days I just feel incredibly odd not even sure what the feeling is - just a mix of frustration at what is and frustration at what isnt but that I wish was. ..

Tues my parents and I did go to the rally against Gov Walker's budget/union proposal which was amazing, exhilirating, tiring and just awe inspiring - simply have expereinced nothing like it before! That day there was estimated to be about 13,000 people and 5,000 in the capitol rotunda and weds, thurs and today the numbers have increased exponentially. Dozens of school districts closed which created an uproar amongst some who are for Gov Walker's proposal and amongst some people not in unions (dont these folks realize busting the unions will only trickle down to them and in the end ONLY benefits the top earners in this state?? I made the comment that 1 is there anyone stopping private sector employee from working in the public sector if they would like the benefits? 2. Private sector makes much more in wages often than does public sector especially in support staff positions which my parents work and 3. These public sector employees have already given up many benefits/raises and their unions are fighting for them - my Dad takes his free time to be President of his union if your not happy w your union maybe more people should get involved. 4. Fox news can not lump every employee into the same pay and benefit bracquets bc each school district and individual union ie teachers/support staff, prison guards/employees, etc bargain and get different insur., pay and pension benefits. And I dont take that statement back bc it is the truth - unions have done so much for this country and people only see the false information out there and that public sector employees do get benefits and they cant look past this. These unions take their spare time to fight for their rights and people need to stand up and do the same.

I believe we are going back to Madison tomorrow- ought to be even crazier though - the Tea party movement is arranging a counter-rally most likealy to try and rile up the pro-union force but I dont think it will work we have been calm, collected and peaceful thus far and I believe this is a force that is united in our message and the message isnt for crazy antics. Our democractic lawmakers have boycotted/left the state in protest against Gov Walkers proposal and so the bill has not been able to move through the assembly or house yet as there needs to be atleast 1 democrat present - go Democrats for finally standing up, uniting and listening to your constituents! At this point no one knows when these democrats will return.

I saw my PM dr on thurs and we spent the majority of the time talking about the provider conference call the other week - her biggest concern was that the insur company by having my care be streamlined through my PCP is trying to limit the providers I see which I said I didnt think that was true. We talked about quite a bit of the call and she was able to clarify some things that I had been really confused about which did provide a bit of relief. I saw my PCP this morning though and she made a comment that she thought my specialists wouldnt see me any longer if I didnt go through her (???) which I thought was slighlt odd bc she clearly doesnt have the time to deal with all their issues before I see them (very confused yet on that aspect) and I still feel my GC should be a bigger part of this picture as she used to be with my former PCP and be available which she is not. I just feel like in the past few years my care since leavin my former PCP has become very haphazard and the only reason these drs communicated this time was bc my CM at insurance company coordinated their call - even my PCP made the comment that she was impressed that many different specialists where on the same call and talking to each other that they all needed to be on a consistent message and plan ie trying to solve the same issues. I guess one thing is my former PCP really reached out to other drs and often told me about different articles and conferences she had read and been at that had information on MPS and I dont get that anymore from my current PCP or any of my drs and I sort of miss that aspect alot. I sort of feel alot disenchanted to be honest. =/ It's like I like these drs and I very much appreciate the strong effort that my PM dr and Cardiol put in to try and reach outside of their specific field in trying to assess and treat me ie cardiol. weights in the MPS, the autonomic issues and willingly treats the cardio-pulmonary issues in treating my cardiac issues and he has never been afraid to give me his opinion of my care in general and I appreciate drs who are willing to do that - it is the same with my PM dr. And by pointing out those 2 drs it's not saying I dont like my PCP and some of my other core drs it's just that I often feel like they dont really know what to do with me bc MPS is so complex and in that I dont fall in to a very well defined category bc I have so many issues that just arent the 'norm'. I think slowly my Insurance CM is working to change this and I never thought I would appreciate that help she gives as much as I do bc it's not that it's overwhelming - it definetly isnt it's just that often you as the patient feel like each dr has a different opinion (even my PCP said as much to this today) and you dont know what or who you are supposed to talk to). I deifnetly just wish I could explain how I feel the past few days.

On a different lighter note I ahd my older nephew last weekend and we went sledding on the hill in my parents backyard for about 2 hours which was killer but extremely fun - when we came in it was about the time my sister and her family came with my younger nephew and new neice. My young nephew walks in upstairs while I was downstairs taking off the outdoor clothes and he yells' "AUNTIE ERICA WHERE ARE YOU!?!?!?!?'", then later on that same night Bella his little sister was whimpering and due to Zander's sensory issues he always covers his ears when she makes even a squeak of a noise and he goes "Bella sto crying, thats not funny" (usually he will say "Bella stop crying" then alternate "Bella it's okay" several times - it's VERY cute! He even gets much closer and watches her facial expressions very closely - I bought him a kid magnifying glass and he has been having a blast with that especially following Molly his dog around and 'examining' her face and tail. Funny and always the cheer I need! :)

More soon -

Erica

Monday, February 14, 2011 7:03 PM CST

Happy Congenital Heart Disease Awareness Day and Valentines Day!

Well what an eventful few weeks weeks for sure - ive kept last weeks update belwo about the provider conference call and will just add on for now - later this week or next I will start a new one altogether. First off I messaged back and forth with my PCP a couple times and am going in to talk with her this coming fri she asked that the appt be 30mins so we have time to talk and so we can square things away with how we will better work together keeping my providers communicating, keeping a consistent message/plan and working together in decision making since up to this point ive really been quite independent and when given provider referrals have really just taken the names and made the appts myself and the same with procedures for the most part. Before when I had my former PCP Dr.Zuccaro and when I worked more closely with LuAnn my GC I relied much more heavily on them for help with these things and on should I see certain providers or should I or should I not have certain things done and since Dr.Z left her practice and I switched drs and since LuAnn and I dont communicate that much anymore I kind of just took on alot of the effort myself. I think in some ways it will atleast for some time create more headache for me in trying to figure out should I call my PCP for her input or what is the right step to take but ultimately I think if we do figure out a system of working together it will be good and it would be great to have her help for sure and be able to run things by her with various things going on with different drs. I like my current PCP so think once we figure it out and if we maybe set up a regular more consistent schedule for fups to touch base it will go fine. I guess we will see, right!? :)

Interestingly I found out today that one of the other patients in the infusion clinic DOES have a Port a Cath I say this IS interesting simply bc my genetics team has always been so absolutely adamant that I not have one - I plan to talk to my PM dr and my PCP when I see each of them on thurs and on fri this week to get their opinions and then will talk to my Cardiol to sometime soon about this since his opoinion is absolutely the most important. I know he had changed his mind in the past to supporting one given the nature of the once weekly every week forever aldurazyme infusions and no end in sight to these despite the potential risks but still if I where seriously going to consider one id have to run it past him and figure out a game plan just in case of problems.

Our Gov here in WI who was just elected in Jan by a very small margin (how people could have voted for him is just so beyond me) is trying to take away the rights of the public/state workers rights to unionize and force higher pension and insurance costs from the employees while not allowing employees to bargain for what they receive in benefits or pay. He is using blatant lies such as state workers (which includes my parents and sister and plents of my friends) make much more money then the private sector workers and that state workers pay much less in to their pensions and in insurance costs - he is ramming this through the legislature and brought it to the floor last week fri and is up for vote tomorrow (where anyone can get up and speak) and on thurs the final vote which is expected to be close since the house and legislature is controlled both by republicans - myself and my parents are headed to Madison tomorrow (tues 2/15) to join the rallies in support of unions and killing this bill. Should be interesting and there has already been HUGE rallies against Gov Walkers plan all weekend and today all over WI. I pray it does not pass and NOT just bc my own parents are part of the state/local school union here in BD (my Dad is president of the support staff union) but bc this will have detrimental effects all on down to people in ANY union anywhere in WI NOT just the state/public employees.

Ive not gotten the hip injections scheduled yet - I talked to my PM drs office today and even despite my Insur CM calling there to try and help get in going the secretary hasnt even submitted it yet to insur and my Appt is the second week of March with my Orthopedic dr and he and his nurse both said the injection needs 2 weeks to see if it will have positive benefit before we'll be able to tell if it works and i'd really like to know that before I fup. I sooo dislike relying on other people for things like this!!!!!!!!!!!!!!!!!

More soon..
God Bless,

Erica

Tuesday, February 8, 2011 11:21 PM
So much to write about and update so I will just start with the provider conference call which was yesterday 1/7

The Provider conference call which lasted 1 hr 15mins approx and included my PCP-Internal med dr, Neurologist, Pa
in Mngmt dr and Genetics team. Initially it was my PM dr and Insur CM the 1st 15mins till everyone else trickled in and my PCP was in and out of the call but there for about 1/2 of it.

The consensus on the headaches is that it is autonomic (ya and not shunt but still in my opinion one is linked to the other - I just am glad all can finally agree on something and maybe realize to that I dont care what the cause is id just like to try to find a solution. There really isnt any simple solution and PM dr and I have tried many things already over the past 3 years as this is a hard problem and no simple answer. ( obviously)

The providers all agree there needs to be more talking amongst all of them, more consistent message on treatment plan and actions with any decisions on managing the headaches/nausea or anything else be it new doctor, new medication, whatever (ahh lots of fun but definetly needed) and that my InternalMed-PCP should be the point person for communication we will use a program called mychart (which I do unless requires cc'ing multiple drs) then instead of unsecure email we will use password protected word document to exchange messages, reports, whatever. Im unsure why but a couple of the provider spparently thought that I was going to see a Nsg at Madison which is the complete opposite direction of where these drs are all at - im not certain where with this information came from since we have no Neurosurgeon and there is no one I am seeing. We do need someone tomanage the shunt in the case of future problems although some feel this if something did go wrong I could just go to the hospital (which one!?!) and any dr would treat me which I dont really agree with simply bc it's not run of the mill issue, there are major airway issues (grade 4-severe) and all of the other MPS factors to combine in - the only thing I can figure out where the madison thing came up is that bc I am working in Madison on a committee at the teaching hospital on post-operative pain mngmt planning to help the PM program there better plan a program for managing chronic pain pts peri-operative and once these pts go home - this isnt affiliated with my own PM dr or any of my drs though and just a committee I was asked to do separate from my own care and there is a Neurosurgeon and Orthopedic drs on the committee but not who I see. Besides that I prefer not to mix what I do in this aspect right now personal with medical care in this case so still back at square one who will manage and watch the shunt regardless of it functioning now. Doesnt mean i'd have to see the dr even on a regular basis just that they where there much like the oprtho-spine surgeon - not really a good idea to have a implanted device and not have a plan for it in the case of an emergency/problem but that is just my opinion. The other thing is Insur Cm talked to genetics team about a central access device and their response was the potential risks with infection and the other issues I have - they are planning to talk to the 3 different nurses which would be interesting as all have different opinions - the one nurse is a central access nurse though and knows quite a bit about port management/risks/who to see/etc and last they said ultimately it was up to me if I got one which is a change in opinion from what was said even just recently but still ultimately I do think it is my body doesnt mean I am running out and getting one bc if I get to the point where I think benefit is better than risk i'll do it. I know other drs like cardiology support this with cautious management so a milestone maybe?
Lastly I guess they talked about the progression of the MPS in the years ive been seeing this group of drs which has been about 3 yrs minus genetics which is 6 yrs now and just concern with that ie especially the PM dr mentioned her concern with the noticeable thickening and there has been in other areas as well such as cardiac but not much that can be done since we are doing ERT already.

So overall not a earth shattering call and no new developments but atleast maybe they realize they need to talk more and will try harder? I see my PCP next week and have messaged with her and she seems very willing to try to take this on and help me figure out how we are going to make this new action plan work.. I think she is good and also trusts me so will be fine.

At the end of the call today my CM when she was updating me on how it all went asked if I ever think about how the disease will and has continued to progress and how this may affect me down the road - ie do I worry about it, get depressed, unconsciously did I think it is something that affects me without really realizing it or how do I manage knowing and living with the changes... Odd question not bc it isnt a realistic question but bc ive never been asked that and just kind of took me by surprise I guess. Although there are always good and bad days ive always tried to look at my life like I was blessed enough to be able to find a dr who recognized what I had at a very late age (21) after years and years of drs who didnt know what was going on and that by the chance of finding this dr I was able to atleast slow down progression of some of the disease and altogether improve other areas - so while there is progression and it will continue it is still better than if I hadnt never made the decision to go see my current cardiol. By making my decision that what doctors where telling me (that none of the various symptoms where related or that it was all in my head) wasnt right and I knew my body and that the cont changes werent normal I was given a chance to help myself and to change the course of my life really by that single doctor after so many incorrect drs before him and was given a dx finally, finally, finally!!! Ive always believed in the very wise motto of one day at a time and one week at a time - not meaning you dont plan for the future but that you focus more on what is in front of you and what you can do now for yourself to make life better and what you can do for others to try to help them make their life better to. Doesnt mean there arent times you dont think about what could be bc thats unrealistic to not think about it at times but still one step fwd and one day ahead. As the saying from my friend Steph goes "Life is a journey with a past, present and future but for now all we have is today" - i've always thought that was absolutely the best saying ive ever been told and totally true and especially accurate w/my life! I also think despite all of the sometimes very maddening appts and never ending insurance, appts, medication/pharmacy paperwork/calls/etc that having a life outside of the disorder is extremely important - even though everything I do is essentially still within helping people indirectly in the medical field through the MPS Society role, my cont from time to time talks for Genzyme and with APFAN keep me focused on making a difference and helping others by sharing what ive learned and experienced and by trying to help others avoid some of the pitfalls ive experienced. And of course having incredibly awesome nephews and neices is the best thing in the world since I wont have kids of my own and I am able to spoil and spoil some more! :)

Otherwise in medical news I guess I will update some of that later!
Take care - God Bless,

Erica

Sunday, January 30, 2011 9:39 PM CST

Sorry for the delay I did see the new orthopaedics dr in Froedtert 2 weeks ago - very nice and seemed to have done quite a bit to learn about MPS before I came and had talked to my other Ortho his colleague. His reccomendations where daily NSAIDs, continued PT and hip injections and if down the road these are not prpviding effective results he said we'd consider reforming/shaping the femus/ball joint. I originally was going to have the injection at FMLH once I decided to go ahead with it but after talking to my Pain Mngmt dr last week am going to do it through her as ive had thoracic and lumbar injections with her and she is good + I trust her - and nice to know who is doing something before you have it done!

Will update the rest soon - hasnt been the best week but lots of new pictures up of my new neices - Isabella was born 1 week ago this past sat and is a doll! Her little brother Zan loves to talk about her although covers his ears anytime she cries and isnt quite ready to go to near her yet.

More soon,

Erica

Monday, January 17, 2011 10:02 AM CST

I thought I would update about a few things and i'll finish the rest once I know more.

Once my orthopedic dr reviewed the MRI-hip results his nurse called and my dr thought it best to send me to one of his colleagues who sees patients with 'difficult, and complicated' issues this will also be at FMLH and I see this dr tomorrow morning. My ortho isnt sure what to make of the results if there is something they can do to help or just watch and see. This other ortho seems well regarded by my current ortho's nurse and the secretary so we will see. I also have other MRIs before that appt for my PM dr for repeat (every 6months to year) brain and c-spine imaging.

2 weeks ago my PM dr requested lab work be done which I did at FMLH and really was a broad array of different levels one of which included glucose (I have an issue with reactive hypoglycemia so when I eat things with carbs for instance). This glucose test came back abnormal around 26 when a normal level should be above 65 or higher from what I understand - it could be a lab error but my dr repeated the labs and also stated she feels we should take this seriously as it could explain some of my symptoms. (vision changes, tiredness for after eating many types of foods and other possible symptoms to. I have to call today and either make an appt or just talk to my dr and am also supposed to see my Endocrine dr sooner. This is sort of ironic really as I was just talking to my Endocrine dr in early dec about this general symptoms and then suddenly got worse and seems to be tied to the hypoglycemia. Hopefully there is a way to treat it and prevent glucose from crashing. We do know this is autonomic related but not why exactly other than the autonomic system is the cause of alot of my issues.

More soon

Friday, December 31, 2010 9:37 PM CST

It has definetly been an interesting month full of ups and downs but overall despite some of the challenges it has been good. Several weeks ago I ended up getting a pretty bad ear infection and resulting sore throat for which I was on antibiotics and after about a week this abated and now just the sore throat lingers for a short period in the mornings until I eat or drink something but nothing near what it was - ive not been that sick with a cold/infection in a long, long time. I do have a cough it actually sounds much like a smokers cough which has started up in the past week mostly when laying down and with this as well as when I was sick I experience episodes of small amounts of bladder loss which is weird. My GC thought perhaps muscle related and advised to talk to my PMD which I will do at some point whenever I go in for some other appt in the future. I have been having these weird episodes of tunnel like vision which have been occuring for about 4 or 5 days now and with it I get fuzzy vision and profound fatigue though I dont fall asleep or black out it is unerving and not sure what to make of it but since it lasts such a short period and occurs on a come and go basis I wont worry about it to much. I mentioned it to my Pain Mngmt dr who was concerned and thought it could be seizure or central nervous system related but since ive not experiencing any adverse problems with it ive emailed this issue to my Neurologist and will see. Like other things it may just go away on its own which would be fantastic time will tell.

I also received the MRI Hip results yesterday and teh Ortho Nurse/dr will let me know what the reccomend next week - it seems to show pretty much the same things as my spine and knees show which is deposition of mucopolysaccharides and resulting damage and arthritis like changes. I dont think it was earth shattering or suprising and may explain the pain I have especiialy when sitting or laying and the question is what will we do to help this? Again we will see. There is really no Adult Orthopedic dr who sees MPS I (or any) pts and so I have to educate myself and expect that my Orthopedic dr (head of his dept) will look at this from an MPS perspectve as much as possible.

I have been doing amazingly well on the diltiazem - I truly believe atleast some of the problem was from the Left Atrial Enlargment which over the past year had progrssed from zero enlargement to mild up to moderate enlargement and along with that the symptoms changed to without my evening knowing initially that the LA was enlarging. I am very thankful for my Cardiol's. being willing to try this class of medication despite his skepticism in how they can adversely affect some of the other cardiac issues I have. It is definetly nice to be able to breathe without actually thinking about it - this diltiazem and lasix combo has been good! And to it is nice to write about a positive improvement/change to once!!!

I saw my PM dr 2x's since I have last updated this and we are going to try a medication called Amrix which works by calming the autonomic nervous system - although we are having huge difficulties in getting any of my drs to work with us my PM dr believes that the shunt either is not working effectively or that the pressure fluctuates enough (which incidentally is an idea my Neurol. also had but she is so busy with other non-practice things right now that my PM dr and I havent really been able to get her help) that I get this headaches and resulting symptoms and perhaps to it is related to scar tissue from past damage related to the hydrocephalus having been untreated for periods of time. Either way I am totally 100% frustrated and my PM dr bless her incredibly kind heart is trying to do the work of many of my other drs to to help me. She is fantastically great and I am very happy to have her.

Next week I am sitting on a committee I have been asked participate in as the patient perspective at UW Hospital - this committee will include Ortho/Neurosurgeons, Pain Mngmt dr(s), Primary dr., Nurse, PM clinic Coordinator and any applicable outside interests to the patients care - although I do not see drs at UW Hospital I have worked with the PM clinic coord on a couple other projects thus far and she asked me back when we where both at the Chicago Pain meeting if I was interested. I will be the sole patient voice so this will be very interesting and I think informative in a way for my own care. The purpose is to look at post-surgical pain managment in chronic pain patients and better planning + outcomes. The initial committee meeting will take place over 1 1/2 hrs. so short but full.

About 2 weeks ago the Medical Affairds Liason and the Patient Care Liason for Genzyme whom I know quite well called after the MCL Karen sent me a text to see if I could talk. We did a 3 way call and updated on all the infusion issues and then the PCL asked if id be interested in meeting whne she came to WI later in Jan. Ive not met this PCL (Judy) as she is newer in this position so I am looking fwd to this - rather than meet at ERT infusion clinic and deal with anyone listening we are going to meet for coffee or dinner and catch up. This will be lovely since ive not yet met Judy in person! I am so thankful that Genzyme has these liaisons who go out of their way to try to smooth things over for patients even when at times it puts them in the hot sear with providers who can be territorial.

I truly hope the Holidays where a blessed time for each of you and I want to thank each of my friends and family for taking the time to come here and follow what I write. I hope that 2011 will bring new and good things and time to spend with family, friends and that each of you will experience good health to.
May the peace of God be with you; today, tomorrow and each day thereafter.
God Bless,

Erica

Monday, November 22, 2010 5:40 PM CST

This will probably be a multi-part update meaning I may not finish it all tonight but really where to begin?

2 weeks ago I was in Chicago for a meeting for the Center for Practical Bioethics 'PAINS' Project which was very interesting and really all about a round table duscussion of pain policy and moving pain forward. There was mostly medical providers including doctors, nurses, hospice providers, a Drug Enforcement Agency (DEA) lawyer (come to find out formerly related to my Pain dr) and 2 Pain pts 1 beside myself but it was a semi-diverse group. The meeting took place over about 6 hours and really was informative and interesting and is a good group to be a part of I think, My Pain dr was headed to the same meeting but in San Diego last week as she wasnt able to attend this one in Chicago (there where 5 or 6 of these throughout the US held in the past few months) so will be interesting to talk to her.

Last week right after infusion I headed to Mn and then took the light rail in to the hotel from the airport which I love their light rail and tues morning began the appts. It started with Neuro-Psych appts all morning and then tues afternoon I had lunch with the former Patient Care Liaison for WI from Genzyme which was nice and tues night spent a little time with the Merrell family who are from St.Louis area and who both boys have MPS I as well. Very nice! ... Weds morning I had an appt with my Neurosurgeon at Mn right away in the morning which I had been hoping he might be able to help us and my Neurologist has really wanted his help - that appt ended up running into my other appts though so I ended up jsut leaving copies of my Visual Field studies from April and May and also my Neurologists last note and the Nsg said he would call me (still waiting =/ ) im not sure when he will call but I guess I will havew to call his secreatary to ask her to remind him. Grr!! After that was the functional MRI and then an appt with my Eye dr at Mn who repeated the visual field studies and though they showed further progression he doesnt know how to explain what they mean which is what we had hoped he could help with. He did say it could be because of pressure but doesnt know. I have to admit I was pretty frustrated and a little upset not at the doctor but just in general after that bc i wanted so badly to just figure out what these headaches are and have somebody validate that it is all for real and not just me making things up which is what I feel some of my drs here at home think sometimes. I hoenstly without my pain dr dont know what I would do bc she keeps me sane and actually believes me and believes that im not just making crap up. (what benefit would I get from making something up???)

There has been alot of mess gonig on around my weekly infusion between my team and I and I will write about that more here soon. For now have to stop typing for awhile.
Love and Happy Holidays.

Erica

Saturday, November 6, 2010 1:23 AM CDT

Apparently in my last update I was married to the word 'otherwise' lol so I promise to avoid that this time!

Not to much has been going on - mostly finalized the Mn trip with airline arrangements being made and appts worked out for the most part for Dr. (Elsa) Shapiro's cognition study which will take place on Tues and Weds Nov 16th and 17th and I will fly in late on Monday the 15th from MKE after my ERT (aldurazyme) infusion which is after my OT appt that morning, I also am trying to work in the Opthalm and Neurosurgeon appts with Dr.Bothun amd Dr.Haines which I had to reschedule a few weeks ago. I am praying these work in as I truly need some help and answers or guidance for my drs here at home on my visual field studies and what we should do next for my visual and for my headaches.

I see an Audiologist this coming Thurs at FMLH, ive not met this one before as my former Audioligst was elsewhere (actually at the hospital where my PM dr practices out of). This appt is per the Genzyme protocol for MPS I and my GC's request + since I have 1 hearing aid already and loss at the SSNRI (sensorineural) level my GC wants to check this. I will update after. .... I see my Hand surgeon in 2 weeks and at this point it has been 7 1/2 weeks post op for the tendon transfer - I saw the OT for the 1st time since the cast was removed and splint made/placed and she started a few very basic daily stretching exercises but no strenghtening till after I see my surgeon when hopefully the splint is removed for good!

This Tuesday is the Pains Project meeting which the Center for Practical BioEthics is putting on - this is one of 5 meetings being held around the U.S. by this organizations and this one which I was invited to is being held in Chicago. It should be good and very interesting to see and hear so many different voices representing diverse organizations from patient and physician groups to I imagine Pharma as well. The meeting runs over about 6 hours at the Westin Hotel in downtown Chicago.

Althoguh my younger nephew was sick last sunday (he did go with his Dad though the day before trick or treating) and so I didnt get to see him in his bumble bee costume my older nephew and I went trick or treating for about 1 1/2 hrs and he was the ghost from the scream movies - which I am updating my photo files and moving to a new site for my slideshow at the top of the page - stay tuned and should be back up shortly. :)

I guess the only other news is that I met with my Genetic Counselor (GC) 2 weeks ago this monday and after ironing out my aldurazyme dosing issue she went over my hip xray results and also faxed these to my PM dr. The hip xrays showed "worsening in severity of degeneration since previous xrays which where in March 09'" (Not that long ago for there to be this worsening!?) Ugh!! No one, neither my GC nor my Pain Mngmt dr are sure what to make of these other than obviously it explains my hip discomfort at night and why they seem to just get incredibly stiff and one of the other side will lock up and thus I end up limping for no seeming reason out of the blue. This has been happening more frequently. My PM dr is sending me back to the Orthopedic dr she sent me to a couple years ago and I see him the end of this month and we'll see what he suggests we can try. Since ive already been in PT for over 9 months and I do daily stretching at home which would id think be the 1st reccomendation not sure what else he might think.

I think that is about it - I know I am forgetting something but need to get some sleep as I am watching Zander (younger nephew) in the morning and shoulders are aching laying adn stting here typing.
Take care + Love,

Erica

PS: the motto of the week which seems many times to apply to my life: to borrow from the election: "Yes we can meets ooh no we won't" This oh so perfectly sums up how I feel at times dealing with so many personalities and opinions and the lack of communication in medicine! I'm not being "down" saying this, it is just simply the truth!

Wednesday, October 27, 2010 1:57 PM CDT

I know, I know I said I would update after I asw my Hand surgeon and yet here it is more than a week later and finally getting it done! The appt went well and though I thought maybe I would get the splint off and to start OT (occupational therapy) but my dr wanted me to wear the splint for another 5 weeks and I start OT on mon the 1st. I cna thankfully take the splint off for showering.

Otherwise I saw my Pain mngmt dr yesterday who is sending me back to an Orthopedic dr I saw at FMLH a couple years ago - this is due to some hip xrays I had which showed "worsening severity" so we will see. I also was supposed to go to Mn today to see the Opthalm and Nuerosurgeon bc of my 2 abnormal visual field studies and the headaches. Anyways the weather here in WI is massive winds and so my flight was cancelled so I am going to Mn anyways in Nov for a study so the coordinator is helping to coordinate these appts with those appts for the study.

Otherwise in the past 2 days ive finally been able to catch up on some sleep - mond I slept 16 1/2 hrs which is plain nuts and last night around 10 hrs which I finally feel slightly human in that respect! It does screw up the day though and this weather makes it very blah but non the less atleast this weekend is trick or treaitng and is supposed to be in the 50's so while cool isnt as bad as some years!!! Zander wanted to be a bumble bee and Quinn is going as a blood ghost with mask, cape and swords so should be fun. :)

Otherwise last week I met with a couple different APF comntacts I am working with - the ASPMN President (american society pain mngmt nursing) and APM Madison person and seems like both will be good to work with.

I guess that is all for now - stay warm and God Bless,

Erica

Saturday, October 16, 2010 0:55 AM CDT

Well it is almost 1:00a.m here and I am still awake though really tired and have been up almost 24 hours with very little sleep over the past few weeks as I just havent been able to sleep well. I dont know if i'll finish this update tonight as it is a little difficult to type without my thumb yet and actually surprisingly painful to type yet with that hand. I will update on that all later or soon but the stitches in the right ring finger, thumh and wrist did come out last week at week 3 1/2 and the cast was removed plus a plastic type splint made which I also wear 24 hrs a day inclding cont'ing to have to cover it in the shower.

I just happened to be thinking about some appts today and over the past week (4 on mond ranging from 7am-OT to 1hr cortisol stim Endocrine labs, ERT infusion and Pain Psychol onto todays with Internal Med dr and Oral Surgeon plus talked to my Endocrine dr who called with the results. And anyways I just was reflecting humerously really but in a funny way on some of my relationships with some of my providres - how there is such a wide range of doctor/patient communication and even respect I guess or trust on both sides alot depending on how drs look at the relatipnship they chose to have with their pt. Ie: purely 'I am the doctor. you are the patient' to the Equal 'I trust you and You trust me'. I was kind of laughing to myself thinking of my Cardiologist for insrance who I actually really respect and think knows his stuff but we do butt heads and with him and I it didnt take many visits till we came to this almost unspoken respect for each others stubborness and ability to agree but disagree and vice versa on that last part. Many drs look at their patient tell them one thing and yet if the patient does that they will still find something they think the patient could do better ((I dont mean that to sound so harsh as it does just that I dont think alot of drs realize they do this) and with drs such as a few of mine they chose instead to respect what their patient knows and is involved in and that their patient may even know more about their disease in some instances than they do. An equal partnership, shared mutual agreements and agreeing to disagree but not taking it as either the patient not listening or as the patient thinking they know better than their doctor. A key piece is patients realizing just bc their doctor doesnt agree with what they may wish all of the time doesnt mean both dont respect each other. Im not sure how to better explain what im trying to say but I guess just that equal partnership between patient and provider can often bring better health results in my opinion as the patient will trust their dr to be doing their best to make the best decisions (vs second guessing) and the doctor will trust the patient if the patient says something isnt right but that the "somethings not right" isnt so tangible or if the patient feels something isnt working. I know I am personally and I say this carefully much more likely to trust the opinion of a dr who trusts me enough to listen, respect me and acknowledge my opinion vs a doctor who believes all patients fall into neat sqaure little boxes and who just believes what they say will work for every person regardless of how the patient feels. It is good to have providers who are open about their own lives or their interests or even just what they want for you the patient - I know with my Pain dr for instance it is because of her continued seeming love for her field and even more her passion for sharing what she knows that has made me passionate to learn and keep learning about this field and sit in on classes on the topic by various researchers and do the outreach work - one persons passion is sometimes just enough to cause it to becomes their patients interest to albeit in different ways! :) I write all of this just because really of seeing my Internal Med dr today - there really is nothing special about these appts I mean they are normal as normal can be most often but it's her interest and seeming interest in learning and to sharing that really sparked this post.
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As for my hand as mentioned above I have the splint on now and go back next week - week 5 post op and I believe they may start OT but uncertain and I am unclear if I will get the splint of then or not - my skin which is soo dry and chemleon like underneath would love it if I did! The OT ive been working with at the Hand center for the most part has been incredibly nice and interestingly lives fairly close to where I do (20mins or so) - I am unsure if that is also who I will work with during OT.

I will come back and update on the many other appts hopefully in the next few days - we will see!
Love,

Erica

Friday, September 17, 2010 10:54 PM CDT

Sorry for the delay in giving an update post-op on mondays surgery... The surgery was as I mentioned last week on here done at FMLH by my previous hand surgeon from last year and took about 1 1/2 hrs. I was able to talk to my Pain dr sun night (she called me actually, how sweet really is that?) and the anesthesia dr also called that night. Monday morning the anesthesia folks wanted to switch from LMA to a regional or MACC (sp?) anesthetic regimen but thankfully was able to convince them the LMA was better, to be safe - as this is also what my Pain dr reccomended given my past airway issues. Once we got past that they did the cortef IV infusion per my Endocrine dr (cortisol replacement which has to be given in a large dose by IV 1 hr prior to any surgery and I take extra doses for 5 days of my same daily hydrocortisone med then go back to the normal dose I normally take. Post surgery was interesting as pain mngmt was never discussed (other than with my pain dr who was fine with letting my drs handle post op care as long as my normal daily pain regimen was also con'td) and so the residents didnt really know what to do with me and needless I didnt get alot of relief and was there from 7-3:30 - finally that night at home I was able to talk to my pain dr again (she had the night before asked I give her a call the night of surgery to update her on how it went and incidentally she cold called today (out of the blue) to check in on how recovery was going to - completely a wonderful person!) and she advised me on how I could tweek what I normally take. Tuesday morning I had a fup with my hand dr and he just updated me a little more on how surgery went and then had a permanent cast put on for 3 1/2 weeks - the stitches (3 incisions in the base of the finger next to pinky, incision in thumb and in area where wrist bends) will be left in until I go back to see my hand dr in Oct. The occupational therapist whom I will work with for rehab post cast removal and stitches being taken out (rehab is usually several months) applied a purple cast - MPS purple :) I had the usual choices purple, blue, green, etc. I am encasted my entire thumb with no movement there and 3/4's of the way down to my elbow. Itching has only started somewhat today and I plan to ask my dr what I can do as there is no way I want to deal with this and worse Iexpect for 3 more weeks as teh swelling continues to go down!!!!!

Please dont forget September is Pain Awareness Month!! www.painfoundation.com to sign up and for information!

Appts in MN with Opthalmologist and Nurosurgeon have been moved due to this surgery to Oct. And also I was asked to participate in a neurocognitive study there which I will do in Nov. ....Otherwise I will update soon,
Take care, God Bless,

Erica

Saturday, September 11, 2010 11:24 PM CDT

Just a quick update - there are other things to update on but will do that later - my tendon transfer (opponensplasty) surgery has been mvoed from the 20th to this monday the 13th. Still at FMLH, same surgeon, etc. As for ym display for Pain Awareness Month at UW Hospital my regional manager at APF is going to sub in for me as is the other co-leader here in WI and I will give my display and materials to this other leader. Disapointed after all the effort of setting this up to be missing it as I was looking fwd to many aspects of it. Such is life I guess and there are other PAM plans and events I will be doing elsewhere to.

More later or at the very least after surgery,
Thanks,

Erica

Thursday, August 26, 2010 6:54 PM CDT

Time for another update - a few new things going on - both medical wise and in general with APFAN work and my life.

I saw both my Hand surgeon and my Pain dr today - my hand surgeon thinks doing a tendon transfer from the abductor muscle (base of middle finger) to place the tendon into my thenar (base of thumb) and route it down to my wrist would be helpful for the problems I have with my hands. We new already before I even had the 1st hand surgery 8 years ago that the theanr muscle at the base of my thumb was pretty wasted and tehre virtually is no muscle left to support grip which of courase affects everything from holding a book for long, be able to easily turn a page, typing, opening containers especially small ones and dropping things. This surgery will be done as out-patient and we will likely do LMA again - I had this Hand surgeon last year when I had carpal tunnel release on my left hand for the 2nd time (a full release and synovectomy) and for that surgery which was relatively quick (I think 30-45 mins tops not including the anestheshia induction) and he is good plus highly reccomended by many people I know both many of my providers especially. This surgery he said will take about 1 1/2 hours as they remove the tendon from the base of the finger, make an incision in the thumb and also in the wrist and 'graft" the tendon from the thenar area down to the base of the wrist. I will be in a cast for 4 weeks with my thumb and wrist immobile. With my CTS releases I was in a cast for less than 5 days with this surgeon! He said after a short bit I will be able to drive even though I drive a stick shift non-automatic car!! That was my biggest concern so I am really happy to hear this news - he said this was because I would still have enough grasp without my thumb to move the shifter!
Here is a description though not that helpful of what will be done:
Palmaris longus opponensplasty (Camitz)

This procedure is performed for loss of thumb abduction and opposition. Camitz transfer provides palmar abduction rather than thumb opposition. A carpal tunnel incision is performed with a distal forearm extension. The distal palmaris longus is dissected free with a strip of palmar fascia attached. The strip of palmar fascia is tubed with sutures. A tunnel is created in the subcutaneous tissues from the distal forearm to the radial aspect of the MCP joint of the thumb. The fascial extension of the tendon is passed through the tunnel and secured to the abductor pollicis brevis tendon. Maximal tension is required with the wrist in neutral position. The thumb should be immobilized in opposition, with the wrist in neutral position, for 3 weeks with splints. After 3 weeks, all splints are removed and range-of-motion exercises are started.25

I also saw my Pain dr today a week early as we are figuring out how to get RFA covered by my insurance which has denied it saying it is 'experimental/investigational' - while talking to my Pain dr about this today she said this in fact is not true and so we are wondering if because there are so many different forms of RFA for different reasons if Dean has the wrong information. So anyways we are going to try to get that re-approved. We switched the long acting pain medication I am on as I had less GI affects with what i'll be on now. (a whole nother story further down in this update). My Pain dr also gave me the name of a rep she works with a Pfizer I believe it is on Fibromyalgia - my Pain dr is a consultant with them and gives talks and lectures on this subject and is always trying to come up with different people she can put me in touch with. Anyways apparently she has previously recently talked to this rep about me and about what I do with APF and thinks we should get in touch. My Pain dr also gave her the pain support group info as my Pain dr is interested in doing more community type events but recognizes that when they are not in-hospital based and instead at community events funding is hard to get for tables, displays or talks. So hopefully this will turn out to be a good contact person and we can work together!

I also saw my Neurologist a few weeks ago and because she is so frustrated tbat basically no dr of mine other than my Pian dr wants to try and help manage my care she just is going ahead and doing a few things. She is havin me do a repeat sleep study the last one being about 2 years ago so that we cna get the bipap question out of the way. I had talked to her about her being my one and only Neurologist as I didnt feel like we where getting anything to special out of the MN dr though he is nice he just doesnt have alot of MPS experience either. We need a Dr.Chanrnas back is what we need we both agreed! So anyways the sleep study will be done and she is sending me back up to the Neurosurgeon at UofMN as she wants his help as no Nsg around here will help her. I am also going to see the Opthalm there when I go next week and see what he thinks of the visual field studies. I have alot of issues with being able to read small print even size 10 print is hard and size 12 tends to blur quickly so maybe he will be able to shed some light for us.

My Case Manager at my Insurance who has been really good other than a few issues which really didnt have much to do with her - she is reviewing a few more medical records I just sent her and wants to come up with a game plan on how to get my providers to work better together. Apparently she even talked to my Primary drpersonally and asked her if any of my other drs ever come to her to get her help and input in managing my care and streamlining my care and my Internal med dr said that other than medical reports she doesnt hear from my providers and would like to help more. Hopefully we cna figure something out because it is pretty bad how disorganized my care is - even my one dr has said multiple times I have the most disorganized team of ptoviders unwilling to take responsibility she has ever seen and she sees alot of multi-provider patients! That says something though wasnt anything I didnt know.

This week I didnt get my Enzyme replacement infusion - it turns out someone at Genetics took a message last week and instead of alerting myself, my GC and my Case manager or atl the very least one of us (~!!!!!) whoever this person was - another GC it sounded like the a message on the GC who sees some of the other ERT pts DESPITE the fact that this GC is out EVERY Fri! Needless I was pissed monday when I found this out and they wouldnt let me do the infusion Tues. I get that they have no idea what it is like to miss an infusion (GI is worst part) but for god sake this drug is weekly for a freaking reason!!!!!

Tomorrow I will be doing teh training to create one of 4 trial test state pages for American Pain Foundation - these pages will be able to be customized to ut particular state and activities but they are only training 4 of us leaders from 4 different states (most states have 2 or more leaders) on how to make this and the set up they would like to use so they are all similar. Anyways I am looking fwd to going through this short training and working on the page and feel honored that APF higher ups chose me as one of only a few leaders to do this initialy!

I took my nephew to the Dodge County Fair this past weekend (a record for me my 2nd fair this year!) and while it was hot and humid and the Fair isnt all that exciting Quinn and I had a good time and I also was able to make another APF contact. Because the BD community hospital had a booth at the fair I stopped by to talk to a volunteer about whom I would talk to with doing some kind of awareness thing for September Pain Awareness Month at BDCH. Anyways the person I happened to talk to was the head of this particular kind of thing and after asking me a couple questions about what specifically I/we do at APF she asked if I would be interested in doing a display during the month and also if I would be interested in a radio interview with the APM Pain dr who comes out to there. I was just today able to get ahold of the marketing person at APM's Madison office and so will write her back tonight and she what we can work out for Sept and will get back in touch with the person I talked to at the fair. Should be a good. This contact made up for the hot and humid weather at the fair!

More later,

Erica

Wednesday, August 4, 2010 0:22 AM CDT

Sorry fellow readers it has been another 1/2 a month - where does time go? I was just thinking the other day my 4 most favorite months of the year are gone again. =/ ...

I am currently working on a new site for this webpage but we'll see how it keeps coming along. I was asked yesterday by my Regional Manager for APFAN if I would be interested also in doing the training for putting together a few 'test' pages with just a couple state Action Network Leaders for each of us to be trained in how to create our own pages representing each of out states and what it going on lcoally. (sorry major run on sentence!) For me this would be a APFAN page for WI and we would be given a template but then have the freedom to customize our state pages as we please. If the few state test pages went well then the other state leaders from the rest of the states would be trained in this as well and in creating their states pages to. Back to my own personal site it will still be a sponsored site - I am currently testing wordpress and so far like what ive been able to do. I would just put a link back to this page so that anyone who wanted could go back and read as much or as little as they wanted from the beginning of my dx.

I have been working with a Pain group in Milw on a support group for patients, caregivers and families as well which we are planning to host in Sept. I met with the Marketing person yesterday in Milw and we have a tentative date set for Sept 24th at Franklin, WI Advanced Pain Mngmt office. This has been really a interesting project and something ive never done before nor have I ever actually been a part of a support group but was something I have been interested in for a while. I am hoping to recruit patients and family from other area faciilities as we would like to get together as many people as possible to share, talk and help one another. The idea isnt to 'steal' patients form other health centers (I after all am not an APM pain patient) but really to empower patients and familiy to help themselves.

Several weeks ago I was in Madison and met with the UW Pain Coord whom I also have been communicating with - she is the person whom my Pain mngmt dr gave me the name of and
so her, myself and Mary (regional manager) met and talked shop over breakfast - after a slightly stressful drive of getting quite lost several times (and I grew up going to madison frequently!) getting to where we where meeting by UW it was a lovely meeting. This person has agreed to act as an advisor to out WI APFAN Leaders and be available to help advise. She has also been helping to try and recruit a few healthcare provide4rs to our WI team and which I am very thankful for. Ideally we would like to add 2 healthcare providers and maybe 1 more leader - usually it is 2-4 leaders per state. Right now it is myself and then there is another leader involved to. Because Sept is Pain Awareneess Month and UW is not planning to do a Patient Pain display for this month Deb is getting info for me on having a display there the 1st week of Sept which I would leave unmanned but will include patient edicational materials such as our pain journals, pain options information, disease specific information and materials which patients can take to help them better talsk to their drs.

I am working on a few other things fro Sept as well. I did email the reporter I worked with directly last year when interviewing for the piece Patti Neighmond did on NPR on Pain and this reporter replied back fairly quickly with the contact info for reporters at both WUWM and at WPR whom he works with on the Health scene so I am going to be reaching out to try and get something there. I also keep thinking about trying to get something on the local radio station(s) where I live and maybe contacting the local hospital which though I dont go there for any providers (mine are all in Milw) I do think they would likely be receptive to some sort of educational display for patients for sept. We'll see!

I saw my Pain dr a couple weeks ago and see her again this week - she is going to be doing further RFA (radiofrequency ablation) at my thoracic spine - it originally was set for last week but I had to reschedule so now that will be the 24th as their other date which was also sooner also didnt work. I also got my MRI results back and not surprisingly they where normal which I figured they would be - I see my Neurologist next week but I can tell she is frustrated and at her neat wits end - the other week she told me that she hasnt ever seen such a dysfunctional group of drs - drs who no one dr will take charge and no one wants to step up and make the decisions that need to be made. Pretty much was what I have thought for a long while - other than my Internal med dr whom I adore and my Pain dr sometimes I wonder why I dont just fire the whole lot of em'? I do really like my Neurologist but she feels like no one can agree on anything and I also feel like this and I feel a little like she has given up.. =/ I think what really just needs to happen is that myself and 1 other dr pick a core group of 2 or 3 providers and they are the only ones who get a say in the headaches for the time being and we all try to figure out a plan. It truly seems sometimes like my Pain dr is the only one who hasnt given up on me.

I continue to do Physical therapy though there has been a bit of a tension or difference in thought (not sure either of those is the write words) about where I do PT due to some scheduling issues - but ive made leaps I think in improvements since having started seeing my particular PT back in spring and my pain level and overall joint moveability is so much improved - yes there is a ways to go but still so much improved. I really actually like this Case Manager - she seems like she is smart, persistamt, actually cares and wants to help + if she says she's going to do something she has done it every single time so far. I guess I want to plesse her but ultimately I have to keep doing what has been working for my body and yet it worries me a little then that more sessions wont be approved.. Ugh!!!!

My neice Alissa age 10 is coming fri to stay until next thurs. Next weds we are all going to bay Beach and this weekend there are 2 different car shows going on which are fun and the one is really pretty cool for the kids. My nephew Quinn age 8 was by me all last weekend and will also be here this weekend and part of next week including Weds. Should be crazy im sure! This last week we took all 3 boys (Jordan age 10, Quinn and Zander age 4) to the Zoo in Madison and then on sun Quinn and Zan came to my parents to swim to - Quinn up until this year has been really tentative about swimming and jumping in the pool and up until last year when he was 7 wore a life vest in the water. Zander on the other hand was already jumoing into the water last year - but always would have us be right there to catch him sort of after he landed in the water. Well now he has us stand at the very back of my parents pool (all one level) and he jumps off of the ladder into the water in a sort of semi-beli flop, swims under water with his eyes open across the length of the pool and into our ours or to the side of the pool and pulls himself up, takes a breather and swims back to the ladder under water! He's never had swim lessons (we never did as kids either but had a pool which is where we learned to swim) but can swim better than most kids who've been in lessons for years! It is truly amazing to see how far that kiddo has come despite his Autism - he now loves to do everything Quinn loves to do and is just hilariously funny because he loves to make everyone laugh. Those 2 boys melt my heart for sure and to hear Zander say 'Auntie Erica' and other related things is the absolute sweetest wirds one could ever ask to hear!!!! I truely feel blessed to have these neices and nephews to replace the kids I wont have!

I am sure im forgetting something but can always come back and update,
God Bless,

Erica

Wedsnesday, July 15, 2010 6:01 AM CDT

Ive definetly had plenty of bad weeks as far as how ive felt physically but I think by far this week has eclipsed them all emotionally for a long time. My sister is not talking to me and so I havent seen my 4 yr old nephew since my 8 yr old nephews bday party a week and a 1/2 ago and I feel like a giant void is missing - I know you might be thinking that isnt that long but normally I see Z 2 or more times a week much less going this long. I hate it that my sister and I arent talking and wish she would talk to me so we could iron it out - I hate conflict I guess and I hate that I dont really even know what I did other than I apparently need to not give advice when asked for advice and when ive been in a situation someone else has been in I guess I need to just let them be bc her husband thinks I am trying to undermine him and push my views on him as far as our similar back issues - and all I thought I was doing was giving advice when asked and trying to help someone else not go through 1/2 the things ive went through to find relief. It just bothers me that ive lost my best friend and can't see Zander. it's hard to explain but bc I have no kids of my own my neices and nephews mean the world to me and Zander that kid has me wrapped around his pinky as far as how much I love him! I know ive done what I can and I just need to let it be and im trying - I just keep dreaming about it and am so tired from sleeping so crappy. I just pray it gets worked out soon.

Earlier this week I tried a new med it is something one of my drs prescribed a few weeks back but I was a little leery of it but decided to try it anyways - somehow I think our unconscious mind knows things before we even try them as that night I (for the first time since right before my spine surgery) I had the blacking out episodes and kept waking up in the oddest places but thankfully atleast not blacked out on the floor so I just stopped it. I see my Pain dr tomorrow and maybe she will have some other ideas. I think the problem with the med might be that it can affect hormone levels in the brain but I really dont know as my Endocrine dr had thought that together with the spinal compression is what played a part in the episodes pre-decomoression.

I see the GI dr next week - actually the PA which is fine I liked this dr well enough and hoping maybe she can help with the GI affects I seem to be having more of a problem with due to some meds and the MPS.

Tomorrow is the Optic MRI after my Pain Mngmt Appt. I will post those results after.

I think my older neice is coming to stay the end of the month/beginning of August - we are planning to go to Bay Beach again this year which is the amusement park and she usually comes for the week before or after. I wasnt sure Lis wanted to come but she was excited when she called the other day so it will be nice. Last year her Mom and siblings also met us at Bay Beach and that was pretty nice to. This weekend we are headed to Port Washington for the Fish Day-car show and Lis and her Mom siblings are also meeting us there. I cant wait to see her! :)

My insurance specialty pharmacy mess keeps on going - ive not missed infusions the last 2 weeks due to extra drug I had left from my previous insur but we have the aldurazyme approved through Dean and they are trying to get it through Walgreens Specialty Rx as that is Deans preferred in network prhamacy but they are saying they do not service aldurazyme despite us knowing atleast 1/2 a dozen other patients with aldurazyme and the other types of MPS enzyme replacement who USE Walgreens specialty! SO we have no idea what that is about but my Dean Case Manager and Genzyme Case manager are trying now through the other preferred Pharmacy Diplomat and have been working on that this week. I to say the least am crazy with it all bc it is stupid how these pharmacies work and just want it to be worked out! Stress!! Thankfully both of my CM's are persistent though - I really like this new Dean case manager she seems the type to stay on things till they are done.

I finally am booking my airline flight and hotel for the Conference in Sept/Oct and though I could only really find a flight that comes back around 12 on sun it is a non-stop flight and I can still make part of the Walk-Run. Next week is the morning breakfast meeting with the US Pain contact and my Regional Manager for APFAN and I also am working on the meeting and plans with the strong hopes that CHW-Genetics approves it. Otherwise I will go to a plan B whatever that is! Another of the Pain groups I am working with (a pain clinic) is going to be doing a support group for Pain pts in Sept for PAM (pain awareness month) and so we are going to try and work on this together a bit as far as getting patients and logistics - they have a psychologist willing to host the meetings and also will do them at their clinic so that is no worry and nice. Last I got an email from the WELS Forward in Christ editor yesterday they may be interested in doing an article for the newsletter which goes out to WELS church members on my situation and how Pain and my faith play a part which would be really good for awareness and a wide audience as I believe FIC targets not just WI but other WELS churches in the other states as well.

I guesss I better go, take care and remember with God all things are possible,

Erica

Friday, July 9 2010 1:01AM
It has been a while again since ive updated - and while I really should be sleeping right now (I have tried several times now but my bosy just wont listen) I thought I would update on atleast a few things.

I think I mentioned in previous notes that I had, had Visual Fiedls studies done in April which where abnormal so the neuro-Opthalmologist who read these thought they needed to be repeated to verify if the results where a glitch or if they where accurate. So anyways those where re-done the end of May and I finally got the results last week - the Neuro-Opthalm who isnt my dr called with the results. They are again abnormal with some odd results which my drs are unsure what it all means. My local Neurol has talked to my local Nsg and Genetic Counselor and she has ordered an MRI which will be done next thurs right after another appt I have in Milw. There thoughts are it is optic neuropath(ies) from either the MPS damage or from intercranial pressure past or current and this may have something to do with my headaches. The lovely thing with MRI's in MPS is that if there where papilledema it wouldnt show up on an MRI but this is still needed as it may give us other information. The very basic information of what is going on or what the V.F. studies showed is:

"issues with fixation on the rigth", " evidence of Superior Visual Field Defect" and "Suggestion of bitemporal field defect compression at level of optic chiasm" - This is just what I have written on my computer the actual reports are sitting across the room in my planner and I am really just to lazy at the moment to get up and get them to read the more detailed summary of what is going on! It all is complicated! I will either come back and update more of that info or just update the MRI info after I get those results depending which I get time for first!!!

My insurance changed July 1st which has been a bit of a headache for all of us - myself, my infusion team, my Case Manager at Genzyme and the perhaphs unlucky (but awefully nice and helpful!) case manager at Dean who is I have helping with all things insurance and health care. Usually you cannot start anything with an insur until the day it begins but thankfully I was able to begin working with my new Case Manager a week ahead of time and we began working on all the paperwork around Aldurazyme cost, how it is billed (pharmacy benefit or major medical, through navitus or another specialty pharmacy and how the benefits lined up ($700/200 deductible and out of pocket then 100which immediately is met with one shipment of aldurazyme and billed to medicare) and all of my providers being out of network but yet many of these same specialists not being available in the DHP system. Confusing I know! :) But anyways because we did alot of leg work prior to July 1st or as much as possible we where able to get the approval from Dean (which is a point of service so more flexibility) immediately on 6/1 for aldurazyme coverage at 100fter the out of pocket listed above and immediately began working on figuring out who the specialty pharmacy is and how it is billed as - which it turns out is under major medical which is why my deductible is met within 1 drug shipment and the logistics of approval from the specialty pharmacy (Navitus) and the (to make it more confusing) secondary specialty pharmacy they work with (Walgreens Speciality vs with WEA Insurance it was CVS-Caremark). This week thankfully I had extra drug already at CHW paid for from WEA which was just left over from being sick or something so it bought us a bit more time to get the shipping and Walgreens Speciality stuff ironed out. Next week is a little dicy on what day infusion will be as drug would have had to be shipped today for infusion mon and that didnt happen because the Walgreens purchaser/approval person was out. Crazy, crazy, crazy!! But atleast other than frustrating my Dean Case Manager, Genzyme Case manager and even GC have really been pushing to get this all worked out quickly.

Ive made some progress on the medical meeting with rental costs, discounts, speakers who are really interested and so just putting the final touches on the proposal. Cross your fingers!

In Pain Found stuff ive been doing outreach with other clinics and offices, sending out information regarding Sept Pain Awareness month and on the 20th I am meeting with my Regional Manager for APF and the UW Pain contact which I actually am really looking fwd to. It is always really interested to get other peoples ideas!

I saw my Hand surgeon a few weeks ago (this is how long my last update was ago!) and because ive continued to have problems with the muscle atrophy in my thenar muscle (base of the thumb) and difficulty with this I am trying some exercises at home and he talked about doing a different surgery after I see him next month in which he would take some of the muscle from the base of one of my other fingers and 'transplant' it basically into to thenar base and attach it there - this is something that was actually discussed before my first carpal tunnel surgery (ive had 2x's each) many years ago and before I was dx'd with MPS I when my orthopedic surgeon at the time had sent me to a differnt hand surgeon. Ive not known anyone who has had this done but I also know that even with multiple carpal tunnel surgeries it hasnt improved..

I'll try to post a few other updates soon but I really do need to try and sleep now - 4 hours until i'll have ot get back up (ugh!!!!) - Im just not a morning person!

Love and God Bless,

Erica

Sunday, June 6, 2010 10:15 PM CDT

It has been a few weeks again since ive updated so I figured I best do that once!

I know theere have been some things going on but honestly I can't remember everything off hand so i'll just write about whatever I do think off!

2 weeks ago I learned from my older brother that his gf is pregnant - she is due in Nov/Dec I think and then funny thing is last thurs I learned another sibling is also pregnant and due in Jan! Wow, talk about totaly crazy! Hopefully both kids will be perfectly healthy - for me I always am a little concerned because there is a small risk of them being carriers of MPS I and if their significant others where also carriers then their child could have it. Believe it or not I do actually know atleast a few people who they are affected and then their nephews where affected also - rare but when you have MPS rare isnt even unusual! Given there is autism in the family there is also that concern to. I dont think they're worried about it much and while I wish they did more there isnt anything I can do but pray.

I saw an Sports Med specialist a few weeks ago due to my ankle issues (I have knock knees and walk on the outer edge of my ankles and this all on top of the high arches combines for some quite painful walking often and standing. A normal person walks with their weight placed evenly throughout their foot. He reccomended some specific ankle braces and I also am having orthotics made for a pair of enclosed sandles I have. I do stretching day in and day out but for this hasnt made significant difference.

My insurance changes from WEA which ends June 30th to Dean Health Plan on July 1st - I just finally will get the account number this week and I can call and have a case manager assigned to me as well as we should finally be able to begin to work on getting approval for ERT. I have a feeling it may be a bit of a mess for a while bc Dean Health Insur is generally only contracted with Dean drs which is a small group of drs in our area and Madison but all of my drs including my Internal Med dr are in Milw out of network. I do have medicare for backup so just am crossing my fingers.

I finally posted some new pictures in the picture thing at the very top of this site. And speaking of Zander from above he will be 4 years old tomorrow! My little guy is growing up!

This next weekend is an Lysosomal meeting in MN so i'll go there from till sat night and then come home sat night and sun is Zan's b-day party which he is very excited for already! He has been talking about his bday all week when I see him, very cute!!!

Ive been working on quite a few different little projects for the Pain Found - there has been alot of media reports and articles done here in WI lately on the Prescription Drug Monitoring Program which was signed into law by Gov Doyle several weeks ago and on abuse of opioids so ive been reaching out to many of these reporters and journalists and giving the facts and personal side of pain mngmt and what it offers to people who live everyday in pain - not just that meds are abused which to many media outlets chose only to focus on. I did receive replies back from several of the TV station reports/anchors so this is a few more contacts which is nice to have. I alao collaborated with thw Advanced Pain Mngmt group (a different practice than that of my own pain mngmt dr) and with ASPM which is the Pain Mngmt Nursing Society here in WI and the Sept Pain Awarenss Month proclamation was signed several weeks ago also by Gov Doyle. Then ive also been working on church outreach and the first contact I made was with the church one of my drs hysbands is actually a pastor at (within my synod WELS) and they have a Cafe-business associated with their church and we are collaborating together on a health fair next fall/winter. I also am going to be working on outreach to other churches not only within the WELS synods here in WI but going to try to reach out to other area churches to do pain displays - especially hopefully for Sept which is Pain Awareness Month. I also want to try and do this very same idea with some of the local libraries. Then one of many other things is APM is considering doing another support group and so I am going to work with them on this to try and get that going as it is something I
think would be very helpful to families. There was an talk by the ACPA Founder on Fibromyalgia this past fri in Madison but I had already had plans and wasnt able to go - on the 22nd myself, the UW Pain Resource Nurse whom my Pain mngmt dr actually told me about and our Regional Manager at APF are planning to meet for breakfast in Madison to discuss ideas for working together as well. I have also been able to make contact with the WI Pain Initiative folks and teh contact I am in touch with there is simply seems to be amazingly knowledgeable and helpful. Should all be good.

On a similar note I have approval so far from Genzyme and am putting together a meeting proposal for the Genetics center for late Fall-early spring for MPS Families to include a few speakers. This is in the planning and gathering info stage yet though I do have ideas for hotel group discount for families who would want to stay, as well as for 2 different meeting locations.

I know there isnt much new in this update but all for now.
God Bless,

Erica

Sunday, May 2, 2010 8:08 PM CDT

I saw the Physical Med Rehab dr fri that my Pain mngmt dr had sent me to in order to get an opinion on my spine muscles - this is actually a different specialist/focus than a Pain Mngmt specialist as they have a different interest typically. - He did botox injections in my thoracic and cervical spine at the siite of my cervical laminoplasty and below this at 3 or 4 levels I honestly am unsure and then he did it also in my shoulders - bc despite very successful PT with the myofascial massage and lymphatic drainage technique my PT uses my shoulders and upper back where still very constricted and the muscles way to tight. Anyways so he hooked up this little machione much like when they do a EMG/NCV and recorded the m uscle activity/response as he was doing the injections and he said afterwards that really there should be no sound but mine where very 'active' as in loud and overly reactive thus the pain and spasms - very odd! It's something I dont really feel (the spasms) I just feel incredibly tight and alot of pain when I bend my neck, raise my arms even a little, reach for anything or lay against pillows - the hope is that this Botox will help this! He is also sending me to a Sports Medicine specialists for my ankles which despite all of my stretching are also overly tight and very uncomfortable when I drive (I have stick shift) or even sit or stand for periods of time.

I also saw my Cardiologist earlier in the same week (last week) and he seems quite concerned maybe even a little frustrated (it is hard to tell with him sometimes) with my whole situation and how treating one problem causes worsening of another problem and is just trying to find an answer for me that can work - we are trying to treat the Raynaud's symptoms which cause circulation problems with my hands turning white, my legs becoming really tired really fast and then becoming sore and other related issues. These might sound like minor inconveniences but when you go into the freezer to grab something out or live in WI with the fluctuating weather and even inability to stand 50's and 60's degree weather if there is wind w/out losing feeling and all color and sensation in fingers and toes. My Cardiol. is very againt using ace inhibitors (which are incidentally often the choice of med with MPS Cardiol for some reason) and calcium channel blockers bc these can cause the aortic and mitral valve stenosis (I also have insufficency) to worsen at a much greater pace than would be otherwise with say a beta blocker which takes the load of the heart) so he and his fellow are looking at otehr med options. In the mean time I am using Ginkgo Biloba which is highly reccomended for circulation and ive not had side effects and seems to work atleast some - if only I could just get my legs to not become so tired with walking/standing (I do stretch out every day, day in and day out with various different pilates and yoga type stretches which are not stressful on my joints but do help a significant amount on the incredible stiffness I experience. I am hoping they come up with some idea to try which might help even better. In the mean time i'll keep taking all of my various supplements and will always do the stretching to which is vital.

Just this past week I was having an icnredible amount of issue with my shunt tubing so I went and asked the Nutritionist at the store I buy my supplements from and she reccomended a supplement called Livaplex to clean out the liver and amazingly I think it is helping already!

We also Finally got the infusion switched into a lower volume so now instead of 250cc it is run/mixed into 180 cc total including w/the aldurazyme and still continues to be around 4 hours - last week the first actual week it ran over 4 1/2 hours so hopefully this week will be the correct rate to time! I do think it made somewhat of a differnce to - the thinking that Dr.Kakkis (formerly of UCLA and of BioMarin and one of the creators of Aldurazyme) explained to me is that the high sodium content in the saline and then the added high sodium in the
aldurazyme conceviably added to my already issue with to much fluid adding to the breathing issues. The other Geneticist and my GC are the ones who finally gave the ok as my geneticist put it off and wouldnt decide and then was gone. I am thankful to finally have it down! Between this and the laqsix I shall be good to go!

I dont even know what my last update was but 2 weekend ago I was in Minneapolis for the American Pain Foun-Action Network training meetings fri-sat. I was able to meet up with the PCL from Genzyme fri after I landed and got in to the airport and then afterwards the weekend of meetings began. Each day was filled to the brim with sessions and part of the time we got to choose which session we wanted to sit in on which also was nice and everything I sat in on and heard was incredibly informative and really interesting! My Pain dr also said to me last week mon when I saw her after my infusion (she is at a different location then my infusions) that she is wanting to try to connext me with some of the people she knows at the Pharma co that make many pain meds like Embeda, Morphine, Fentanyl, etc. I think it was King pharma but simply cannot remember off hand and cannot remember exactly which meds this company makes. She will be going to a Pain conference for AAPM I think it was but im unsure and anyways this Pharma is always there and is going to talk to them about me! How cool! I have to say I am incredibly lucky to have the Pain dr I have not only bc she is good at the pain side of it and helping me manage my pain but also bc she has such a passion about pain as a whole and learning more and helping her pts and in my case helping me to biggere and better things in-stride with what I get to do with APF! I will say I consider my Pain dr a bit like a mentor and find no fault in that but I love learning all the things she teaches me and then having this knowledge to help MPS families especially but then also the public!! :)

I also got in touch with the WI Pain Initiative late last week and heard back from them - this also is a group I found out after having contacted them that my Pain dr (not surprisingly) is affiliated with - I had emailed my dr a ques and she responded w/ a little info on this. The WI-Pain Initiave has the focus more on educating the medical community and I focus alot on the public while also working some with various medical groups - they are interested in partnering and so we are going to work on this to see what we can figure out and go forward with. I am excted and think it will be really informative!

Last but not least I also got permision from Genzyme to begin working on a MPS Family meeting here in WI and so the same PCL I had dinner with in Minneapolis and I are going to be talking on mon and begin working on the logistics of this! Should be great especially since so many families here do not have the opportunity to go to the Nat'l MPS Conferences.

I best run for now, God Bless and please take care!

Erica

Wednesday, April 14, 2010 10:24 AM CDT

It seems I owe an update all the way back past easter - eeks! I think I tried writing one and then lost it all so just posted the easter passage and went off!

Can you even believe it is mid-april already?? How amazing!
The wearther here for the most pasrt has been gorgeous - yesterday 50's today 70's! Time to get my car ac fixed it seems!!! (yey to another care expense lol!)

So right before easter and right after seeing my Pain mngmt dr I went and saw a Physical Med Rehan (PMnR) dr whom she used to work with and highly reccomended. My dr wanted to see if this dr had any reccomendations to see if we couldnt help treat the spinal muscles which are waaayyy overly tight and the cause of some other of my spine problems. This dr initally reccomended muscle relaxers but bc I never had a good effect from these and had lots of side effects he tehn mentioned about trying botox. So that is what we inevitably decided on and it was originally to be set for tomorrow-thurs but bc I am going out of town fri I was a little worried about possible side effects such as increased soreness and having the fact that i'll basically be sitting most of the weekend. So it is now scheduled for teh 30th and we'll start with my thoracic area.

I think I posted about having saw my Cardiol and about his having me go back up on the lasix to every day which i have been doing and while it's not perfect it is a little better than every other day. I also figured out that the infusions where causing more problems with breathing for the day of infusion despite lasix and so i talked to one of the drs who helped create aldurazyme and then sent an email to my Genetics team who my geneticist agreed to try a lower volume of fluid mixed with the aldurazyme. (150cc vs 250cc) Part of the reason the infusion from what I was told can cause increased breathing issues is not only the fluid but the high sodium content in the aldurazyme and in the actual saline fluid.

I also saw a Nsg at Children's Hosp about my headaches - who actually turned out to be extremely nice and also knows my Nsg in MN. He unfortunately also doesnt feel anything shunt wise will help my headaches and the only true option to rule pressure in or out would be a ICP study again which I just dont want to do. So he'll continue to be the local link and i'll continue to look for alternative things to help the headaches - if tradt'l med cant seem to help then maybe alternative med (supplements, massage) can. Who knows.

This weekend (fri-sun) I am headed to a meeting for the Am Pain Found-action network leaders which although I wish wasnt this weekend I think it should be informative and interesting - I know last years really was and I defiently learned alot! I am still working on the display to do and yikes so far behind it feels like!!!!

More soon,
Love and God Bless,

Erica

Monday, April 5, 2010 5:17 PM CDT

"When I saw him I fell at his feet as though dead. Then he placed his right hand on me and said: "Do not be afraid. I am the Frist and teh last. 18 i am the living one; I was dead and behold I am alive for ever and ever! And I hold the keys of death and Hades. 19 Write, therefore what you have seen, what is now and what will take place later. Revelations 1: 17-19

Sunday, March 28, 2010 8:54 AM CDT

Romans 6:8-11
Now if we died with Christ, we believe that we will also live with him. For we know that since Christ was raised from the dead, he cannot die again; death no longer has mastery over him. The death he died, he died to sin once for all; but the life he lives, he lives to God. In the same way, count yourselves dead to sin but alive to God in Christ Jesus. (NIV)

I cannot believe it is alreadt Palm Sunday - 1 week until Easter which officialy means this is Holy week within our church. To me Lent and Easter are simply an amazing time of year not only did God give us his son to die on the cross and days later he rose again to give us eternal life in heaven! Lent and Easter also represent new growth and warmth and the beginning of life all miracles of God for which I am amzed and thankful.

This last week I had ERT as usual which was a little rocky, the infusion seems to take a quite a bit longer than it ever did at our old infusion clinic and I am unsure why sometimes - someweeks it goes perfectly at 4 hours but then other weeks like last it ran over 6 hours which is really lnog and I ended up not leaving until lcose to 4:00pm! Ive also been having it our with my genetics team here and there over silly things in my opinion and I was not feeling well that day which all compunded into just one really rotten day - somedays I really consider trying to get my infusions elsewhere and honestly the only reason I dont is because of a close friend there - he's just like family to me in a way now. I dont know I just wish providers didnt always make things mroe difficult than they have to be and I guess I am trying to teach myself to expect less or with many things go to other providers. For now it is fine but it just seems silly!

I saw my Cardiologist last week whom bumped my lasux dose back up to every day and is having me come back in a month again - I have been having still more off the breathing-suffocating/someone sitting on chest feelings where I literally feel as if like whenyou choke on something except im not choking on anything! It is very weird and the closest ive come to anxiety when it gets especially bad and struggling to get in air -it isnt even like being SOB (short of breath) it simply is like when you are underwater to long and rush up to get air or something alon that lines - very weird and very unerving at times! So I am back on the lasix and the toprol Xl which is a beta blocker is the same - we will see I just hope it helps and I can tolerate this I am trying to do as much as I can t okeep from getting dehydrated and keep potassium and other levels normal! My Neurologist is also starting Diamox which they called in friday that is another similar but lighter diuretic which works in a different way than Lasix and which is used in High pressure to either tryand keep it down or to give an idea if there is pressure going on. That will be 2x's daily.

I have been watching my 3 1/2 yr old nephew all this weekend while his Mom and Chris work and because he has the high functioning autism he has been delayed in some areas - but this weekend he has been potty training I jsut decided yesterday to see if he would try it again as his Mom and Chris had been doing it here and there but he wasnt going and with a slight bribery the 1st time he went potty and then the 1nd and 3rd time yesterday he went without anyything other than praise and this mroning he went again!! He is very proud and so are we!!!!!

This weeks appts include OmnR 9physical medicine rehab) and my Pain mngmt dr. both of which are thurs. ERT is monday of course.

Happy Lent and May the peace of God work wonders within you!

Erica

Wednesday, March 17, 2010 11:04 PM CDT

What a week is about all I can say I think - I really wonder what is up with idioso people and am just incredibly frustrated with doctors though to be fair it is one team of specialists who over see my infusion every week. It is like they promise to help with something and they don't. I ask for help with something urgent but that will take just a couple minutes and they are 'so busy' they can spend 5-10 mins typing out an actually very rude messages whne in that time they could have completed the info I needed. I ask for help with symptoms and in general and they are 'to busy'. When it comes to fup appts they are 'ooh so busy' that they cant see you on the day of your infusion but yet a few weeks after your appt which ends up being on a completely different day than infusion, requiring yet another couple hour round trip drive to see them and thus wasted time when you are already at their office.... but they can manage to find time for another person with the same disorder on the same day as our infuions = WTH? It wouldnt be irritating except when your provider specifically tells you that she cant see pts on infusions days bc she has other clinics every mon elsewhere every week.

Last week the Dean Insurance Rep asked to meet with me and so I went to her office and since my Dad is the union rep in charge of the vote for keeping WEA or changing to Dean he also was there. So initially she thought no problem with getting me covered onto their policy if Dean did get voted in (the vote was tonight-weds) but by monday I had to get her more paperwork and met with her again tues to drop off a few things and go over the strategy. Tonight-weds the vote is complete and no answer yet. I do think this rep is trying and she seems very nice I just feel very discouraged about this whole insurance thing now. I was just getting ready to leave for church (Lent service) when Dad called around 6 tonight they had already tallied all the votes and unfortunately Dean won by a landslide I guess this time. The school district kind of pressured the union members and support staff and also threw in over a $1 a hour raise this time so it's not a surprise it won just is crappy bc WEA has been such a good insur. I do worry now about Mn drs if medicare covers there, and how much harder is it to get services and see specialists if I dont get onto Dean? I am so nervous and just feel sick to my stomach and not in the normal take some zofran kind of way either. :( I hate having my life in other peoples hands. We will continue working on the Dean thing trying to get them to cover me but who knows, just who really knows. It is all just frustrating lately between my infusion team and this insurance stuff.

Since ive stopped taking synthroid my pain has improved much - this isnt to say it's not still bad if I dont take pain meds BUT I take less pain meds and what pain med I do take seems to cover my pain soo much better! Now to just figure out the shoulder issue and get some energy back again? I ended up chopping some more off of my hair but finally I (and my hairstylist) can tell where the old, damaged hair is from the cortisol issues/hydrocortisone as well as we think from a different brand hair dye I had tried to get my hair back to it's natural color. It is 1/2 grown out to it's natural dark brown color and once I get it all back to normal (my hair does seemto grow like a weed atleast) i'll probably start to grow it out long again to where I had it. Right now it is still layered but around chin length.

I see my Pain dr tomorrow and hoping she will help me out will teh insurance forms since my infusion dr was less than helpful mon as having this piece might help us.

I'll update again whenever I know something, just please, please pray for something good with this insurance business.

Erica

Friday, February 19, 2010 10:56 PM CST

Last weekends MPS event at the Milwaukee Wave Game was really ncie and went well - initially myself and my nephew Quinn went with Jake (Skywalk Pharmacist) and sat down on the Wave bench which ment we got an up close and personal view of the players and got to talk to them. After this I went out on the field, the announcer did his thing on MPS, did the kick off with the ball and then Jake, Quinn and myself went and joined my family (my parents, sister and B-in-L as well as former infusion nurse and 2 friends and The Wave won in a very awesome game!! Afterward we went down on the field and got pics and autographs with Wave Players and Green Bay Packers! Very fun!!

Believe it or not I actually have my core group of drs talking to each other and sharing ideas via email on the headaches and what to do which is good! They are trying to figure out if it is Hydrocephalus/pressure-shunt related or if it is something else and how to proceed but atleeast they are talking! Saw my Cardiol. Thurs and he is keeping meds the same (lasix and beta blocker) and also is going to talk to a Nsg he knows at CHW to see if he has any ideas for either someone local to follow me or if this dr would follow me.

Can you believe it has been 5 yrs now since I was dx'd with MPS?? Sun is the 'official' day I met with the docs/GC but got the info over the phone a week prior from my former GC who handed me off to my current GC at that meeting.

Going to try and upload new pics so check above!
Love,

Erica

Friday, February 5, 2010 7:23 PM CST

I try to be a very patient, patient and think I give more than enough slack to drs but I am just pissed right
now and fuming. Lets start with i've already called 3 times to schedule a fup w/one of my drs but noooobody seems to no what the heck to do to schedule a 'special' spot within my drs full schedule - with her old Nurse/secretary this was never a problem and I just incredibly irritated. They say the'll call back but they dont ever. Then drs say they are going to work on something 'ASAP' and get it done by the next week or atleast get things in motion but then you dont hear from them so of course another phone call or message (all these people must think patients have no life and just want to feel aweful. Then bc your not in their office when they do respond they decide to change their mind and 'ooh lets talk to other drs'.. Well im sorry and I fully realize drs have other pts thus why I give them more than enough time but a year ago, 6 months ago, 3 months ago you couldnt have taken care of talking to each other when the tests where done??? Im so sick of providers (other than drs) saying they'll help with something and give a deadline when they'll have it done then the deadline comes and they never even bothered to touch it and dont bother to let you know so your hanging in the lurch with paperwork or things that are already due and to top it off they dont bother to give a reason why they didnt do it or offer to help straighten it out. And oh because it nevers ends Im sick of people who are supposed to be helping with medical related stuff, insur, etc but they dont return calls, tell you to do it yourself (when it is things that are their job and they are getting paid for) plus really dont care if you need their help. Just really, really sick of it. And yes im well aware some who read this will think im whining (I am and dont care right now actually). We as patients pay our drs, and the support staff so providers should not give empty promises. And yes I do have a couple good providers who actually do what they say they will help with but they are the minority.

Tuesday, February 2nd, 2010

I just wanted to update on a Cardiology appt today-my internal med dr had talked to my cardio last week about my breathing and they both wanted an echo and he wanted to see ne after. So he thinks it may be multi-factorial (cardiac, neuro and restrictive lung mix) but that my aortic and mitral valves are not pumping as adequately as they should be so he is starting low dose lasix for fluid and beta blocker to help the heart pumping. I'll fup in 2 weeks and in the mean time will have PFTs before then and he said he may do an exercise (bike) echo which would show how well my heart is coping under stress vs how we did todays and all of the past ones at rest.

I also had the Radiofrequency ablation yesterday (2/1) and went extremely well with no complications!!! My doctor ablated levels T6, 7. 8. and 9 on the left side (facet joints) and we will go back and do the right side in the future. Relief if it is going to work should start very soon (I can tell some benefit in my arms I think already) and full benefits within 2-3 weeks. It was done under conscious sedation so that they can ask a couple questions to verify correct location of the needle (facet joints are not visible on fluoroscopy) and to stimulate muscle in the area to verify correct location which wasnt painful just a weird sensation and after that I think my pain dr gives a little more sedation so as to both put me further to asleep and so that I would not feel the actual ablation procedure (burning which lasts 60-90 seconds each level) and not remember anything after all was said and done. I am curious to see how this works or how I feel especially after the injection site muscles heal at each level! Between this and the myofascial muscle and lymphatic treatment (PT)and the Graston treatments (Chiropr) I am hoping I will get really great improvements!

My Neurol is also working on trying to figure out how to test for ICP pressure above the actual shunt in my lumbar area so hopefully she is able to come through with this and we'll get answers there. I am just extremely glad the RFA went well!!

I also have started (just tonight in fact) talking to a research MD one of my drs put me in touch with and whom she used to work with. This dr is looking to get into a more people/patient centered-makes a difference area of medical research and so has questions for me about genzyme, MPS, the other Pharma's, etc. She seems very nice and I am grateful my dr believes in me enough to think of me when other drs are looking around! I think this dr and I are going to meet in Milw (she is in Madison, I am in Juneau, but we both go to Milw often) as she asked if I would want to do this otherwise we'll talk on the phone as emailing back in forth with all we have to ask each other would take forever I think!!

Wedsnesday, January 27, 2010
Today I saw a new PT whose focus is almost purely Myofascial release and in the future will also work on lymphatic drainage to help clear out inflammation and swelling. I think he was genuinely shocked how tight my muscles are despite even my doing daily exercises at home but does feel like he can help me. I got the permission from my Nurse-CM at WEA the other day to switch my providers as I just didnt think I was having alot of luck with the other therapist and getting pain relief.

I also got permission to try Decompression-traction with my Chiropractor from my Ortho-Spine surgeon which is neat and though I am nervous I hope it will help and I think others of my providers are curious to see what it does to.

We also got the okay to go ahead with RFA (radiofrequency ablation) and so my Pain drs office managed to get me in this coming Monday (I am incredibly nervous and dont usually get this way!!) This will be in my thoracic or lower cervical area and is to try and take out a piece of the pain puzzle - I was talking to one of my other drs the other day who used to work directly with my Pain dr and she has seen her do this procedure and she said it really is an amazing thing and can do amazing things as far as pain and taking out a neuropathic aspect of pain if it is targeted at the right level. (a little tricky in my situaiton as I have such a wide area of pain and involvement and not always easy to pinpoint the worst-painful spots. I will post after when I can - my dr does does sedation for this so generally your not really aware or awake enough to feel what she is doing and the process to get to the facet joints which are what is targeted with RFA in the spine is pretty similar as an injection of steroid/anesthetic.

Today I saw my Neurologist at UofMN and was explaining to her how frustrated I am with having dealt with these headaches or so long and feeling like im just not getting answers and as if my drs because my situation isnt easy to figure out have kind of given up trying to find an answers and that I dont feel like I should have to live my life feeling this badly with headaches, nausea, breathing issues, endocrine issues as a direct result, etc. I dont know if she will really be able to help but she did seem like she wants to and as if she still cares her comments where that the headaches are more than likeally "mechanica" or "plumbing" related and not something which medication will treat (as we've found out) and that a big problem is likeally the stickiness of my tissues due to the MPS Glycogen storage and how this complicates matters. I told her about how earlier this week my Nsg responded to a message I sent him about my breathing issues over the last few weeks especially when laying down and the vision thing with feeling like there is a 'fuzzy' quality or barrier between everything I see. He had responded that the breating things would be an unusual symptom of shunt problem/pressure but that if my drs wanted him to order a spinal tap to measure opening pressure he would be willing to do that in the office. My Neurologist is talking to my UofMN drs and going to get back to me within a week or two and tomorrow in the mean time my NP at my internal med drs office wanted me to come back in and see her so I will do that. I will update whenever I know something further.

I also 'fired' the Oral surgeon I was going to have take out my wisdom teeth this week as he was adamant that I did not need any extra precaution when he sedated me and during the extraction despite the swelling there would be and my already very narrowed airway. All we had wanted was this to be done at a hospital setting sothat if needed in an emergency a Anesthesiol. would be there! Good golly I hate when new drs who barely know me or have met me for all of 5-10 mins try to tell me they know my history and more about MPS that I do!

PS: the Milwaukee Wave Soccer Game and MPS Awarenss is Febr 13th and there will I just learned the other day also be Packers players there!

Will get up pics from my Sisters wedding as soon as I can,
All for now,

Erica

Monday, January 18, 2010 7:08 PM CST

What a crazy day today has been - starting with the CT scan right away this morning for chest to 100% rule out a clot (blood test was done and pretty well ruled it out last week but as a precaution since the chest xray was normal and my dr is trying to figure out the cause of my symptoms. Since they needed to start and keep an IV for this scan I actually got them to leave it in this way I didnt have to have a 2nd one started over at CHW for ERT infusion. After infusion which is 4 hrs I went to yet another hospital and saw my Pain dr which was fine to. We have an entirely new (almost) plan of attack given what I am doing right now for my pain just isnt working that well. My dr talked about a bunch of different options including the new forumaltions of Fentanyl (a trnasmucosal, a 'lolipop' and there where some other things to ) but these are only FDA approved and regulated for cancer pain (she was equally unhappy as I am about this given the LARGE population out there who could benefit from medications which do not affect the GI system and thus have less side effects. We've decided I need to go out and have a talk with these regulatory folks (joking...sort off..!) We then talked about Methadone which
is a different category of pain med than what I currently take but she is concerned with the cardiac side effects methadone can have (and withdrawal effects are down right nasty) including prolonged QTc interval leading to ventricular arrythmias among other cardiac affects so that is ruled out. Other categories such as Oxycontin are ruled out due to the affect on neurologic.Somewhat short list we have! So after quite some discussion we decided to go back to a med I was formerly on and increase the dose of another med I am on. She also is talking to my Pharmacist and wrote a script for a med to be compounded using lidoderm gel and ketamine (a anesthethic usually given before surgeries to put patients 'out' but in a compounded form can work effectively for pain apparently as it is sometimes mixed with still other medications and the stength can be taken down. The other thing we are going to do is RFA (radiofrequency ablation) in my upper back area to try and take out one aspect of my pain

This weekend is my sisters wedding - other than the fact I still havent found shoes I think we are all pretty excited and I am getting my neice on thurs and then we'll take her home sun as my older brother, her Dad does not want to stay for Sara's reception and Alissa wants to. (he has some work xmas party he wants to go to believe it or not vs his own sisters reception) In any case i'll post pics sometime soon after.

Have to run but more soon!

Erica

Tuesday January 5, 2010 9:18 PM CST

Hello, Just a quick update, I happened to stop in at the Childrens Hospital Pharmacy yesterday and the Pharmacist whom I know a little came over and asked if i'd be willing to represent his Pharmacy-Skywalk and Children's at a Milwaukee Wave Professional Soccer game and I also thus will get the chance to sit by the players as well as address the stadium at one point during the game and speak on MPS!!!! What an AWESOME way to raise awareness of MPS to a large population!

Friday, Januart 1, 2010
HAPPY NEW YEAR - May 2010 Bring Happiness, Joy and Health!

I wont make this real long as I am having super sore shoulders this week as a result of falling on the ice last week and then of course also the PT ive been doing in which we've been focusing on my shoulders quite a bit as part of the cause of my mid-back pain we think. In any case
I thought I would start the new year with an approprately new entry! :)

I dont think I wrote at all about this last time but I had asked my WEA L.T.M casemanager about having a Nurse-case manager assigned to me whom would be the sole person in charge of approving therapies, surgeries, etc. vs having a different person to deal with in customer service every time I/we (any of my medical team) call. Well last week Erin my other CM called with the name and to say the request was approved and this week my new Nurse-CM called herself to introduce herself. This will be especially good for Physical therapy and related services and with the many other things to that we deal with related to ERT, etc. Joane has also initiated trying to get my ERT shipped directly via Genzyme vs going through a middleman as we do now with Theracom and their Pharmacist is currently negotiating to try and get a same/comparable deal with genzyme ie drug charge. Normally it would likeally be cheaper to cut out a middle man but atleast in my case bc Theracom is part of WEA's caremark pharmacy 'family' they get a cheaper price through them bc it is in-network. My GC had also left a message the week before Christmas that she was working on something within her hospital re the Aldurazyme shipment so I need to fup with her and find out what that was and what she has found out. My PT is very happy we have 1 person to be working with now as we tend to think her knowing my case and background history better in time will allow her to see my needs for PT are different than a general say surgery/rehab patient.

ERT went fine this past week other than the usual annoying IV issues and with about an hour left and the IV literally would not stop beeping unless the nurse held it and I stayed in one position (impossible for me bc of back pain, legs falling asleep and discomfort of what we have to sit on/in. I finally told her I would love (kidding sort of) if she would just restart the IV (the 3rd time) in a different location as I was a little irritated with having someone stand by me for the last 30mins or more holding my arm and the beeping was clearly driving both of us quite nuts. I think she was relieved I was willing to let her restart it, but I dont know of anyone else but the beeping was incessant and on top of the daily headache making me grouchy and neither of us really wanted to deal with it for another hour. I dont think ive made it out of the clinic in under 6 hrs any of the weeks now since we've been at the genetics clinic location - it takes so much longer for the nurse to start the iv, mix the med and then seeming problems - the last 2-3 weeks ive increased the rate by 10 to get out atleast within a reasonable time due to having to get to other appts elsewhere (usually PT) and still the infusion has never been done in less than 4 1/2 hrs!

This coming week I see my Pain dr, Neurologist, other Pain person as well as Opthalm (one at FMLH that my Internal med dr reccomended me to) and PT, yikes!!! I also have a zillion phone calls to return from my fellow Pain leader here in WI, to insur people, to application-renewal forms, and just friends whose calls I havent been great about returning in the past couple weeks. (sorry guys!!!!) My nephew has also started his ABA classes and literally almost every day is in his classes either the ABA (usually 8 hrs a day I think) and preschool for 10 hr days so I just remind myself if Zander a 3 yr old can handle the craziness with only a little grumpiness I can to! ;)

Today-New years Day my Insurance Lifetime max reset to a lovely $0.00 balance and $2million more for another 5 yrs!! This is AWESOME news bc not only was I precariously close to maxing out my previous $2mill LTM but given how much Aldurazyme costs and costs of all the other MPS related things - surgeries, procedures, drs., meds., etc. this is all a much needed amount!! (our LTM renews every 5 yrs back to a 'zero' balance and 'fresh' 2million balance to work with. I do have secondary insurance but this private insur vs my secondary is heads and tails better as far as where I can go, who I can see, etc.

My New years resolution for 2010 is to continue to learn as much as my mind cna take in on nutritional aspects of health and what I can do or take i naddition to what I already take for myself as a means of getting to a healthier, better feeling place and hopefully at some point as place with even less need for prescription meds other than the 'vital neccessaries' like Aldurazyme and for now Pain med but ultimately maybe I can get to a point where I need even much less of this - if I came as far as I did in 09' in nutritional aspect I think I can come even further in '10 it is simply a learning curve and process! By learning what I can I can use this information to help other interested MPS families, Pain individuals and my own family especially my nephew - as with all the leaps and bounds he made in the past 6 months without having been in any ABA program (till a couple weeks ago as the wait list is long) or other learning program other than daycare and then preschool in fall but having been on several key supplements (namely probiotics and fish oil w/flax seed oil and multi-vit occassional other things by trial and error) I totally believe nutritional aspect plays a huge role in autism and hope I can cont to learn more for his benefit as well!!! (if you where to see his speech now vs even a year ago, and his other skills you'd think it was perhaps a different kid - he is able at 3 yrs old count 1-10, ABC's, sing a song or two (namely twinkle, twinkle) on his own and ask for what he wants plays much more focused with his favorite things - trains, kitchen set, connect 4 (with us, but he waits his turn and has even won several games which is doing better than me most of the time!) and loves to talk about his salamander.) My other hope is to cont to be able to just plain be a source for MPS families to call or email or whatever and help out with the support committees ive been asked to.

I better run but wanted to post atleast somewhat of an update!
Again Happy 2010-May God bless and keep you!
Erica

Wednesday, December 23, 2009 11:05 PM CST

Going to try and make this short as I can only seem to sit for very short periods of time the past week or 2 without my legs falling asleep again and thus painful!

First I want to wish all of my family and my friends a Blessed, Merry Christmas!
For my friends whom have lost family I pray for you on this holiday season as I cannot begin to imagine the pain you feel.

My parents and I returned from the MPS Disney Conference at
Disney World in FL on Sunday where the weather was averaged
in the 60's-70's the entire time and quite lovely compared to the snow and cold here! The conference was held at the Coronado Springs on Disney grounds and was just a HUGE hotel which made for hard getting around it as everything was quite far from our room though we where only in Bldg 3 of like 8! The conference was in Bldng 1 I want to say within our Cosceitas block. Other than venturing over to check out the larger pool with the slide and playground I never did go in there as it was atleast a 1/2 mile but I think further walk around the Lake from the room and meetings and by the end of the day was purely exhausted every day! The dinner w/Genzyme on Weds was very nice, we inadvertently sat at a table with all Genzyme employees and was very lovely, though I have yet to meet anyone from that Com whom I havent liked. Thurs. panel was also good and seemed to go well - they had one of their physicians speak for a small part of the time and the rest was QnA and us Panel members speaking. There were an addt'l 4 MPS I families this time in addt. to the original 4 families from 2 yrs ago and though alot of the teens/young adults didnt say anything or were very quite I still think it went well. I tried to share some things I thought would help some of the other families and vice versa and I also was able to get some ideas from the genzyme employees on my infusions, etc. Thurs night we went to downttown disney shopping/browsing and then after getting back I went and spent some time with some families including my close friends from Il the Marcotte's whom Lucas their middle child saw me coming down the hall and ran up to me, which was so cute bc it has been atleast 2 yrs since ive saw them last in person and the last time I saw him, he did the same thing when they picked me up at the airport! Friday I did not go to the morning sessions as my parents rented a car and we drove out to Wal-Mart to get a few things and then they drove to Daytona Beach to the Daytona 500 speedway and I came back to the afternoon sessions where I got to meet my friends the Clendenny's, Michelle Butts, Amanda Craig and the White's and Kelly + Caleb Waddel amongst other families of course. Very cool! I deifnetly wish I had not been so exhausted as I would have LOVED to spend more time with Elizabeth, Kelly, Michelle, Amanda, and others! At fridays GALA dinner I did sit with Michelle and Amanda )plus hr adorable girls) which was really nice bc we talked quite abit about different things and seem to have alot more in common then I would have even realized in some ways. Sat my parents and I decided on going to Magic Kingdom and WOW definetly not like I thought it would be but still fun I just never would have made it w/out the pass for skipping the lines! I cannot say enough thank you's to my Internal med dr for writing a short note for me for this!

I do ahve to admit I was happy to come home I suppose mostly bc it being so close to Christmas - and other than our Luggage having got lost (we did finally get it on mon) all was well! Now I am writing this as my nephew sleeps curled up with the Christmas goofy my parents bought ( I really, really wanted it but didnt know if I wanted t buy it since I had already bought a few other things and they decided to buy it since otherwise they are always only buying me little stuffed animals when im in the hospital for surgerys! (as you can imagine I have quite a collection of big and small stuffed animals!)

The monday before we left for Disney I was about 10mins from Childens hosp. when my case manager called to tell me not to drive down to the hospital bc they didnt have my drug there -it hadnt been ordered/shipped! Of course this was already 1 hr into my drive and I was to put it plainly quite irate that this would happen and know 'check' was in place on thurs or fri the previous week to make sure infusion drug was ordered and there. Then to top it off I get up to the genetics clinic to talk to the other GC (not mine) and my Geneticist walks by and says 'ooh well it wasnt our fault' and starts to walk away - AS I was talking to him yet! I firmly believe to drs need to treat patients exactly as they themselves would want to be treated and bc I was so tired from getting up early, and frustrated/mad about the whole situation I basically just blew up at him yelling at him that he needed to atleast try and pretend to care a little about his pts, and to show a little courtesy to pts when we are talking to him. He wasnt thrilled with this all but I do not care and refuse to be treated like I do not matter and like my time isnt as important as someone elses is just bc that other persons happens to have fancy training. I just was sooo, soo incredibly mad and anybody who knows me well knows it wasnt a finest moment not that I cared or would ever apologize! This week he comes by (and he NEVER bothers to come by during our infusions) trying to impress the residents/fellows who where with him and spouting off this information about me and different MPS related things and with any other normal dr I would be very nice or at the very least hold a conversation bc my other team of drs actually seem to CARE! Anyways so he's saying this different stuff and things which where totally not true but basically he was trying to impress them like for instance saying I was in the original clinicl trial for Aldurazyme bc of him - which I wasnt even dx'd at that point! I point blank corrected him and told the residents not that I was trying to be rude (and I wasnt but I do think drs should know the basic information about their pts and most DO!) but that I was only diagnosed 5 yrs ago and the trials where some 10 yrs ago I think. (not sure exactly off hand) and then told them a little about the IT trial. I dont want to come off as a smart alleck by writing this all I simply want is for those of my drs and providers reading this - keep in mind behind your patients are still actual humans and if you cant remember something about them but want to share it with someone else, all you have to do is ask your patient bc patients usually are just as informed and knowledgeable in alot of ways in their own care history. Non the less you can see why I rely much more heavily on other providers on my team.. and I do have my GC whom although to incredibly busy is a good general resource when I absolutely need her help.

There are other things to update, I will come back and do that later. For now I want to wish all a Blessed, Merry Christmas!

May Christ be with you in Christmas,

Erica

I posted pictures above from Disney.

Thursday, December 3, 2009 10:33 PM CST

It has been 1/2 a month since I last updated, actually it was right after the surgery (carpal tunnel on left hand for 2nd time, scar tissue removal and flexor tenosynovitis in wrist. Anyways although I just got the stitches out yesterday (they left them in for 16 days) and wearing a compression sleeve for another few weeks I am getting some more movement back and this is sligthly easier than the last time I updated.

Other than some new pain med trials of a new long acting med and a new PT location there hasnt been alot going on. Im not really in love with the new pain med as it seems to have even less of an effect than the other long acting I took before did and makes me really tired. This one is taken 2x's a day and ment to last 12 hrs each whereas the other was ment to last 24 hrs and also had an immediate release med built into it. I think alot of it has to do with the way I metabolize meds apparently and thus not always a good, lasting quality. I saw my Pain dr today and told her about these symptoms but she is just going to have me take one of the immediate release pain med right alon with the long acting in the morning and in the evening to see if this might help. Im not thrilled to have this extra tiredness though and ultimately think that is going to be a problem as we where just getting to a fairly ok place with my cortisol dose and my energy level as is was!!! But I am willing to try it for atleast 5-7 days and if no change in pain level and in especially to the fatigue i'll call her office. Otherwise I am to see her in a month.

Funny she told me today several patients/people she knew heard the interview on Pain Care on NPR and asked I guess if I was a patient of hers - so it is good that this interview is raising awareness out there and getting people talking!

She brought up about how far she has seen me come since she's known me the last couple years in terms of coping, fighting for myself and for my own best care and how strong i've become to her. It's nice to hear someone say something like this to you bc sometimes I sure don't feel strong and sometimes when im sitting here and feel so aweful and am so tired and cant get anywhere I guess it's the really important people like family, really close friends and drs that actually care who get us through. Sometimes when i'm literally throwing my phone in frustration and just want to strangle someones neck and call it all quits I guess knowing I am making a difference in peoples lives maybe makes it slightly easier? Today I was sitting talking to my former infusion nurse (we did lunch) and we where talking about another patient who happens to now be good friend of mine and about all their frustrations and the shittiness of their situation and I just couldnt help but think that atleast this family has someone like our former nurse to step in for them and care and to help - sometimes I wish I had someone who I could really go to, to help me with some of the stuff. Some of the things my Genetics team or someone is supposed to help with (finding specialists, getting into specialists, getting letters, making calls, etc) I am really, really glad my friends have our nurse bc I know they desperately need her support I guess I just wish... maybe I just wish I had someone who listened and didnt always look at me and see me as stoic and strong and instead looked beyond the face value and would realize I need someone sometimes to listen to me to? I don't know I think anyone who has that quality of being a good listener would tell you that you in turn have a hard time finding someone to listen to you bc it inevitably gets turned to how the other person is no matter how well meaning a person is. I think sometimes that might be one of the hardest, most invisible parts of my particular form of MPS I being you look so normal, so if you take time to do your hair and wear a little makeup people forget you have something. Granted im thankful to look normal just is hard sometimes when you just need someone to vent to! Tonight a former ex called I had, had to cancel our dinner bc I wasnt going to be home in time and anyways he called on his way home later tongiht to chat and I just told him I couldnt talk after like 2 mins of listening to him talk about his night, his plans for tomorrow and for the weekend.. I get sick of friends who it is ALL about them! Sorry for all my friends reading this I am not saying dont call me I am very willing to listen just for non-MPS friends consider that other people somtimes could use a listening ear to and dont forget life isnt just all about your life and awesome plans.

I better go b4 I tick off my entire cb reading population in form or another though. Please dont take my words personally, it has just been a long few weeks.

Erica

Tuesday, November 17, 2009 11:26 AM CST

http://www.npr.org/templates/story/story.php?storyId=120381128

A copy of the interview from NPR - alot of each persons interviews where left out but thats to be expected w/time constraints in radio. But any awareness of Pain is typically good awareness non the less!! Myself and 2 folks directly in the pm field are interviewed in this article. Thanks to Patti Neighmond of NPR for doing this!

Todays CTR surgery went well - originally they planned to do a nerve block for the surgery but then opted fo LMA for safety-airway. Dr.D cleaned out the synovial tissue, released the carpal tunnel and freed up scar tissue. Other than the soreness at site, stiffness and welling am doing fine. (typing one handed) I'll update more when I can type a little better!!!

Love,

Erica

Erica

Saturday, November 14, 2009 0:27 AM CST

Just a quick update - earlier this week I ran into my Pain mngmt dr while dropping off a form for her secretary at her office which isnt where I see this particular dr. Anyways so we talked for a few and she took me off the Nucynta and wrote a new script right away for a short acting opioid which is supposed to be from what i've read morphine derived but stronger. I started that earlier in the week and can't say I love it but I don't hate it either and don't feel terrible like I did with the Nucynta. We're going to give it a couple weeks to see how it works but if I really need to she said I can go back to the MSIR. Im unsure if that is whats causing my incredible tiredness (more so than even normal) this week or something else - who knows, it's never clear cut. I saw her yesterday (thurs) for the regular scheduled fup appt and she started a new long acting medication also in the morphine family which the pharmacy is ordering and I should start monday or so given theres no insurance issues. (I did check online and it was covered atleast by my Part D insurance w/no prior-auth. supposedly needed.)
This med is written for 1-2x's daily depending how a person metabolizes it and given how I metabolize the avinza in such short order my dr just wrote it for 2x's daily right off the bat hoping it will last longer.

I havent heard results from my Endocrinologist yet which I am slightly frustrated about as it has been over a week now and I did leave a message. She is normally one of the best drs I ahve about calling with results but the last 2 times hasnt and im unsure why - I emailed her tongiht hoping she will respond bc honestly it is my body and I think as the patient I deserve to be kept fully aware of all test results to my all of my drs. I also sent out a group note to my core group of providers including this dr asking for their help in dealing with my headaches, asking them to work together since alone we are all getting no where and they each have their own ideas so maybe as a team they can get somewhere-working together. I am usnure but what I do know is I am tired as all hell of these headaches and will keep pushing till I get an answer somehow on how to treat them one way or another.

Monday is the carpal tunnel surgery - 7 yrs and 3 months since I had the original release done and this time my surgeon (different hand specialist) is also planning to do a release of surrounding nerve and clean out scar tissue if needed. So at this point im unsure how extensive it will be.

The interview on Pain care and hospital experiences also airs on Monday this week the 16th on NPR's morning edition where one can look up their local station times. I will also post a link here once it is up on morning editions site.

I had a call today from the coordinator at Genzyme pharma who is doing all the meeting arrangements for Disney conference and our Patient Panel - apparenyly Genzyme is giving us a speakers fee of $150.00 which seems a pretty decent amount in my opinion given they also are paying airfar, meals and hotel!

Wish me luck Monday,
Love,

Erica

Tuesday, November 3, 2009 9:40 PM CDT

I was driving back from Milw today and realized a year ago today was the c-spine surgery-WOW! Maybe this will be the Christmas I acrually get to spend in my own place with my own tree, lol!?

My Pain dr called me yesterday due to my increasing neck pain which had worsened even over the weekend and she is/was concerned enough to want to schedule an c-spine (neck) MRI for this week which is thurs and then will see me that afternoon. We are also planning to likeally change up my pain meds as I have grown pretty 'tolerant' of the doses I have been on now for several years..I will update after that appt but I seriously do not know what I would do without this dr!??

I heard from Patti Neighmond the NPR reporter today that the Pain interview will be on Morning Edition on Monday Nov 16th which ironically is also the day I have my left carpal tunnel release. It should be a good piece and I will post a link to it here once it is online at morning editiion.

I saw my Neuro Endocrine dr today for 3 month fup of hydrocortisone/cortisol levels and thyroid - despite the shunt results and Dr.Haines opinion at UofMn she feels given the continous up and down cycle of my endocrine levels in tune with when I get the headaches worse (ie before the shunt was put in the levels where down, then they improved after the placement, went down before each subsequent revision and levels subsequently improved after each revision and are down again and along with my headaches, weight loss, and nausea that she is still concerned about it somehow being shunt related especially bc the two shunt studies where so similar. (the one before the last revision and this recent one). Ironically she works with Dr.Sinson my former Nsg who put in and revised my LP shunts on alot of other neuro cases and I explained to her why we switched from seeing him, given his refusal to deal with my situation anymore but she is still wanting to talk to him an to Dr.Peltier my FMLH Neurologist and explain her thinking and is going to do this when in clinic with him later this week. I don't think either of us expects this to go anywhere realy it's more I tihnk she feels like she should do everything she can internally to try and help. Another one of my great drs, and is really sweeet! She even said she thinks she should ahve one of the fellows write a case study on my situation with the MPS and how the endocrien issues and Headaches have presented for future Endocrine drs and doctors in general trying to treat MPS pts with similar issues especialyl she said bc most pts will not see a Neuro-Endocrinologist who can truelly delineate differences in causes and symptoms.
Dr.Rothman my Neurologist at UofMN also called this evening and is getting in touch with Dr.Peltier, i'm not sure why they hadnt ever talked before but non the less he very much wanted to get in touch with her..he is a nice guy as well, definetly much more laid back that my former Neurologist there was and always ends his emails/calls asking if there is anything else he can do for or to help me - it's amazing how different drs can be even drs in the same specialties!

I will update when I know more...
God Bless.

Sunday Nov. 1, 2009 6:12PM
What an aweful but still semi good weekend if that could possibly make any sense. My neice Jordan who is just 3 months younger than Zander (3) came up Sat and I took her with Quinn and I to Waupun where we went trick or treating with my sister, her fiancee Chris and his son Jordan in the 40 some
degree weather with extreme wind! Crazy!! It was fun though and will post the new pics. I havent been sleeping well due to really bad and new neck pain which is causing pressure, worse nausea, burning type sensations right down my arms and just bad pain which made yesterday harder but I went anyways. I think though if i'm still feeling this way tomorrow-monday I am going to try and get into my Internal med dr after infusion is done (first day at new clinic at Children's Hospital-Curative Genetics clinic) and see what she thinks or if she thinks xrays should be done - as this has been getting progressively worse for the past week. Just please, please pray it goes away and maybe just an adverse swelling from the thoracic facet injectios though I think those are supposed to reduce swelling on the spine not increase it. I am so frustrated with this!!!

Saturday, October 31, 2009 2:54 AM What a week is about all I can say! Thank heavens it is Halloween - it is windy as all get-up outside and supposed to stay like that right on through the day to trick or treating but atleast the week is over. Quinn my npehew was sick this week but his Mom sent me a text tonight (fri) that he was feeling better so i'll pick him up this morning, we'll go over to my parents where my brother is going to look at the tye-rod on my car and also tires I guess and then we'll head over to my sisters make sure Zander gets a nap before 4 (starts at 4 until 6) Zander is going as a very cute puppy dog and Quinn is going as Chucky as in from the movie though I have yet to find bibs but have the makeup and a similar shirt and sword for him and we'll add lib the rest - probably have to get creative with the rest of his costume - I was going to paint his hair orange but if it s going to be so windy not sure what i'll do since I want him to wear something covering his ears. Grr WI weather!!

Earlier this week I started seeing a different, more hands on PT that my pain dr reccomended he seems good and interested in learning so it should be alright as long as we get Insurance to give the ok although I do have 2 backups. I also finally got Dental approval through PSI so having root canal and couple crowns down in another week. And then the 12th I have the carpal tunnel.

I had the interview with Patti Neighmond of NPR on Tues and was interesting but I think it should turn out well - the air time will be the 16th bc of the Flu stories they are putting on and she is talking to a Pain mngmt dr and another person in Salt Lake I think for this story and has to put it all together. Interestingly she did say I should keep her contact info for next May given she's their health reporter and is interested in MPS. Very cool. I will update when I know details of final date and a link to find it.

Mn finally got the 06 shunt study images downloaded and my Nsg emailed me today just a short not, kinda frustrated he wouldnt have called with this results/his opinion given the differing opinions but he said the 2 studies are very similar and that this study from 3 weeks ago appears to be working. I asked how they could have the same results and yet back then it was revised by my former nsg but now it is 'ok'. Kind of frustrated that there still is no answer and still have no way to treat these headaches. I will wait to hear from some of my other drs and will make sure we find some way to treat this bc I am just tired of them itnerfering with my life and making me feel aweful.

After Chris and I got all the way down to Elmbrook yesterday we where told my Pain dr was out sick (an 1 1/2 hr drive each way) for which I was pretty upset the hospital oupt dept couldnt have called and told me this infor. especially since I live that distance away and had already been to milw 2 other days that week for other stuff. Needless to say my Pain drs secretrary did reschedule for today at a different hospital she is at on fri - I have been to this hosptial but had been like 2 yrs since the last time and my brother who went with today bc of the sedation (Chris is my to be brother in law) and I where a we-bit lost! Then bc of my increased pain level lately I hadnt slept more than 3 ish hours the last couple nights each and was pretty exhausted after the injections so was feeling pretty out of it. I came home and slept till mindnight which would now be why I am up, have a headache, nausea and some pain so unable to sleep. Go figure!! My pain dr did say she thinks we should talk about at my next appt switching my current pain meds to newer, different options bc I am no longer getting the same pain relief benefit and no sense staying on something which isnt working that well. Anestheshia especially when I am so tired always makes me sooo, sooo forgetful well I definetly too that to a new level today - I kept forgetting things I had done yesterday as well as parts of conversations we had before the sedation and inections began. It is so weird the silly things you can remember before and during these but then always forget the more important things which drives me just about bananas! I am one of those organized-disorganized people meaning I forget EVERYTHING unless I write it down and ALWAYS have notes all over in my planner and on my desk for what I need to do, need to ask, need to get, etc. and so being in a situation like the sedation where you forget everything always makes me want to have a second copy of me standing by to remember! Hmm maybe I should make my twin brother my new secretary?? hahahahaha. :D)

Wish me luck I don't go postal on someone about these headaches (I really am only joking) and that we have a great time with the kids trick or treating! If im feeling ok i'll go with otherwise i'll hang back and pass out candy.

Levitivus (verse 12) I will walk among you and will be your God, and ye shall be my people . . .

Be well my friends, my family and all,

Erica

Thursday, October 22, 2009 10:35 PM CDT

I've not heard from my Nsg yet which is a bit frustrating but have other news to share!

I had received a request from our APF media coord the other day if i'd be interested in doing an interview for NPR health reporter on my experiences with Pain and pre/post op hospital stays ie if pain was addressed, managed, etc. So I spoke with the Public Radio reporter today (i've heard some of her prior peices on the radio and they are very good) and afterwards she asked if i'd be willing to do a taped portion for her at one of their stations (likeally W-UWM in Milw) and I guess either she will phone in via conference call to do the interview or have one of the radio people there do it! This is awesome for raising awareness of Pain as awhole, for raising awareness of MPS especially and for helping increase the word about APF and our work!! I'll post the actual date of when this will air, though it probably will vary from state to state.

Wednesday, October 21, 2009 9:35 PM I don't know anything from my Neurosurgeon in MN yet to update on other than I know they received the cisternogram disc this week from FMLH, where uploading it to compare to my study 2 weeks and my Nsg is going to compare the two and will get back to me.

In other news (rarely dull I guess) I saw my Hand surgeon at FMLH and he is proceeding with a 2nd carpal tunnel release of both hands - my left being first as EMG's show this has clearly reoccurred and is quite significant once again. The last releases where done 7 years ago almost exact in August and sept which was 3 years before I was dx'd with MPS I and 4 years before I started ERT which may be why it reoccured who knows. (it is pretty uncommon from what I know atleast for it to reoccur once a patient is on ERT). They are in hte process of scheduling all that.

I also saw my Cardiologist at CHW and Echo stayed the same from a year ago with moderate stenosis and leaking of aortic valve and mild/moderate mitral valve. Despite all my joint problems and the pain I even improved on the stress test which was impressive to us all! It was funny to see that I saw him not only on Halloween a year ago but that was 3 days before my Neck surgery, so glad that much is atleast behind me!

I will update when I know more...off to have Thoracic facet injections tomorrow.

Wednesday, October 14, 2009 1:07 PM CDT

See the previous post for all news related to last weeks Lumbar puncture-Nuclear med shunt study. ... It seems it will be next week until I officially know what my Nsg thinks of my current shunt - he is out of town right now at
a conference and though the official radiology report reads the shunt as 'functioning' he wants to compare my previous Nuclear med studies from 06 (previous to the 2nd revision) to this current studies to see if the same phenomena is going on I guess before making any decisions one way or another what he thinks. It seems he isnt ruling in or out that it is truelly my shunt causing the headaches and subsequent other problems and symptoms but wants to cover all his bases before saying it isnt the shunt, I am unsure. This is basically the same predicament we where in back in 06 at that point there where no studies to compare to and my then Nsg said a revision could work or it might not work relieving symptoms wise and it did when I decided to have it several months later.... this time I just know even if some drs don't believe me that the shunt isnt flowing as good as it needs to be ie a partial obstruction or something. I found this article and found it interesting.

http://www.ajnr.org/cgi/reprint/21/4/721.pdf

This is the email my Nsg sent to me, I normally wouldnt post these on here but it kind of explains his feelings better than I can.

"As you know, the official interpretation (which was only recently posted on the radiology site) is that the shunt is functioning. I will need to compare with the older studies when I return.
Your April MRI shows what I would call slight enlargement of the central canal of the spinal cord. We all have a spinal fluid containing canal centrally in the spinal cord. Yours is a little larger than most but not large enough to be considered hydromyelia or syringomyelia. More when I return next week."

Please, please, please pray for answers and for all the different things to be taken into consideration and to point to an answer.. especially given I don't quite get why the Ns resident and Neuroradiologist clearly told me the shunt was not flowing as adequately as it needed to be which isnt the same of course as it simply working or altogether not working but none the less grr is there ever justt a clear cut answer when you know?

Friday, October 9, 2009 4:59 PM CDT

It seems like when it rains it pours and today has been the pouring rain after the shower yesterday. Not only did I end up not falling asleep for several hours bc of trying to get comfortable but I was really upset from some past medical reports I was reading from several drs. and just was floored that the one would even write such stupid remarks and put them in a report which goes out to many other drs of mine - do some drs ever stop and think about what their remarks in reports mean and that they make it even harder for us pts to have our drs believe us when we say their is a problem? Just bc MPS is a total body involved disease and takes alot of drs to care for many different areas of problems and bc I push for answers (bc I live in my body and I know my body) means that I am basically in this drs words making stuff up?? I am so glad now that this dr has left and moved elsewhere and just irritates me how wishy washy he could be when it came to helping - ie: in the report previous to this one (a month earlier I think) he talked about how it was likeally neccessary to have the spine surgery as he had seen several pts with long term complications bc they waited to long for surgery but yet when I am having blacking out episodes, numbness, pain, and other symptoms which where clearly visible on their little tests I was making it all up and to focused on my care???? Give me a freaking break... don't ask me why I even care I guess bc like with my shunt this time I kept saying it was pressure related somehow and probably something with the shunt being partially occluded in some way but very few drs believed me (was I ever wrong about htepressure h/a's before the shunt was placed or b4 each of the 2 previous revisions??). I just think there has to be a change in the way drs and patients work together and that drs need to learn to trust their patients more and patients need to speak up and not be afraid to push for their bodies when they are the only ones truelly experiencing the problems.. I am not saying all of my drs frustrate me bc I know some of them genuinely try and have tried their best to help get answers but somehow this crap that some drs write in their reports, the mis-information they get and not trusting their pts is not great. This all said some of my drs the truelly great ones know that I wouldnt write this about them..

I went to PT today and another added blow, my therapist doesnt feel like he can offer a whole lot more to help with my back pain as he doesnt think ultrasound would work bc of my shunt location, and that we may just be stuck at this point of pain and problems with muscle tightness and contractures. Given how much he was able to help me with my knee pain and knee stiffness i'm really surprised and disapointed which I know sounds funny but we where just getting to a point where I felt like I could notice a difference in my pain w/in my mid back and the subsequent radiating pain to my arms. I know it will be nice to not have that appt 2x's a week but honestly I just rather would have to take theat 30mins for a while longer and get to a better place hopefully before we stopped. He reccomended I do water aerobics, swim laps and/or do Pliates all of which i'll look into and i'll keep up with the exercises both of course for my knees and for my back and just hope and pray I am able to get to a better place on my own. He told me that it is harder with my back bc they would normally do 1 set of exercises/stretches for people with disc bulging and completely different ones for people with spinal stenosis but since I have both of these things going on to a pretty significant degree that progress will be slow and if I did pilates i'd have to stick with
the beginner type things. I guess i'm just kind of discouraged this week but all the various crappy things and so tired of constantly trying to improve my well being physically and yet I come across cosntant resistance and disbelief and things like with PT where basically insurance companies make the decisions and tired of being in pain from my back and from my headaches and just wish for once I could get ahead of both of these things. It is so funny to me, for instance my therapist who is a nice guy expressed disbelief in even doing surgery for Hydrocephalus which he clearly didnt quite get what it was/ment symptoms/secondary problems wise and he himself admitted he didnt know alot about it was asking me alot of questions. So as I was leaving he said 'good luck with whatever decision in made about your shunt and if you need another surgery, I don't think I would go through it'. The thing with that statement being that 1. I don't think anyone in their right mind ever wants a surgery and 2 who in their crazy right mind wants someone placing a mechanical system within their spinal cord to drain fluid BUT at the same time then you just let subsequent secondary symptoms keep cropping up which you have to deal with, including endocrine issues, weight loss, nausea, vomiting, vision problems/loss, etc.???? I think most people don't quite think about the ramificications of doing nothing with MPS problems - the same as spinal cord compression how many times I heard 'wow I could never let them do that to me' but when it is your body it is happening to, you have all these scary symptoms and you have no control over your quality of life and ability to go out and function normally I think you do what needs to be done to help your pain, your symptoms and to prevent even worse problems. I just wish people really understood or knew more about MPS before they said some things.

I am going off my soap box now but just rememeber only you or your child truelly know how you feel and YOU need to be the best advocate for yourself and/or your child that you can be even if it means being pushy, stubborn or persistant at times.

Thursday, October 8. 2009 10:15PM
Today takes the cake for worst day in a long while and while it wasnt probably even that bad I just am exhausted beyond exhausted, have a horrible lower back ache and just seemed like nothing with the tests could go right. I guess by this far into my experience with shunts I should know far better than to let a resident or anyone other than a Interventional or Neuro Radiologist do my Lumbar punctures as they almost always have tons of experience with these and always use Fluroscopy... but going against my better gut feeling I let the Neurosurgery Resident attempt (key word) my LP's today and he didnt want to use Fluro guidance despite both my and the Tech telling him he really needed to bc of my shunt and bc of my 3 lumbar spine surgeries and many past Lumbar Punctures... I just had this sinking feeling it wasnt going to go well and was I ever right.. i've never cried during a LP in the past (have had many of them to) but this resident just could not get the needle placed into the correct position and kept hitting nerves which then sent a fireworks display of pain down my back and legs which was worse than just about anything I can describe and this happened with all 5 of his attemps to get a needle placed - he kept having to remove them and start over and this went on no lie for well over 30mins until the Tech said he was calling the Neuro-Radiologist who came down, had me lay on my stomach flat and used imaging to guide the needle into place so that he could inject the needed dye-tracer for the Nuclear-Med study. He also commented how much scar tissue there is and how much thicker the ligaments they need to go through are in my spine that in most people and that he had troubles to but bc of the fluro-guidance could advance slowly thus pretty well avoiding hitting nerves, etc. ....

So then after they got the actual LP done (they didnt measure opening pressure) I was so shook up, and chilled and just completely rattled I think my nerves where shot for the rest of the day. They then take you over to the Nuclear-Med dept where they do a series of images so they can see how the tracer (dye) is moving through the shunt. At the 1st shot 20-30mins after the LP was finished the dye hadnt moved at all so they waited another 15 mins and took more pictures and the dye still wasnt really moving so they waited an hour this time and the dye had moved a little ways and was a tiny bit in my peritoneal cavity which is where the CSF fluid drains from my shunt but the Techs and Neuroradiologist said that by this point in the imaging most if not all of the dye should have been already emptied out of my shunt. So another hour went by and more pictures with still not satisfactory emptying results so they originally wanted me to come back tomorrow (fri) but I didnt have any clothes or meds along and is a 4.5-5hr drive from WI to Minneapolis so they decided to do a test that combines the Nuclear Med imaging along with CT scans so they could take smaller slices of pictures and follow this to see if they where able to pinpoint problem areas. After about 6 hours (4 pm by this point and we had left BD at 5am) the tests where done and the NS resident reviewed them and came out to talk to my Dad and I. His opinion given what I told him about the last Fluro study at Froedtert showing these exact same problems with slow moving dye was that there is likeally a partial occlusion/blockeage preventing CSF (spinal fluid) from being drained as much as needs to be but he'd like to look at the images from that Fluro-Nuclear Med study in 06' and be able to compare that one and todays with Dr.Haines (Nsg). He feels given my headaches and these results that a shunt revision might be neccessary but it is up to Dr.H and he'll talk to him i'll call them next week to see what they've decided next. He is trying to avoid making me come up for a fup appt to just talk to Dr.Haines if the end result is going to be a shunt revision and so depending what Dr.H says i'll either go back and see him if he doesnt think a revision is the right thing or we'll go with a revision I guess. My concern is of course if they do decide to revise the shunt I am going to push lock, stock and barrel to have it completely moved into a VP shunt given my lower back problems, all the scar tissue and all of the problems I have had with all of the LP shunts so far. It is nice to know that atleast my feeling of the shunt being partially blocked or not draining as much fluid as needs to be is pretty dead on, bc know matter how well one knows their body and how many times you go through something when you constantly have people telling you they dont think it is in any way pressure/shunt related then you 2nd guess yourself at times.

I also had EMG's done earlier this week for my Hand surgeon but done by my Neurologist which showed pretty well the same thing as last years but my Neurologist is not reccomending a 2nd release on my right hand (it has been 7 yrs which isnt altogether a long time) bc she feels the nerve and muscle damage there is to severe and permanent but does think that it would help to have my left hand done to prevent further damage. I see my hand surgoen in a week or 2 and we'll see what he thinks.

I'll update when I know more shunt wise,
Take care,

Erica

Monday,October 05, 2009 8:427 PM CDT

Just a quick update-Matt the scheduler for my Neurosurgeon at UofMn called today and the Lumbar puncture and dye flow study will be this Thurs w/check-in at 10:30 and procedures at 11. This means Dad and I will have to leave WI around 5am (yikes!) - i'll drive up to MN and Dad will drive back, this way my Dad can get a little extra sleep hopefully since he still has to get up for work fri and who knows what time we'll get home! The actual lP should be quick and the dye study anywhere from 1hr to several hours depending on how well the tracer they inject via the LP (after he does an opening pressure reading) flows through the shunt but hopefully we can get out of the Hospital and leave Minneapolis by 2-3pm, get home by 9-9:30. I'll update after I get home.

Tomorrow I have the EMG which is for my Hand surgeon but being done by my Neurol. who has done my past 2 of these as well.

Thursday,October 01, 2009 7:47 PM So today I had another appt this one in Milw with my Pain dr who I generally see about once a month. Another good appt which makes all good appts so far this week, amazing really!
She is putting me back on a long acting (24 hr release) morphine (Avinza) bc of my up and down pain cycle throughout the day on 3 immediate release morphine tabs - I have been on the avinza before and was a decent med with no side effects. and i'll drop down to 1 a day of MSIR. If it means sleeping through the night bc of no break through pain and better control through the day then I told her I think it is a good idea to vs what is right now. She also is going to do some more thoracic facet joint injections where she injects steroid and anesthetic into my facet joints which will should reduce the inflammation, and pain around the joints, nerves, etc. This is the mid-back area and right below where my neck surgery was done - out of my entire back my thoracic spine is probably the least affected with just a small syrinx (fluid filled cavity) running from T1-T11, disc bulging and degeneration a curve which might sound serious but given my c-spine surgery and then how my lower back is moderate and severely narrowed in the facet areas, degenerative diseases, disc bulging, stenosis, etc. (not o mention the 3 surgeries for the LP shunt in L2-L3) you would think that would be where most of my pain was but it isnt for whatever reason! But anyways my dr thinks this might help my pain as it did seem to help my lower back pain atleast reduce it when she did an injection there. These ones she will do both sides and at multiple levels under sedation. (she's trained in Pain mngmt but also trained in Anesthesiology). While I normally wouldnt do sedation under any other dr bc of the severe probs that drs have with getting me intubated I do trust her immensely and she is very aware of all problems going on.

I also am next on the wait list at 2 different appts back where I lived before (waupun by my nephew and sister) and also just found out today if I want an upstairs apt there is an opening finally at a 3rd apt.

More when I know it!

Weds, Sept. 30, 2009 8:18PM

Well I went and saw the Neurosurgeon at UofMN whom I had seen in the past (local Nsg who put in my shunt several years back refuses to see me and has for the past year or more despite my headaches. I just explained exactly what has changed in the last month and how I realize it may or may not even be the shunt in the first place causing my new endocrine problems, weight loss, nausea, etc., but that non of my drs can agree on what to do and that thus they seem to think just bc I get up and function everyday I really must be having that bad of headaches and that the other 1/2 of my drs do not want to look at other possible causes of the headaches (I already take meds for migraines which this headaches clearly are not the same) bc it might be the shunt or whatever. He agreed that it sucks, and did X-rays of the shunt right away, and then ordered a Lumbar puncture which he will do himself and dye study under fluroscopy to see how the shunt is functioning if it is partially blocked, completely blocked or no problems. The dye study is done right after the LP and bc he is doing this will take a couple days to get scheduled but I am just relieved he looked beyond the ICP bolt study from 2 yrs ago almost that he did and is willing to rule in or out the shunt as a cause.

This has been a crazy week already but monday I was at FMLH
for Brain MRI and Ultrasound (thyroid nodules) for my Endocrine dr. which I havent heard any results yet but probably nothing to enlightening for us. She just wants to see if there is flattening I think it was of the pituitary gland, hypothalamus or other areas being caused by pressure or whatever which could explain my decreased cortisol levels and other symptoms.

Thurs I see my pain dr in the early afternoon just for fup but hoping she will do something with my pain meds as I am miserable with back pain. I also need to call and schedule an appt with my Ortho-spine surgeon - the pain is even going into my hands again which isnt a great thing. Week before last I saw my Hand surgeon who ordered new EMGs for Carpal tunnel to get an idea if it is reccurent or being caused by another nerve area and these are being done next week at FMLH by my Neurologist actually who also did the last ones a year ago.

I heard from Genzyme Pharm. today and though I had kind of known about this already for a few months as a friend there asked me about what I thought of getting the panel back together again I didnt really know till today if it was going to happen. So anyways they are going to have us come to the Disney conference (not sure of logistics/timing yet as far as during or before the actual conference but am excited simply bc of getting to see families I havent seen since Vancouver and since the last panel 2 yrs ago. That time my infusion Nurse went with me but if I can take someone with this time want to take my parents bc they do so much for me and I know they would love it. So I am hoping I can take them should get all the info in next day or two in the mail!!

I also posted new pics in the slideshow at the top of the page earlier this week and though there all out of order bc having probs with my computer they are there atleast! The pics are from B-day party at my sisters a couple weeks ago, a family get-together on my Dad's side of the family, various car shows and from this past weekend at my parents 4 of us 5 siblings and 3 of 4 neices/nephews where there. (Older brother and oldest niece where missing though I may be getting Lis for halloween!!) Enjoy!

I will update when I know MRI/ultrasound results and when I know the date for the flow study/LP.

God Bless,\

Erica

Monday, September 14, 2009 10:59 PM CDT

For background on the Endocrine stuff see the last post before this one..

After my Endocrine doc literally playing phone tag with me 4 different times last week, her calling, leaving me VMs that she'd call back and her calling back again the next day and so on till she got me fri she gave me background on
what she thought we needed to do. As I said before the ACTH
test was normal my body produces cortisol when it is stimulated from what I understand but s/t is going hay-wire where I under produce thus low levels of cortisol. Cortisol is essential for many different hormonal processes
and likeally a partial cause of my extreme fatigue and weight loss, nausea, and muscle pain even. She has put me on a lower dose of steroid (hydrocortisone) as asynthetic version of what I am not properly producing enough of. I now take 10mgs in the Am, and 4 mgs at 4PM. She also has ordered a new brain MRI to look at and see if their is 'flattening' or 'squishening' of the areas related to these which in my case would likeally be caused from high intracranial pressure which is the original primary cause of the Pituitary insuff. and under-active thyroid.

The past many days i've felt quite aweful much like a truck ran over my head and literally like I havent slept in days even when i've went to bed earlier than normal. I am going to the health store tomorrow to get a few things and hope this new med will help some and also just pray so hard that the appt on the 30th in Mn with my Neurosurgeon will go well. While im not holding my breath I am hoping my dr will look at my changing situation and consider doing something-some kind of test on the shunt like a flow study or whatever so we know.

Otherwise I have new pictures to put up and a lovely, interesting 'story' to tell of the guy I was seeing but will do that all later...
For now God Bless!

Erica

Sunday, September 6, 2009 1:07 AM CDT

Just a quick note add on to below, Endocrinologist called back this fri (4th) and amazingly I happened to pick up my phone! (a rarity) So anyways she told me that my cosyntropin stim response test to cortisol injections was good w/my body kicking in and reacting or using what was injected through the IV each of the 3 times but that she wanted to wait for the ACTH labs to come back and would call me back Tues. She is thinking she wants to start a low dose of synthetic cortisol (hydrocortisone) anyways despite this basically normal stim-response test bc of the previous Lab work she did 2 weeks ago which showed low Cortisol level (not sure how this all works off hand, i'll admit I was driving while talking to her, trying to get from ERT to another appt and was late leaving clinic) and the fact that I have had such a big loss of weight again, such easy fatigue and crazy tiredness, increased headaches and even muscle pain/fatigue apparently can be from this problem she said! So she wanted to wait till she got those other results this week and then we'll go from there if she'll start low-dose hydrocortisone or not. I guess if it helped me with energy i'd be all for it and especially if it helps with muscle fatigue! I'll update when I hear from her, she is one of those drs who will call if she says she's going to also.

Thursday, September 3, 2009
Well i'm back and your reading this so this means you are back to, whoever you are that is! :D)

Last week friday I missed a call from my Endocrinologist at FMLH with the results of the labs she had drawn. In her message she said that some levels have decreased some while the thyroid level had jumped up a little. (this is not unusual with a synthetic hormone like synthroid.) Growth Hormone which i've not had a problem with in the past decreased on this testing (last testing was 6 months ago) which could be bc of the headaches if they are pressure related (the Hydrocephalus is the original secondary to MPS cause of my pituitary insufficency which affects the thyroid and other hormone levels.) So she said that for now she'll just watch that level make sure it doesnt go down again and that she isnt going to do anything about it bc of my age so atleast that was 1/2 good news to the change atleast! The other level which decreased is Cortisol which is produced by the adrenal gland this gives a person energy and helps the body respond/adjust to stress. So today she had me do a 'short-cut' blood lab test which compared to the more indepth test she did several years ago when I first began seeing her this was relatively short (1 1/2hrs). The treatment if this proves to be problematic to a point that i'd need medication is prednisone or hydrocortisone which of course increases peoples appetites so it could be a good thing given the recent 10-15pd weight loss. We will see sometime next week how it turned out.

Other than that I fup with my Pain dr today, I guess I must have looked as tired as I felt bc the Nurse there told me dr I looked like I was about ready to cry! Yikes, just over tired!! (nothing to new there I always seem to be 'over-tired'!) Sometimes I think if I could have 1 wish with MPS it would be that drs truelly could get a picture of just how 'up and down' the disorder makes your body feel and how day-to-day and week-to-week is never the same, 1 week I feel decent pain wise especialy and the next week it totally seems to flare. I know we all want progress and most drs are not very used to seeing this up and down cycle and thus having to ideally change the treatment plan often..but it seems to me like with MPS especially it is so mis-understood and drs are so afraid to go backwards so thus the first response is 'well it's emotional' or 'lets wait and talk to other drs' and the patient is left with headaches, pain, and lesser quality. I know our medical system is set up unfortunately that drs have to see so many pts in each day and is around Insurance companies and all these negatives things but when does it truelly become about just listening to the patient? Don't get me wrong I am not dissing any of my providers I just am speaking in broader terms for myself and for MPS pts and patients in general. Maybe I am just frustrated with never seeming to get anywhere with the headaches, always being tired and the never ending seeming need to defend how my body feels and how I see (and hear from other parents and affected adults) their kids feel. Just frustrating I suppose!

On a completely lighter note Zander my nephew started preschool this week and when I was there weds night after picking him up from daycare he still seemed to be full of p and v! I also am casually seeing a new guy and though I just am the type who likes being single, being independent and having my time to do with as I please.. this guy does seem pretty nice so we'll see where it goes. I do know i'm just not really looking for anything to serious but we have nice chats and have fun doing things together. I also started PT for my back which has been going well, this started last friand tomorrow will be my 3rd time for this particular focus-issue. Right now my same PT who helped me with my knees/hips is doing massage to try and loosen up the muscles within my spine and shoulder area and although makes it pretty sore hopefully in the end run it will decrease my flares of back pain and overall pain in general, I hope!

Several drs have told me they really thought I should go see my Neurosurgeon at the UofMN and so after talking to my pain dr today about it somemore (she also wants me to see my spine surgeon there and make sure something hasnt changed/worsened) I made an appt on the way home today and see the Nsg on the 30th of this month.

All for now, will update when I have some news or need to vent or likeally both!
Remember Sept IS Pain Awareness Month!
Erica
ps: one of these days I will post the link to the Pain/MPS article published about me.

Tuesday, August 18, 2009 8:55 PM CDT

Thursday, August 20, 2009 10:54 PM CDT

I thought I would just update on todays appt with my Pain Mngmt specialist. First wanted to let all know in case you havent noticed and want to watch there are new pictures up of my family from our recent day at Bay Beach in Green Bay, see above.

So anyways I am slightly disillusioned with medical care and why it is okay that pts have pain of any sort - keep in mind that I truelly do think my Pain dr is great and tries what she knows to help me, but sometimes I feel like medical care could do so much better if it where just willing to embrace alternative therapies and supplements more. We talked about doing more injections like the 2 i've already had both in lumbar spine (2 levels I think it was) and thoracic spine which was 3 levels of injections. We either could do more of these same injections at further levels
in these areas or my dr talked about in the future trying something called RFA (radiofrequency ablation) which basically deadens the nerve endings (ie the facet joints) which are part of the overall problem. Rather than decide to go forward with this though right away she asked me to talk to my Ortho-spine surgeon see if he had any reccomendations on my spine and on my shunt or headaches or even if he thought the headaches and spine problems where inter-related (which I think they are) and then she left it up to me to decide on whether to see this new neurosurgeon of whom she had previously given me the name just recommending though that I make sure my Neurologist wouldnt be offended. (which to me it is not about if my drs are offended or not, while I care about their opinions and value their input and help I am the only one who truelly knows how bad these headaches feel and this is what I told my dr today) I think I will keep the appt bc I really want a local nsg and I just so badly want to find a dr who will listen to me and hopefully be open minded, if in the end this dr by some small miracle of all miracles was willing to test the actual shunt by doing a flow study and it ended up not being the shunt then fine I have no problem saying I am wrong if that would be the case. I do have a problem that no dr is willing to do a fairly simple flow study on this shunt given the spine problems, my ever increasing headaches and nausea and the weight loss. I'll continue to pursue finding a neurosurgeon locally until one is really willing to just listen to my side of this to and look at the full picture. So anyways i'll wait to see what I find out from these drs and will see my pain dr again whenever I can get back in to her around when my meds need refills. The only thing which sucks about this all is that given her schedule at this hospital vs when I saw her at the other clinic she used to also be at (an outpatient imaging center) is that there is no 'flexibility' or atleast I havent found any with her staff and if she wants to do a procedure in a week or see you back in 2 weeks you end up having to wait 3 or 4 weeks and so it will be atleast 2months i'm sure till I get any further with treating and easing this back pain and with trying any of the things we talked about today which is slightly discouraging.

I spent the evening over by my sisters playing with my younger nephew which is always nice, his hugs, laughs and silliness cheered me up some from these headaches, my overwhelming tiredness this week and this appt today. While we where all eating tonight Zan was sitting next to me outside and he was eating corn cut off the cob and I was eating mine on the cob and all of the sudden he reaches over and pulls mine to his mouth and from then on wouldnt touch his loose kernels but would only eat off of mine and it was beyond funny and adorable!!

I can't stress enough even if I am having a harder week that 1. I am not in any way trying to put down my drs for whom have done so much for my care just that I wish it where not always such a fight to feel better.

Remember to fight for your own needs and for the needs of your family, you are the best advocate you have and no one else is going to fight for you. Remember also to look at all sides of the coin, western AND alternative medicine have a place in medical care, there are plenty of qualified, well trained providers in holistic medicine willing to work with medical drs and though it is another hard process it can be done getting these 2 sides to work together!

Tuesday, August 18, 2009 8:55 PM CDT

An eventful day I guess it could be said. I had appts with Endocrine @ FMLH, Orthotics @ Children's Hospital and an appt with another dr after all that.

The Endocrine appt went great though I did forget to ask her what her thoughts where about the ANS (Autonomic Nervous system) issues and how to best regulate them but she reordered the neck ultrasound (to visualize I think it is the adrenal glands or something which is enlarged within my neck better that had shown up on previous MRIs) and said she would call me with those results once she had them so I will ask her then what her thoughts are. She also ordered repeat blood work of thyroid levels (done every 3-6 months) and cortisol levels which is what the pituitary gland makes/produces. She is concerned that the residual fatigue and now presenting weight loss I have again is perhaps related to that as I have Pituitary insufficency which is the subsequent cause of the under-active thyroid. If these cortisol levels look at all funny she said she'd go on to a 'fancier' more precise test in which they test blood levels 3 different times over a specific period of time within a short period of time. (couple hours)

The Orthotics appts at Children's Hospital was just to get the custom made orthotics fitted for my shoes and was fine and easy other than adjusting to several different pairs of my shoes. And my last appt with my Pain Psychol. I was only able to make it for about 1/4 of the appt but always nice to see this dr, she is sweet and we get along great what with having alot of shared commonalities.

This thurs I see my Pain mngmt dr and have several questions for her, she is also a great provider and very helpful resourceful so an appt I usually dont ever mind other than another trip to Milw. on top of fridays addt'l 3rd drive there! And then next week monday I see my Genetics team at Children's Hospital which is always eventful if nothing else. I haven't talked to my Genetic Counselor alot in the past 6 months so will be good to get caught up. I am curious to get her take on this weight loss although not looking forward to my Geneticists 'opinions' on it, as he tends to not have alot of tact. I am thinking perhaps maybe they will have a few insights on some other areas we've been dealing with to, including orthopedic, PT, and of course the ANS and the Headaches.

Then later next week I see a new Neurosurgeon which my Pain dr has reccomended for quite some time and am hoping maybe he will offer a new opinion and willingness to look at my headaches and shunt with a possible new set of eyes. I am not holding my breath but feel that I need to pursue all areas for my care and this headaches and this dr happens to be one of them. My pain dr has reccomended him to me more than once and so I made an appt and will talk to her more about him on thurs. I've gathered up Imaging discs (MRI, CT and X-ray) and Operative testing results from various surgeries and varioues procedures and tests. I'll update on that afterwards sometime.

Last week our family (my parents, my sister, her fiancee, my 2 nephews, myself, my neice and her Mom and brother and sister all met at Bay Beach in Green Bay at the Amusement Park for the day which was a blast - I have a ton of pictures to upload to here and will do so either after this or soon. It was a hot, muggy day and being that I completely don't regulate to hot or cold temperatures my body was hotter than a pistol and sweaty but it was great fun running around the park with Alissa and Quinn and Jordan (future Brother in Law's son) and watching Zander on rides and riding with Kirsten and Nicholas as well who are Alissa's siblings from her Mom's marriage. They where so cute - they always call me Aunt Erica and all day where calling my parents Grandpa and Grandma to! Way to adorable! Quinn had been with me since friday before that monday and then Alissa came home with me as planned to spend the rest of the week with me to, so her and Quinn spent the majority of the week playing together, riding bikes, running around, playing games, going with me to appts, and just being kids which was fun. I was really tired last week but I really enjoyed having Alissa here and always love having Quinn to. :) In fact just last night while looking for my cat in the dark I tripped over one of the grass 'areas' they had made as part of their house ie an area for sleeping, for eating, playing, etc. (They begged me to let them sleep outside). I have my own bike which I ride (bc of the joint problems especially with knee and hip and back it is alot easier to bike than to walk or run as it is none weight bearing) and I have a miniature mountain bike for Quinn so we can ride to the Parks or go on the trail in the area and so Quinn and Lis rode quite a bit with Lis riding my bike and I usually would just walk along if they wanted to go outside of my parking lot or block. Quinn was gone one of the days so Alissa and I went to FDL and did some miniature golfing and had a mini picnic plus then did a little shopping and a movie night that night. The following day I had the Radiothon interview for Children's Hospital so Jamie Quinn's Mom a good friend of mine watched Alissa for me (it was in Appleton so this way Lis didnt have to sit in the 1.5 hr drive w/me there and then back) and Quinn came back to my Apt and spent that night again and they came to the clinic fri for ERT.

The radiothon interview went really well I thought and only 1 small error on a statistic when comparing MPS's rarity to a more common, heard of disorder. I even managed to hear it myself while driving home! My ERT infusion Nurse happened to have her radio on as well and heard it! I am hoping to get an copy of this interview as my parents where outside of the radiothon station hearing area and would be lovely for them to hear it.

I have one of these fantastic headaches again so am going to sign off. Will update again soon.

God Bless,

Erica

Saturday, August 8, 2009 6:57 PM CDT

Hello all,

Since it is I just realized today but a few days from the radiothon interview for Children's Hospital I thought I should list the radio stations who will be airing this (live) throughout the day but my interview will be at 3pm. These stations will air this:
95.9KISS FM,
105.7 FM WAPL
104.3FM The Breeze and
1150AM WHBY

Last night while my nephew slept soundly away in my bed (yes my bed!) I sat out in the living room of my Apt talking on the phone for 4 or so hours with a friend. One of the things we talked about was if we would want kids, about adoption, being a foster parent and about disability.
Well needless I am slightly perturbed by part of this long (and to be continued!) conversation simply because of the fact that this friend who really is a pretty nice guy 1. would not want to raise a special needs kid "bc he feels like he wouldnt be available enough', 2. that he would have a problem dating someone with a disability (this struck me as exceptionally shallow) and so I asked why because of haing to explain to people you know that your gf does not work but is on disability? (nothing like looking at the valid reasoning behind why a person is on that particular said disability) and I asked if it was because he didnt think his girlfriend would be worthy enough just bc of what they receive? I asked isnt it kind of 2 faced to say you are a strong christian but then be ashamed that your gf wouldnt be a 'so-called productive member of society' (the feeling I get). The conversation definetly isnt over and I want to know why if he likes somebody (in general) why not look past what they have, look at the other accomplishments they have?? All I can say is how messed up is our society that people can't look past a 'name' to see the whole person especially if otherwise you like that person! Keep in mind I am not really looking for a relationship these where questions I was just curious to know what he thought and I fiercely believe people are and can still be valued members of this world regardless of whatever it may be they have.

I've continued PT which slowly and surely seems to be improving things and I also saw the dentist this week (very frustrating and I won't even get into it) plus the new Orthopedic dr my Pain dr wanted me to see (a colleague of hers) which was fine although reallly, really long (like 3 hours) with maybe 30mins of that with the Ortho) bc of a screw up. He said that unless they where to clean out the area in my knee of the GAG buildup there isnt really a lot else he would do and even that he would be concerned it would just build back up. I really do think PT is the right way to go though and is helping slowly but surely.

This past week I did a phone itnerview which will be in the
Sept issue of the Pain Awareness (Am Pain Found) newsletter
i'll try to publish it here once I get a copy.

Otherwise monday our family is headed to Bay Beach which is near Green Bay, sits right on Lake Michigan and an awesome cheap kid and adult friendly amusement park with lots of carnival type rides but which are there all year round.

I am going to sign off for now but take care!
Remember each and every member of our society has something to offer and has a value, never discount somebody just because they are 'different' and do not fit into society's very narrow minded standards of 'perfection!'

God Bless,
Erica

Saturday, August 1, 2009 10:00 PM CDT

Well I need to turn off the light and go to bed considering it is 1:00am and I will get back up at 7am but I wanted to update on jsut a couple things.

Earlier in the week I met the new dentist at MUSoD who i'll see in the Advanced Care clinic - other than a very annoying and hard time getting the full mouth x-rays with the Tech (extremely small mouth due to MPS storage/stiffness to) the Dentist seems really nice and interested in learning about the causes of my issues. She asked if she could contact the dentist I have talked to at the UofMN (an MPS specialist) dental center and also if she could contact my Cardiologist (antibiotics are no longer reccomended but she wanted to get some background from him) so i'll see her next week and we'll go from there.

Thurs of this week I had Thoracic Facet (joint) injections done at 3 levels under anestheshia by my Pain dr (an anesthesiologist trained in Pain mngmt and PM n R) which went well. The after part was tougher this time both shaking off the anesthesia affects and the pain from the actual injections. It almost is like a step backward to hopefully get a couple steps forward (increased pain from the needle injections but hopefully in the short-long run further reduction of pain medication. I hope! I didnt really talk to my dr afterward - she stopped by and talked to Chris (my sister fiancee who took me) but I was getting a chest x-ray she requested to be done, I actually don't even know why she wanted that test other than I assume bc the area of the injections is very near ones lungs. I also don't see her till the 20th as the only other opening is the day I agreed to do the Radiothon for Children's Hospital (I know, I know I still havent listed the 5 radio stations it will be aired live on, but I will!) So I am curious to talk to her see which levels in my thoracic spine she settled on and her input.

This coming week I see the Orthopedic dr at Froedtert - which my Pain dr after reviewing the MRIs commented that she was a little shocked the very same things going on in my spine are also going on in my knees - anyways so this appt is more or less to see if there are add'tl reccomendations the Ortho dr might have for myself and my Physical Therapist and Pain dr as well as to have an Ortho dr for knee/hips on board. I am going to start counting pretty soon how many different types of orthopedic specialists can I accumulate on my team of drs! (:)

I will write again when I have time or have something to say, Need to really try to fall asleep!

Please keep my friend Lea's son Gabe in your prayers he will be having VP shunt surgery monday at UofMN for Hydrocephalus, with the same Nsg I have.

Love,

Erica

Tuesday, July 21, 2009 10:27 PM CDT

I don't know alot to update but happened to be reading an article online a bit ago and thought I would come post a small update.

Last week in a slightly (very big!) embarrassing moment on my part I messed up an Appt time and date, which this appt was supposed to be last monday and in fact I thought it was this monday - aahhh embarrassing! I ended up seeing this dr this monday anyways she had an opening, but sometimes I cannot believe the 'duh' moments I have!!! I also saw the Orthotics person in that clinic at CHW monday
which was fine, he will make custom ones for me and also is
talking to my Physical Therapist I guess to check a few things.

Last fri I had MRIs done of my knees at FMLH after ERT and
got the prelim reports already friday night! Pretty cool. These came to my 'mychart' inbox which is something I have so that I am able to get test results from my my Internal med dr. and communicate to with her team. (although we do email as well) These where normal as far as anything outwardly being wrong but the 'normal' MPS degenerative changes and GAG build up are evidenced in all areas. Ironically that same night I got an email from a Geneticist I know who did the original Aldurazyme (ERT) studies and he said that in his opinion skeletal survey's would be better as they give a clearer picture I guess of bone issues. This was something my primary dr (internal med) and I had talked about doing to, so I will fwd her that email message.

I have a bunch of new pictures to put up which are absolutely adorable I think, from this past weekend. I went
to Fish Day in Port Washington where I was able to spend most of the day with my 2 neices and as well my oldest neice Alissa's 2 siblings Kiersten and Nicholas. This is a huge city fundraiser type event which has a 2 hr parade, walk/run, carnival rides/games, car show (my Dad took his 67' Chevelle) craft show, and so on. Very fun! I should get to see Alissa I think in 2 weeks as I think her Mom is going to let her come to Bay Beach Amusement Park with my parents, my nephews, my sister, her bf and myself. Alissa's sibs where so cute, they kept asking if they could come stay with me for a week to next time Alissa came
as they wanted to sleep over - these are an adorable, well behaved bunch of kids! I'd gladly take them!!

Not sure what i'm going to do the rest of this week, not much going on, but next week is the MPS Family mtng at UofMN Sat August 1st. It sounds like my good friend Lea's son (Gabe) will have his surgery next week likeally, so hopefully I will see them and the following week is the Iowa fundraiser i'm planning to go down for.

I'll update next week soemtime, and will post the new pics when I get a chance.

Erica

Friday, July 10, 2009 11:19 PM CDT

hello my frusted and true friends,

Although I don't take a lot of time to think about what i'll write here before I actually sign in to write an update and in summer especially I tend to go a week (often longer between updates) today I have a little bit of updates and otherwise just some continued thoughts on a subject.

As you may know from reading here my youngest nephew age 3 is diagnosed as having 'high functioning' Autism for which his main problems are constipation, GI, and constant infections along with neuro-speech delays. I love to visit him every time and see the small steps forward he is making each day and week with the things my sister has done so far in a series of eventual biomedical interventions she is pursuing to help him recover. We have both been reading books written by Jenny McCarthy (actress) ie: Mom to Evan also dx'd with Autism and I have been doing a fair amount of research and talking to friends (also doing these biomed things for their kids wit great improvements) to figure out which are the best biomed treatments she can do for Zander to help him. While it is a struggle with her Ex bc he has said he is doing things but it turns out he doesnt seem to be - I really love helping Zander bc I love him beyond belief and there are so many things that both our Vitamin Shop store owner (a Nutritionist) and friends of mine using these same interventions have given us as far as showing this stuff isnt just 'make believe' and really does help. The other night I spent a couple hours printing off info on several biomed interventions my Sis sent to me and asked if I knew anything about for Autism so that she could read up on them more and also so we can give all of this info including this books by Jenny McCarthy on Autism to Zan's Dad and maybe he will do more to - i'm hopeful bc I know he loves Zander to.. Unfortunately it just seems to take guys alot longer to believe things that 'arent prescribed by a medical dr.'. But seeing the small improvements my nephew has made with the few things my sister does her 50% of the time with him (ie more speech, seems better able to concentrate, less irritable, better bowel movements.) should convince anyone! (especially given the changes we see when Zan doesnt get these same things for a couple days). ...And please anyone who has a problem with my writing what I just did about my sister and my nephew and her Ex - just spare me the crap because this is my personal blog where I come to share what I have learned and to just write what I am feeling at the time... it is not a forum for others to critique or criticize bc I actually don't care what you think, i'll continue to write what I like. I am in no way writing bad things about anyone or bad-mouthing anyone and I am not listing names so back off!

A close friend of mine whose son has MPS III has started a Virtual Bio-Tech company and asked me to sit on the BOD for this which is neat and will be another area of commitment but I like helping MPS families and this is a perfect outlet for us to make a difference for these truelly under-served children for whom research just is lagging behind making any actual improvements for the kids. Our focus with 'T.S.F' (intials not the actual name of the foundation) is 1. we have an organizations whom has written a start-up plan for us, we have an initial source of funding (personal families) and we have a project Manager working with us in addition to the person leading this outside of our T.S.F. BOD members. The focus and mission as a certified Non-profit Foundation in the Virtual BioTech. Our mission is to with the combined work of several different researchers from different locals test on Sanfillipo mouse models both FDA approved drugs in an 'off-label' fashion as well as OTC supplements. For instance many MPS III families now use Genistein bc of the Studies done in Poland and there is research out now on the relation of ALzheimers and Sanfillipo (P-Tau tangles are affected in both disorders although in Sanfillipo this is only a small part of the combined problem) and so researchers will look at the use of teh drugs in treating Alzheimers but for Sanfillpo and at things like Methylene blue, Coconut Oil, etc. Keep in mind please I am relatively new - actually very new to T.S.F so I hopefully have this info all correct but will keep updated on each new step of the Foundation progress!

Physical Therapy continues to go well - this week I can actually sit fairly comfortably in bed, straighten my legs out in front of my and my knees do not hurt as much!! Before I couldnt even sit comfortably with my knees bent up bc of the tightness in all the muscles, ligaments and tendons! I am so pleased at the progress so far - I truelly hope to get to the point where it does not hurt to stand on my feet and be able to run alongside my nephews without my knees/legs seeming to give out! (it's a weird thing that happens but they knock together I guess and my ankles are weak so I stumle alot). I continue to wear the Patellar-tendon knee brace which most days is fine now and does help - the difference is amazing with this brace keeping my knee cap in the groove it is supposed to sit in so that it does not move in and out of alignment constantly.

I also saw my Pain dr and although im a little frustrated that i'm not able to see her for 4 weeks when she wanted me to fup in atleast 2 weeks so she could let me know the info from her talking to the dr she is sending me to and also her findings/decision as far as my thoracic spine goes. But non the less scheduling there is not always the easiest and no le-way with this secretary so I finally called back today and asked to just schedule the thoracic spine facet joint pain injection and my dr can let me know that day if there is some reason she isnt comfortable doing it. Her concern was just being able to visualize my t-spine and lumbar spine areas with fluoroscopy before an actual injection to map out her 'playing field' and a safe distance injection wise away from my shunt in my lumbar spine (it is anchored in L2-3 but loops around and back down into my side where the flow valve is anchored and then on into my peritoneal cavity ie stomach area basically). But her office called me back and my dr said I could just schedule this facet injection (I think she knows this is one of my worst pain areas and has been going on for a long, long time as it is already plus anything we can to take areas out of the 'pain picture' for my body. So it is set for July 30th - 3 weeks from now.
The dr she wants me to see is an Orthopedic dr she used to work with quite a bit at Froedtert when she was there - she said she was going to call/talk to him, to give him a heads up on my situation, the MPS, knees, etc. basically just so that we have an ortho-dr for this. (my ortho dr in MN does only Spine and then there is a Pediatric Ortho dr there who has MPS experience but only sees peds kids)(he is willing to give us his opinion of the MRI imaging though which i'll send up to him this week after their done). So anyways I got the number from my drs office today to bc I actually didnt know what it was - just figured I would get it after my dr talked to him! But given the delay in fup want to get things rolling. So i'll keep everyone up to date when that Appt is and what I find out from the knee MRIs, etc.

Please, please keep my buddy Gabe Creese in yur prayers he is having some problems with his Port-A-Cath (a clot in his vena cava at the top of the port) which blood thinners have not been able to successfully thin out/dissipate and he also will be going to the UofMN this coming July 22nd to see the Neurosurgeon (my dr) for Hydrocephalus surgery. I'll be in MN the following week the 30th-1st-2nd but am going to try and go up and stay with Lea for atleast part of the couple days she is there. I consider Lea one of my very closest friends and probably the one who listens the best when I just need to vent - she isnt the type who will ignore your calls or say she'll call you back and never does - she is always there and I try to always be there for her. Really she and I are extremely stubborn, don't tend to put up with BS from those drs (definetly not all drs., we each have some very good ones on our teams!!) who think they know better than we do ie refuse to atleast listen or accept us as an integral part of our teams. A patient and a Mom have insight that ALL drs need to pay attention to - the good ones already do and the bad ones jsut need an attitude adjustment. So anyones she and really 2 other friends are whom I talk to the most although I do have tons of MPS friends whom I help out and who support me - you do find out quickly enough who is just putting up an act or show.

Anothe month from now will be the Children's Hospital of WI
LIVE (!!!) Radiothon which will be broadcast on 4-5 different channels (I just received the radio stations participating today). This is where CHW Public Relations has asked me to tell my story of MPS and basically how CHW helped me (the diagnosing center through my Cardiol.) plus what i'd really like the public to know either about my story or about CHW. Should be fun enough although is in appleton which will be a fancy fun drive away (1-2 hours I think nothing to far jsut havent been up there by myself in a long while!). Should be good for getting lost a couple times despite my GPS if I use it!! I'll post the radio stations once it gets closer.

I'm sure there are things I am forgetting but I am tired as all can be so time to cut this off!
Good night or Good day which ever it may be my friends!
God Bless,

Erica

Thursday, July 2, 2009 3:59 PM CDT

Frist let me preface this by saying that I am tired so this likeally won't be anything but a mundane update w/a little side of frustration thrown in..

Alissa my neice went home on Sat - I took her back and miss her terribly, I definetly look fwd to whenever she can come back!

Physical therapy has continued to go well with teh continued as exepcted excercises both there at the clinic and a 'program' here at home my therapist has laid out. I do some of these 1x a day and others 2x's or more and although the actual knee pain hasnt improved I do think the way over tightness is improved if not alot then atleast there are small changes towards the better which is
all I can ask for! :) Bc of the 'clicking' of the knee cap (patella) both when walking and spasms when sitting we have tried a knee brace which although a little irritating (under-neath knee + I pull it up a zillion times a day) it makes some improvements which again are good. I basically only use it when not home and not at night. It is amazing to me the things I (and many people i'm sure) do wrong as far as how we support or carry ou bodies and where our weight falls - like in my case my ankles tend to turn inwards (mostly when sitting I think not sure entirely) as do my knees and although one should be walking with their weight falling on their hips to knees most of mine is carried into my lower back as a result of the over-tightness (lower back my therapist said is 'lax' or over-bendable (not the right word!!!) and my hips are over tight same with knees thus why he thinks some or alot of the lower back pain, disc bulging and degeneration is occuring! Who knew!?

This week I have had several people make off the cuff comments about staying positive and not letting the disease get me down.... I don't think either ment what they said to make me feel bad and I don't neccessarily - I just wonder what vibe do I give off to some drs and friends that I totally don't give off to other friends? I say this bc on the flip side of this another of my drs yesterday who on a personal level bc of her own experiences really gets the having a chronic illness, the drs, etc. said to me that she thought I did a really great job of staying positive and moving fwd when alot of people these same things would cause to give up.... I personally don't think I dwell on MPS to much and I don't think I let it get me down - yes there are the days which are a little rougher than most but EVERYONE has these.. I always wonder what is wrong with wanting to help others who are in the same boat and try to make a difference for them? Try to save them some of the frustrations i've felt? I really enjoy helping others and like the involvements I have with the MPS community and with APF advocating pain change.. I don't think anyone should have to live in pain and I don't think anyone should have to feel alone in their struggless or isolated bc friends or family or drs don't get it..

I saw my Neurologist earlier today which went fine - nothing new and nothing she knows to help, not that I expected any different - she is a fantastic dr but I really just don't understand what is wrong with modern medicine when it is 'just okay for patients to have pain or to have headaches' it frustrates me a tiny bit..I think the bigger part that frustrates me of it all is that drs expect that we need to be lying in our bed or on our couch, in the dark doing nothing all day long bc they have no idea what to do to help. I refuse to be my disorder or any one symptom and am going to do the things I want to do and maybe this hampers my actually getting an effective treatment at times but I just am going to enjoy what I do have and what does make me happy..

I need to wrap up - am exhausted today (sleep the lovely elusive idea!) but watching my youngest nephew for a litte while tongiht.

More later.
God Bless - may you enjoy time with your family this 4th of July,

Erica

Saturday, June 27, 2009 0:02 AM CDT

Well what a week it has been - my almost 10 year old neice is sitting (sleeping) here next to me in bed (somehow she keeps 'falling asleep' here in my bed although I have an actually wonderfully comfy futon out in the other room!) Really it is bc she just likes being close I guess and I don't mind - I miss her as I don't generally get to see her alot so having her this entire week has been great. Im used to having Quinn here all of the time and Zander!

So while she is sleeping away I am pondering some things from today and the week while trying to ignore the knee pain I have which is causing spasms underneath the knee cap
and knee area even while jsut sitting here - as if the 'clicking' isnt annoying enough when I am up and walking around!? I had PT again today (2x's a week) which con'ts fairly well - my PT Brian and I are really trying to work on loosening the calf, thigh, IT band, hip and ankle areas while also adding in strenghtening for these areas and to try to 're-train' the patellar-femoral (joint?)area. He has been awesome about trying to learn about MPS, asking lots of really actually fascinating questions and making me think of ones I really am interested in finding out (like why 'normal' people can take Glucosamine n Chondroitin and it re-builds cartilage but yet in MPS I we don't break down this very substance (heparan and dermatan sulfate but dermatan sulfate is chondroitin if im getting it right and since we don't break this down to much of it builds up in virtually every organ, tissue and joint so why do we have joint pain? Shouldnt we have less joint pain given we have addt'l 'cartilage'? Or is it bc it is broken down into something else..just teh way my brain works I guess, always wanting to know these things! So anyways my PT and I have talked about several things ( possible options) one including wearing a night time brace to keep my knees straight in order to try and 're-train' my body in a sense to straighten my knees most fully during the daytime.. we opted to hold out on that and not do it atleast for now, it may be something we try down the road we just don't know yet. Today he talked about a brace to wear I guess during the day (or all the time, i'm not sure, he wanted to talk more about it at next weeks appt) to try and keep the patella (knee cap basically) in place so that it does not move back and forth in the 'crevice' and thus hopefully cause as much pain, and clicking. This may reduce the swelling there is to, I don't know on that part though - we'll talk more next week I guess. He did say I probably wouldnt like it though as it would be hot (lol maybe that comment comes from the fact I always wear sandles in to PT and not 'normal' shoes which he said might provide more stability (I guess I just really, really like summer and not wearing socks and slipping into open toed things!) :@) Atleast after more talk on the whole shoe thing he did say atleast my 'higher' arches which are otherwise not normal are probably better for my knees! Ha!

I also saw my Pain dr this week and she did the first of several Lumbosacral Facet joint injections (below my LP shunt) under light sedation - she is a very skilled and capable anesthesiologist so despite the fact bc of the airway issues I would never let any other specialsits do sedation (upcoming endoscopy for instance is already being planned under monitored anesthesiology care) without a secure airway I fully trust her both bc of her anesthesiology training and experience and bc she fully knows my background and has seen the reports. The IV access itself was amusing (as it always is at a new place)
bc they started out with 1, then 2, then 3 nurses looking over my veins trying to figure out where to go (the 4th vein finally held and worked) and we where all talking about the back of my hands which are notoriously bad although they look great and in walks my Pain dr who says to them 'just listen to her, she's done this way to often (ERT and surgeries, procedures, images, etc) and knows what she's talking about' - it was nice to have my dr KNOW and trust that I am quite aware of my body and pay attention plus am not just going to say something unless it is true! Needless the Nurses found out quickly and will be on alert for the next injection - they all where very nice though. :) I fup in 1 1/2 weeks and then we'll talk about when to do the same Facet joints but on the opposite side of the lumbosacral spine area my dr did this week. (She does right or left, 1 at a time)

My Neurologist at UofMN also got the sleep study stuff arranged I guess and they called today plus the 'consult' which he specifically asked to be avoided in order to prevent 2 different trips is on for July 30th - he sent me a note tongiht and said I should just call them and tell the office stay exactly what him and this Pulm.-Sleep dr talked about (just doing the sleep study and avoiding the pointless 15min consult). So anyways one way or another it will get straightened out. I know that my Pulmonary dr and Sleep med-Pulmonary dr here are aware of what is going on as is my local Neurologist (whom I see this coming thurs) and the 2 pulmonary drs did not want to repeat sleep testing but I guess I am open to the idea, if it atleast gives us an answer one way or another, it is the only way to rule this Co2 thing in or out as the cause of headaches.

Otherwise this week I really have not been able to get really much of anything MPS or APF wise done but then again
in summer I also get alot less accomplished then other times of year bc I love being outdoors!! :) I do have several things to work on next week or after though.

I received a 2nd reply from Mystery Diagnosis from an original email I sent to them on their casting for Season 8 (?) of Mystery Diagnosis and they sent me some paperwork to fill out - they want to try and get me on for this coming seasons show! Wow - how AWESOME would this be for raising awareness of MPS I 'mild' variant? Perhaps this would highlight and help some un-diagnosed families out there whom have MPS (or a Lysosomal disease for that matter) but are mild and thus their drs really do not recognize various symptoms (such as mine all where varied) are actually caused by one disorder? This would be my only hope to help even just 1 family...bc that road to diagnosis when you don't know what you have and your drs don't know is really, very tough.

I best be getting myself to bed - morning comes calling early with Alissa around!
Love and God Bless,

Erica

Monday, June 15, 2009 1:39 AM CDT

Once again it has been a few weeks.. I've just returned from 4 days of leadertraining with the American Pain Found.
in Minneapolis, which was amazing but also simply exhausting! I am so glad I was given this opportunity to attend the training, participate in all of the various meetings and work-shops and meet so many other leaders and APF staff whom I had previously only talked to on the phone or emailed with - it is always nice for me to put a face with a name! The Leader 'showcase' friday evening went
really well I think - I had made a poster on 'MPS and Pain'
and put this up along with hand-outs on MPS from the Nat'l MPS Society and 'Join the Search for MPS' and had a few different videos going (at different times of course!) of which 1 was a video I had done with Genzyme, another was from the MPS Society and another was a joint video from the Pharma co. and MPS Society. Many people (persons in pain, APF staff and medical personnel - all of whom are affiliated with APF stopped by and had many questions on MPS and what it is, how it affects a person and other great ques. So I can honestly say that many, many more people now know what MPS is and dozens of pamphlets where taken - alot by medical personnel who now if they ever see an MPS pt may be able to diagnose them!

Some of the meetings and work-shops included - working with the media, legislative advocacy, online communities we can work with APF on to reach broader audiences, and training on so, so much more! It honestly was 3 1/2 days of all meetings and work-shops from 8:30am till 8:00pm but very informative and I have alot of add'tl great ideas.

On an personal note I missed this weeks ERT due to the Conference but will be back next friday to a more usual schedule. I did miss the Chicago MPS Family meeting Sat. due to the APF conference which was a little sad bc so many friends of mine where going from in the midwest but I know atleast if there where handouts they will give me copies. A good friend of mine Jamie Fowler is having a Golf fundraiser in Iowa in August for her son Jack (MPS II Hunters) to which I am planning to go - god willing - stay by my very good friend Lea for a few days and we'll likeally drive up. Something along that line - I just know I really want to see both of these 2 and they are probably
some of my closest MPS friends. Lea and I met last summer at the Vancouver conference and have become pretty close since then I think - we often talk on the phone, exchange emails and texts just being there for each other - her son Gabe has MPS II as well. The UofMN meeting is officially being set for August 1st in Minneapolis although know times of schedule of speakers has been released yet - likeally that will occur sometime in July. This is always a nice conference and hopefully my former Neurol. whom left
there for Shire Pharm. will attend!? It has been a bit of a long, slow process with the new Neurologist there at the U.

Otherwise in Appt news - I have the swallow study at FMLH for next week ( I resched.), Orthotics Appt at CHW that same day which was put in by my Neurologist at FMLH. I also see my Pain Psychol. that same week whom is one of my favorite providers, we really have a lot in common and she is really the one person I really know who gets it on a personal level locally. Otherwise I have been doing PT locally (BD) at the Orthopedics Clinic/center there - that isnt my Ortho dr I actually need to get one but that is another story :) ) and this has been going well. He seems very knowledgeable and always asking me interesting questions about MPS, plus he has on his own shared about the disorder with the Ortho clinic staff just bc he wanted them to know what it is! I have very tight IT band, calf muscles, thigh muscles as well as an out of alignment knee cap which are all causing really excessive, odd pain when I am on my feet and even when I sit really, which we are trying to help. (By strenghtening and basically re-training) We may end up doing Knee MRIs anyways as he said that X-rays wouldnt show muscles, tendon or ligament damage and that is what he thinks is likeally a problem especially if the pain con'ts despite our doing all this 'work'. So we will see but atleast he knows what he is talking about and takes a multi-area approach versus just working on one area (like IT band) for the knee pain and hip pain. And other things going on, include weird chest pain which I think is acid reflux but i'm not positive either - the only thing that eases it is laying down! Crazy!! I do have the GI Endoscopy already scheduled for July 1st at FMLH so havent done anything about it since that will show us if this is truelly the problem or not. I see my Pain dr this week and we'll re-visit the thoracic/lumbar injectoins - I still havent figured out what I should do??? In some ways I think I should jsut try it - could it further help the benefits I get from Chiopractic and would I be able to get off more of meds and in other ways I think I shouldnt do it - I don't know! So i will ask lots more questions of my dr whom is fantastic and I know will not mind. I know there are other Appts comingup just can't think of what they are!

I havent seen either of my nephews now in a week and think I will see if I can get Quinn this week for a few days, and
still havent figured out my sister - she's apparently mad at me about something (it seems like) - but I really want to see Zander to! I miss him alot - I thought about him and Quinn so much the last few days..

Since I havent slept well, including not getting much sleep in the past few days I better try to go to bed so much to do tomorrow.

Good day all, God Bless,

Erica

Weds, May 20, 2009 7:31 PM CDT

I actually am back here updating the rest of the news believe it or not! I even have some new pictures to put up and will try to do that right away to.

1st though yesterday the 19th I was able to hold the Awareness 'event' at Skywalk Pharmacy in Children's Hospital
- I worked with Ledia the Student Pharmacist and think it was actually kind of fun getting ready! She is really nice and we talked alot about how different it was for her to meet me in person vs just reading about MPS on a website as well as she had alto of questions for me on what I thought of various things and my experiences with diagnosis, out-reach, etc. We both made cookies and frosted them (I was even able to find purple food coloring!!) and decorated these with purple ribbons and then packaged them, tied them with purple ribbon and a little template which had the word 'HOPE' and the purple ribbon on one side and on the back had the www.mpssociety.com website. I had also brought in brochures/pamphlets from the MPS Society and from the Join the Search program (www.jointhesearch.org)
and made posters with photos of kiddos with the various types of MPS on. Overall I think we covered many basis with this all and I think if we even reached out and raised awareness of MPS to a couple medical providers and a couple families (this pharmacy is extremely busy!) we are doing awesome!

I started taking a new supplement last week called 'Ligaplex II' which is specifically for people with degenerative spine problems, disc bulging, spine trauma (such as surgery), and ligament probs. Since then I have been able to cut down on my pain med dose by over 1/2 (and may be able to stay off of the long acting morphine all together, I am just trying this so will see). It also helps
if a person is unable to hold Chiropractic adjustment which although I was seeing good results from my Chiropr. it always would work it's way back out after a few days to 1/2 a week, which now last week I last ed all week (minus a couple days that where a little worse but not as bad as they would have been) and this week seem to be doing well to since I saw my dr Monday so far. I highly reccomend this supplement to anyone who has spine problems. I know that I got my sister's BF to start it the other day.

Sara, her BF and I where all at the health food store before I went down to Milw. My sister and I have been talking to a couple people about my nephew Zander and starting him on supplements for his Autism and on the GFCF diet atleast on the days hse has him. All agreed it was better for Zander to have the benefits of these things atleast part of the time even if Sara's ex-husb. refuses to do it. (sad) So she bought some probiotics for Zan's GI system health, a mood calming agent and I think there may have been one other thing. Zander is extremely smart what with being able to run through his ABC's, signs parts of a couple kids songs (or rather say them), with a couple of his favorite books when we read them he will fill in the blanks if we stop and always the right words in the right places. And he also can count up to 6 already. He will be 3 on June 7th.

I am still debating whether I will do an cortisone/steroid injection next week as is planned at this point. This would be done by my pain dr - one reason I am leaning towards doing it is the possibility of reducing pain meds even more and on the opposite end am thinknig maybe I should not do it atleast at this point bc of the improvements i've seen with the supplements I take? I really just am not sure yet.

I also saw GI the week before and they want to do an Endoscopy and a swallow study - I have to reschedule the swallow study but that consists of them placing a small camera down the nose and then watching you swallow. The other they place a camera/tube down the throat into the stomach, etc. to look at the various 'GI' areas under anesthesia. That one is the 1st of July. I wasnt able to do it sooner bc of other things I have already going on teh dates that where open and I am not really in a hurry for it anyways. It will more or less just help us.

Next week I see my Neurologist at Froedtert and need to get in touch with my Neurol. at UofMN before then to find out what he wants to do next. I am guessing maybe him and my FMLH Neurol. will talk. I did hear back from
my Sleep med dr and she confirmed what she suspected last thurs that the Co2 levels where perfectly normal in both of the previous sleep studies and there
where no indications of hypo-ventilation. (under-breathing)

All for now. Love and God Bless,

Erica

Monday, May 18, 2009
This is over due simply bc i have been meaning to get on here and update since before last friday which was Int'l MPS Awareness Day! This weds the 20th it will have been 4 years since I started ERT which is simply amazing to me! Yet at teh same time I can tell it has been 4 years bc I am tired of driving down there every week and have come to the point when there is something going on that I would have just missed in years past now I will skip infusion and
go do whatever it is. Granted I don't do this alot but I do it.

I made cookies with purple frosting and purple ribbons decorated on w/edible gel for friday and sent out an group email on what the day means and to remind others to spread
the message and what to look for in the cases of those providers that I sent it to. Bc it was ERT day the real
that we are putting out to. This is a high-flow (busy) pharmacy and plus alot of people walk past it bc of it's location to get to their Appts in teh clinics or hospital so we'll hit alot of families and providers to with what MPS is.

There is so much else to update and i'll come back and do that another day - hopefully i'll get some time but it has been crazy busy!! So many things going on and then with it being light out longer I am outside alot more even just working on my patio planting/potting flowers, etc and at my
nephews house. Then I had been working on the Pain fact sheet, POPAN leader stuff, etc. I will get back here and update on a couple of Appts I have had to.

Last but defiently the best of all, my youngest nephew Zander just started saying 'Auntie Erica'!!! This is a Huge, huge step in my opinion bc of his autism - him and I am super close and the other day when my older nephew and I spent the night at my sisters (due to watching all of the kids) Zander would bring his 2 favorite books the next morning (sat) and crawl up into bed with me and snuggle and then get up, leave, but then he would be walking around the bed and I had swore he was saying 'Auntie Erica'
while pointing at me - turns out while I was gone for a little while that afternoon he was standing at their picture window saying it plain as day except 'where is Auntie Erica' over and over! Sunday he said it several times again right to me. (this is big deal given his autism) My heart just melts when he runs up and gives me his hugs, or cuddles in my lap with his favorite 2 books ('Tooey to the Rescue' and 'Goodnight Moon' both of which he can fill in the words at the correct places if you stop reading for a minute!) or when he says my name which his ginormous smile, ookay just everything about that kiddo makes me melt! (and the same with my older nephew to, whom I had all weekend as well)

More as soon as I get a chance,

Erica

Sunday, May 10, 2009 0:37 AM CDT

Relatively new update on the next page but I did go to MN Weds-Thurs and saw several of my specialists there. What with our not knowing my former neurologists plans (Dr.Charnas) as far as seeing pts very part-time eventually
possibly at UofMN again my local Neurologist and I decided
to just go ahead and see neurol. who took over his pts. I had met this dr previously through Dr.C when I was in-pt.
at the UofMN for the ICP study (him and Charnas tag-teamed me that week) He is very nice, alot quieter then Charnas and yet still very personable, and funny. I asked him about
our issues locally with getting a new Neurosurgeon to manage my shunt and work with my drs in that area, and how
the ones my lcoal dr talked to would refer back to my former Nsg. as he was the one to put in the Shunt. But that
this dr did not want to over-see and/or work with us in any
way really even despite my Neurologist and him being i n the same office and her having spoken to him several times.
So anyways he said that he really likes the neurosurgeon (Dr.Haines) who did the Bolt-ICP study (where I first met him)and that we should go ahead and consider him a part of our team (am I confusing anyone yet?) and our 'official' neurosurgeon. He also said he was going to take the MRI images to Dr.H and look over them with him, concerning my question could the shunt be causing the lower back pain as
well as the seemingly worse looking discs, facets and lumbar area in general? So he is going to do that, and get
back to me. He did tell me that if I hadnt had the ICP study and it's results having been fairly normal a little over a year ago he would say my symptoms where hydrocephalus/shunt related. But as it is he would like to
look at atleast one other area and is/has talked to my Pulmonologist.. he told me that although my sleep study results for this have been normal bc of the narrowed area and 'under-breathing' (I think thats how he described it) that can happen in MPS he would like to try a bi-pap or c-pap if possible for just a couple days to see if this may help the headaches. His thinking was that although it would
be expensive, if the headaches are caused by Co2 build-up
from un-diagnosed sleep issues then maybe it would be an easier fix to the headaches. I usually send an update to providers after appts in MN and did so this time to including to my Pulmonologist who in turn must have talked to the Sleep Med-Pulmonologist I used to see and asked her if they could get me in as soon as possible. They did want me to come in Monday but I already have other appts then so
I will see her Thurs before my pain dr fup.

The Neuropsych testing went fine, no big changes other than
with fine motor which was a concern to them until I told my
dr that I had just had EMGs done by my Neurol. back in winter which show 'chronic' carpal tunnel. So between that CT occuring and the spine stuff it all adds up but I sitll function fine i've learned accomodations for myself.

Ortho-spine surgeon went fine he really just said to watch the thoracic and lumbar spine issues and that my neck was
healing well. He also brought up about the possibility of doing injections into the facet joints in the spine which is something my Pain dr and I had talked about the week before when I seen her. So he gave his blessings for any kind of treatment/injection she would like to try.

---
This coming monday I have the bone density test at FMLH and
then after that I have the GI appt. which is a new provider. I am hoping she can help me figure out what is the cause of the abdomenal pain I seem to have been getting
for a bit now in addition to teh very mild swallowing and heart burn issues. It will be interesting. My Pain Psychol.
saw a different dr in that clinic and so I have some idea of how it is run. -- Then Thurs I see the Sleep Med Pulm
per my Pulmonologist and Neurologist see what she says. After that I see my Pain dr and hoping she will want to go ahead with the injections and see if they help. Also curious to get her opinion now on some of the MN happenings.

I skipped ERT yesterday to go do something with family and was fun! I'll go back next week but don't feel bad at all about taking some fridays off now if I have something I really want to do.

Please keep these families my friends the Butts, the Craigs and the Bohannon's in your prayers.

Erica

Monday, May 4, 2009 0:50 AM CDT

I owe a real update and have been meaning to for a couple days or more... the last week especially has been busy!

Before I update though I want to ask that all keep my dear friends in their prayers. Sadly 2 more children with MPS I have lost their battles with this horrible disease and went to be with our dear Lord in Heaven. Brinley Craig and 'LB' Butts touched many of us and our prayers are with their families. Their webapges are caringbridge.org/visit/brinleycraig and carepages.com/lb

My friend Hali her son Gage is also back in the hospital with complications from his transplant for MPS I, and his first engraftment study showed just 17% so please pray this increases and Gage recovers quickly. carepages/com/gagebohannon.

I had the Brain, Thoracic and Lumbar spine MRI images the week before this last and this week went and followed up with my Pain dr. The results kind of stunned us both I think it is fair to say and we are working to figure out what they mean. I guess I figured and I think my dr did to since it had only been a year since the last MRIs that they would essentially be the same with degeneration and bulging. I'll copy here what I emailed to my Neurologist, and several other specialists to get their opinions for us.
I also do happen to see my Ortho-spine dr, my Neurologist and the Neuropsychologist in MN on Weds and Thurs this week so that will be good. I guess this also may be the likeally cause of my headaches and my pain dr thinks a definate sign that the shunt needs to be removed and moved
as she thinks the brain images report clearly points to Hydrocephalus. Although I don't want something further wrong (but I knew there was as my legs wouldnt feel like they do when I sit and stand for to long and I wouldnt ahve the pain and the headaches that I do) it would be nice to get this shunt out once and for all and just moved. I don't know what will happen and what the docs will concur on but it would seem like to me that the shunt together with the MPS could be a very likeally cause of the lower lumbar problems. (not to mention the brain). Here is the key parts of the report:

Brain:
There is no intercranial area of abnormal signal
intensity, contrast enhancement or mass effect. The ventricles, sulci, and cisterns have a normal appearance, aside from a slightly large cisterna magna or perhaps a small retrocerebellar arachnoid cyst.. Normal flow voids are present in the major intracranial vessels.

Lumbar:
(much more to report but again the key)
Minimal disc bulges are again noted at each lumbar level, greatest at L4-L5 and L5-S1, but no focal herniation. Minimal degenerative signal change is
noted in the L4-L5 disc, but it maintains normal height. Mild multi-level facet joint hypertrophy and perhaps ligamentum thickening is also noted.

Con't - The above congenital and degenerative findings combine to cause mderate spinal canal stenosis at L3-L4 and L4-L5, milder elsewhere. Foraminal stenosis is severe on the right at L5-S1; moderate on the left at that level and bilaterally at L4-L5; and mild on the left at L3-L4.

The lumbar peritoneal shunt is at L2-L3.

In other news my nephew Zander who will be 3 in June is beginning to put sentences together such as 'me no touch', 'hot tea' (everything is hot and no right now!) and
'choo-choo tracks'. He also points out many things and says
them plus points at the clock and can say the numbers he is pointing at such as '8' and like yesterday he stopped when we where walking by a picture on the ground, pointed at a picture of flowers in a vase and counted each individual flower! (3 of them) He says his ABC's now and is just learning so fast!! His favorite things are the swing and his trains. It melts my heart when I see him he just breaks into the HUGE grin and comes running into my arms, and if I try to leave he cries and cries just like when his Mom, my sister leaves! Between him and my other nephew Quinn I am a very blessed Aunt! I only wish I saw my 2 neices more!!

I can't really explain it right now but I have been struggling a bit with all of these children losing their lives (in the last year Hurlers children during transplant I think there has been atleast 6 losses if not more which is sad as these children are often the same age as my Zander and I just am saddened for their families and for what MPS does to all of us.

I have been 'supplement shopping' and experimenting with different things which together with the Chiropractic have been helping a quite a but - I do think I need to as my chiro. about possibly increasing to 2x's a week ago and for some exercises for my mid-back and neck as by sunday it just is really tight and painful again and I see her on tues. Some of the supplements my friend Patty has told me about that I have tried, include Calcium/magnesium, hyaluronic acid and fish oil. I am thinking about trying 1 or 2 others depending. My pain dr is also thinking again about trying spinal injections to help relieve pain in my lower back and hopefully in my mid-back which could aid the chiropractic but we need to get my spine drs approval and plan first this week.

I will update when/if I think of anything I have forgotten.

Yea, though I walk through the valley of the shadow of death, I will fear no evil: For thou art with me;
Thy rod and thy staff, they comfort me.

Erica

Sunday, April 26, 2009 10:49 PM CDT

HOW COULD YOU? - By Jim Willis, 2001

When I was a puppy, I entertained you with my antics and made you laugh. You called me your child, and despite a number of chewed shoes and a couple of murdered throw pillows, I became your best friend. Whenever I was "bad," you'd shake your finger at me and ask "How could you?" -- but then you'd relent and roll me over for a belly rub.
My housebreaking took a little longer than expected, because you were terribly busy, but we worked on that together. I remember those nights of nuzzling you in bed and listening to your confidences and secret dreams, and I believed that life could not be any more perfect. We went for long walks and runs in the park, car rides, stops for ice cream (I only got the cone because "ice cream is bad for dogs" you said), and I took long naps in the sun waiting for you to come home at the end of the day.

Gradually, you began spending more time at work and on your career, and more time searching for a human mate. I waited for you patiently, comforted you through heartbreaks and disappointments, never chided you about bad decisions, and romped with glee at your homecomings, and when you fell in love. She, now your wife, is not a "dog person" - - still I welcomed her into our home, tried to show her affection, and obeyed her. I was happy because you were happy.

Then the human babies came along and I shared your excitement. I was fascinated by their pinkness, how they smelled, and I wanted to mother them, too. Only she and you worried that I might hurt them, and I spent most of my time banished to another room, or to a dog crate. Oh, how I wanted to love them, but I became a "prisoner of love." As they began to grow, I became their friend. They clung to my fur and pulled themselves up on wobbly legs, poked fingers in my eyes, investigated my ears, and gave me kisses on my nose. I loved everything about them and their touch -- because your touch was now so infrequent -- and I would've defended them with my life if need be. I would sneak into their beds and listen to their worries and secret dreams, and together we waited for the sound of your car in the driveway.

There had been a time, when others asked you if you had a dog, that you produced a photo of me from your wallet and told them stories about me. These past few years, you just answered "yes" and changed the subject. I had gone from being "your dog" to "just a dog ," and you resented every expenditure on my behalf.

Now, you have a new career opportunity in another city, and you and they will be moving to an apartment that does not allow pets. You've made the right decision for your "family," but there was a time when I was your only family.

I was excited about the car ride until we arrived at the animal shelter. It smelled of dogs and cats, of fear, of hopelessness. You filled out the paperwork and said "I know you will find a good home for her." They shrugged and gave you a pained look. They understand the realities facing a middle-aged dog, even one with "papers." You had to pry your son's fingers loose from my collar as he screamed "No, Daddy! Please don't let them take my dog!" And I worried for him, and what lessons you had just taught him about friendship and loyalty, about love and responsibility, and about respect for all life. You gave me a good-bye pat on the head, avoided my eyes, and politely refused to take my collar and leash with you. You had a deadline to meet and now I have one, too. After you left, the two nice ladies said you probably knew about your upcoming move months ago and made no attempt to find me another good home. They shook their heads and asked, "How could you?"

They are as attentive to us here in the shelter as their busy schedules allow. They feed us, of course, but I lost my appetite days ago. At first, whenever anyone passed my pen, I rushed to the front, hoping it was you that you had changed your mind -- that this was all a bad dream... or I hoped it would at least be someone who cared, anyone who might save me.

When I realized I could not compete with the frolicking for attention of happy puppies, oblivious to their own fate, I retreated to a far corner and waited. I heard her footsteps as she came for me at the end of the day, and I padded along the aisle after her to a separate room. A blissfully quiet room. She placed me on the table and rubbed my ears, and told me not to worry. My heart pounded in anticipation of what was to come, but there was also a sense of relief. The prisoner of love had run out of days.

As is my nature, I was more concerned about her. The burden which she bears weighs heavily on her, and I know that, the same way I knew your every mood. She gently placed a tourniquet around my foreleg as a tear ran down her cheek. I licked her hand in the same way I used to comfort you so many years ago. She expertly slid the hypodermic needle into my vein. As I felt the sting and the cool liquid coursing through my body, I lay down sleepily, looked into her kind eyes and murmured, "How could you?"

Perhaps because she understood my dog speak, she said, "I'm so sorry." She hugged me, and hurriedly explained it was her job to make sure I went to a better place, where I wouldn't be ignored or abused or abandoned, or have to fend for myself -- a place of love and light so very different from this earthly place. And with my last bit of energy, I tried to convey to her with a thump of my tail that my "How could you?" was not directed at her. It was directed at you, My Beloved Master, I was thinking of you. I will think of you and wait for you forever. May everyone in your life continue to show you so much loyalty.

A Note from the Author: If "How Could You?" brought tears to your eyes as you read it, as it did to mine as I wrote it, it is because it is the composite story of the millions of formerly "owned" pets who die each year in American & Canadian animal shelters. Please use this to help educate, on your websites, in newsletters, on animal shelter and vet office bulletin boards. Tell the public that the decision to add a pet to the family is an important one for life, that animals deserve our love and sensible care, that finding another appropriate home for your animal is your responsibility and any local humane society or animal welfare league can offer you good advice, and that all life is precious. Please do your part to stop the killing, and encourage all spay & neuter campaigns in order to prevent unwanted animals.

Please pass this on to everyone, not to hurt them or make them sad, but it could save maybe, even one, unwanted pet. Remember...They love UNCONDITIONALLY.

Now that the tears are rolling down your face, pass it on! Send to everyone in your address book and around the world! This IS the reality of dogs given up to shelters!

Saturday, April 11, 2009 1:16 AM CDT

It hasnt been so long this time since the last update but being that it is the morning (ever notice these updates are always in the morning? I can never sleep!) after Good Friday I wanted to wish all a Blessed continued Holy week and may you have a Happy easter filled with the knowledge of our Savior having died and risen again for our sins so long ago. I hope you have a hippity, hop happy day but never forget the true reason for this Holiday season!

I am up again and just decided to come here write about a few goings on with the week. I am not really sure why I decided to write this entry right now given the massive headache I have which has been maknig my vision all out of sorts all day but I am here so I digress. I am hoping the glasses as much as I do not want them will atleast help my vision and how weird it has been. .... Yesterday (thurs) I saw my Pain dr and Pain Psychol and then later on in the day saw my Chiropr. all of which went fine. My Pain dr gave
a few insights into the Fact sheet we are working on for the MPS Society and for which I will then give a final copy
to the American Pain Foundation. We are really trying to be
pretty comprehensive in order to help our MPS families as MPS pain is so different in so many ways than 'standard' pain and we're trying to cover the many different aspects which play into causing it like the neurological affect and nerve function which is commonly affected (neuromodul. type medicats. helps this type) as well as the joint type
pain and how it can and is commonly treated and then further are looking at post-surgical treatment of pain and
as well what types of providers best can treat this all what qualifications to look for. It's pretty in-depth but so is MPS (!) and Amy and I are just trying to put together
each of our unique outlooks on it in order to best serve families. It is a highly mis-understand area especially for
MPS pts and from my own experience to many drs mis-treat it
and mis-judge you. it has been defiently eye opening to do this but actually kinda fun - I am glad the MPS Society and Amy are allowing me to help on this. Anyways back to why I started writing this my Pain dr offered to look at the final product for us either before or after Amy and I send it to the committee and my Pain Psychol. is interested
I guess in putting this out as a copy in her office for pain pts in general.

Because the pain in my lower back continues to act up occuring from time to time but at the same time having 'spread' to further down my lower spine my pain dr
and local neurol. have ordered MRI's to be done. They are doing these partly bc it has been about a year since the last ones where done and the reccomended schedule of tests from the specialists is 1x a year. The 2nd and more important reason they are repeating these is bc of the headaches, the shunt being in my lumbar spine and this same region and below it being where the pain is where the disc bulging, degenerative issues, etc are. basically in other words just to double check there isnt anything changed. The MRI's will also be done of my thoracic spine and my Neurol. would like to include brain imaging to. (My Genetics team reccom. this as well).

For any MPS pts/families reading this it's pretty important
to know that in MPS the ventricles of the brain do not generally change even if there is pressure within bc of the
GAG storage and the rigidity this causes.

If you will please say a prayer for my sister's boyfriends family - earlier this week his uncle committed suicide and I know they where pretty close. It is sad and aweful that anyone should have to feel they had no other choice but to take their life. I personally believe there is someone each of us can always turn to no matter how tough times can seem and I am sorry he did not feel another
choice. It is sad. This week my parents also along with others of my family went to the funeral of my great uncle - someone I did not know well but which I do know was a great person and for whom I know is in a better place free of earthly pain. I pray that his family is at peace.

I know it was probably not a great thing to do but I skipped out of ERT infusion today again - I just am so burned out on how tired it makes me feel and the fact of driving back and forth every week for what feels like forever now. I love my 'family' there but wish I either lived closer (but then would miss my family here and seeing
my nephews and spending as much time with them as I do) or there where some other arrangement. As tired of MPS as I am
and sick of feeling crappy I am it's weird that I have no problem helping other families and doing things with APF - I guess thats just replacing what I had been doing job wise
and school wise before MPS started to affect me so much?

In any case there was something else I wanted to write about but have forgotten (whats new there!) so am going to sign off.

Happy Easter!

Erica

Tuesday, April 7, 2009 2:16 AM CDT

Afte spending the last 15 mins trying to figure put the new
set-up to this cb I am finally in here and actually don't feel like writing anymore but I will.

I was thinking today when it was one of my harder days bc of how bad I felt how truelly great this special Holy week of Lent is to me - it brings the promise and reminder of God's son dying on the cross to save me and rising agaon Easter sunday. Easter has always been my very favorite holiday both for the springtime beauty and for the renewed promise of God's salvation and a blessed home in heaven to be reunited with family and friends and free of MPS and it's earthly pains one day. I love that I am able to look at and see what my savior did for me and see what promise there is for me one day in Heaven. I love all things easter Lillies and in fact have one sitting on ym kitchen table and the beauty of renewal of these flowers during the
special holiday season. I lvoe that despite my earthly sins
my father in heaven is there watching over me and knows his easrthly plan for me and for all the days of my life.

I also was thinking alot today after something I wrote to a
friend of how lucky I am to have despite all of the rocky
'relationsships' with some providers how truelly great others are. I especially feel blessed to have my one dr whom shares similar (but different) chronic) issues which unite us and bring us a friendship in a provider/patient sort of way. We share a commonality with our church and beliefs, with many of the types of specialists we see and with our ambitions to help others. She has helped me trewmendously just by being there to support me, to listen and to get what im feeling many times. I think and truelly believe just as her and I once decided not that long ago that our heavenly father brought us together and knew we would be a great support system for each other through the things we each experience with our own disorders. I feel very blessed to have her.

My pain dr and I have con't to play phone tag with each other which is funny but I do see her this thurs if nothing
else and have some things to show her and know she will have much great advice for me on the pain write-up we are writing. I have been focusing on the supplement side of pain this past few days and am fascinated really by what there is available and so have written down doses, sites and types for the article of the ones that seem to be most
mentioned such as Calc/magnesium, Ginger, and there is a host of others it's fascinating really! My next step after this is to ask my Chiropr. some question for that portion of the article and get her input so we can provide the best
possible inform. to our MPS families. Amy the Mom whom I am working with on this and I have been exchanging emails and each have really interesting ideas and view points plus experiences to bring to this article for our families which I think in the end will be really great. :)

I was reading a latest Genetics report I received from my Febr appt via mail and although I knew this was one of the things wrong with my t-spine I was surprised with myself that I never looked up what it was for whatever reason. Apparently I have a small syrinx extending from T3-T9 which is a fluid filled cavity of space in the spinal canal/cord which is not supposed to be there and can cause progressive damage to the spine. Ugh this on top of the degenerative issues, stenosis/myelopathy, disc bluging and
other things it's any wonder my spine feels like it does? Today for instance I was up all last ngiht -about 2 hours of sleep due to bad pain - nerve pain I guess it could be described as and though I was tired it just radiated. And this hasnt been like this since before my surgery. :( I hope i've just over-done something and it improves. Tonight I managed to fall asleep for about 2-3 hours again.
I am off to the vitamin store tomorrow to pick up a few more things to try that a friend reccomended after I talked to her abotu some questions I had. My chiropr. was able to do a little today to relieve what has built back up for whatever reason into a very toght spine (the dr there for my own chiro whom will be back fri said it was 'one tough spine' and 'good thing I had as much breakfast as I did' meaning it was extremely hard to work on. I asked him why he thought my spine would have tigethened back up so much in my thoracic area after it had
relaxed and felt so good for several weeks there after starting seeing my chiro. He said probably bc there has been so much work above the area of my spine which is naturally meant to move but now doesnt and this puts undue pressure on my mid-spine constantly in most all movements I did including bending which probably caused the increased response again. Especially since they have such a small area to work with (lumabr spine has the shunt and has had all the revisions so they are not able to do anything there either) So the one area of the spine that moves the least now has to bear the most pressure, so I guess this means it will be a forever project on my spine to keep it 'relaxed' and get it in shape. I hope my chiro.
on fri is able to get it feel looser again and less painful
as this stinks! I have seeminly lost the wires to connect my TENS unit from the actual unit to the patches (probably the cat has it somewhere as they hide everything and I find things in the goofiest places!) This really would have helped right now so i'll have to call the company to try and get a new leds/wires. Bummer!!

Otherwise there really ahsnt been alot going on - I see my chiro friday, and my pain dr and pain psychol. on thurs.

Much love and God Bless,

Erica

Monday, March 30, 2009 8:17 PM CDT

hello all, I saw the optomestrist today (I see my Opthalmologist at UofMN in April) but have been having more
problems with vision so decided to go locally for the time being since I knew i'd need a new script for glasses anyways. My vision corrected is 20/40 and the new frames are pretty quite. I do find glasses annoying but maybe will get contacts after awhile if their covered anyways. The astigmatism, far-sightedness and corneal clouding, are there and about the same I think.

Otherwise not to much new - will update when I know anything new. :)

Tuesday, March 24, 2009 3:31 AM CDT

I wanmted to share a little more on exactly what the c-spine surgery I had in Nov. did to relieve the cord compression and degenerative disc and disc bulging problems. basically it's 'fancy' name is Cervical Decompression w/ 'Open-door Laminoplasty and instrumentation + bone grafting. (the bone grafting is like a 'fusion' of the vertebrae to prevent de-stabilization.

open–door laminoplasty in which allograft bone and titanium miniplates were used to treat cervical myeloradiculopathy while maintaining functional cervical motion segments. This type of laminoplasty has the advantage of providing immediate rigid stabilization of each opened lamina, maintaining the posterior cervical tension band, providing a segment of bone that heals under compression and is quick, effective, and durable.

Explanation of what is done:
During a cervical laminoplasty, an incision is made on
the back of the neck. A groove is cut down one side of the cervical vertebrae,creating a hinge. The other side of the vertebrae is cut all the way through.
Then, the tips of the spinous processes are removed to create room for the bones to pull open like a door. The back of each vertebrae is bent open like a door on its hinge, taking pressure off the spinal cord and nerve roots. Small wedges made of bone are placed in the opened space of the door.At the end of the operation, the door of the vertebrae swings shut, and the wedges stop it from closing all the way. The spinal cord
and the nerve roots rest comfortably behind the door.

I know this may be boring to some to read for a update from me but I am amazed every day at what my body can handle - it really is amazing!

I apologize now for any sp errors - having a 15 pound fur-ball (cat) sitting in your lap does not help type!!! But really otherwise things have been quiet - just 1 appt besides ERT this week which is with my favorite provider. Alot of phone calls to return/ and resched. to do for some Appts. to.

I am working on the 'Fact sheet' on Pain mngmt with a friend of mine (MPS I parent) for the MPS Society and have a phone call with my Pain dr tomorrow as she is an amazing wealth of knowledge and is answering a few quest. I have.
She is great! Otherwise I also see my Chiropractor this week and know that my back needs it as it feels tighter for whatever reason since last week. (sleeping wrong or bending to clean, who knows! I'm still getting used to having to use alternative ways to sit, clean, sleep etc!

All for now,
More soon,

Erica

Wednesday, March 18, 2009 2:40 AM CDT

Hello all, just thought I would write a little note. For whatever reason I always seem to write this in the middle of the morning - am not really sure what that is about!

Not to much new mainly bc I have been just doing the small things in life that make me happiest - including spending time with Zander (youngest nephew) - I watched him Sat night after my Sis, Zan and I did a little shopping and she
took me out for dinner. Zan and I always have a great time
playing with his trains and playing hide n seek behind the couch. Zan hides in the front of the couch and I behind it and then we duck down and pop up which makes Zan crazy happy and he laughs and laughs while his eyes just light up. If he wants me to con't or if i sneak around to his side he does he more sign (fingers together and then each hand touch in a pinched sign. - Hard to explain but it's the sign language 'sign'. Then he will push me back to my side and we'll do it all over again. He just lights up my day and makes me so happy. So I generally go to his house 2 or 3 times a week to hang with him and my Sis'. I also have had Quinn alot both spending the last weekend and then
usually see him one day during the week he comes over and I
take him home. He's totally into this race care track set my Dad has and also playing with my Dad's train set when we go over there. Otherwise he loves Junior Monopoly, Sorry or any game like that and 100piece or bigger puzzles.
So it'a always fun!!!

On the MPS side of things the Chiropractic cont's to be really great with my mid-back really only hurting if I over-due it such as sitting on my knees or bending over. My neck is okay and my lower back where the shunt it and lower is acting up yet again but unfortunately due to where
my shunt is my Chiropractor doesnt want to mess with it. (the area) So it justs is and I try to minimize the things that I know really aggravate it.

My Neurologist and I have exchanged a few emails on the Neurosurgeon situation and so she said she would put in a call to the 2 drs she knows and talk to them about me and seeing me about the headaches. It's a little complicated bc
of my current shunt, the spine issues and bc the shunt was put in by my former Nsg alot of times other drs in the same
field don't want to mess with someone else work. So hopefully my Neurol. can do magic and i'll have a new nsg.
She is going to let me know.

I talked to one of the MPS Society board members who is working on new fact sheets and 1 in particular on pain mngmt and apparently s/o else (a member) called and told them I knew alot on pain care from experiences with it and
from just doing alot of reasearch plus POPAN work. So she said she'd be happy to have my input so that will be neat.

I also just did the media portion of the POPAN (power over pain action network) co-leader training on Monday which was
pretty informative and interesting. I also have my itinerary scheduled including the Flight/hotel for June's Summit meeting/training in Minneapolis. In the mean time I have been doing some thinking on what would be really informative to others and getting our message out about pain awareness/care. So I talked to my Pain Psychol. who also happens to be a Professor at Wisconsin Lutheran College and she is working to hook us up with one of the other Professor's classes to present about Pain care and POPAN. (Plus MPS and the tie between the two I guess) So I am excited about this and hope we are able to do this. I also am bringing her some info for her office on the various Pain fact literature we have from APF and POPAN. Another idea we have is we are going to try and get onto the NPR radio and speak about our nessage. So i'lkl con't to keep everyone updated!

I was supposed to see the GI dr tomorrow (weds) but due to a few things rescheduled I havent really had any major problems in the past month and think that getting off of a few of the meds I was on and lowering other doses has helped.

Keep this little guy in your prayers - Brinley also has MPS I and is in transplant at the UofMN right now. www.caringbridge.org/visit/brinleycraig

Wednesday, March 4, 2009 2:41 AM CST

Alot of people have asked me how the Children's hospital/ALSA charity dinner went Sat. so i apologize for not updating! I have to admit it was set up alot differently then I thought it was going to be from what I
had been told and I guess if knew the way it was I would have brought pamphlets and a video on MPS...ooh well atleast it was another awareness event on MPS and educating
people. I do have to say it was weird speaking in front of
former classmates - I much prefer a room of strangers!!!

I was reading a bunch of medical reports from various providers of mine this past week and though I knew that some of my specialists come here and check this out/read the updates from time to time it was still really weird to
see in one of the reports my neurol. mentioned a pics.
I emailed to a group of people including her right after the spine surgery in nov. and that these same pictures where also on my blog. Ooh well atleast providers ger an honest look at my life and understanding where I come from, from this blog I think.

I had my second adjustment from my chiropractor and feel actually pretty good tonight since! I didnt take the 2nd dose of avinza this morning just 1 30mgs tab at night and right now 1 msir in the morning and today was the first time I was able to do this and feel pretty good still!!! YAY!!! I am very excited. She also gave me a few supplements like multi-vitamim, fish oil (DHA/EPA), calcium/magn, and B-complex which these should also help with energy.

I also on the med front have gotten down to just 300mgs from *600-700mgs) of the Lyrica in the last week and still trying to figure out how to get off of the cymbalta w/out the aweful withdrawal effects. I am taking topamaz yet bc it genuinely makes a difference and it seems unless I can find a different treatment/supplement i'll be on the protonix as when I don't take it I have aweful heart burn. And Synthroid is the same. So I am working on it! I guess I should add that I thought I was a pretty good advocate for my self and had learned alot in the last 4 1/2 yrs but
from now on I will always, always find out the risk of waithdrawal side effects such as the case with cymbalta, I don't even need the med for the nerve pain anymore as what I do have left (healing) is taken care of well by the Avinza and Lyrica! It definetly sucks being on a med just bc I can't get off it bc of se's!!! GRRR!!! I also have officially gotten off of the midodrine as I am pretty sure that was a big huge culprit of my crazy bad itching and weird vision blurrines. (which I already can't hardly read the print on cans/packages, etc and many things not at all
but this made it so much worse!=. So it's been a full week since i've been on the 3x's day dose and 2 or 3 days since i've been on the 1x a dau dose and as of yesterday guess what the itching was like 100% better and vision is improving! So I don't know what will happen there but I won't go back on it and won't try florinef which I was on in the past and hated how it made me feel + the blacking out hasnt gotten any worse in this time frame actually none
which is about what I was having on it - 1-2 episodes every
few weeks...so makes me think it wasnt really doing anything anyways...

I have been as I said last time looking up home made recipes for all varieties of things including cat - ear mites (from running out in the hall at my aprt complex and/or the neighbors cat the few times it came over for play date with my youngest kitten 'Grey'. In any case lemon steeped in hot water over night then sprayed on the car works well as does vegetable oil in the ears to smother ear mites, vit e helps inflammation and brewers yeast apparently sprinkled in the cats food helps keep pesky things away! lemon, virgin olive oil, vingera and Apple cider vinegar work amazing in the hair and there's just a ton i've found inmcluding for cleaning whjich is fun and awesome!! Which BTW salt in the vaccuum bag/compartment kills fleas. So happy hunting! :&)

Have an Absolutely splendid day!

Erica

Friday, February 27, 2009 11:06 PM CST

A big thing a have been doing lately is research online on both the meds I take and on natural OTC supplements. I am tired of feeling so well tired so am working to find what I cna to help the fatigue and take away as many of the prescr. meds I take except those ones I really know are needed. I am working to find my options of feeling the best I can this includes looking into alternative therapies in conjunct w/standard care I receive. I basically think I am the only one who is really going to get myself feeling better.

One of those things I have just started doing is seeing a Chiropractor. I just began this earlier this week and she believe it or not knew what MPS was and about it. She wants
to work with my providers and she is being very conservative on the methods she uses to help my back. I just have really liked her the 2 times i've saw her already
and think it only can help especially with how careful we are being. The methods she will be using include an electrical stim therapy similar to the TENS unit I have and also the stadard treatment options - which will be slightly different than what a non-MPS chiropractic patient
would receive.

I absolutely know this will cause a few people reading this to raise their eyebrows (chiro care) but to feel as crappy as I do and have to have no energy I have to try everything I can. I do want to say that the decompression/laminoplasty surgery absolutely did help my neck pain and was the right thing to do - this feeling bad/lack of energy is related to some other part of the MPS a big part I would be willing to say is the headaches/pressure and how bad these make me feel.

Each of us makes very personal decisions for our well being each day and I am glad to have options. Maybe it will help, maybe it won't but I won't know unless I try.

I also have maed a decision after giving it alot of thought and after talking to my genetics team to switch Neurosurgeons completely - I have a bit of a background and hx w/the 'Chief' Nsg whom has MPS experience at UofMN and am contacting him again. We also will be looking for a nsg in the Milw. area but outside of
FMLH as to many of my providers are not able to get anywhere w/the dr who implanted my shunt and he does not apparently want to 'help' manage my headaches anyways. I am a very big self advocate for team providers and feel my
Neurologist already has had to take on a ton for me and she hasn't been able to get this surgeon to take any interest despite their being in the same
hospital/clinic setting either. I'm just tired of battles, constant battles! I as a patient feel like our providers at the very least owe it to us to maintain what they decide to implant in us even if we are not 'easy' patients and it irritates me to no end how ridiculous this struggle has been.

Tomorrow I have been asked to represent Children's Hospital of WI at a Charity Event - dinner. I will represent CHW and there will be a speaker on behalf of ALSA. Before the 2 of us speak there will be an open bar, a dinner and a few other little things and then a DJ afterwards. It should be interesting and another opportunity to share my story and what MPS is.

All for now. Gid Bless,

Erica

Monday, February 23, 2009 0:48 AM CST

I know I owe an entry and I am sorry to my fellow readers but I also know you'll understand atleast somewhat when I say I have been feeling pretty crappy headaches and even pain wise over the last week especially and I just havent done much of anything except lounge at my Apt. I never even made it over to my parents or to my sisters (and boy did I miss my nephew!!) during the week. Then friday night I took this new med the geneticist gave me to help with itching for the days after ERT and I ended up sleeping for almost 24hrs I literally went to bed at around 11:30ish and
didnt wake up except once till 7:30 sat night! Talk about extremely bummed that I slept all through Sat - so needless
no more of that med I refuse to let a medicine control my life. MPS already does enough of that. So I am hoping this week will be better and i'll feel better.

I just have had such horrible, aweful headaches and it's like I can't get any of my providers to really take them seriously and help me figure them out. My Neurologist tries
but we need Dr.Charnas really and my Pain dr has an arsenal of meds we try but she is afraid to try anything more I can tell to. So I am weaning myself off of atleast 1 med (cymbalta-on it for nerve pain but I also take Lyrica) hoping this makes me less sleepy. And my memory and
focus is just like in the crapper pardon the pun but it's the truth and I hate it. So I also have been doing a little
research on supplements and next week going to go back to the health food store and try a dfew things including this
high energy multi-vitamin my sister takes from there. I am so tired of concentration being all over the place and even typing is hard. Grrr!!!!

Ironically it was exactly 4 years ago on Sat (21st) I got the call that the blood/urine tests where positive and I had MPS I. So I had a dx after a long and frustrating battle to find out but dangit if I would have had even the slightest inkling then of how abd things would be or I guess I shouldnt say bad but different how different my life would be I think I would have cried honestly. I think this is the exact reason God does not let us know what is ahead of us and gives us only what he thinks (knows?) we can handle. So while i've come leaps and bounds in that 4 years so much has also changed and in some ways I miss what
was then. I sure wouldnt want to start over with assembling
a crazy team of providers bc that was like treading water while taking baby-baby steps WHILE stuck in super glue and for the most part I really like the providers I have today
especially my pain pschol., pain dr and neurologist. I do wish my GC had more time bc I could really use her help some times but I just have learned to figure it out on my own and search on my own till I find what can help many times this has meant going to providers that are not even on my team and asking if they could help with information.

Enough of that though - another ironic thing since I could
not get my genetics teams help on the dental-medical insurance thing I tracked down some info in pubmed on some research articles, put together the Society brochures/print-outs and by contacting my NeuroPsychol. and the BMT coord. at the UofMN I was able to get in touch with the Dentist there who has experience with MPS and she
wrote me back and is sending me some info she was going to put together. She seems very nice!

I also was able to get in touch with the coordinator handling my former (still somewhat) Neurologists schedule (BMT coord for MPS at UofMN) and she and I exchanged a few
emails but are going to talk this Tues. I sent my neurologist at FMLH an email last week asking her something
else and then asking her if she was ok with my still seeing
Dr.Charnas and the 2 of them working together w/me sharing
my care I suppose as she has expertise on Dysautonomia and he on MPS I) So anyways she emailed me on friday and thought this was very good and was happy to con't to work with him bc of his area in MPS I and he could help guide us on things we might need to do. So I am happy about that.

I also have an Appt with the Eye dr there again but it's not till like the end of March or early April I might email him directly as he had told me to do that if I ever had problems as this vision issue and not being able to read things is driving me crazy. (just print on like containers, back of books, larger print say signs is fine)

There are other things to write I guess but will come back and finish.
Be Well and cherish each day for what it is,

Erica

Wednesday, February 11, 2009 1:41 AM CST

Just a few things which i'll update later but I saw my pain dr yesterday and things are going fine other than what we all con't to think is the overly tight muscles which is a 2ndary cause (tertiary really I guess to the MPS and then the compression). Basically bc I had and have such weird
spine structure and had the severe stenosis and then compression for so long the muscles in my spine overly compensated trying to protect not only this area of compression but the areas below where I have disc bulge, structural issues and narrowing. So we
are keeping things the way they are, considering trying an NSAID and i'm doing PT. I did forget to ask about what injections do and if they might help but in due time I guess. I can always call or email my dr.

I am on a hunt to find my former MN Neurologists new contact info at Shire
Pharmaceuticals so anyone that can/could help please help!

More later...

Saturday february 7, 2009
I just wanted to start a new entry but i'll have to finish it later. I just want to write a thought i've had on and off over the last year especially it only surfaces from time to time for instance when things are rough. But i've always wondered why do specialists wait until their is damage done before they fix or find a better solution to a problem? For instance with the spinal compression now 13 weeks out from surgery I seem to be 'healing' from the surgery in a whole new way. Bc of the nerves in my spinal cord that where compressed for such a long period of time and for which created so many long lasting problems my body seems to want to be trying to heal the nerves that are
affected by this area of the body and I just wonder why wouldnt specialists especially in MPS do surgery before this damage is even set in to prevent the problems from occuring long term? If this c-spine surgery where to have been done a year ago where would my body be today pain wise
and healing wise? On that same note if the Hydrocephalus had been treated sooner and even just dx'd sooner where would I be health wise ie dysautonomia, headaches, and pituitary which thus secondarily and even at a tertiary level affect blood pressure regulation, under-active thyroid, etc? And going back even further if the MPS I where to have been diagnosed even a few years earlier where
would we be? Isn't it amazing how it all trickle down effect, affects the other systems? Just a thought really, one that I have thought more abotu as I work with various specialists trying to get my body back into a basic, healthier shape post surgeries.

I have been up for over 36 hours so I need to get some sleep - and it is another early day tomorrow, my bf and I are going to the Public museum to see Imax and Titanic exhibit if it's there yet just walk around. The tomorrow night Quinn (nephew) will be here with Chuck and I and he's spending the night with me (quinn that is not C.) and Sunday am watching Zander my other nephew and Jordan my Sis's bf's son and Quinn to. Busy.
**We went to MPM and was nice, I think we both had a good time and afterwards we went down to a frozen custard place called Leon's on 27th.

Have just a bare minimum of time here so will be coming back to finish this for sure later on.

Part of the reason for the Neurology fup (which will con't to be every 3 months) was for the blacking out episodes-dysautonomia which has improved vastly on both the Midodrine and as a result of decompressing my spinal cord. I do occassionaly get episodes but my Neurologist told me she has talked to my Cardiologist (they work closely on this bc of the effect it can have on the heart if BP is increased to much) several 'good conversations' and they both agree that
raising the midodrine to the 3rd dose a
day if we keep an close eye on bp readings looking for to high of read-outs. So i've since increased to the 3rd dose a day, we will see if this is the final trick to this. The problem with Midodrine is bc it works by increasing the blood pressure their is an increased strain on the heart bc of the blood pumping through the heart at the increased rates it can put even more 'pressure' literally on the over worked 'diseased and bad' valves and cause the chambers of the heart to swell to. I hope i got this right but anyways thus the close eye. I do take a beta blocker which decreases strain on the heart by slowing down the
conduction system/rate and that is sort of a small cause of the low BP's. But w/out the Nadolol my bp's ran in the moderately high range (140's) so one drug slows down the conduction system while the other drug raises the pressure/rate I guess of blood racing through the heart. A bit confusing and looking at it now I think I need to read up on this some more. I do know that the trade off is that the Florinef which is one of the other drugs for decreasing/eliminating blacking out episodes increases fluid/sodium in the body - yuck! My Cardiologist knows alot about adult problems caused from congenital heart issues (I guess in a way MPS can fall into this and the affect ot has on the heart over time) and my neurologist is very well versed on neuromuscular problems and dysautonomia. They are and have been working really closely to try and solve this in the best way and so far things have been going good
since starting the med a couple months ago and also since upping the dose. My major providers are involved and keeping in the loop on this to.

We also talked about the CTS (carpal tunnel) and my Appt w/the Hand surgeon. Anotehr thing we talked about..

To be continued have to run...

Erica

Sunday, February 1, 2009 1:28 AM CST

Just a few things to write about - note first that I updated the pictures at the top of the page and also added a new video 'little boys and their choo-choos' further down. Just something fun.

I met a new family this week they are heading up to MN today (sun) for the work-up week fo their son Brinley's transplant - please keep the family in your prayers Amanda seems so nice as does Cara her cousin. Their webpage is: www.caringbridge.org/visit/brinleymeador

Earlier in the week I saw my Pain Psychol. which she is probably one of my favorite providers- really we just have alot in common life wise and share what works for each other, who we see and whats happening. Really she's more like a friend in an odd kind of way considering that she is
a provider but even she said this week 'I really think God put us together for each other'. Just very, very down to earth and I know I could tell her anything and she wouldnt judge. She also is the one whose sister taught at the parochial school my siblings went to years ago before we moved to BD. (at the school actually here in Juneau where I
moved in August)

I also ventured up to MN to see my Ortho-spine surgeon this
week for the 12 week fup and x-rays. Bc I would have had to
stay overnight there to fly with Angel Flight (bc teh appt was at 1:30 and by the time we would have flown home nad then the pilot flew to wherever they lived would have been after dark - just rules in spring, summer and fall it's less complicated) I chose to drive or I should say my Dad drove! Usually I go to MN alone and up to 2-3 years ago I otherwise would have driven there alone but this time decided I needed company so my Dad took off and drove which was sooo nice off him. My parents really are pretty great!! When I go back for fup my Sis's bf wants to come so
i'll fly then and he'll take a vacation day from work to come.

The Appt went fine and X-rays look awesome, the screws are so real like - it looks like I could unscrew my spine and then use that to screw something say to a wall, etc! Weird but facinating and hard to believe thats actually in my neck!!! My surgeon said it's healing great and looks good
so far. He'll see me in 4 mnoths for x-rays and another fup. He did say the pain between my shoulder blades is likeally caused from tight muscles secondary to both the surgery (what they have to do to the muscles higher up during surgery affects the muscles lower in the spine apparently-I did not really know..) So anyways the other thing is he things the degenerative and structural issues caused from the MPS I are affecting this area and pain. (tighter, smaller area for muscles, disc probs, surgery etc it seems all contribute) His reccomendation was alot of PT, massage and ultrasound, and also he thought trying either Pilates or Yoga could help - all to help loosen the muscles and relieve pain.

My Pain Psychol. is writing the letter of reccomendation on
my behalf but Ann Corley from APF (American Pain Foundation
- Power over Pain Action Network) emailed me mid-week to say she read over my stuff and was thrilled to have me come
on board and be a part. She will call me after she gets the letter so we can get any quests. from each other answered but then I can hop on board anytime I like. So I guess I am now a part of the APF-POPAN team. Very cool! I am excited to jump in and hopefully somehow make a difference for others and learn anything I can to take back to our MPS families.

Today we moved my oldest brother from Port Washington to Sheboygan - I got to see the not so little kittens! This is actually the first time I saw bella and demon (now foxy and jr) since they left me, and their Momma Cat! I wish I would have remembered my camera to get pics.

Keep our families in your prayers, each is facing a difference struggle and each day is definetly a different day to be faced. With God ALL things are possible I do believe though.

Night,

Erica

Wednesday, January 21, 2009 1:21 AM CST

I am actually writing a new entry sooner than later, believe it or not! I was really looking fwd to todays Inaugaration and the new Obama Presidency and I ended up catching only bits and pieces on NPR between Appts! Go figure!! :) I will catch it off of CNN.com or youtube. I just think beyond the significance of today, beyond the historic nature of today I am just looking fwd to having a new President and hopwfully in time HOPE for our America!

Yesterday I had a couple Appts but 1st back up to over the weekend and getting a lack of sleep due to just not being able to fall asleep, not feeling the best and having things
I wanted to get up and do. (like hang out with Chuck) :) It was a nice weekend somewhat calm and other than being w/Chuck and hanging out with my family for a couple hour sunday night I didnt do much things just always seemed to fall in the morning like church, etc. .. So yesterday I saw my PT, Ele and she did heated ultrasound and massage in
the area of my c-spine surgery which felt really good bc of the tightness and resulting soreness. I was so exhausted I ended up falling asleep! I'm not always the best company!! After that Appt I went and saw my Pain dr and she has made a few small changes in the pain meds including switching backlofen to a muscle relaxer called Skelaxin. We do think the increased pain is from being out of the neck brace and teh tightness of the muscles in both my c-spine and the thoracic area. I had mentioned to her how I was getting involved with APF (american pain foudnation) and how I hoped to bring what i've experiences and learned to APF but also take away what I learn from APF to help our MPS families. Well my pain dr told me she is involved with this group and works with them as a part of the pain organizations she does alot with and also told me she fully supported my doing this, and if I ever needed any advice or help I should come to her she would be very happy to help me and back me up. Very cool!! I am excited about being involved with APF and the potentials to learn and to help others but it is nice to know that someone like
my own specialist whom is very well informed and very smart is willing to help me if I need it!

Today I saw the Hand surgeon and they are a littel puzzled what the next step should be so to give us perhaps a little
le-way he opted to try an injection of prednisone (steroid)
and anti-inflammatory in to the carpal tunnel space and into the joint of my fingers. At first I was like I didnt know if I had just made it worse but now the numbness from the injection has worn off, it can apprently take a week or even longer to see results if there are going to be any.
In the mean time I am holding off on the other hand. He seems like a really nice dr, nice humor. There is still the
very real possiblity that this whole hand problem is related to my whole spine issues either the cervical or even the area below that which have issues causing this and apparently if I don't norice any difference than this may indicate that the problem actually lies in my spine and not carpal tunnel. Interestingly the EMG's my Neurologist did showed CTS and nerve damage but again.... and it is even more complicated bc i've had carpal tunnel releases over 6 years ago.

I also saw my Internists NP (they sort of tag-team me) about the heart burn and GI stuff she agrees with my Internist that Studies need to be done and is talking to her about what exactly they should do and she'll then call/get ahold of me and order the tests. One thing she wanted she said was Bronchoscopy which is where they place a tube down your throat to visualize your upper self. Fabulous. But I guess we need to figure this all out. I also talked to her about the side pain (right) and how it has been going on for atleast a year but has defiently worsened to the point of often severl times a day and still the only thing that helps is pressing on my side where the tubing is. She decided to order an X-ray to see if this might show anything and thinks they need to look into it further - i've let me neurologist know and I do see her as it is on the 2/4.

I'll keep everyone updated.
God Bless,

Erica

Wednesday, January 14, 2009 1:39 AM CST

I must apologize to the followers of my blog for the long overdue entry it has been a particularly trying few weeks on the pain front both spine pain wise (nerve and muscle healing pain and thoracic spine issues for which I have and
which I had probs even before the c-spine surgery 2 months ago. Additionally although i'm not sure if it is a sort of refered pain or if it is from carpal tunnel (EMG testing does show CTS and severe nerve damage) but my Neurologist did not know at the time she was doing it if this was from past carpal tunnel surgery damage, from new CTS or from nerve damage from the just decompressed cspine. That testing was done a little less than a month ago and I have
an Appt with the Ortho-Hand surgeon whom is also at Froedert Hosp. This Appt is next Tues., I originally had it
w/the Hand surgery clinic 2 weeks ago but had to r/sched. So anyways that is the long and short of why I havent done a new entry since Christmas. My apologies!

This pain is really kind of different than pre-surgery in some ways and I guess I just need to wait and see my spine surgeon in MN the end of the month (3 month) and Hand surgeon next week before I try this pain med decrease. My pain dr has been fine w/my staying at the doseages I was on
so I just need to give my body the time. I am very tired of
laying on my back though!!! (this is one thing that kind of takes away the pain when I am feeling especially bad or don't want to take any more extra. It's sooo slow and frustrating and in some ways or many ways maybe feels alot like 3 steps backwords for 1 step forwards.

This week I have Appts with my Neurologist at FMLH (f/up) and my Internist (some fup they are wanting to do ie my Internist and the NP) Next week should just be my Pain dr (I see her every 2-3 weeks) and the Hand surgeon/clinic. Other specialists I need to schedule with include the Opthalmol. in MN for 1 year Appt and Neurol. there or call his office atleast.

I'm sure there are things I am forgetting but in other news
I have been talking to the American Pain Foundation Midwest
Power over Pain Action Network (APF-POPAN) Leader about becoming a volunteer there and we have come up with a couple different possible areas. For now I am going to likeally do work as one of their WI patient volunteers and
this summer this Leader asked that I consider attending their new Volunteer Leadership training to consider becoming a Leader of the volunteer activities. Right now I really was interested in becoming involved with them bc of the interesting programs they have going on to reach patients in pain and the info I hear from my Pain dr about her involvement in the Pain found(s) she is involved with ie I want to know as much more as I can about pain as a whole to give back to our MPS families bc I know how woefully inadequate many pain specialists and doctors (mine is very wonderful I should add) make pain treatments
for our kids and adults. So just a way I can be a more empowered patient and part of my own team and help others as well. :)

Time to leave off or my hands will fall off i'm afraid.
Thank God for the miracle of another New Year and for the miracle of life. Though I may not understand why I have to deal with and live with this MPS and it's associated pain I
will try to the best of my ability to live a good and gentle life.

Happy 2009,

Erica
APF

Thursday, December 25, 2008

1. In those days Caesar Augustus issued a decree that a census should be taken of the entire Roman world.

2. (This was the first census that took place while Quirinius was governor of Syria.)

3. And everyone went to his own town to register.

4. So Joseph also went up from the town of Nazareth in Galilee to Judea, to Bethlehem the town of David, because he belonged to the house and line of David.

5. He went there to register with Mary, who was pledged to be married to him and was expecting a child.

6. While they were there, the time came for the baby to be born,

7. and she gave birth to her firstborn, a son. She wrapped him in cloths and placed him in a manger, because there was no room for them in the inn.

8. And there were shepherds living out in the fields nearby, keeping watch over their flocks at night.

9. An angel of the Lord appeared to them, and the glory of the Lord shone around them, and they were terrified.

10. But the angel said to them, "Do not be afraid. I bring you good news of great joy that will be for all the people.

11. Today in the town of David a Savior has been born to you; he is Christ [1] the Lord.

12. This will be a sign to you: You will find a baby wrapped in cloths and lying in a manger."

13. Suddenly a great company of the heavenly host appeared with the angel, praising God and saying,

14. "Glory to God in the highest, and on earth peace to men on whom his favor rests."

15. When the angels had left them and gone into heaven, the shepherds said to one another, "Let's go to Bethlehem and see this thing that has happened, which the Lord has told us about."

16. So they hurried off and found Mary and Joseph, and the baby, who was lying in the manger.

17. When they had seen him, they spread the word concerning what had been told them about this child,

18. and all who heard it were amazed at what the shepherds said to them.

19. But Mary treasured up all these things and pondered them in her heart.

20. The shepherds returned, glorifying and praising God for all the things they had heard and seen, which were just as they had been told.

^ Luke Chapter 2-20 ^

I wanted to share this verse from the Christian NIV Bible. This has been a passage for which I treasure and for which I remember the words to going all the way back to Grade school and the Christmas recitals we practiced and practiced for each year and which took place on Christmas Eve either at teh 5 or 7:00 pm services. By the time you as a student reached 8th grade this entire versus where recited by Grade K-8 and 8th grade reciting the entirity.

This has been a quiet week with a few small bumps which I just grind through
and of which no Appts and no ERT where
had. This next week if I can actually get a family member to take me I have Appts monday which I am scheduling over to Tuesz. with Ortho-Hand surgeon,
Pain dr., Internist and possibly other pain provider. Also have to try to get in PT somewheres. I am continuing to heal day by day, week to week and really by accident have went off of and down on a couple meds. If my Pain dr will let me stay off of it I am not taking the muscle relaxer (Baclofen )anymore..I just don't know if they will
want me to take it yet cause of the other spine things. Also am down to just 1 (2pills) long acting morphine and generally 0-1 doses of the short acting morphine a day. I am not taking the Topamax right now either, although not sure what I will do when I do get migraines - maybe just a medication dose when I have one instead of a dose every day. The midodrine is seeming to go fine except of course I still havent
managed to fit in the 3rd of 3 doses in
a day! There are some other med changes
but won't get into it all.. just suffice to say I am healing and think I
am doing waaaay(!) better aat this point 7 weeks out from surgery than I was doing before surgery! And I am so thrilled for that! I do hope that at the end of the healing from neck surgery I will be totally pain free on
that front. I of course know there are other things which can/will bring on pain but am so happy to be finding relief. If I could actually get to sleep at night I might even not be tired as the days I do get "a normal amount" of sleep I feel energetic and that is something I just have not felt in a very long time!!!
We celebrate X-mas with my parents and siblings on Christmas Eve morning but due to baaadd roads my 2 older brothers, SIL and neices where not able
to come up. So due to not having Quinn and Zander today and my 1 older brother
and SIL not being able to come up today either bc of X-mas on SIL side of
family. Well so we just opened up gifts from my parents, those Secret Santa name exchange siblings that where
there (Sara, Aaron and I and Chris whom is Sara's BF) and gifts from all of us kids to my parents. I received a surprise couple of lovely gifts as well
from Sara (shirt, frame for our 'Sister
pic) and I also received from my Nephew
and his Mom a lovely gift. My parents bought be the 'light' upright 'stick' vaacum I had been looking at (much easier I think to vaacum now with this than my regular vaacum which is heavy! I cannot wait to use this vaacum on my carpaets and especially each day on the cats litter they insist on getting
all over!!!) I also received some very
cute glass 'knick-nack' decs., teh Brita water pitcher and insert and many other things. I am so blessed to have the best family truelly. I had so much fun today playing card games with siblings, my Dad and family and taking videos as well as on Sat. we will go to my Grandpa's on Dad's side and celebrate the Holiday and being together. I truelly, truelly am blessed
to have this family and to have my parents and siblings who are willing to
stand by me and to help me out so much.

May you remember the childhood Joy of the past and may you always look to the
future and to what it holds.
God Bless and Merry Christmas,

Erica

Tuesday, December 16, 2008 11:10 PM CST

Just a brief update or atleast somewhat of an update. :)
This past weekend was pretty crazy - every year we decorate dozens and dozens of cookies, my Mom, sister and I. Well this year I think we went a little over board - i'll post pics when I get my laptop back hopefully Weds. but between Sara and I decorating and cutting out and also my 6 yr old nephew and Sis's BF's son we where at the cookies for well over 4 hours I think it ended up being! I have to say I am not a huge cookie fan anyways and now especially not !!! but it was fun. That night Sara asked if Quinn and I wanted to come spend the night by her, so Jordan and Quinn could have a sleep over and then sunday we
went shopping for hours which my legs definetly felt it to!
(the shoes I was wearing) ...

Today I had a couple Appts in Milw which included the cervical spine x-rays which i'll send off over night to my Surgeon in Minneapolis and also an EMG which was partially done by my Neurologists and which showed damage to the nerves but she really doesnt have a way to know if it is re-current Carpal tunnel or if it is caused from the spine disease - she is sending me to the Hand center at FMLH her thought was they may be able to do injection(s) in my hand(s) to see if the hand pain gets better and if it does we generally know it's re-occuring CTS and if it doesnt we know the damage is further up in my spine and will just take time. Overall for the most part the past week has been better than any other week pain wise although it ebbs and flows, sometimes it's great and soemtimes it's not. One
contributing factor among many things is how I sleep as there seems to be only 1 way I can lay and 1 way I can sleep now w/out causing pain. (The problem is it's not easy figuring out what that way is somedays I just am lucky to wake up having slept 'ok' and sometimes not. Hmmm.

I am very much hoping to be able to go back to driving after I get these results, relying on someone else for every thing and for getting to Appts is incredibly hard not
to mention it's hard enough making 2 schedules work add in a 3rd and it's virtually a nightmare... this has defiently
been a bit of a stressful to ut it mildly recovery.

Try to update again soon.
Remember the reason for this Season.. Celebrate the Birth of Christ with family, friends and loved ones... God Bless,

Erica

Sunday, December 7, 2008

Just a quick update to say that instead
of going up to Twin Cities tomorrow (thurs) to see my Spine surgeon for the
6 week Fup I worked with his PA and Secretary and my Neurologist here in Milw. and am having the orders for X-rays sent directly to my Neurol. who will order them, and give the Radiol. a heads up. I'll have these on Tues. and then the films and report will be sent to my surgeon at TCSC who will review everything and decide both if I can start to wean out of the neck brace
and if everything is going okay including this films will show if theres a/t up with that 'bump' which is under the incision. I'll update a new journal when I know more.

Sunday, December 7, 2008
Hello again.
I just thought I would add an update to something I said in the entry below about my neurol and about my Cardiol.
I talked to my Neurologists office friday morning and my dr. has ordered an EMG of which she is doing on the 16th - a week from this Tues. to see if that gives us any insight. I also talked to my Cardiologists office (see below entry for more) and he is in fact ordered the Midodrine which I start probably monday 3x's a day. This is a tricky drug given it raises BP and
how it has to be taken 3 hours before laying down ... and if BP gets to high this puts increased strain on the heart and the valves which are already under strain, the purpose though is to raise the BP to get circulatio through the heart and to the brain increased w/out that side effect! Well with MPS I spend alot of time atleast right now laying/sitting and when I am not recovering I don't always know when i'll lay down. Given i'll take it 3 x's a day and having to be careful of some of these issues like with lying down blood pressure through teh heart can become dangerously high.. so it will be interesting to see how it works and my body handles it!! Hopefully well! I'll go back and see my Cardiologist again January 6th and I also see my Neurol. in Jan. in
clinic. I will with the
Midodrine have to keep atleast weekly BP's but that I can have done at ERT and as well at any of Appts.- easy enough.

Happy Holidays, may you always remember the reason for these Holidays as we Celebrate the Birth of Christ at Chriatmas,

ET

Friday, December 5, 2008 2:40 AM

Well I guess this is just a wee bit over due but only a week or so... :) updates, updates, updates.... things have been quiet most of this week until today anyways and the wonderful time with my siblings and parents on Thanksgiving all of us together except for my 6 yr old nephew ( :( ) whom was with his Mom. The day was lovely and as these days
go with my siblnigs very funny and fun! We've exchaned our
names among us siblings and b/f's, g/f's and my SIL. This year I have my sisters boyfriend Chris whom I have gotten to spend just a great amount of time with through the year as he held my sister up, as he helped with my nephew and as his so played with my other 6yr old nephew. So he'll be fun to have! Both he and my Sis' are going through evil divorces but they shouldnt be looked down on by anyone bc the support they give each other is just wonderful. Chris is the truelly most amazing guy you could imagine and perfect, perfect, perfect for my Sis. Her Ex has been nothing but down right nasty through the divorce and just takes Zander my nephew whenever he wants and doesnt call my sister, won't be at home or won't drop Zan. off when he is supposed to. To prove this point just the other day no lie while he and my sis where both on the phone with Zander's guardian ad' lidem (in these sort of non agreed cases one is appointed to look at Zander's best interests t/out bc no agreement has been reached. Anyways my Ex-Brother in Law was cussing at my sister right on the phone with this guardian. Ugh.

Recovery is sooo painfully slow, the 1st weeks where so much 'quicker' and now is just the slow part.
I can go around and do things and go out if I am wise on my energy-effort into outings but the pain in my neck and more intensely in my hands is just terrible especially towards the end of the days and standing seems better than sitting. ... I am writing this cringing at the pain. I email back and forth with my neurol in Milw and she talked about doing an EMG- I don't know if she will but it is not wonderful!
There is only one way I can lay to partially relieve it - right now I am laying down with the laptop balenced up on my knees to do this. I just want a break from PAIN!!!!! I wish so badly it would go away and soon. If it is carpal tunnel that is worsened I will get it re-done otherwise it's just my spine and it will either take time (my surgeon said it can take up to a year)
or who knows what the cause is. I do know I just absolutely cannot imagine the entier next year even the next months or more like this. =( I just needed to talk about this for a bit as alot of my 'viewers' are my friends in MPS and you get it like no one else can...

Rather that re-type what my Neurologist emailed me I will copy and paste it here and not put her name for her sake but for this one instance since it is a generic sort of note I will post part of it...... I do not think she would mind. :
"just spoke with Dr. E. directly, finally.

We have a plan. Would like to start low dose midodrine to increase the blood pressure and see if drop attacks stop. His office would like to coordinate this and be sure your home monitor for checking blood pressure is accurate. If that doesn't work I would like you to consider another hospital stay when I am on service in late December and we would try to taper medications one at a time that could be contributing. A so-called 'drug holiday' to try to clear the slate and see what is happening."

I also had a missed call and Voice Mail from my cardiologist himself telling me a little of this and asking me to call him back tomorrow. (today-fri) I will update after or sometime this weekend
more on this.

Otherwise I thought it funny listening to one of my favorite Politico shows - I am generally all for Gov't for
our Nation - but this mess our current President has us is and then for him to go on all these talk shows, NBC and ABC, etc and he talks about his 'other'
'new' retirement home (hmm Mr. Pres. does that make it 3 homes now???) yet others have no home or are losing theres??) he talks happy as a lamb about this new home he just bought - what a joke and what a joke this presidency has been - I will be thee happiest person when Jan 20th Inaugaration comes and Bush is soo long
gone along with his croonies. One these shows he talks about how he is happy he will not have to get up and worry about the economy, the wars and so on and ooooh yes He's Having FUN! Wonderful, great fun! Gee Mr.Prez you really don't have a clue do you? All for your buddies and let those whom truelly need the help go under. That man should be forced to go out into the 'real' world and volunteer w/the homeless or with charity's such as food pantries to see what has and is happening and he celebrates
himself. Sooo typical of his style.

I just had to post this basically as a way to say be grateful for what you have but never forget the big picture and take responsiblity for your actions - it is nobody's fault but your own when things are bad. Yes of course some things are out of our control but I mean the little things, the little blessings that make life what it is. And please do not be offended by my rant!

God Bless my friends,

Erica

Monday, November 17, 2008 7:55 PM CST

11/19 this is deeply person al to me and I am writing this honestly more in hopes of a cathartic effect. Physically I am doing ok with the recovery I think, other than not being able to do things on my own such as (and yes these are selfish)
style my hair, and drive, be at my Apt
and the list goes on) The actual recovery of my body and neck I think is
going well I am learning to not do to much and for that it is extremely hard for me. I am learning to try and take it easy, and I well I will let you know whate else. But emotionally I am having a hard time dealing with this - not the surgery that was fine but the actual having to give it time to recover. It is only 2 weeks out and already I am bored upon bored, I am not doing well with not being able to drive myself whenever I need to go someplace and I am just overall tired (in the emotional sense) of not just being able to go see a friend when I want or run to the store whenever I need, and not being able to take the time I need to do things but instead having to require someone else being willing to take me and that person either coming back to get me or doing their own shopping. I am just an incredibly independent person and so tired of how this year has been the ups and downs, ups and downs I think have finally worn me out. the surgeries, the hospital stays and so much relying on other people on so many avenues I am just spent. I just can't wait for this new year to get here and I pray so hard it isnt what this year was I can and hope to do without any more hospital stays and surgeries for a very long time. I just need time to recoup and just as much more than a few months without having to stay witho someone else for weeks and in this case even longer but instead time to spend atleast say a year or so or forever at my Aprt and i'll come and visit instead of staying.
I feel like i'm all grown up and yet I still can't ever stay at my Aprt full-time. Yes I know it has been months since the last time I had to stay somewhere other than my Aprt but I would like to spend every night there. It is just incredibly hard to convey my feelings - it's not that I don't loev my family I do very, very much it's just that I need my space and I need to be able to get up in the middle of the night when I want and to cook when I want and it's just so many things. I guess all I can say is I just need to write please don't judge me.

11/18 - I also forgot to say I uploaded new pictures most from the actual days I was in the Hospital. Amazing how 'drunk' a person can look in looking back without actually remembering it! Also the incisional swelling has went down and the incision
although well healed definetly does not look as good as it does in that 1 pic I have amongst these - now it looks more like a bony-mountain range of hills and valleys!

I just had a long, long update written which has taken me nearly 30mins and reaching over to read something on an article I wanted to post here I deleted it! =( So I will start again and am pretty sure I will end up re-entering some of the things I wrote before tomorrow or later in just editing this entry I am currently writing now.

I saw my Internal Med dr today for a fup sfter surgery and also her NP Laurie stopped in not bc she was scheduled to with me but just bc she wanted to say 'Hi' and offer some help with the issues going on. So very sweet of her! My Internist wrote a letter on my behalf regarding the Madison ERT 'push' still going on by CHW. She also is helping me as is Laurie for that matter with the Swallowing stuff that has been going on for some months and for which continues on and off. She suspects it is related to the other Autonomic dysfunction stuff and is going to send me to GI docs at FMLH (her hosptial right next door to CHW for those not from WI! who wonder) Bc of the current Miami-J collar I am wearing up to 3 months out from surgery she is going to wait until then to get me in with these drs for testing. The reason being is they would not be able to do the swallow study/camrea studies to figure out what is going on until atleast after this is off. She also gave me all of the recent medical reports she has received today which was nice - I always think it is funny to when you receive medical reports from those providers who don't either dictate in front of you or whom don't tell you even the littlest of details on your testing and then you read a medical report of theirs and read something that although might not seem important to them is important to you, to explain something going on within you. I understand that specialists and my providers do not always have the time to explain every detail I just guess I wish providers shared the little things if even just to give me their medical reports as some of my providers do.
To go back just a bit on a prior thoguht above I wanted to share a statement my primary Internal dr wrote in the letter she wrote on my behalf. Keep in mind there is writing from her in this letter above adn below this one statement but she wrote: 'She has consolidated her medical care to the Milwaukee community and has helped to facilitate open communication amongst all of her providers". I always appreciate these providers of mine who consider me a part of the team and consider me when actually making decisions. It is a little amazing to think how much hard work and 'trial and error' it has taken on my part and behalf to get to the actual point I am at today. Not just the last year (but I have definetly found a host of really great providers this year alone who have come on board) but the last 4 years of working with and looking for providers and and specialists has really brought me a pretty great team of drs and providers whom I consider a part of my team. It's actually amazing.

So another reason for actually coming to write this entry tonight was I wanted to share some info I hope might help other families going through cervical spine surgery and I also find the info interesting. This is just some info I new but didnt know the exact details of (bc exact details werent and couldnt be known until after surgery) I received in the records from my Internist today the operative reports from my main surgeon, as well as from the Surgeon-colleague whom he asked to participate (these 2 work in tandem on all past MPS surgeries I found out to!) these 2 reports also included the 3rd Surgeon Kate whom is Dr.Schwender's Assistant.

The imaging they did before hand showed there was in fact just as my Surgeon suspected Compression on the spinal cord especially at levels C2, C3 and C4. I first want to say that the Mayfield tongs that surgeons use - I assume most if not all Ortho and neuro surgeon use these take atleast a few weeks and longer for the 'pain' and numbness to go away. This can be uncomfortable more so I suspect for female patients bc of our hair?! Anyways this may seem a little thing but for families children this can be unsettling and uncomfortable. Another interesting, although probably useless fact, I personally would think Medtronic was for Cardiac and even Hydrocephalus implant devices but they also have devices (in my case the Medtronic Laminoplasty screw set and Medtronic Sofamer Danek laminoplasty plates) for the spines. Much more Diverse company than I ever thought. So anyways although I don't know much about what exactly tehse are except for knowing their purpose Lipped plates where used on C3 and C4 levels and non-lipped plates on C2 of which where fixed with screws laterally.
Apparently also bony fragments where used atleast on C2 for fixation. And last this amazes me from what I can tell in the reports 9 (!) screws total now reside in my c-spine and where used to hold the titanium plating (instrumentation) in place which stabilizes my soinous process/spinal vertebrae bc of their being reconstructed in the new 'form'. Very interesting and kinda cool - maybe I only think so bc having gone through it I think you should find something to learn about each surgery and procedure you have and find 'sight' into knowledge. And I think it is amazing what is all really in my spine if you think about it - the Lumbar-peritoneal Shunt for the Commun. Hydro and then all this hardware in my neck.

I will update the rest tomorrow or try soon!
May God Bless and keep you and your family close this Holiday Season,

Erica

Monday, November 10, 2008 6:33 PM CST

I just wanted to let everyone know know I am home from the Hospital and also home from MN. I also wanted to apologize as part of this is a aprt of an email I sent to many friends and providers.
- I was able to get out on Friday after havimng the surgery on Monday 3rd which with having 3 surgeons lasted about 3-4 hours. Not bad, I am glad I had those 3 surgeons! Also I think Anesthesia went really well for the first time in honestly the last 7 surgeries, my airway is still very narrow they just knew what to use for an MPS pt.

I did get another piece of good news, from Dr.Charnas my MPS Neurologist at MN who stopped by my room to
say Hi and see how I was doing how surgery went/what they did. He is working it out with UofMN to come back from Shire Phrma once a month to see pts as their
MPS neurologist. I am very happy about this!!

My surgeon I kid you not was pretty near excstatitc on how the surgery went - he did do the decompression but instead from levels 2-5 (vs 2-6) which was a good
enough thing. I did have to lay flat for a day or so after surgery until they
could figure out an neck brace that would work - they thought about doing a soft collar for nights but non from size x-s up to med. worked on my neck bc of it
being so short. So then they tried 4 different hard collar types including Philadelphia and finally the miami-j which orthotics ended up with sn x-small size. I also was srill pretty swollen and am still in my airway some what. I started OT and PT in very short segments (15mins) 2 days after and they worked with walking, balance, and a grabber for at home. PT also fitted me with a Cane bc of not being ablt to look down and so I walk with that when up and especially at night and when outdoors.

Dr.Schwender did tell my parents and then me the next day that the compression was at all 4 levels aand definetly needed to be taken care of as the risk of permanent damage was very high had we waited, the imaging just did not show the extent of it. At this point it should be reversed though and once swelling goes
down even better.
I am having alot of difficulty breathing and even more so swallowing both I suppose from the swelling and pressure on my spine now due to the plates and screws adjusting in that they used during surgery after the decompression and laminoplasty where done to stabilize my spine. I hope the breathing settles down!!
Pain control now is actually basically only at my incision and spinal cord bone and managed somewhat but definetly not the best by the Avinza, MSIR, and Neuropathic meds like Lyrica, cymbalta, etc. and then Baclofen and they added Valium for spasms which if I take it at all I take jsut 1/2 a dose. And Dilauded also a narcortic.
Really the pain is from where they screwed teh bolts in to the sides of my skull during the surgery (didnt feel pain at first, weird!) but now eeek and is makes
it hard to have anyoe wash my haiir or pull it up out of the way of the collar (I normally wear it up as most know anyways!) I also cannot really feel the sides of my head also I guess from those screws being in during surgery.
I do have a cane to help walk with since I cannot walk klooking at my feet or see much right in front under me and also a reacher/grabber to help pick things up. It's still hard to rely on people!

I apologize fir the bad typo's it is hard to see the screen and have some double vision going on. I hope/wish i'd see you this week at ERT!!!!????? If you have any thoughts or reccomendations for comfort please let me know!!
God Bless and Thank You So MUCH for ALL the prayers - they Worked!!!!
Erica

Friday, October 24, 2008 2:15 AM CDT

I wanted to post a new journal although I don't know alot that is new. I'm going to this weekend first and then will go back to the past week. My 6 year old nephew Quinn is coming friday, I will pick him up on my way home from ERT and he is staying for the weekend. Trick or treating is Sunday so I imagine I will be out doing that for a while it
is 2 hours in my city and 2 hours where my parents live, I
am not certain where Quinn and I will go yet - I am sure though that I will despise the cold and be sooo tired after! Ooh well even if I don't feel good still good exercise and I can always trade off after a while with one of my parents or Quinn's Dad. .. So back to late last week I saw my Neurol. on Tues which I wrote about in the last entry and then last Friday bc there had been some communic. with my Cardiol. and a couple things she called me on Friday returning an email ques. AWe are going to hold
off on the med. to raise my BP till we get a snapshot so to
speak of what is going on with my BP during the blacking out episodes. She has written a script so that I can get one covered by one of my Insurances and I also am at the moment doing a Holter monitor for my Cardiol. as he wants to see what my heart rhythm is over 24 hrs. I will see him next Thurs. i think in the past 5 years I have seriously kept the heart rhythm machine companies in business! Anyways though that is where it all stands with that, we might if we're lucky get it figured out and a decision made
before I have the spine surgery but we'll see! It's taken
since march so whats the rush now.. well except blacking out after the surgery and causing harm to the newly worked on vertebra and spine maybe.... but then again as said before it may be the spine and shunt causing all of this blacking out stuff due to an autonomic effect definetly as my Neurologist says no simple answer and a very complicated situation all encompassing. Atleast she has kept pushing along seeking out different avenues for possible answers, some I have and or have had in the past for specialists (definetly not any of my immediate very close providers but..) would have blown it off. But i'll keep posted!

I saw my Audiologist finally this week, my fault purely on
that end I simply see and have so many specialists and this
year has been well a little bit difficult for 'organizing'
bc of SOO many different things going on that I put off what things I didnt absolutely need. So she had ordered a new hearing aid for the one I lost and I picked it up, bright new and shiny, hahah for a grand smacking nice total
deductible payment (which I now owe to the "Bank of Dad', which I don't mind I am always borrowing from my Dad and repaying and then re-borrowing and repaying for this and that it just well he lets me bc I don't have lots of choices for soem things). Such is the great, grand fun of MPS. Hah, Not! There is a small chance I could get the deductible covered by one of my Insurances if I choose to try (yey to that fight) but I don;t know if I can deal with
hours on the phone when I already spend so much time on it
for that sorta stuff.... I barely ever even call my distance friends anymore bc I despise the phone so much! I tend to email or send a short text but someday will call my
friends - I think people, family included think I ignore my
phone! Most of the time I just have it tuned out! =;)

10 days/11 days to surgery and to Elections - I ate supper at my parents tonight and was updating them on all i've heard election wise and we got to talking about who we're voting for and my Dad was like I knew 6 months ago who I was voting for, I just am so ready for this to all be done. I was like I love the excitement around it, and can't
wait to find out, I try to follow it all closely and cannot
wait until next Elections come up (not bc of speeding up time) bc I want to get involved in an actual campaign volunteering with getting out the vote effort, etc. I have
been so disapointed this 'season' bc it is so 'exciting' to
me I love all things political but I just have been so aweful feeling physical wise I literally am in so much pain
when I am up for a long time and so exhausted it sucks! But I have HOPE and BELIEVE that that in time this surgery will in time help my body to recover most of the neck, upper back pain and the nerve pain as well as the fatigue I really think I will get back to the point where I was before this back pain really got so out of control. And I think these Headaches will lessen if not completely go away. I truelly think and belive and pray for this. And I know that I have many of a special providers wishing the same things for me, I have gotten atleast 4 emails from providers wishing me a speedy recovery and saying they where thinknig about and praying for me. And I know as well
that my friends and family and best of all my Lord and Savior are standing with me through this upcoming trial. What more can I or anyone possibly ask for?

I have several friends whose children are going through similar things (well many really but these couple, their kids are under going testing to prepare for their spien surgeries) and I ask you to pray for them as well. Although
i've met Melanie and Jake just this past June at the B.C. meeting and not met Robin and Logan they are family to me. We are all friends in MPS.
God Bless,

Erica
will be alot less tired and be

Thursday, October 16, 2008 10:13 PM CDT

Please see the journal entries for the entry from Weds. (yesterday) I don't normally write two entries so close together!

I actually wanted to write a littlle (alot maybe?) about what is exactly is happening to my spine/spinal cord and what this means. 1st though I wanted to update about s/t else I wrote about below. My neurol I think I wrote below is interested in trying a medication to
increase my BP due to this low blood pressure being the possible cause of my
blacking out episodes at night. 1st though she wanted to talk to my Cardiol. about the beta blocker med and the dose I take. Well so I traded a
few emails to w/my cardiol and at 1st he was okay with going off this med altogether but now has decided he wants
to have me come in and wants to do a repeat Echo (ultrasound of the heart) before deciding what to do I guess. He did say he was okay with my Neurol. trying this med for BP though. I will keep updates here on this all. Given that in just the last week I have fallen probably 4 of the 7 nights and many of those it happens repeatedly and I have I am pretty sure done something to a couple fingers I just want to get to the bottom of it! Given that I didnt have any of these episodes
while I was in the hospital for the 5 days a month ago, I am just thankful my
Neurol. is still trying to pursue an answer and help me. My worry is often that providers just won't believe what I am telling them when things arent clear cut like this for instance. There is the possibility though with this blackingf out episodes that they will completely stop after I have the surgery, as another concern my drs have had is with the spinal cord stenosis and compression in my c-spine and then my Shunt for the Hydrocephalus being below this area in my Lumbar spine are these 2 things basically working against each other and causing a 'dynamic' situation as my Neurol. puts it and causing autonomic issues. Whatever the case I guess we will find out soon enough, I just will be happy to be on the road to healing soon and hopefully not be passing out to, would be a double bonus if both occurs so close together! (ie blacking out/med and then surgery)

So to give the 'educated' background on what exactly is going on with my spinal cord and body secondary to the MPS and the c-spine compression. As most here know the stenosis and compression are caused from the mucopolysaccharide (aka GAG) storage building up in the spinal cord and on the bone causing the thickening of the spinal cord and bone which leads to little to no space for the spinal cord (CSF) fluid not being able to get through and thus compression and damage to the cord and bony ligaments. (hope that is all said right, feel free to correct me - I know what is gonig on but it is hard for me to put it into words!) This compression of the spinal cord then leads to headaches, weakness, and neuropathic pain in both upper and lower extremities as well as abnormal reflexes and so on. Some of the things I have include myelopathy (damage to the spinal cord and that listed above so I thought I would give the educated look at what some of these things are doing/why they are occuring. Since
I want to know everything that is going on w/my body and why I thought everyone else could have a bit of an Anatomy lesson to!

Myelopathy: is a term that means that there is something wrong with the spinal cord itself. This is usually a later stage of cervical spine disease, and is often first detected as difficulty walking due to generalized weakness or problems with balance and coordination. This type of process occurs most commonly in the elderly, who can have many reasons for having trouble walking or problems with gait and balance. However, one of the more worrisome reasons that these symptoms are occurring is that bone spurs and other degenerative changes in the cervical spine are squeezing the spinal cord. Myelopathy affects the entire spinal cord, and is very different from isolated points of pressure on the individual nerve roots.
The stenosis/compression begins to press on the spinal cord and the nerve roots, and that pressure starts to interfere with how the nerves function normally.

Clonus: Clonus is repetitive, rhythmic contractions of a muscle when attempting to hold it in a stretched state. It is a strong, deep tendon reflex that occurs when the central nervous system fails to inhibit it. Clonus is not the same thing as myoclonus, which is irregular and uncontrollable jerks of a muscle or group of muscles.

Babinski's reflex occurs when the great toe flexes toward the top of the foot and the other toes fan out after the sole of the foot has been firmly stroked.
Reflexes are specific, predictable, involuntary responses to a particular type of stimulation.

Lhermitte's sign: An electiric-shock like sensation shotting down the spine when flexing the head forward.

Hoffman's: A positive Hoffman's is the involuntary flexing of the end of the thumb and index finger - normally, there should be no reflex response.

Tandem gait: any difficulty in walking heel to toe

Scissoring - knees and thighs hitting or crossing in a scissors-like movement when walking. Everyone who walks next to me always say I walk like a drunken sailor and am always unintentionally walking into the path of whoever is walking right beside me!

There are other things I could post what they mean and other areas which the 'test' have been abnormal related to neuro exam. Basically I just hope this can help anyone else
going through spine issues to - MPS is certainly knows no boundaries! (sp?)

Thursday, October 16, 2008 8:43 PM CDT

Weds Oct. 15, 2008Hello all, 19 days to
surgery/19 days to Election
I would like to update on a couple Appts. this week with my
pain dr and with my Neurologist both of whom are in Milw. I first would like to write my thoughts on something I found out over the weekend from a friend. For teh past 2 1/2 years I have saw a Neurologist at the UofMN whose specialty is metabolic related neurologic disorders. (I hope i just said that right) Anyways I found out that Dr.Charnas will be leaving for a research and non-patient care position in Nov. Back when I was having Headaches every day and no one here at home knew what to do anymore to diagnose what was causing them Dr.Charnas immediately recognized the signs of Hydrocephalus secondary to the MPS.
After 3 months of repeated LP's and repeated relief he fought to get a shunt to bring relief. A year later when it was found I had Spinal cord disease again related to the MPS he helped to get me into the Intrathecal Study - he
was there trying to get the best, least riskiest treatment for the c-spine disease. And fast fwd to now, when the IT study was no longer an option, my symptoms where increasing
and I needed surgery he was right there making sure I was able to get the best possible spine surgeon for my particular disorder. My reason for writing this all is simply to say that I really was pretty lucky to have his care and both myself and I believe many of my other providers will miss his knowledge and his willingness to help figure problems out. I am glad atleast that he will be
around the UofMN when I am there having my surgery I only hope he will be able to stop by after.

I am very glad I atleast am set up with a Neurologist as fantastic as my dr here in Milw is, I don't know honestly what I would have done if I had no neurologist other than Dr.Charnas, and then found out this late in the process of his leaving that I didnt have anyone for this care.

Monday I saw my pain dr and bc of the continuing blacking out episodes at night we are trying to cut down some of the
doses of medications or weed out even a few depending of course on how I can tolerate the changes given the issue of
high pain already present. This is something I truelly look
forward to being improved after the decompression and laminoplasty in Nov.

Yesterday (tues) I saw my Neurologist and she is trying to
also help in many areas, the biggest one of which the blacking out she is involving some of my other specialists
to come up with the best plan. She talked about 2 different
medications which might help given the very low Blood pressure I have and which they found during the admitted Video EEG falls even lower at night. She is talking to my Cardiologist about this and the beta blocker potentially being the cause. We will see what becomes of this, I do think she and some of my other providers are concerned about the blacking out and this happening especially after I have the surgery.

I will keep everyone updated.
There is more to write and to update but I am very tired and so will finish later.
Remember to vote Nov 4th!

Erica

Thursday, October 9, 2008 9:57 PM CDT

I made a relatively simple although very chaotic and sometimes frustrating day trip out of going to Twin Cities
(MN) today going and coming back in one fell swoop. I do have a bit more info and a much better understanding of what exactly will be done (cervical decompression with laminoplasty procedure) which like I posted in my last entry will remove bone and ligament compressing the spinal cord and in essence cut the Lamina in half to create an 'open door' effect on the various vertebra to create more room. These are held open by the titanium plates and screws. For the surgery which should (not including intubation) be around 3-4hours in which i'll be placed on my stomach flat and then a halo traction will hold me head in place with screws which are tighthly screwed into the head (without actually causing burr holes I believe) my surgeon did say on top of the other aches and pains these spots will be pretty sore after. After surgery I won't have this halo device but instead the miami j collar (3-4 months depending how the bone is healing) which while still uncomfortable is better than the halo. After surgery I should be able to get out of the hospital in 4 days wearing the collar at all times at first it sounds like. I think I sort of knew how long total recovery would take but am still shocked to actually hear my surgeon say the lenght! For the bone to heal it takes 3-4 months, and to regain some movement in my neck up to 6 months with full recovery could take as long as a year. Thankfully bc my surgeon is doing the Laminoplasty with the decompression I will still have most range of motion in my neck and should
only lose some but the length of recovery just to get even a little of the range of motion back initially is staggering! After surgery I will have to stay with someone and about the only things they want a person like me to do after this surgery is really rest ALOT (ugh) take it extremely easy (after 2-3 weeks I can lift a whole 5 pds - one of my cats ways waaaaay more than 5 pds alone not to mention nephews!) =( brush my teeth and ADL's gradualing building up to other things I guess like showering and so on). The prices we pay to feel better!! My surgeon said his
goal 1st for me is to not get any worse (see below for issues I already have) and if 2ndarily I improve symptoms that is great. My goal is to improve in any way from the way this C-spine stuff has affected how I feel! The last interesting thing is that most patients get off their pain meds completely within a couple weeks but bc I am on heavy duty opioids already ahead of surgery and have been on pretty high doses of these 2 meds for almost a year (comparatively high doses for my body weight I guess) it will take 4 weeks just to get back to my baseline meds I have been on (these opioids) and then down from there on these. Definetly interesting, but at the same time these meds and my pain dr have been truelly a God send as the pain is aweful enough with all these meds but without them just is unbearable and I don't know how I would have made it this far. I think that is all, I can hardly believe it's
just 2 weeks from this coming monday to surgery date! EEKS.

The symptoms causing me to want and to need this surgery are staggering I ambasically writing them here for other families with MPS/ML so you may know what to possibly look for. All told the symptoms have included Numbness, tingling and even occassional burning. (although the 3 different Neuromodul. meds I am on for neuropatchic pain have greatly improved that) There is weakness in my legs which has started in spring but is now noticeable even to me the past few months, and mild gait impairment.
Brisk reflexes, (hyperextensible I guess) aweful Headaches, some blurrier (sp) vision, fatigue beyond even what was there before, increased forgetfullness, neck and back pain like you couldnt even believe, and then there are
things like clonus, and neurologic signs.

I won't list risks here as they as well are staggering and no need to scare everyone off, I understand and accept them
which is what matters. The risks of not doing surgery are to high.

PS: bc I won't be home for elections i've already filled out my Absentee ballot - for everyone else Remember to VOTE Nov. 4th!

Yesterday I learned from my sister that her ex- MIL passed away, 2 days ago 10/7 although Sara and I no longer talk to most of her Ex's side of the family we always got updates either from his younger sister or from when I would run into their Dad at FMLH or elsewhere. Pam fought cancer from before my nephew Zander was born with 1 remission in between there until 2 days ago. If you ever needed a nicer person to meet Pam was the one, she always
asked adn cared how I was doing and Sara and I missed talking to her the most over these last 6 months of the divorce. She truelly lives on in a better place and I believe is an Angel now in Heavens wondrous gates. many prayers for Jerry and the family, although I no longer talk
to most of them I can't imagine losing my own Mom so young.
Pam was 56 years old I believe.

Yesterday heaven called home another special little MPS I child I spoke of ALyssa in the last journal entry and she lost her fight early last night. I know the Angels in Heaven are rejoicing but here on earth her family must go on. My prayers are with them as well.
(alyssaloudenfund.com)

God Bless and may you have a wonderful, peaceful day, while never forgetting the blessing of family and love.

Erica

Monday, September 29, 2008/Friday Oct. 3, 2008 11:15 PM CDT

I should note that the reason for this surgery is that because of the MPS I Scheie Syndrome dx. I have as a secondary dx Severe Stenosis and myelopathy (compression) in the Cervical spine. I do have spinal issues including disc bulge (lumbar and thoracic spine also) and stenosis elsewhere just not anywhere as severe.

As you probably know I will be having cervical spine surgery Nov. 3rd at 10:30 in MN at the Fairview (U of MN) Hospital. The reason for this surgery being in MN is that my spine surgeon Dr.Schwender an Orthopedist is specialized in MPS and spine surgeries and he will be working with Dr.Perra as far as I know. (a colleague of his due to the complexity of my case) My surgeon will be doing a decompression (removing ligament and bone that is compressing or putting pressure on the spinal cord) and then removing/cutting the lamina from C2-C5 and opening the lamina up (to create the effect of an 'open door' which is then held open with titanium plates and screws. This to create more room from the stenosis and compression. The Laminoplasty involves the small metal plates and screws to help keep the spinous process in the correct place.
I will then wear a hard brace (cervical collar aka miami j brace) for 3 months as the bone heals. I
go up to MN to Twin Cities for Pre-op next Thurs the 8th. My Anesthesiologist will be a Pediatric dr. and familiar with my case as my airway is very narrowed and every surgery of recent years has been very hard for even the pediatric drs using small tools to intubate. The Anesthesiologists at my last surgery in
May wrote down for my records and for my othr drs the 'grade' of my airway (1-4 w/4 being the worst and means least visualized in the airway and chords.) After that last surgery they told me they understood why so many drs including themselves have to try for quite a long period of time even when using bronchoscope or fiberoptics to intubate as my airway was at the very severest involvement and narrowing grade. Eeks!

Otherwise not to much is going on, it really has been very quiet and I do mean quiet! I am not sure what to say, very unusual I know. I will keep this updated.

I should post though that there is a bill before the represenatives in Congress and of which some good friends
of mine co-wrote and are getting sponsors for among congressional staff.
Well anyways they have asked me to write a letter to my District Rep and I will be passing this potential bill info on to my friends in Genzyme and at
the infusion clinic who are affected by Insur. difficulties. Basically the bill seeks to get approval for patients
on orphan drugs like Aldurazyme to be allowed to get on Medicare/medicaid without facing the money restriction once their LTM's max out with their commercial insur.

I also today (friday 3rd) had the very wonderful opportunity to meet my Case manager (relatiely new to my team bc of my former CM Steph having to give up
myself and her one other patient in WI) Sylvia Wallace from Genzyme. I have a cute picture to put up from our meeting at the clinic today and was nice.

I have not heard anything on any plan what they will/are planning to do with my current Shunt for Hydrocephalus either during the surgery or soon after. I need to find out.

I do want to ask everyone to keep some other MPS I families
in their prayers. Both families are experiencing very hard
times. Little Liam Larrow passed away due to CMV this past week during his BMT. His website is: www.caringbridge.org/visit/liamlarrow Please pray.

Sweet little Alyssa Louden is experiencing a very rough peri0od and needs ALL of our prayers. www.alyssaloudenfund.com

God Bless, good night.

Erica

Monday, September 22, 2008 12:42 AM CDT

I wanted to post a few new things but at the same time leave the information below for a few more days atleast. My Spine surgeons Nurse and Secretary both called this morning and I learned
that the surgery will be about 3-4 hours long (expected anyways) and they also scheduled a pre-op Appt to come in
Oct. 12 (a Thurs) to meet with Kate and
Dr.Schwender to go over more details and so I can ask questions. I am very glad about this even if it is in MN and
an extra trip. I am sure if my good friend Karen is in town we will end up
having lunch and that is always a bonus!

Tomorrow I go to Madison to meet the Metabolics-genetics GC and Geneticist so in case it absolutely comes down to my having no choice (and we are fighting to the very end to stay at NS
bc of cost and resources my other physicians) I will know these 2 providers and the way the clinic works.
Incidentally I would still keep my Genetic Counselor at CHW in Milw. and my numerous other specialists.

Last I wanted to say that I am very excited to be volunteering this weekend
at the Fon du Lac Barack Obama Campaign office as a part of Move on.org I will update after! I realized
in thinking about this that my surgery is the day before Elections so I will have to find out how to vote by Absentee Ballot as I absolutely want to
vote! I have always voted via a regular
ballot so have to confess I do not know
exactly how I go about doing an Absentee but will find out shortly! I will have to have my parents vote this way to and make sure they tell me the results. GO OBAMA! =)

Saturday September 20, 2008
Just a short additional update - my Spine surgeons Secretary and also his NP called me back today and I have a few more details to add. I am sure I will be adding things from now till the day off surgery and then after!

The procedure they will be doing is called a Laminectomy (I think actually a Laminoplasty but now I need to look at what I wrote down!) and I will be in a Cervical Collar for atleast 3 months after surgery until they feel the bone is healed well enough in the c-spine area. Only after the 3 months will they start Physical Therapy. My Neurologist here in Milw. emailed
me today and she is going to have my local Neurosurgeon get in touch with my surgeon about my shunt and make sure there is a plan w/ what and when they will do s/t (if anything..) with that. I also talked to my GC and she gave me a few things to ask as well before the surgery. She wants me/us to look into a medical alert system for here at my Aprt. as I live alone. Once I find one that is the best fit she will help to push getting it. Right now we are talking to my other drs for their opinions on this.

I need to talk to my spine surgeons staff and make sure everything is up to date with my medications and especially the pain medications I am on and they know which specialist takes care of what. I may not have to do a pre-op with them which seems odd but they did have me sign forms and do
x-rays while I was there in August and have the films. As long as they have all the info and are in touch with the key people like my Neurology and Pain drs. One (of many things!) other thing I need to do is make sure the Anestheshia dr they use has MPS experience which I know atleast 1 if not more of the Anestheshia drs at the UofMN has this expierence so it should be ok on that end.

Thursday, September 18, 2008
Hello All, many have received this surgery date via email fro me but for those who have not I wanted to post here to. I also want to say I am always greatful for everyone that visits. ...

Earlier today I talked to my Spine surgeons Secretary and unless my surgeon wants the date for this surgery moved up sooner they have the surgery date set for
Nov. 3rd at 10:30 at the UofMN Fairview Hospital (where Dr.Haines and Dr.Charnas are as well as this is where the MPS I knowledgeable Anesthesiol. is) In the mean time
Dr.Schwender's Secretary did say she was still going to talk to him next week when he is back and make sure he talks to his colleague, Dr.Perra and so they get details worked out for the surgery.

My surgeon also does still need to talk to my Neurosurgeon up there about my shunt and if they are going to do anything or if they will do something at a later date with the shunt. His secretary told me today that bc it was taking so long she was going to talk to this other surgeons secr. as well and they would just set a date for the surgery, so that they could atleast get it on the schedule.
So thus she called me back with the Nov. date and w/a few small details on the hospital stuff. I should be getting a few questions answered about the logistics of how they'll
be doing the decompression and possible fusion and if there
is anything needed before hand.

After the past few months and especially the past month I am just so ready to have this over and get back to life and feeling better! I of course know I won't feel 100or probably months and still have other things that cause pain but this is a HUGE, huge source of the pain, fatigue and headaches for me right now not to mention the blacking out episodes that I am not even nervous at this point, just very happy to have a date. I am definetly singing my praises to the Lord and know that my firm belief in his wonderous ways will carry me through. Until then I definetly believe a positive attitude can only help and that I have one of the best surgeons for MPS I to do this incredibly risky procedure.

I am sure when the date gets much closer I will become more nervous but until then I think I just need to do what I can on my end to make sure this has a good outcome!! I will keep all updated either through emails or on here- my CB site.
I am soooo looking forward to feeling better!

Erica

Wednesday, September 10, 2008 4:42 PM CDT

Last week I went to Froedert on Weds and was admitted in early afternoon - the EEG leads where all hooked up and then I was taken up to the neurology floor where the video
testing began. For the next 5 days and 4 nights I could do very little on my own except stay in bed which drove me just about crazy. I didnt have any of the episodes which considering how often they have otherwise been occuring the
weeks before being admitted it was frustrating not being able to see what was going on and figure it out. My Neurologist had an Epilepsy/Sleep Neurol. on my case and they both decided this atleast is very unlikeally seizures and more likeally either from my medications and my already
low BP due to the beta blocker or teh c-spine disease/shunt. I came home on Sunday and slept that night from around 11-1 the next morning that is how little actual sleep I got in the hospital!

I ahve been talking to MN every few days and just spoke to
them again both yesterday and today - my ortho-surgeon apparently has to talk to his colleague this Dr.Perra but they are looking at a couple dates 1 in Nov. and then otherwise if he wants it sooner they will get this moved up. This has been the longest, slowest most frustrating wait since back when I was first diagnosed and going on ERT. I really hope a date is finally made soon as I want to have energy back and feel better, no or atleast very little pain and not be so limited in what my body can take bc the spine causes so many issues pain wise.

Right now my kittens are laying next to me and my biggest cat Pandi (not the Mom) is laying on the chair arm-rest. Grey keeps licking my arm and then biting me - they are in to everything and so incredibly feisty! I think my oldest brother may be coming this weekend to pick out one of them and my Mom I know is falling in love with them to, I am trying to convince her to take one! ;0)

I see my Pain dr tomorrow and will update after on what if anything we do differenctly.
I also know there are some things I am forgetting and will update those when I remember!

Erica

Wednesday, August 27, 2008 2:44 AM CDT

Sept. 3rd will be the admitted Video EEG at FMLH and the lenght of stay will
be dependent on how long it takes to capture a episode and then carectorize it.
-I had been emailing back and forth a little with my Internist on s/t and related to seeing her in the office - well she is very limited in her days but bc I had ERT this fri (and every friday) and couldnt miss due to missing likeally this coming friday she
offered to see me on one of her none clinic office days and is going to meet
me at her office Weds morning before I go to another Appt and right before I get admitted for the Video-EEG at FMLH.
I thought this was to nice and very loveally of her!

I also talked to my Ortho-surgeons NP today and the hold-up has been that he is coordinating schedules with 2 other surgeons - 1 is a Dr.Perra a Ortho-surgeon in Dr.Schwender's office and then Dr.Haines who is the Neurosurgeon that did my Bolt study last Jan. and he
will be working on my shunt at the time of the c-spine surgery. Given how
bad the last week and weeks have been I
hope this gets moving soon I am tired of being tired and having life pass by!
I wnat to be back to my busy self!!!

** I have to comment on yesterdays and today's historic Events: is it just me or is amazingly, amazing that we have just put our first Democratic
and as a whole Black Nomination for President? Yesterday we celebrated the historic end of a historic fight the fight between a former 1st lady and now Senator and a Black man to win the Nomination. One day after we celebrated
the vistory so long ago of Women's right to vote we celebrated this very great historic event. One day our great Nation will truelly become Free.

Wednesday, August 20, 2008

Un-finished journal parts of this where
written on 8/20 and still others today the 26th.

It's been a bit of a stressful week - I must add of course that the week was still fine and flew by as I was busy but some things happening. ..

I lost 1 of my 4 kittens this week due to we think the Momma being over-protective and having dropped her off the bed which is a pretty high up one when I was playing with them. 'Ghosty' was a very adorable all white kitty with just one singly splotch of grey on her head and then a grey tail. I still feel badly she suffered. This is the kitten that my Mom had most fallen in love with, everytime she came here now she picked up that one first to play with. One the note of my kittens they are somewhat time consuming right now, as I am trying to teach them how to begin eating regular food and not off of their mom-cat. (Ally) They are litter box trained just this week though which is neat to watch them playing around in the litter.

Last Tuesday I think it was my Neurologist at FMLH called with the ANS results, she was surprised with the Tilt table test being normal for the most part but on the other test the Sweat test it showed that my body doesnt regulate temperature I guess and I don't prodiue sweat in my lower body/legs. This my dr told me she believes is likeally a result of the Cervical spine disease and may be reversed with the decompression.
Today I talked to the Ortho-spine drs office his secretary has been asking my dr to get the surgery order forms filled out so she can swchedule everything, she also said that when she talked to my dr today he told her that he had talked to one of my other drs. (not sure if that would have
been my Neurologist at UofMN or my Neurologist locally but in any case he said he'd get the form done and she said she'd get bacl to me with the plans in the next few days. I am not sure how they are working in the Shunt op but I guess the Neurosurgeon that did my Bolt-ICP study last Jan.
is going to be involved. This per my MN Neurol.

Last week I began having even more pain then normal, a deep aching, electric like dual issues which no matter what I tried just werent getting under control. I saw my pain dr last Monday and we had decided to just let meds be the way they where and see if this would just be yet another of these bad cycles. Well by Thurs I was completely miserable especially any time I sat still - friday I truelly was miserable although I could still function, I wasnt sleeping hardly at all at night, the blacking out episodes where occuring every night and the Headaches where occuring far to often. Friday night I sent an email to my Pain dr letting her know what was going on and a little while later she called me. I tend to misplace or not answer my phone
alot at night but was thinknig it was going to be my sister - a good thing I picked up! My dr and I talked a little bit and then decided on some dose changes which I have been doing since Sat. My Pain is still worse than it was
even 2-3 weeks ago and my dr thinks this to is likeally the c-spine problem
vamping up its level and was really wanting the MN team to get things scheduled and going as soon as possible. I will update details when I know more. I called the Nurse today to ask about further changes to meds for this pain issues, she is going to call me in the morning. Which this reminds me last week when my pain dr and I where talking about this surgery, etc. she was giving some tips to prepare my
body to be at it's best health-wise and
also told me something that really meant a lot to me. I have said it before but I feel blessed to have found
the many wonderful providers I have.

On the front of infusions and Northshore clinic vs UW Hospital I have
gotten my genetics team to hold of making any moves until I get more info back. ie: from my Insurance Case manager at WEA and word from CHW's Patient Relations. My WEA person has looked into the billing and told me that UW hasnt been willing to work with
WEA on ordering the Aldurazyme through Caremark/TheraCom and also that the actual infusion bill will be likeally higher. Erin has told me and I hope I get this right but that the risk of maxing out my LTMs $2mill. is not likeally at the current billing rates of CHW's outreach clinic and their ordering drug through Caremark who is WEAs preferred pharmacy provider. With UW ordering the Aldurazyme direct from Genzyme vs through the Preferred provider and UW billing WEA (vs WEA paying for the drug before it reaches the clinic even and the money goes straight to Caremark) after they get the drug to the Hospital, thus the Hospital can 'upcharge' which frequently happens and I know firsthand
how much more expensive it is. Then you add in that the infusion is being given right at the Hospital vs. at a outreach clinic and the cost their goes
up to. So anyways back to the point I was making, my WEA person Erin has said
that the chances of 'maxing' out my LTM
is much higher and of course our goal is to reach to 2010 without hitting $2million so that my benefits restart at $0 with a full $2million more to use.

On a different Note I am here in my 4th
new Aprt and new Address this year, I now live in Juneau, WI vs. in Waupun.
The Neighbor guy and I have been hanging out almost every night, he is cute and sweet and nice company to.

If you will please say a prayer for my Sister and my Nephew Zander (2years) who has just been diagnosed with a milder form of Autism 2 weeks ago. The odd thing with Zan and his Autism is he
has all of the traits EXCEPT he is very
cuddly and loves to snuggle and give Hugs! He likes to play by himself vs with other kids his own age but when it comes to older kids liek my 6year old Nephew Quinn - Zan always seems to gravitate towards what he is doing! I am trying to help Sara look into programs and options now and Zan is in Birth to 3 plus they I guess are on the wait list for a program. Sara is also looking into OTC supplements to help.

Friday, August 8, 2008 8:43 PM CDT

->I wrote this while sitting at my Ortho-spine surgeons office:
I am sitting at Dr.Schwender's office right now - he is the Ortho spine surgeon I hope it goes well I have to give him a note from Dr.Charnas as Dr.C wants him to call him after my Appt.
I saw Dr.Charnas yesterday and I am not sure if I should say it went well or not!? He is again really pushing for surgery, him and my Neurologist at home have been talking alot and they are really concerned with all of my symptoms - ie: numbness, tingling, burning, tremors/spasms, and blacking out as well as electrical shiock type sensations. He said he has saw 2 MPS I pts in the last 2 months who they waited to long to do surgery on and they had tons of complications and he is really concerned now. My Physical exam is huge for alot of things including extremely brisk reflexes which just means when they use the hammer I kick alot. ;) The surgery has a ton of risks which they are all concerned about and is why there have been so many MRI's lately and Xrays today of spine watching it and seeing what they should do. The risks of not doing surgery though include bowel and bladder loss as well as paralysis so it is a hard call for all to make... The other thing they know is that when/if they do spine decompression nad fusion they will have to either put a pressure-flow valve on my current shunt and revise it or move it altogether to a VP. Which this is what I will push for as there are other spine issues in my thoracic and lumbar spine and I don't think the current shunt helps this any... I also think they will probably coordinate to do that surgery at the same time they do the spine surgery - for anesthesia risk reasons but am not sure either. .... I sure don't want to have to go under twice......if possible..

Update: Also written while in MN yet:
I am done with the Ortho-spine Appt and waiting to fly home. Anyways: bc of all the symptoms and progression in
the last 6 months Dr.Schwender is reccomending a Laminectomy - he doesnt know if he'll have to do a fusion till he gets in there. The surgery date will be set in the next few days and within the next couple months atleast he said as he wants to be able to revers all the symptoms I do have as much as possible. I don't know much other details yet like lenght of hospital stay, shunt ,etc. I am going to call Dr.Charnas later today/tonight to see what him and Dr.Schwender got figured out regarding the shunt, etc. Dr.S did think that would be at a later surgery (although I should have brought up the very difficult intubation problems) and he thought the Hydrocephalus might be reversed, although Dr.Charnas and my home Neurologist have not thought this and think the shunt will have to be revised or moved. I personally want it to be moved to a VP if they will be doing something to it I am so
tired of the side pains adn lower back pain which have all began since I had this LP shunt put in.

My local-FMLH Neurologist emailed me back not to long after I sent out an update today and she said she was very relieved and althougth she knew just like I do that spine surgery in MPS I is incredibly risky she also feels that doing something definitive is the only way to really see if
we can bring the symptoms under better control. She offered
to set up or participate in any Conference call with my drs in MN including Dr.Schwender (Ortho-spine) and to be sort of the one in charge of setting up my Fup care here in
WI for after. Very nice of her. She didnt say anything about ANS test results yet so I guess there not back yet. She asked me to keep her closely informed of all updates. She is so nice and I feel so blessed to have her on ym team!

Yesterday (thurs) I had a brief Lunch meeting with Karen O'Neill my good friend and area PCL she just was interested in updates on my meeting as well as helping in any way she can with the situation at ERT. We ended up having a really great lunch with my good friends the Restemayers as well as my good friends the Kleins and it was just really, really nice and so free flowing in the information we passed to each other!! Always the very best ways to get updates and just if you are in any way stressed
a great way to release to friends who understand! In my case I am not really stressed about anything but I am frustrated about the ERT and believe genetics cannot make me go elsewhere without my own willingness. So they just of course try to help and offer advice. Incidentally both friends on ERT, providers and family all believe I cannot be forced to go to Madison and that it is unconscionable that they are forcing me (or atleast their trying!) I did get the contact info for the Genetics team in Madison and emailed them to see if I could sit down and talk to them. my friend Karen (genzyme) said they are so very nice and I
might just talk to them if nothing else - but of course she also is supporting and trying to help me.

More news on a promising new drug below:

http://www.genzyme.com/corp/investors/GENZ PR-071708.asp

See the above link, Genzyme has bought part of the
company working to bring PTC 124 to the larger
Genetics community who live with atleast 1 missense
mutation. (in the case of MPS I forms none of the A-L
iduronodase) PTC 124 is currently in trials in the
Duchenne Muscular Dystrophy and CF population with
this same misense variant. From what I understand PTC
works by by-passing the missing mutation and thus
'fools' the body into creating the enzyme, this in
turn in both persons with 1 misense and those w/2
misense mutations would effectively become the
equivalent of Carriers for their respective disorders.
For further info see the link above or under the
'News' section of the genzyme website www.genzyme.com
probably the info there is much better
than my own!
The reason I am so interested in this of course, is
that I do have 1 misense mutation and thus when this
does became a treatment available for MPS I it can
replace ERT infusions!!
I also emailed the VP at Genzyme working on this,
whose email I was given by my PCL also at Genzyme. I
will put new info on my web page when I get any.

Please Keep Zander - my youngest (2yrs) Nephew in your thoughts if you would he has his Developmental testing to see what is going on with him
if he is just delayed or if there is really Autism or something else going on, on Monday.
Hugs,
Will update more soon.
Love and Thank you for your support,

Erica

Wednesday, July 30, 2008 3:41 PM CDT

hello all, I thought I would update some things from this last week or I guess this Monday really. I first saw my PT
where she is trying a TENS (which is I think stands for Trans-Electrical Neuro stimulator.) unit to be another measure in with the neuromod. and opioids. After this I saw my Genetics team which has had me very frustrated for the last 2 days since then.

I don't know how else to say this except that what they are
trying to do is unconscionable and I can't imagine there isnt anyone who won't agree. You see the clinic I receive infusions at weekly has very, very limnited staff (1 Nurse 1 week and 2 Nurses the oppositse week) and need to move patients out to the clinic from the hospital or start new pts or something. Well because Children's Hospital can't and I do literally mean they are not able to get more Nurses for whatever reason so their answer is to force me to go to UW Madison which many of you here know is in a whole other part of the state completely away from all of my providers, my resources and my Nurse and friend whom I often spend nights with especially Thursdays and/or if not feeling well after infusions she will have me come sleep there. Also in Winter I spend almost more Thurs at her house than I do at my own Aprt. These are all resources I will lose and the family I have at the clinic whom I have gotten to be very good friends with many of the other fabry
and the one other MPS I pt.

Just this morning I made a flurry of phone calls - 1 was from my actual Case Manager at WEA about my LTM and some ques. she had. So I talked to her about it as I was going to call anyways with ques. for WEA. She is checking a few pieces of information for me and will call me back when she
has answers, then I called my GC talked to her again about
this - she incidentally is going to go back I guess and try
to find out some things and push back again, then after that call which was on the north end of 30mins I talked to my CM at Genzyme for atleast 30mins or so and she is trying
to find out a few pieces of information for me and then last I talked to my good friend Steph also at Genzyme and just if nothing else felt good being able to vent to her.

Big reasons for not wanting to go to UW is - no resources other than 1 former dr I am now friends with but no friends
I actually am super close with. Winter driving. UW Hospital will be buying the Aldurazyme (drug) directly from Genzyme so the 'middleman' will be cut out generally that means lower prices but in my experience talking to other pts. - the drug actually is 'up-charged' significantly and becomes twice to 3x's as more expensive to the patients Lifetime max. (LTM) basically bc the Insurance's pharmacy isnt well the Insurances pharmacy (hope that makes sense) so the Hospital bills the Insur. medical benefits after they get the drug - and well has a drug EVER been cheaper when you got it from the hospital then when you got it from a pharmacy? NOT EVER in any of my experiences. So I am pushing back telling them I cannot be forced to go to another center in a complete different part of the state when it will likeally greatly impact my LTM and chances to keep it below the 2million dollar mark until 2010. Right now I am just below $1million and if it does not max out before 2010 my benefits will return to '0'
and I will get to restart with another 2million $'s!
So I am pushing back telling them I will fight this till the end - they want me to start the end of August and I am flat telling them they can't force me to go to another Hospital infusion place in another city just because CHW cannot get it's own more resources for NS clinic and wants to start other pts. So we will see but I can tell you this I don't think I as the patient can be forced to do anything
in this regard - I have never heard of such none-sensse, and am furious, so again we will see.

Otherwise the genetics Appt. went fine - it would have probably been a great Appt had it not been for this but how
often does ti work that way? it was nice to see my GC and I
imagine we'll be in touch even more so in the next few weeks as we go back and forth with this.

My kittens are getting big - believe it or not 1 of them the little fuzzy gray one whom I think would be cute to just be named 'grey' has had a cold the last 5 or so days
including congested chest, wheezing and sneezing. I had never known a cat could get a cold but the week or so before that my older cat 'Pandi' had been sneezing quite a bit. Strange! The kittens are getting bigger and turning to
look like quite the little fuzzy felines now with eyes open
and meowing, and the like.

back to Monday due to Genetics Appt going so long (2 1/2 hrs) my Pain dr and her Nurse asked if we could reschedule
for next week Monday - by this time it was already 5:30 so
it was fine. I havent received word on the ANS test results yet - although am thinking maybe sometime in the next week?

This past weekend I decided not to go to Mn - for the MPS pt meeting, I had been asked to speak on a panel with 2 MPS
parents but bc of some things that came up decided to stay
here. I did miss everyone there and received some nice sentiments back from the head-genetics director there who invited had me to speak. very sweet what he had to say about their missing me and it not being the same without my being there. (I think that last part was a compliment anyways!) ;) I do go to MN next week though to see my Neurologist and Ortho-spine surgeon. I also will be having
lunch or something along that lines with a good friend of mine at genzyme who is the PCL there.

Will keep updates and write when I know something more!
Love and God Bless,

Erica

Wednesday, July 23, 2008 4:51 PM CDT

Hello All,

let's see how much I can get written here before I go crazy
bc of itching! We'll see. ;)

I didnt update again after I wrote here last week that my
newly adopted stray cat 'Ally' was having kittens, well I now have 2 Adult cats (both Female) and 4 kittens! very cute and cuddly although Momma Cat is protective and wants her loving to. *I just thought i'd share a bit of unrelated
to medical stuff news once!

Today I woke up bright and early after getting very little
sleep bc of a restless night to head out to FMLH for Autonomic testing. This consisted of about 4 hrs of tests
ranging from a tilt table, to breathing to Sweat testing and then the finale a 1/2 hr in a sauna like room with sand
like grit all over my body which progressively turned to purple as I sweated and heat rose. In the end I looked like
a Purple people eater or better yet Barney! Hopefully these give my Neurologist some more clues and insight and she can have some treatment or atleast answers. Today in the mean she called in a new script for Zofran for me the Nausea definetly has been getting worse and is aweful. I am thankful my local Neurologist really has been great and con'ts to push to help and to get others to help.

I was supposed to be heading out to Minneapolis, MN tomorrow for Appts on friday including with my Neurol. there. I called today and asked his scheduler if she could
try to get that Appt. the same day/week as the Appt I have
with my Ortho-spine surgeon in MN I havent heard if she's been able to or not yet. I did just get a pre-recorded message reminder about the Neurology Appt. so am going to say that so far it hasnt been changed. We'll see, I really do want to be able to hang out with friends this weekend and do some mundane, silly things though so am not sure what i'll do. I do know I most likeally am not going to go to the meeting on Sat in MN I just have other things I want
to do here! I do want to hopefully meet a couple families up there when I go in a couple weeks though. Mainly I think one of the best reasons for having the Appts both the same day/week is that i'd have the ANS test results, can get feedback from both the same day and can take all the info from both drs back at the same time to my drs here
at home.

Monday I go to Milw. yet again but for PT, then a 6 months
Genetics Appt with my Genetic Couns. and last an Appt. with
my Pain dr. Busy! Tonight I can finally start the new Neuromodulator - Gabitril see if we can get this to work for me as an add in to the 2 other NM's and the opioids I take. I so far have had good success with the increased muscle relaxer dose and have to say this week when I had to
stop all but the opioids bc of the ANS testing today (each med had to be stopped on different days, some as far out as
last fri) that I could tell a difference in the muscle tension related back pain and the pain that the NM's help and was happy to restart them all this afternoon!

All for now, will write again soon.
Love, Hugs and God Bless,

Erica

Friday July 18, 2008 4:40 aM CDT

Holy crap I woke up this morning and I do definetly mean this morning! (good thing I took a huge nap after Quinn's Mom picked him up yesterday!!!) anyways my 2 cats where hissing and meowing and 'cat fighting' each other..
well... it turns out this 2nd cat I 'adopted' as a stray about 2 weeks ago is having kittens! Ooh crap-oola.. So far there is 1 that looks like a mini of her and a black one that could be my other catsw mini-look alike (cept Pandi is a female to and fixed!) My Allie cat was going in on Monday to be de-clawed in front and fixed and shots so I don't think she can even have that done yet. .... I have no idea what i'll do with these kittens when they get old enough? And I am moving in a few weeks eek... and to top it all off Allie's 'nest is on my bed! This is got to be so nasty-eew!

That said it has been a relatively busy few days with Quinn and I have been exhausted. He can be my little prince and he can be my little devil in training! This week it was a mix as he was waaay over tired. I hope to get some work done on the meeting at the clinic today, we'll see though. Next week I am in Milw. Weds for ANS testing, Tues for Pain dr Fup and Thurs-Sunday traveling to Twin Cities.

Guess this is all - wuish me luck with Allie and her kittens!
Tues. July 15th, 2008
Just a brief update.

I think I have a date settled for the MPS and ML Family mtng! My 2 speakers (that I want) are both available the 1st Saturday in Nov. Yey! I know this is a change from my
original goal of Oct. but I am ok with it.

Now last week I wrote a brief bit how I wished that CHW would get behind this mtng........ after some emails back and forth which basically where only about my dr wanting to know who my speakers where and agenda info. he approached me on Friday at the clinic to talk. I told him a bit more about what I have been working on and my speakers and he told me that he thought (if I where willing) that CHW genetics should help plan the meeting and be behind it! So this is good news! Now I just need some time to get info to them - life has been so, sooo crazy!!!!!

I saw my Pain dr today and we are adding a 3rd medication to Neuromodulators to try to combat and 'settle down' the nerve signals as I have reached a pretty near max dose on the other two (max dose as far as how high my body can toleraste not how high they go dose wise) We also made a few small changes to see if this will help the overall pain issues more. Otherwise her and I talked alot about the test results and what my Neurologist (local) is doing. Both are really great drs and have helped alot. My local neurol is really trying to push fwd with finding answers which is great. I truelly wish my NSg would be more assertive and really just more involved and helpful. I do as my dr said always have the choice of the other Nsg she sent me to back when I first started seeing her, we really are just waiting to see what my local neurol can do or get done first.

I'll update more soon!
Jenn I love yah and am thinking of you, Anisa and David!!!

Erica

Friday, July 11, 2008 4:49 AM CDT

A likeally short update and a likeally partial vent! I apologize but this is truelly going to be a "Erica's" blog session!

I am up late (early?) as I can't sleep -I am very tired but just can't fall asleep. I did take a couple hour nap last night though. Which was the routine of the week, between this week and the one before I have had an increase in the 'coming n going' pressure headaches and this week has just worn my body out completely. I have had a little bit crazy schedule lately to with alot of busyness
(sp?) in phone, Appts and especially my nephew but I ended up sleeping straight through the time for supper every night but one this week -averaging 3-4 hour naps and then sleep t/out the night to. My body is just feeling the effects and I can tell. beyond even the H/A's is the tremors and or twitching whatever it may be coined my arms, hands and legs, etc do at night and in the mornings +
some during the day. Then of course there is the usual array of things. Ahh well it is what it is.

I have been busy working on the meeting/family get-together
for Oct. I have decided to drop the fabry component which is a shame but it just was creating more headache (no pun intended there) w/ the Hospital that is apparently doing their own meeting. Although and I write this hesitantly it is interesting that no one knew about this meeting until it
was told to me when I talked to them about planning what I am doing. Rather than compete for speakers I have decided to just go with a MPS/ML Family get-together and have 2 speakers over the Lunch. I just confirmed the 2nd speaker I
was hoping to get today and we are working out the date - if my first speaker (pain dr) is available what is the 2nd week of Oct. (or is it really the 3rd?) i'm not looking at a calendar right now but think it is the 13th. Anyways all reading this are probably completely confused! I'll be setting the official date within the week!

I am talking to my geneticist tomorrow he wants to know the details of my meeting - I am unsure why and thinks I am
being 'secretive' in not sharing but really I am just moving fwd slowly and cautiously planning. I wish that he would decide the Genetics group wanted to be a part of this
it would definetly be beneficial --not that I want to give the planning to anyone but for the backup. I do know my GC is interested as are a whole host of my other providers who
would like to attend and meet other families w/the same disorder as I have. I am not sure how that will work in but
I think it is ok to have a family get-together and have non
MPS folks come to, for a chance to meet and for families to meet other providers and talk to. I am very excited about my 2 speakers the 1 being local and 2nd being from L.A.!!

I know I still havent uploaded pics here - I feel badly my laptop is completely crashed (twice) so I need to re-call dell and have them come out and actually look at it and replace the hardware. Thankfully i have the warranty for that! I promise to try to upload soon!!!!

I love, love, loved my Apt in waupun but last weekend after a final huge argumentn with my sister I met my final straw and moved to my parnts. I am looking at Apts. tonight
though and plan to be moved atleast by the beginning of the
next month. (aug) Love my parents, but can't stand being there! I am so looking fwd to looking at tehse Apts! I will be completely on my own then with just my 2 cats although my oldest nephew spends 3-4 or more nights with me. Incidentally my sister and I are talking again but both agree it's better to be apart. I sooo miss being with Zander every night that he is with Sara though. =( (1/2the x)

So I'd like to write this and keep in mind everyone this is simply my thoughts. As a 25 yr old with MPS I, I sometimes feel like I have my place in the world now doing the things I do with advocating when I get the chances and
especially I feel 3/4 a part of the MPS world but almost on
an 'outer edge' persona - I meet and mingle and associate and know alot of the MPS individuals both on affected family/kids/individual level and on the professional MD's level which has offered many great chances and experiences.
But I also feel like I don't quite live up to expectation to many or as if I don't quite fit in maybe bc I don't have a college degree, maybe bc I keep a smaller, closer, tighter group of MPS/ML friends whom I talk to often and then many, many others I know well from meetings, and speaking and being invited places and tehse are friends but
on a different level and with some in this group I don't quite fit the mold... I don't really even know how to explain the feeling it is a funny feeling to have so many people and really so many of my providers who are just that
my providers (albeit some get the updates on meetings and tests, or life bc they've had a place in my road to dx or are just special in some way) believe in me, and offer insight into all they see of me and imagine for me and their interest in what I do MPS wise.. Then there are my friends completely unaffected by MPS in any way except through me and although they completely have no idea what it is like and they are frustrating the things they same sometims it is funny how they try to be unassuming and to the best tehy can understanding. It is so completely two different worlds I havent figured out yet and am trying and
only hope this doesnt come off sounding slef pitying or conceited.

I want to close by saying jenn if you are reading this I miss you and Anisa and Dave and we need to plan a trip 1/2 way soon!!!!

"May Thy word be a Lamp unto your feet and a light unto your Path." psalm 119 : 105

Erica

Thursday, July 3, 2008 11:19 AM CDT

**I think I have a date set for the October meeting I am putting on for MPS
and Fabry families. : the 2nd week in Oct. I also have 2 of hte speakers set and 2 more am talking to.

Neurology Appt Update
Hey a short update from Neurol. Appt. I
had that yesterdy at FMLH and went fine. My dr sort of left it up to me to start a medication to try for my blacking out basically but we basically both agreed it would be like a shot in the dark. She does think that my Ortho-spine surgeon in MN needs to see the Evoked Pot. (SSEP) results. She also said she doesnt really think the episodes are Seizures but more ANS related secondary to the Compression, Shunt and thus delayed body response times. I emailed her a ques. last night I had forgeotten and she wrote back today that if the testing we are doing next (Autonomic nervous system ie: Tilt table etc) comes back normal than next would be an Admitted Hospital (over night or more) EEG to try to capture this happening. Also she is going to help set up a phone conference call between my U/MN Neurologist, Ortho-Spine surgeon and NSg locally if not also the NSg there to go over and make decisions on what is all going on. For the mean time she is having me get Compression stockings to try (I just thought of this but I do get alot of swelling in my legs by morning which I wonder if is related or not) as this would help episodes if they are ANS related.

Her last comment otherwise was that my case is complicated and there is no one right answer.. (which I agree and in my message last night even wrote that I felt like there was a disconnect in my care these days - sort of haphazharded where each provider passes off to the next and thinks one or the other of the other providers is taking care of things.) I did write on my end that I didnt blame her or any one in general more that it just needed to be addressed or I felt like maybe we could do something differently for all providers in the main team. (neurol., NSg., Internal med, Pain, Ortho-spine) I don't want to say that they arent pulling their weight or helping me - my Pain dr and Neurologist here are fantastic and have done so much to help and they believe me which is a nice feeling.

I think my Neurologist really wants to find an answer an we'll get there we might just go crazy in the mean time. Her other parting words where 'you'll just have to be verryy careful getting up at night, sit on the side of your bed for a bit, pay close attention to symptoms. Hang in there and keep me posted" Quinn my Nephew enjoyed (he likes going to my Appts for some reason - for all the dr Appts he's had you'd think he'd be freaked out! But he sits non-chalant or lays more like it at both this week relaxing, watching his video and chatting away about himself and what he was doing this weekend!

For those of my friends I promised info from the Conference, to Study, to meeting to teh researcher in Manchester I promise, promise, promise I will send it soon!!!

Weds July 2nd, 2008
To save time this is part of an email I sent!
I just got home Monday from the Conference (Int'l MPS Symposium and joint Family MPS mtng.) monday and have confirmed 1 more of the speakers I am going to have at my Oct. meeting. I have 2 confirmed at this pt. and in the midst of talking to the Pharma com. (Genzyme, Amicus, BioMarin, Actelion, Shire, and also will have represenatives from ISMRD and Nat'l MPS Society.) I also have a tentative date - the 2nd Sat in Oct.

The conference was great although I have to admit I was entirely exhausted and completely out of it sick the entire time with aweful headaches. The joke amongst all I was with was "how many naps is Erica going to sneak away for today!" =) I do think it was the atmosphere/elevation as well as weather being right there at the Mountains and Ocean. I am feeling better atleast H/As are reduced to a tolerable pt again! Now lower bask and upper back is something else I dont know where i'd begin with that! It most definetly was Pressure/Hydro issues the w/HAs though. I had the great pleasure to have dinner with Patti Dickson (former IT dr) and her Husband and Kids who where at the meeting with her Sat. night before the Gala dinner which was so nice! I love her a d her family - Daphne always remembers me and we have fun together. I have tons of picstures to put up here including new ones her Hubby took of us. I also have pics from the Sat night overall Gala dinner which was so formal (Fri nights US MPS Society dinner was dressy but a little less so) I ended up wearing a short black dress and very high narrow heels. (which i love but harsh on ones feet!) I also found out 1 of my very closest Canadian (Toronto area I think - Sick Kids) friends and her hubby and daughter (age 3 MPS I HS) live only about 8 hrs from us so we are planning a
trip to meet 1/2 way. There where over 150 drs in attendance and 500 people at the over all meetings which made it busy as can be but the new drs I met where very nice. 1 is Joe Clark of Genetics at Sick Kids whom my friend told me the next day was thinking "how can I get that girl to my practice" just bc of my hands and how mild I look given everything! Funny! I also saw many others of my friends - including old IT rewsearch coord. Alla, Barb Lyons, and many, many of my friends in the MPS anmd dr world.

A whole flock of the genzyme marketing Dept where at this mtng (I have a neat video to show you) and they wanted me to do a Photo shoot so we did that on Friday
skipping out on 2 hours of talks to go to the beach and took the Ferry out to Granville Island. The pics are nice but was to funny! They also had a video streaming of myself (from last May) Nick andn Denise and also a poster of myself also from then at the beach. That particular pic doesnt even look like me anymore though bc my hair was shorter (chin lenght and reddish) whereas now it is long and it's natural brown color. I had sooo many people come up and introduce themselves and also just ask questions about myself and/or questions about MPS they had for me.

I see my Neurologist at FMLH later today
for the EEG/Evoked Potential results which both came back abnormal and may explain my blacking out episodes.. Also the latest MRIs show some changes overall. I will post an update sometime after this Appt.

Will write again soon and post pics when can here!

Erica

Saturday, June 21, 2008 2:40 AM CDT

I have my SSEP (evoked potential) and EEG results I received the reports in the mail today (not sure when I will talk to my Neurol.) I also received my MRI results back on Monday from the Lumbar and Cervical imaging.

I am just going to post the results here only for the sake that other MPS families who come here if it in any way can help them. Rather than write everything though I am just going to write what was 'abnormal' but first let me just say that I don't know what this all means in terms of what I all have going on or even in the overall course-history of MPS I in general. As you all know I have the Commun. Hydro, I also have the MPS I, and for the past several months (3 at the least) I have been having blacking out spells ONLY occuring at night and what we/I call mini spells (which are mainly just similar but repeated and not actual blacking out.) I also since last early winter have had twitching/tremors in my hands and feet on and off
and other symptoms.

The EEG results:
"Mild intermittent slowing in a generalized fashion,
as well as intermittent slowing noted in both the left
and right tenporal head regions which where done
independently, and these where noted to be sharply
contoured slowing at times."
- This is an abnormal awake and asleep
electroencephalogram due to:
1. Intermittent generalized slowing which is
indicative of mild diffuse encephalopathy which may be
toxic, metabolic, structural, infectious etiologies.
2. Independent left and right temporal slowing. This
indicates focal structural/vascular or physiologic
abnormalities in the right and left temporal head
regions.

SSEP results:
-Abnormal posterior tibial somatosensory evoked potential due to slight prolongation of cortical responses (P37/N45) bilaterally. These findings suggesat the presence of a lesion located above the lower medulla and below or at the somatosensory cortex bilaterally. Flexion and Extension maneuvers show no changes from baseline.
-Median SSEP was normal

Cervical MRI:

(my own words- Essentially the same findings w/the exception of the CSF surrounding teh cord is nearly effaced. (C2-C4)
(my own wording again- Others levels include various findings.
*New finding of small bilateral thyroid nodules that demonstrate increased signal intensity on teh gradient echo axial images. ---I do have Pituitary insuff. w/hypo-thyroid due to the previous un-diagnosed Comm Hydro.

Lumbar MRI:

1.Slight mid lumbar levoscoliosis
2. Mild central canal stenosis at L4-5 due to cong. short pedicles, a circumferential disc bulge, and ligamentum flavum hypertrophy.
3. Mild circumferential disc bulge and bilateral facet degenerative change at L5-S1.
The central canal is not stenotic at this level but there is moderate foraminal narrowing on teh right.
4. Mild bilateral foraminal narrowing at L4-5 due to disc bulging.

If you remember I have a Lumbar-Peritoneal (LP) shunt in place for the Hydro and I forgot or did not think of to ask my Pain dr on monday when she was talking about these results what she thought the Lumbar results ment as far as the shunt. My Neurol. locally was wondering if there was problems being caused from the spinal issues and the shunt aggravating teh headaches and I am so curious to know. Only bc I would Love, love to have these H/As gone and maybe some insight. Maybe I shouldnt say this here but I regret so badly and have always that I ever let my Neurol at the MPS center and my NSg here do the LP shunt...
-----------------------------------------------------------
Now on to better topics; ;) I have been watching my Nephew Quinn many a days this week and having him over night those same days and we have had some pretty good times! 1 day we went and got a water gun so he could join all the neighbor kids at my Aprt. in a squirt gun water chase and another day we spent all morning and that night at the Park on my Apt. site and across the street and then
another day we did more park, and games, and with teh neighbor kids and just alot of activity! I LOVE summer! I will Miss Quinn and Zander this next week that I am in BC!

I leave for Vancouver early Weds morning and then get there
early afternoon -coming back the following monday. It will be great to catch up with friends from here in the US (teh ones that are going - I think s/t like a handful of us members only) and the rest from other countries-to fun! =) The meetings look really great and i'll have the choice to go between the family and professional sessions of which I
have more professional talks I want to sit in on right now.
(just bc I want to as always learn as much new as I can and
about any new and emerging therapies as well as just research in general in different areas 1st for my own self and 2nd to bring back to the families here not attending and 3rd to bring back info for my providers here so they can know more about the disorder. I also will be doing the Video product. for Hide and Seek (unless like I said in the
other post ((bc I missed Bryan's phone call and need to call him back)) we do the video locally or in L.A. at HnS Headquarters. That video is titled and Bryan or Steph can correct me if i'm wrong! - 'many faces of Lyso disorders'

Today I also received in a email from Laurie at our MPS Society office that there is a Production co. which works with Dr.Pastoures (Greg Pastoures-NYU) that have previously
done educ. videos on MPS I, II and Gaucher and they are looking to find patients to do complementary videos on MPS I and II. I have sent an email to this Louise-since I will be in Vancouver I think it would be neat to meet and talk to her and her prod. group as well as Dr.Pastoures. (whom I
have met and talked to but not long I believe in the past)

Otherwise my parents have their pool open now and I think myself, my sister, her Bf, and my nephews are all going in - it is nice here tonight but was hot and humid today. (fri err Sat morning!)

I otherwise will update when I find out what the above results mean for me and if anything changes otherwise will
try to update soon.
Have a great one!

Erica

Tuesday, June 17, 2008 1:34 PM CDT

i'm not to sure how long this will be as I am typing with my small finger out of commission and makes this a little difficult! I saw my Pain dr yesterday and she asked to look
at it and after doing so wanted me to go to Urgent care and
have it looked at which I am going to do. .. I havent had a chance to quite yet bc I had to get home to meet my Nephew's Mom so I could get Quinn from her as I have him last night, today, tonight and tomorrow.

He is outside playing with a group of kids at hte moment - if I lived anywhere but at this Apt. complex I wouldnt let him alone out there but we have a cop on sight and is alot of people around as well there are plenty of toys for them to climb around on and grass area to play.

I have been contacting people and getting feedback on a Joint Lysosomal meeting - this has been extremely interesting to say the least bc I have interested pts and families and even interested speakers and represenatives but at the same time bc I am not associating with the childrens hospital (long story and i'd rather not tell it except to say that 1 person I talked to in the Genetics group is interested there and 1 is completely not) there has been alot of bureaucreacy. (sp?) I have a contact now at Amicus whom asked me to call her so I am going to see what her thoughts are when we speak. Otherwise I will just
put aside what could be an outstanding, informative meeting as well as a great chance for different Lyso disease groups to mingle. I will then go to planning just
a MPS/Ml get-together like last year but probably invite someone to speak I think still in one of the high interest
MPS areas like IT (bc of the trials going to be starting for MPS II and MPS III as well as the next one that is going to be starting for MPS I in the next year to 2 years.
This all frustrates me but in no way discourages or even upsets me I am very beyond anything at CHW getting to me.

My Appt. went ok yesterday no changes to meds, our only thing is to get the test results from Neuro testing at FMLH
and if something happens that I get back into having severe
pain again I have to try to figure out what the brought it on or what pre-symptoms where there first.

Next week I leave for Vancouver for the Int'l MPS meeting
which is 4 days of near all day meetings and the Gala dinner Friday evening which is always fun. The speakers will be from all over including the U.S, Germany, England, France and Italy and Canada to name a few. My former IT (intrathecal) dr (and now friend) will be there with her family and we have already planned to have dinner and spend some time - her daughters are soo cute!!!! Daphne is my little sweet heart! There also will be my good friends from the Hide and Seek Foundation whom I will be doing a Video production with (unless we decide to hold off and meet either in Wi or L.A.) I also will meet my canadian friends the Purcells and my friends Jenn and Anisa Elder. Of course i'll also see my very good friends the Hollands, Amy is a complete sweetheart and I wish the entire world that I could do more to ease her pain and aching loss. Her daughters Maddie and Laynie are so incrediblky cute and I
just always, always wish we lived next door to each other!!

There is so much more I could write and I will update again soon.
All Love and so many Hugs,

Erica

Thursday, June 12, 2008 9:05 AM CDT

I was at the Chicago MPS/ML meeting this weekend which was a great time, I had the chance to finally meet Lynn Diedrich whom is a Sales Rep with Genzyme and formerly covered Wi. I also had hte chance to meet the other PCl for
WI - Laura whom was also very nice. My friends Carolynn and
her family, Kris and her family, and Teri and Jenny Klein where at the meeting so was a good time. Terri and Jenny actually spent the weekend with us at the Nagy's which is also where I stayed - and was nice. I am looking fwd to going there again next month and Linda and I are going to the MPS meeting in MN the end of July. Incidentally my parents are also coming to that so they will get the chance to meet some of my friends!

I have had an otherwise busy week - between Quinn being with me, a little bit of work on the Lysosomal meeting I want to put together here in Wi and dental Appts. yesterday
and today many other little things which have all added up! Needless to say Quinn and I where both exhausted yesterday afternoon and took short naps together! I also had the C-spine and Lumbar MRIs on Monday at CDI which took
up the afternoon and for which I should get the results of on Monday. I should alos get the results from my Opioid Metabolite blood (levels) testing and also the SSEPs (evoked potential) and EEGs soon!

I see a Physical Therapist whom is very excelent down in the further reaches of Milwaukee and for which it has been
a little difficult to work out... She realy is great but with everything else and especially with gas prices now I am going to talk to her and see if there is someone she might reccomend nearer to my Pain dr or other specialists
offices.. I very dislike how high prices are!!!!

I have a few pictures to upload from this weekend and going
to do that now - I may put them in a separate area though
see how it looks.... Since the other pictures are quite lenghty in how many there are!

I have an Appt. now with the Ortho-spine specialist in MN July 3rd for which my PCl at Genzyme and I are going to try and do breakfast together.. and then i'll see this dr again. You might remember I saw him back in Dec or so and he said to come back in a year but my Neurol. up there wasnt so sure that long of an lenht between Appts was so good as he thought surgery should have been done then... Well given all of the symptoms and new symptoms as well as
changes my pain dr and I decided it might be a good idea to see him. The concern from some of my drs is that the c-spine problems are causing or being caused to worsen by my LP shunt and causing a 'dynamic situation' in which one makes the other worse and vice versa. For instance if the CSF fluid isnt able to fully get past the c-spine area this could be causing the shunt to not be able to drain and thus the headaches... or it might be that the c-spine and other issues are feeding of the shunt and or just causing the headaches in and off themselves.. I know thats probaby confusing and I hope I got it right!

Have to run, Take care and sign the guestbook!!

Tuesday, June 3, 2008 7:50 PM CDT

I apologize that this particular journal entry in it's 3 parts is so long..

I spoke with my Genzyme Case Manager today and I feel like what is happening
there rocked my world. My CM whom I have known since I was diagnosed 3 1/2
yrs ago and who has been a 2nd Mom to me since is no longer going to be my Case Manager as she hasnt covered WI for about a year or two now and now is giving up myself and her one other pt she still had here. I know that Steph and I will remain close and I know I mostly did my Insur things on my own and have an assigned Nurse-Case Manager
at my primary Insur who has said when we talked previously that she will check in monthly to help and see if there is anything she can do for me so I will not lose Steph and I will still
have someone thankfully right within my commercial/primary insur. to help. I should add that I will be reassigned to the 2 CM's at Genzyme that cover this state.

The reason I have grown to feel like Steph is like a 2nd Mother to me is bc
of her guidance along the way of this journey I have been on the past 3 1/2 yrs. She has been there to listen to me, to talk to and to support my endeavors and introduce me to others at genzyme who became friends to when she couldnt be there. I guess I know her not being my CM doesnt change anything with our relationship I just will miss her help with questions and her just being there to help when I deal with different insurance issues with the 3 I have.

I know there is nothing I can do to change not having Steph now but I would like to say here I am glad you will still be around as a friend and alli (sp?) Steph and I will miss your help but I have been lucky these 3 years to have you. Thank You.
_______________________________________

Monday June 2,2008
I saw my Pain dr today and she is re-doing cervical and lumbar MRI's next week bc of worsening problems and symptoms. I fell twice last week and now have a huge amount of pain in my neck/upper back and in my lower back upon doing any activity including even just sitting here typing. She thought the area was bruised in there where I already have significant narrowing (probably swollen now to) and that the area being 'fragile' already needs to be re-checked and watched closely. She started a new topical anti inflammatory
medication for the painful areas and increasing the IR med I take to help. We also talked about adding in another Neuromodulator at night and I think we where/are going to do this and should talk to one of them tomorrow. This would help any addt'l numbness, tingling and burning I have. At this point I take a relatively low dose of 1 and a moderate (high for me I think) dose of another both in different families but work at that Neuro-level.

Otherwise my dr was going to send a message to my local Neurol. about moving up my Appt. from July with her to sooner and about the new developments I guess. I do know that my
local Neurologist and I will talk after
I have the flexion/extension (great fun the way my neck is I bet) SSEPs/EEG
and she gets the results.

Other things included talking about my Shunt and my once again worsening H/As and which local NSg I should see. My Neurol. works with my NSg at FMLH and knows him well but he didnt want to do anything with it when she talked to him(I wish I could quote what my dr today said-it completely sums up my relationship with that NSg! it wasnt bad or anything just not sure she'd want me to put it here-it went along the lines of the difficulties her, myself and my other providers have had with his office though and neurol. at some pt would experience it) So anyways
back on track my dr today and I talked about Dr.D the new NSg she set me up with she thought he would look at the over all picture and consider moving the shunt. I guess what I need to do is
talk to my neurologist..

We also talked about spine surgeons again and though she and I both think it would be easier and almost better to
have decompression/fusion done by the local Spinal-NSg my FMLH Neuro team reccomended (this spine dr is also in their staff) when we talked about the nitty gritty of the MPS playing into the picture we thought the Ortho-spine surgeon in Mn who has extensive experience with MPS spines should probably be the one I see again, have re-eval. it and do surgery. As my dr put it "you don't want to be some spine
surgeons 1st MPS case even if they have
alot of experience and are good". True

I forgot to add this yesterday I got word back from an Accupuncture provider
I contacted on Sat. She has her MSN in Surgical Nursing and her experience in that was in Neurology and Neurosurgery.

I don't expect i'll know much this week
but will update when I do or have something to say. ....

Sunday June 1st
It has been a slightly eventful last week - interesting if nothing else! I moved a week ago and our new Apt. is very lovely, very nice. My Nephews love the park right on site, if fact my soon to be 6 yr old Quinn woke up this morning, threw on clothes and went out to the playground right away.

It is a half a day later and back home we went on a family picnic up to Oshkosh-rainbow Park which was nice. Quinn and I and Dad went fishing (yes i even baited my and Q's line!) on the shore and Sara, Zander, Jordan, Chris and Mom
went around the park, did the few rides, swam etc. The highlight of the day you might say was feeding and petting the ducklings and their mother in the water. Incidentally I
later went on to bait the Mother Duck accidentally (she went after my bobber and bait) and after we finally got her and the line out of the water, we had to hold her and work to get the hook out and then not 2 hours later one of her little ducks hooked on to my line and I ended up holding that duck to while we un-hooked him! We did catch 23 fish in probably 2-3 hours but let them go.

On friday I received an email from a Patient Care Liason at
Genzyme whom works on the East coast - this was the first time 'meeting' this person whom was very nice when I later called her. She is working with a newly diagnosed MPS I family in VA and had gotten my name from the US Aldurazyme Marketing people as a MPS I patient and contact to run questions by and to ask for suggestions I might have to give to the family. So I called her back vs emailing and we talked for quite awhile, she was as I said very nice! I was able to give her a list of suggestions and help for the family. She is going to meet them tomorrow (monday) and
then said she'd call/email me an update later in the week probably fri or so. I'll update and I will probably talk to the family at some pt to)

I am in the very early stages of work on a MPS event for later in summer, most likeally early fall. Right now I am contacting Golf locations and also have some new Physician
contacts whom are the Directors of the resident training in
Internal Med and Med-Pediatrics at MCW whom I just contacted late last week about a resident-education speaking on MPS Awareness.

Another thing I have been working on since late last week is my nephew Zander and getting him in with the Child Dev.
providers at CHW and received a contact back the next morning from the Director (head doctor) and he asked us to
send more infor. on Zander and also contact numbs. Fri that
office called my Sister and she is to call them back tomorrow (they called her cell not her direct line at work)
I am helping Sara get Zan in there and will call for her and ask and write down the ques. she has and also pick up the questionnare so that she can fill it out right away and
I can re-drop it off tues when I am there for SSEP's and EEGs at FMLH.

I otherwise have just had a few things going on medical wise - last week I fell half in and half out of our boat when trying to sort of 'jump' in to it from the pier. Yes I know hind site a really stupid idea, but was pretty funny site atleast. Then that same night at the bottom of my parents basement steps I fell and hit the hard cement hard and so since thurs have been extremely sore to the point I can't do much (even taking a shower my lower back aches so, so badly. My neck is painful to the point I can't even tip my head back enought to rinse the shampoo out it makes quite eventful! As you can see from above I havent stopped doing things and just sit alot doing acitivities like today (some anyways) and as well see my Pain dr tomorrow.

I will update whatever else I have forgotten later or otherwise when I hear any news on the new MPS I family and how this meeting went with the PCL I 'met' on Friday after she calls me again. =) Otherwise just working on alot of other little things and need to go take a shower right at this moment.

Love,

Erica

PS: PLEASE SIGN THE GUESTBOOK!@

Tuesday, May 20, 2008 4:54 PM CDT

I am sorry I havent posted a new update since I had surgery last week.. -- The overall day was long (as are mnost on any days i've had surgery?!) but the Anesth. where
well prepared and did their homework ahead of time. I asked them ahead of time to write down for me on a separate
paper what type of intubation and size they used (grade) and they did for me which was nice. They 1st of did a Laryngoscope to view my airway (after I was asleep as this encompasses passing a device down) which told them my Airway is at the most severe 'Grade' where nothing including the Epiglottis can be viewed. -- Anesth. rate these as Grades 1, 2, 3, and 4 with 1 being least severe and 4 being most with nothing being visualized as they intubate. As noted I am at a 4 whereas 1 year ago this level was at 3 in febr. 07. So my Anesth. (2 and 1 resident) chose to use LMA #3 as the surgery was relatively short. (2- 2 1/2 hrs)

My Surgeon had planned to have Mesh available but did not think she would have to use it if my hernia was small enough but afterwards she came and talked and said that it was Larger than she had originally thought so she did place Mesh around it. This will give a larger likealy-hood of the hernia not recurring again though atleast. (2nd repair-the 1st was as a kid) I have to tell you that surgery was so incredibly painful and i don't want to go through it again and am SOOOO thankful it is this Tues. and not Last week at this time! I could barely move, walked completely hunched over and it hurt to do anything! Atleast now it only hurts to get up from a lying position, drive over bumps, and to cough! I saw my Pain dr yesterday and she wasn't real pleased with the Pain mngmt used as she said they should have given me a PCA pump and then I should ahve went home with this on top of my original pain meds I am on daily.

Off to look at a new Apt right now - yes we want to move again where we live is a DIVE! We have trhe Landlord from H**l and I don't say that lightly! Since I won't post this till I get back will update on the new Apt if we like it! ----- 2 hrs later I think we are moving again, lol the Apt. is open already (a HUGE multi-bldng complex) this is also in Waupun on Rensway Ct.

I have to email my local Neurologist (FMLH) and update her on a couple things my Pain Specialist and I talked about on Monday (19th) including my having more and con'ting to increase 'mini' spells which may be autonomic or I guess she thinks more likeally neurologic in nature. One other thing was some swallowing difficulties that have been occuring over the past month which I would describe I guess
as difficulty in getting the food all the way down. There seems to be something else but I can't think of it at the moment. (have it written down)

The other thing from my Appt. yesterday not related to my Neurol. has been the 2 Labs we have done (Pain dr) have shown that my drug (medication) levels are non-existent (0)
even though these are drugs that I take in a 24 hr release form and then 1-2 addt'l pills of immed. release (IR) and the labs have been taken always just 4 hours at the longest after i've taken the Extended release (24hr) capsule! Another medication I take the Labs have shown and incredibly low rate in my blood stream as well even though I also take a high dose of that. So my dr is checking blood-metabolite levels to see if her nad her research partner can figure out what is causing this she said it is likeally being broken down and binding to proteins in my body and then what is left goes through very
quickly vs the drug staying together and thus lasting the 24hrs. I may not have said that right or even close to! But she is also setting up a research study if you will in a few months where they will do Enzyme assays of how drugs are metabolized I guess and she said this would give us even more explanation about why my levels are what they are and help explain why I thus feel symptoms sooner.

I can't think of what else I was going to update - am sure there are some things but will update soon, again.
Love,

Erica

Thursday, May 8, 2008 2:18 PM CDT

5/13/08 - Surgery FMLH

5/19/08 - Flexion/Extension SSEP's 8:30am
EEG's 1:00Am
Pain Specialist 3:00

1st my surgery is scheduled for next Tues. and I have met with the Anesthesiologist who was quite well informed and also spoke to my Neurologist.
2nd I spoke with my Neurologist at FMLH on Tues. evening - I must say she was very sweet considering how many times I
missed her call due to being on other calls! (2 differnt days)
-So she is ordering the above testing as this is what both her and my NSg at FMLH felt might yield answers to the H/A's. She also brought up the idea that her and my NSg talked about of having a local Spine Neurosurgeon (yes, in addition to my Nsg. for the Shunt. I really did not know what exactly to do ie: having saw the Ortho-Spine MPS dr in
MN and likes him although I think honestly he may have been
blown away by the fact I look so 'un-MPS'. )(and yes I know
very improper English!) That said I would have trusted him
and Dr.Charnas very much trusts and likes him. So we decided my NSg will put in a referral to this Spine-NS colleague of theirs at FMLH and I will see her. Either she can just follow my care with my local Neuro team and I could eventually when it is decided have the surgery still in MN or I could have her do it and she could communicate with the Ortho-Spine specialist in MN about the 'different-
and complex MPS spine surgery. So I will update when that is.
There where a few other things we talked about but those are the major areas and i'll keep updates.

I wanted to put something here that I have been thinking more about on and off since last week. I think that people who are not around me or really talk to me enough have this pre-conceived notion that I only focus on my MPS... so this said... please remember all that I started and keep this blog as a means of educating others but also as a source for my own self to 'talk' for my sanity. =)

--I guess I do not talk enough perhaps about the NON-MPS things I am up to here and I should maybe so everyone can try to grasp my 'normalcy' to the degree it is. So here goes as they say 'NON-MPS' things I am doing (although I find absolutely NOTHING wrong with the time I do spend working on MPS things as I do it purely for others and the enjoyment I get is my exchange for the loss of the career I really wanted but no isn't possible in the way I want. (Nursing) - I am taking a class and going back to college this fall taking several more classes at a time at my pace. I spend alot of time with my nephews and this summer will be watching my oldest nephew 3 days a week, including his trucking with me to Appts when neccessary as he for whatever Quinners (his name is Quinn) reason loves this and we'll make fun trips of it doing things. And on top of that I am applying at the moment to be a Camp Counselor in MN at a Camp for kids with heart disease which my cardiologist also volunteers at (there are 3 different 1 week long camps to choose from and this is something I had been accepted to do 2 years ago but at the very last minute had to have very emergency surgery. So anyways a crazy busy summer which is what I like.

I think people under-estimate me my providers and friends that see me enough talk to and hear me (I mean local friends I don't mean what I am saying as anything against my friends-you guys.)
know that I have a divide but I also don't believe in trying to live 2 lives 1 in MPS and 1 not in MPS - for me it is a continuum 1 day I have MPS things to deal with say many Appts and I may feel great which is a strange feeling and then the next I may have non-medical things going on but feel worse, my body may not be at full speed.... I am always amazed that how different each day and each night and each week are for me and for how I feel - an it has nothing to do with how much I focus on this disease, it is just what my life is. (I think some under-estimate how much time it takes with complex issues to be your own co-ordinator and make things flow to when there is many different areas going on!

I think people don't realize that - and I have to say this may be the HARDEST of things - I look good, I don't have all the normal MPS features and so people figure well I must feel fine, I must be ok. But the thing is a person can put on maKeup each day, a person can dress niceally each day and style their hair but that does not mean that they feel well, it does not mean that MPS isn't kicking my butt! Sometimes the days I don't wear makeup and I dress the most casually are the days I feel the best bc I don't feel like I have to put a front to the world.

Anyways I just don't know I don't think I will ever stop having to explain myself and my life and how I am and live - it is just what I can do and is what I want right now. I am working to make my life better, but I also know even if I get that college degree I may or may not ever use it in a job but it will be another accomplishment for me. I just wish people would stop underestimating me and listen more or perhaps realize that I am doing the best I can and just support me. I have a great support network, really great I really do, just somedays it is just hard when you havent slepped in days and the neck and headache pain is so fierce you don't know what is up or down and you have providers on one side all out campaign to find answers and providers on another side second guessing and pushing when maybe all you need is to talk about someting completely different to them and they could just once not bring up those topics and try to step into your shoes. Anyways sorry, I am waaay rambling - I am sorry. It was nice to write these feelings, thanks for letting me.

I hope this may give or help in my trying to
give a realization that as MPS progresses you do what you can each day and each week and sometimes you can't always look out at the huge, big picture it doesnt mean you don't try to plan though. And sometimes it is just nice to, to focus on the MPS but for someone else, not for you. IE: the get-togethers and events, and such I do are for others not for me and yes it seems like alot of time on MPS but it really isnt and better though it helps.

Think about MPS and what it is and means to an MPS Adult and realize it is no cake walk but this just makes us stronger and more self-reliant and better and advocating for our needs.
I Love you all,

Erica

Saturday, May 3, 2008 10:21AM

Pre-Op Surgery Anesthesia - FMLH 5/6 10:30

Surgery FMLH 5/13 @ 10:30

Hi All, I am just updating a few things
and also with the surgery info. ... I am currently surrounded at my Apartment
by a flock of very noisy kids! Zander is climbing behind and taking out his toys including as many blocks as he can master getting out all over our living room!! Quinn and Jordan are throwing there 'Dora' ball at each other and then tumbling all over the Dora chair. (both Zander's) Needless the action here is liveally!

I spoke yesterday with my GC for quite a while - I had been awaiting a call from my Neurologist (FMLH) and of course as things go she happened to call while she was leaving work and I was on the phone with my GC! She did leave a VM with some info and said she would try to call back on Monday. She has info for me after having spoken with my MN Neurol. and NSg. She also said in the VM she would like to speak to my Anesthesiol. before the surgery on the 13th.

The conversation with my GC was interesting - we don't have info yet on what we are trying to do at CHW for MPS Awareness and we also talked about the surgery and other medical things going on. Then we got to a heated debate about my future and potential career and potential work which anyone who knows me really well knows that I don't have a cake walk for a life - not that it gets me down just that it is consistently alot of mmedical issues/health wise. I don't want to elaborate on the whole of the conversation but we eventually agreed to disagree I think somehow I need to give providers like my GC a better glimpse into my life seeing how they see me for an couple hours maybe 2-3-4 times a year. I think and I know that alot of people especially my new providers agree that I handle all of this MPS things remarkably well and am a fighter who is always, always looking out for my better interest in finding the best potential answers. One thing my GC said was "what if you never get better than you are now, what if a couple years from now or more you feel or are worse than this then what will you not work then either?" I think that some of my providers are just waiting for me to give up or stop fighting or waiting to find a hole in my exterior and interior person but i'll never give in nor will I ever stop defnedinbg myself or fighting to find answers bc if you stop doing this then who will care for you and who will look out for your (my)best interests? As one of my providers says I am my own best advocate and I know my body better than anyone else and I should always push for what is right for me.

I do want to say that despite the debate my GC and I had/have we are still close and will still work together this is just the type of relationship we have - to keep each other on our toes. As she said she will always bring up these things to keep them in mind and is always trying to look out for my interests to.

I will update after Mondays Appt with Pain dr or if I talk to my Neurol. otherwise after Anesthesia/pre-op Appt on Tues.

Wednesday, April 30, 2008 5:22 PM
Hi All,Post-General Surgery update. (a day late sorry)
I saw the General Surgeon tuesday and after his disbelieving there was anything wrong I told him to look at the Ultrasound as that was why my Internist sent me so quickly. So he went out looked at it, and then came back in with one of his colleages (he is chief of general surgery so wanted her to do my surgery) and said that in fact I did need surgery fairly quickly and this dr would do it and so her and I talked. She is going to use Mesh to repair this as this is on my Umbilical area which was already repaired when I was much younger. She said the total surgery would take about 2 1/2 hours (given the bowel/fat entrapped whichever it is) and would be done under General Anesthesia - I should get out the same day.
Will keep updates.

Now if I could just get some sleep! Will upload new pictures hopefully tonight, have them on a CD to put on computer now.

**************************************
Tuesday, April 29, 2008 8:34AM

I'll have to come back and update this later but yesterday I had an Ultrasound ordered through my Internist at FMLH and which the results I received within the hour after from her office. I apparently do have an Hernia and it is caught with Bowel which means it is an emergency of sorts apparently. I think they call that strangulated hernia. Anyways so I have to go back down there this afternoon and see the General Surgeon.

Last week I saw my Pain dr and we took away the pain patches I was using due to rash and breaking out severely.
She started a jelly form of Lidocaine and as well we talked about starting Joint injections into my spine. She is first talking to my neurology team in MN and we will reconvene on the idea next week. She did say she wanted to makea decision and move on it within the next month. What I know is I truelly cannot wait for something to help this back-spinal cord pain.

I have been emailing a little with my local Neurologist (FMLH) and she was waiting to talk to my former NSg. - I hope to hear from her soon with what she talked to my MN Neurol. about and what her ideas are for moving forward. I did get her report yesterday and she states that (and I knew) she is still concerned about the possiblity of pressure causing my headaches and hte dynamic situation of the shunt in my lower back and then the spinal cord problems in my thoracic and cervical spine. (thoracic is less stenoses than cervical which is severe)

The beginning of June I will be a chaperone for my Nephews class trip - his Mom called and asked me. Quinn and I also will be spending much more time together this summer as I will be watching him several days.

I'll try to upload newest pictures soon. And will update after Appt when I can and If I learn anything else new.
Love,

Erica

Friday, April 18, 2008 7:05PM

Hi All, I just wanted to add an short update tonight. I saw several of my drs
yesterday and although the over all Appts. went well I did let down one of my drs regarding something I misplaced. I feel badly for this matter and although I do not think I can elaborate on what it is, I know I just need to be even more organized and
careful. I guess the reason I write this here is that I want everyone to take in to consideration that your providers are human and that when they truelly care try even harder, beyond your hardest to honor the relationship. I hope this makes sense. I have been so lucky or rather blessed in the last 4-5 months since having this provider come into my team, there have been so many less struggles, and so much less problems over all. Yes, I still have all of the same health issues but with this provider she has helped put together a really great 'new' team to work together with the 'old' team and we have been working ever so steadily to get on top of different symptoms. Definetly a slow process but non the less I am getting to a much better place than I was last November! I would be lost without this main provider she is sweet and caring, and at the same time in charge and keeps you in line if you move out of place.

I received a call from my MN Neurologists office today as they needed to reschedule the Appt. in May. So we where talking about when to make it for and then she went back and talked to my Neurologist to ask him something, which he wanted to talk to my local Neurol. So I got ahold of her and she told me that she was going to get ahold of him today, he was on her list to call as it was - otherwise she was hoping to reach the others by the end of next week and would get back to me. So I might be getting some answers by next week.

I will keep updates.

Wednesday, April 16, 2008 8:50 PM
I promised I would update when I could after the Appt. with my Neurologist so here it is. I saw my local Neurologist today (at the Medical College) - although she has been active on my medical team since December I 1st met her today. I can say she is extremely thorough and very nice as well as really wants to cover all bases and tread the waters but by that I mean talking to other specialists further over the next few weeks and discussing with them some options or ideas she has. She has been in touch with much of my medical team already before today and especially with my pain dr. but wants to email my MN Neuro team as well as my specialist I saw in CA and talk to them about options and treatment choices.

She told me today when we talked about my former NSg that she knows him very well and would work to find out for me where the 'melt down' happened and why he stopped "playing nice in the sandbox". In other words why he stopped doing anything as far as helping us (my medical team) find answers. She really wants to have him back on my team as she (and I agree) said he is a great surgeon and nice guy. - Incidentally I (we) do think much of the problem lay with his NP.

I've never had a provider say this to me but at the end of the consult as we where saying our good-byes she said "thanks for choosing me out of all of the Neurologists to join your team and your really great group of providers - I look fwd to working with you." I thought that was a realy nice and unexpected comment.(and I do have a very good team)(she is sending me her report as she said she wants to make sure she got it right, which is always a good sign of a great provider)

Some of the tests she is considering include Autonomic Nervous response (ANR) , more SSEP's this time doing flexion and extension and full body Imaging-MRI as well as potential EEG.

The things she is putting to my other specialists is whether or not my Spinal cord problems are causing the H/A's (likeally) and what about moving my LP shunt to a VP as the LP shunt I have now is acting like a drain and causing worse pain and even probably the Headaches as the tightness/stenosis is severe and when I lay down fluid is most likeally not able to get through down to the Shunt. - The other thing is doing the actual Spinal Cord decompression and fusion and at the same time reassessing the shunt/drain. Then the 3rd thing was to wait and do nothing and continue to just monitor very closely.

She thinks that the blacking out spells and also the twitching are likeally related to the spinal cord and to the ANS which the testing would help reveal.

Tomorrow I see my Pain Psychologist (colleague of my pain dr) and as well as my Pain dr. I will add an update if there is anything new after or when I get a chance.

Monday April 14, 2008

This week I see the Neurologist at FMLH
- she is the specialist my pain dr set me up with so as to have a local Neurol. to. I will continue to see my neurologist in MN several times a year to as he is the 'expert' on managing the Spinal cord issues I have. The local Neurologist we are hoping (my dr knows her well) can help us with the blacking out spells as well as the twitching that is in my hands and feet.

I see her on Weds and then on Thurs I see my pain psychologist and my pain dr which should go well. I did misplace one of my scripts which I needed to refill and so now am doing other measures to manage the pain as best as I can until Thurs.

I found out the other day that I was eligible to stay on my parents Insurance which is excellent news! I do have medicare and medicaid but the commercial insurance affords so much more ease and even opportunity as far as seeing drs in MN. This news made me extremely happy!

The other thing going on is that we are packing and painting and moving to a downstairs Aprt. of which we hope to be completely done and moved in this weekend!

I will update when I can after the Appts.
Thursday, April 10, 2008 2:35 PM CDT
Last night I happened to flip on the TV and while going through the channels I came across ABC 2020 w/Dianne Sawyer. The hour was based on the man Randy Pausch and his work at Carnegie Mellon University as well as his journey through Pancreatic cancer and ultimately his dx of 4-6 months to live. The reason I ended up watching this had nothing to do with this man's illness but because of his truelly awe inspiring personal and emotional acceptance of what his life was and his desire to help other people. He co-authored the book 'The Last Lecture' of which I just ordered online and this is based on his Lecture given to colleagues, family and friends and then widely spread throughout the internet via YouTube. The deepest piece I took away from this Man was his will to make a lasting impact and to leave a legacy for his children. His quote " I was unlucky but it was not unfair" deeply resonates with how I feel about my own disease and desire to make an impact.

I have been pondering the thought of starting to slowly write a novel, I thought since I come and write here and so many providers, friends and people have told me I should write a book I wonder - even if it took me awhile I think a memoir would help raise awareness and be a tool for my own sake. I don't know though.

I have been seeing one of my newer specialists on a weekly basis, of all that I see she has been the most encouraging, the kindest and I think she has pushed me the most to stand up for me and be my own advocate amongst all that I see and work with. She herself is really such a sweet person and truelly wants to help - I have many, many providers who are kind and helpful, but this dr just takes that role to a completely new level of which I am so greatful to her for. The providers she has set me up with have also been very nice and helpful. Last week this provider said something to me that I thought alot about, she made a comment about her life as represenative to Real Estate - ie: how one person is really such a small piece of all that is around. I thought about this and thought about it, and came to the realization that although we are each only one small piece we can make as big of an impact as we choose and unknowingly many make very lasting, large impressions to everything around - I firmly believe that we can be just like a great piece of real estate, when we put in a little extra effort we makes everything around us worth that much more. I think that we all should think about this and make the biggest, best impressions we can but in the kindest possible ways.

I need to sign off now but will write more soon.
Thanks to all who follow my writing. In just 3 years we've had 9773 sites!

God Bless,

Erica

Saturday, April 5, 2008 5:26 PM CDT

I just wanted to add a short update and will try to do the rest later. This week has been a little rough but over all I guess pretty good. I wanted to share something my dr thinks may be going on though - lately for the last few months in addition to having twitching and spasms in my hands and sometimes legs I have been experiencing what I thought of as really just falling asleep standing up. Well my dr and I where talking about this in the setting of my recent sleep study (2 weeks ago) having come back normal (study was done per this drs request but through another specialist I formerly saw) I see my local neurologist in another 1 1/2 wks and my dr is going to have her evaluate the study results and potentially do other testing. Well back to the falling asleep - this has occured on numerous occasions in multiple settings at multiple places and including being in the setting of sitting down and this happening to standing up and having this occur. My dr thinks that given what has been occuring that I may be experiencing seizures at night (frontal lobe I think she said?) and to tell my Neurol. who she would reccomend to do EEG's and testing. Apparently this can be treated but what I thought was relatively innocent may just be another affect of the Hydrocephalus having gone untreated for so long (no fault of anyones really) or just another effect from the MPS and it's storage on the Brain.

On that same specialist front we have the new sleep med Rozerem on board (a prescriptionthat has Melatonin in it) as well as the new NSAID Pain Patch Flector.

Other news would be working on getting the ok to hold the MPS Awareness Events at the Hospital both CHW and in MN and having close contact with the Genzyme people I am working closely with. If for some reason we cannot get approval then we have back up plans in place.

Will update again when I can.
Love and God Bless,

Erica

Wednesday, March 26, 2008 7:35 PM CDT

After all this years of diagnosis, I am astounded yet how callous and un-understanding (not a word I know) my siblings can be. I don't need life to be all about me, the opposite is true in fact - I would rather help someone else than have someone help me. But I just can't believe how superficial people can be, especially this person - it is like it is her way or the high way, the minute I have a bad day or a few bad days ie: tired and cranky I am a well you know with a temper no matter that I still support her 100% and listen and help her out and am there for her. Does she ever stop to think maybe I could use her to be there for me to? Instead of being her piece of garbage who comes in 2nd. I just completely don't get her and wish she would realize what she is doing to herself, her 'friends' (myself included in there) and see how she treats people. All I want is for her to act like a sister and a friend and a roomate and not use me and think about the things she says taken into context of how my every day is compared to hers.

Last night I had the sleep study at FMLH which went fine until the part where I had to lay down - that anxiety feeling I get when I have mo nitors all over me and am in an unfamiliar place. This is truelly only a feeling i've gotten at each surgery i've had and at studies like this where I am in the hospital. The feeling did dissipate after a while thankfully. I guess I fell asleep after awhile but woke up repeatedly and as well as the Tech haviong to change around monitors. I should get the results soon.

Tomorrow weather permitting I see my pain dr again - we are on a week to week basis, I havent been able to implement any of the meds she chantged and added last week so I don't know what this Appt. will go like. I do know I truwlly, truelly hope she can help with this pain it is so bad.

I need to update some other things and will tyrto do that soon here.

Thursday, March 20, 2008 1:12 AM CDT

I wanted to add a new journal for the first part of this week but I also would like to put here a quote from another MPS parents site and for which I thought was fitting.

"It is my privilege to walk this road less traveled; the road of MPS I."

This week has been stressful at the least on an physical and a little bit on emotional level to. Mostly on a physical level though as my body just is not happy and I do not know how to make it happy. Between the lowered opioid dose from a week a 1/2 ago and the increase in neck pain and H/A each day was rough enough, then add in a haphazhard sleep schedule (in the middle of a sleep cycle issue again I guess) and to top it of stomach pain in the belly button area I could use a break! Then there is the issue of all the Appts and on different days and trying to consolidate days and so many this weke have been cancelled making me want to just about cry at some points! (frustration although I do understand things happen and are out of all of our control)

I want to write about a conversation I had with one of my drs today, since her and I work on the pain things from a more body/emotion/ approach we have been doing things like guided imagery and today something called progressive muscle relaxation. In addition to working with these things with her in the past 3 visits I have slowly gotten comfortable with her. (I am so lucky to have hte new team drs I have, including her, who are so open, so personable and so easy to actually talk to!) I usually have a hard time talking about myself, my fears, my expectations, my life in general and even just how I am doing day to day - ie how things as individual and as whole are affecting me. I am much more the type to help others, to listen to other people and to guard my own self even to the very closest of people. Today I guess maybe bc so much has happened and so much is going on lately my unconscious mind needed to talk. (I hope this is all making sense, the screen is a bit blurry as I am extraordinarily tired!) This dr and I started to talk about last week and how I have been feeling and I actually talked to her about things I havent ever talked to anyone about and I have known her for just a very short time! I think the thing is you can tell when someone really wants to listen, when they care and when they can sense almost from things you might say what is going on in your mind w/out your ever saying full out about the particular topics. (I will elaborate I promise more in a day or two and am sorrry this is confusing!)

Something we talked about today and for which I've never I don't think talked to any other provider about is mortality, my expectancies for how long I will live and what my drs have told me. Another thing was accepting that I do have this disease which has caused disability.

There is just so much I want to share and this is in a very different type journal/blog then I normally have done I think in a long while. (and I honeswtly think she would not mind my sharing this here. My dr today asked me about how I feel about not having a job (not asked in a judegemental way) as I talked about my fear of disapointing people especially family and even more so providers bc I already have to do so much to gain credibility for myself. I talked about my not having a normal job and how that is ahrd for me and how some of my providers had for so long been pushy about that issue - I explained how I think of my job now as helping other MPS families, raising awareness, and traveling so that I can learn the latest in medical research adn bring this back to my providers. I think of my job as a job that although not 9-5 it does take alot of energy, it does take mind/body effort and the most important is that I truelly am making a difference and have made differences and how many people in the normal world really say that when look back at their life? I hold a non-traditional, but completely important position in life. The reason I share these things are that I think it is so important for other MPS people to acknowledge these same emotions, to not be harsh on themselves for what they cannot do or can no longer do and instead celebrate the differences they can make.

She then asked me how I feel about being disabled and do I cope with that? And you know I don't know. I think I try not to, but there are so many reasons for why I struggle with it and many of the reasons come back to other providers and how some of them from my 'old' team have looked away or tried in so many ways to get me to do things that just are not possible.And have instead of helping, discouraged. I know, I know I am so evasive - just trying to not step on toes, but still be honest and yet talk about how I am doing.

Another topic I really want to share that we talked about was my life as a whole: She asked me what my drs have told me about how long I will live? Another thing She also asked was how long do I think I will live? - No dr of mine has ever ventured a guess, I just have so many drs who tell me I am not in the typical MPS I pt. - I do not follow a normal mild nor moderate pattern but rather my body has followed its own little schedule and done things not expected and still yet there are things I dont have that don't go along with things I do have wrong. So besides drs saying they havent ever seen another MPS I pt quite like me, no one ventured a guess and to me that is ok. For me I simply told my dr I had no expectency for my life here on earth bc I have no control over it nor does any of my drs and we can only appreciate the time we are here, make the best of this time and leave behind a legacy of some sort of what we have done and in the end although we leave our earthly bodies we go to a place so much better than this. God is truelly the only one who has the book written on how my life (and all others) will turn out and when the day will come that I leave my earthly body and its pains and join my savior in heaven in the most beautiful place of all. I talked about this and my dr just seemed so at ease with it, most drs just would not accept nor believe in this or atleast that is how it seems?

One day my heart is going to stop, and that will be the end of my body-- but not the end of me.

May God's wonderous love and glory be around you today and always. I this week walk hand in hand with my Lord to the cross/

I am going to end this for tonight and come back tomorrow or in a couple days and finish the entry as a whole for my blog this week.

Please stay by me - visit again.
Love,

Erica

Thursday, March 13, 2008 10:32 PM CDT

3/16/08 Palm Sunday

I wanted to add an update on to this journal for last week.

This last week Weds (normally a 2nd service in the WELS church is added on Thurs. nights but during the Lent season this is changed to Weds nights.) The Thurs night service is otherwise for those people who are unable to attend on Sunday. Anyways since moving over here to Waupun my Sister and I have been going to a different church but on Weds. we decided to go back to our 'home' synod and found the local WELS synod here. Our Savior Lutheran is a much smaller church than I grew up going to (and was confirmed in after attending the parochial school for K-8). Our Savior though just brought such a peaceful feeling, and a sense of calm. I have found my home again and while living here will attend there. It is so joyous to be upon our Saviors Holy Week - I so look fwd to Good Friday and Easter.

On a completely different note going back to how I have been feeling this week I feel sort of like I am walking on egg shells with providers - yes, true, many of my new team is so easy to talk to and work with but I still feel like I need to watch everything I say here and I really just intended this to be a sounding board back when I began it 3 years ago. (and a learning source for other MPS families) Now I have such a huge following of friends, providers, and medical people that I have to be more tentative about what I write. Earlier last week I wrote an email to a provider who I have had troubles with (many of the trials have been journey'd here) locally and b/c it had to do with who my providers are and where they are). Well I cc'd this in a separate email to a few people who I thought needed to see it as I felt they needed to be in the loop about what is/has been going on, being said and now I just feel like should I even call providers on issues that are not really of their choice? I don't mean this to sound like I am trying to be rude or like I know it all when it comes to my care but I am an Adult and do get to choose who my providers are, and who I think should be a part of my main team. I don't have this hard time with my new drs but with old 'in the beginning' speicalist. So I don't know. I just hope that things go ok and that Appts tomorrow are good.

I see my Pain Psychologist 1st whom I really like and maybe I will ask her about this all. I have a hard tme asking for help and talking about how I am feeling over all but I do need to talk to someone on this. After that Appt. I see my Pain dr whom I love, and after this week really am looknig fwd to seeing - in hopes we can come up with different options.

If anyone who has had Spinal cord issues and MPS has suggestions on things that have worked for them pain control wise please email me so that I can share with my team. I do know that MPS pts typically have a whole different body response to different treatments and so would love to know what has worked. I do completely trust my dr., she is great and is doing everytthing she can to help thankfully though.
March 13, 2008
----------------------------------
Hi All, if you've not noticed I updated new pictures from the recent trips above.

This week has been a little odd, but i'll get to that. Monday I saw 3 of my providers beginning with my new Internist and I have to say she is extremely nice and I think will be very nice to work with. We seemed to hit it off immediately and she does seem like she really is interested in helping in any way she can. The 1st thing she did was fill out the paperwork for me to get the Disabled parking placards for when I have to park longer distances or am just having a day when my bodies not working with me. After that I saw my Physical Therapist and then on to my Pain dr. which we are continuing to work on my care plan with the different pain aspects and this week increased the Lyrica again and also decreased the Avinza.

Now although I know it is good to decrease that dose and we need to, for whatever reason am having alot of pain in my neck and radiating from there. I am hoping there is something else we can do to help this until to either supplement the Lyrica or until we get to an effective Lyrica dose. I can't even describe the pain, have to try to think about that for monday.

Otherwise the thing this week has been sleep or I should say lack of - Monday night I could not fall asleep despite all the different things I tried I ended up being up about 24 hrs before I fell asleep for about 4. Then Tuesday night despite so little sleep the entire 24 hours before I ended up lying awake the entire night and not being able to even sleep the next morning I literally was awake for over 30hours then before I could finally fall asleep last night (weds). The only reason I finally fell asleep weds night though was bc of taking a sleep medication my dr had given me a script for the last time I had sleep cycle issues like this a few weeks ago. So we'll see if tonights better.

If there are other things will update later,
Night,

Erica

Wednesday, March 5, 2008 4:08 PM CST

This day March 5th is my 25th B-day and how more fitting than to be on the phone with medical providers, Insurance and Medical people all day (literally) I truelly do live the life of an MPS individual - there is no escaping this reality!

tonight my sister and I (who I share an Apt. with) and my Nephew Zander are going to FDL to have a B-day supper and do a little B-day shopping - she is such a good and kind sister. I am so glad I get to help her through her problems now with her separation nad I am even more so blessed that she is here to help me.

This past weekend I was in Texas as mentioned in the past journal for a friends Memorial service who passed away from MPS several weeks ago. Spencer's memorial service was beautiful, touching and moving and I was so honored to be there knowing that I had the experience and time of knowing Spencer for these past 3 years of my MPS journey. The Holand family truelly is special to me, they have been like a 2nd family to me many times over in the past years. This past year in addition to seeing them all at conferences I had Amy ewith me at one of my own IT treatments in CA. Amy always told me I reminded her of her kids so much and every time I was with them I truelly did feel a part of the family. It was great to see many of my MPS friends and Research/dr providers again this past weekend although under such circumstances it was truelly sad as well - we all shared many tears.
I had the chance to see my good friend Barb Lyons again as well as Patti Dickson and Emil Kakkis as well as other families I had not met but had either talked to or known in the past. I was able to spend an extra 2 days with the Hollands before coming home and wish I could do so much more to help them, in the grief and the days ahead. The little things I have been able to do just seem not enough.

Before I had left on Friday I was staying with my ERT infusion nurse at her house and upon leaving for the Airport I got my car stuck in her driveway. After much stress (and digging) I ended up taking a taxi to the airport whioch was a bit hairying. Then on Monday when I got home that afternoon I took a different means home but nin the mean time my infusion nurse and I had misunderstood each other on as far as rides and we ended up in a diagreement monday ngiht for which I was felt terrible b/c the day had already been very trying b/c both before and after deplaning I was on the phone with Insurance companies almost constantly - in the end on Tues. we got past it all though nad I guess are ok.

Yesterday (tues) I had many Appts. and in between was again on the phone almost constantly with providers and insurance companies. The Appts. with Endocrine and Physical Therapy and Pain dr went fine though and progress being made. The PT is working on musclew relaxation, massage, and gentle exercise which is somewhat very neat! She also has a class which works to retrain the bodies motion/movement, I need to ask my dr about ti but sounds interesting!

My pain dr is so sweet she has been talking to 2 colleagues of hers who live in texas and 1 of them who is in Dallas is going to see the Holland family - my dr and I just have to pass along her info - I am so greatful to my dr for helping me and for educating other drs and being willing to help others of my MPS friends to!

Today I got a little further with the Insurance companies and atleast will have my Medicaid reinstated thanks to a whole lot of persistance on my part (!!!!) (never, ever give up!!!) in 7-10 days and should hear from WEA by the end of the month hopefully at the latest.

Other things going on are that my primary dr - Dr.Z left her practice at the end of febr. =( so I now have a new dr at Froedert who is an Internist and has interest in Chronic disease and who Dr.B-pain dr set me up with. This dr has interest in my care and I see her next week for the first time. In addition I have been seeing a pain psyhologist also through Dr.B and we have just started working on guided imagery/relaxation also and next week will start Hypnosis with the other 2 things. This dr is very sweet and wants to help be an advocate to my other drs about my emotional health and the fact that I am not depressed as many of them try to say. Today I also found out my old Sleep dr is ordering a Sleep study for my pain dr and I - and this will be at the end of the month (25th) to record sleep (for possible apnea), as well as leg movements, brain activity, sleep patterns, lung/breathing ( can go along with apnea to some extent) and Oxygen level.
Next week I also have a Cardiology Appt. from the day when I passed out several weeks ago - I just havent been able to go sooner b/c I have been traveling so much.

Will update as things go.
God Bless,

Erica aka Affected Girl
those in Texas will understand thanks to a new nickname from AH lol!

Sunday, February 24, 2008 12:56 AM CST

Hello all, I thought I would just start a new journal entry since I never returned till now to finish the rest of what I was saying early last week.

before I went to Las Vegas I had been having alot of health issues - some of it maybe related to a medication I was on. I had been having these spells of waking up repeatedly at night and walking around the Apt. but then faling asleep in random placves for no reason b/c I was that exhausted, I literally was falling asleep standing up. This resulted in walking into/falling nito things, falling asleep for hours at a time on the bathroom toilet and just for no apparent reason passing out. Since we have stopped that medication things have improved and I am sleeping better, I don't feel as restless at night and I don't wake up as often. As a result of the passing out I am having to go for a Sleep study, go see my Cardiologist and had to make ER visits at the time of hte passing out.

LV was great, after some flight issues I made it there, only to run into two of my good friends which was great an unexpected treat! Weds-Fri reasearchers, Doctors and scientists from around the world (Italy, US, France, Germany, Canada, England, etc.) which was great - the presentations they gave occured every 15mins. and in between at the everyone mingled which was great - very, very nice to be able to talk to all of these different 'experts' from all over the place and all where so willing to shre their knowledge and where so interested in talking and fielding questions from me! Awesome! At teh Symposium I also saw my team from MN, my former IT study dr from CA (her and I talked several times and then while in CA we had dinner with her family which was sooo nice, I LOVE her family!!!). I also saw Bill Cummings from Genzyme and him and I had a great time catching up and brainstorming ideas - he is such an awesome person! I also saw the people I know from the other drug companies.

On Sat. instead of flying back to CA., Steph, Brian, Kat and I all decided to spend more time going around LV and then drive back (about 4 hour drive) and then on Sunday since we got in late Sat night we just hung out with Stephs's son Tristen who is 12, has Nieman Pick type B and is such a cutie, I loved him and we had a great time!!! On Monday we did a little site seeing and then went to dinner with Patti and her family. Tues. I actually off the top of my head can't remember what we did but Weds we went to Disnwey Land which was Awesome! And then on Thurs I flew home. I am so thankful to Steph and Brian for letting me come! And for anyone who doesnt know check out the Hide and Seek Foundation website! This Foundation run by Steph, Brian, and Jonathon Jacoby exists to raise awareness of LSD's, support Grants for research and create collaboration among researchers in the different disorders within Lysosomal diseases.

Now that I am home I am taking it easy but tomorrow I start Physical Therapy as well as see my Pain Dr. in which we will con't on our path to treating/helping all the pain symptoms. One of the ways my dr had talked about was through spinal injections into the areas where pain signal is coming from or I guess into the facet joints using Fluoroscopy and small needless which are coated with steroids and pain medication.

This coming weekend I hed out to Texas - a close friend of mine passed away several weeks ago at the age of 18 from complications of MPS I and I am going to attend the memorial service. His family has been a huge support to me and I want to be there for them.

God Bless,

Erica

Thursday, February 14, 2008 1:47 PM CST

Hi All, I am here in Las vegas at the World Meeting for Lysosomal diseases and have talked to lots of my drs as well as some of the drug/pharmaceutical people I know and has been great. Right now I am sitting at the Hide and Seek Foundation informational table and talking to people/drs. etc who come by.

I suppose all would like an update but it has been crazy so I apologize I will do what I can right now and more later.

Since being home from MN and the Bolt study I have saw my Pain dr and we are working on some treatment options. I also met my new neurosurgeon who is local who seems very nice. I also received a call yesterday form the local Neurologist and have an Appt. with her - Dr.P. and also seeing a new Physical therapist and having to find a new Internist as Dr.Z is leaving at the end of the month.

Last week I passed out which is either a Neurologic thing or more likeally possibly a Cardiac cause - I also have been changing medications with Dr.B and we went off of and changed around meds since then. My sleep has been on and off some nights better wth waking up 2 times and other nights 3 or more times.

More later. Love, Erica

Monday, January 28, 2008 9:01 PM CST

Hi All,

I have talked to some of you and so you know that I am
home from MN and was able to get the ICP Bolt removed
Friday afternoon after 5 days of the testing and then
moved up to Neurosurgery floor (after ERT infusion was
done) from NICU. I talked to Dr.Charnas and Dr.Haines
(Neurosurgeon) both friday night and Sat before going
home and we have a new direction to take for working
with the Headaches, managing them and who is on the
team.

I had the sutures removed with the Bolt on friday but
will have the 10 or so staples from the other incision
in my head taken out by the local Neurosurgeon who my
pain dr (whom I saw today) is having me meet later
this week. The other reason for the Appt with the
local NS this week is Dr.B wants me to meet him and
begin a relationship with him, as well as have him
evaulate the MRI films so he can watch this as time
progresses. I will then have this local NS as well as
Dr.Haines in MN - the local one will follow me, make
diagnostic decisions and then if we have surgery needs
say with the shunt then i'll go to MN and Dr.H will do
the surgery. Both Dr.B., Dr.Charnas and Dr.H have all
formed a working provider relationship on my behalf
and have come up with many ideas to try and help.

I will also be seeing Dr.Peltier, a local Neurologist
(she works with Dr.B) a Physical Tx. who they won't
let do very much except gentle massage of my neck and
spine and also a Pain Psychol. for several reasons not
just pain mnmgmt. In seeing Dr.B today she had come up
with some treatment choices with the 2 others and
which include going up on the Neuromodulators,
Spinal/Facet joint injections (if this doesnt work to
help pain then RFA into nerve/joint of spine) Both of
these are invasive but still the alternative to the
cervical decompression. Some of the other things she
is doing include having a Thoracic Spine MRI done in
the next week or so before I go to LV and CA (so we
can begin injections once I get back after the 21st)
She is also (as is my GC) helping me to locate a new
Internist as Dr.Z. my PCP is leaving her practice at
the end of Febr. =( (I see Dr.Z the 7th and am sure
she will give me help with a dr to if she knows anyone
down by FMLH area.

I think that is about it for update - so maybe
complicated news, but Dr.B today said this doesnt mean
it was un-useful news, (they learned that for instance
even with severe spinal stenosis ICP can apparently
still get past to the lower lumbar area.) Also it
doesnt mean that my headaches aren't from pressure,
just that it isn't overly high (at most on record in
high teens, 20's) and that my headaches are likeally a
result of the pain signal and sensativity to higher
pressures I have had in the past and not sure how else
to word it b/c I can't think of the right word but my
pain signal over-reading this brief/higher fluid
increases in the brain. So thus between that and
probably some H/A relation to the spinal problems we
have a new plan, it may be complicated and will be
alot of work but at the same time I am so glad to have
such a great team!!!!!

All for now, remember how precious our one and only
life is!
God Bless,

Erica

Prayers and Thoughts to Steve, Amy, Maddie and Laynie Holland ^Angel Spencer^ MPS I 18yrs old

Thursday, January 24, 2008 2:35 PM CST

So I think I owe this blog/journal to my Neurology team and
even hte Neurosuregeons - Dr.Rothman who has been following me this week for Dr.Charnas with his team of drs including Dr.Freeman (sp?) I hope I got that right.... came in today and teasingly said that he had a "bone to pick" he was wondering why I was giving all the free publicity and press/attention to Dr.Charnas when it was he who was following me? ;) I of course had to ask what he was talking about or how he knew who I was talking about and to who and he said Dr.Charnas reads my blogs and has been following them! Huh, interesting to know! Guess they realy care and are really interested! Just is to funny!! I do have a great group of drs and nurses here though. Since I hadn't updated here all week I am thinking someone is fwding my emails to my Dr.Charnas or he is going on one of the MPS forums and reading nwhat I write! They did say he thought it was funny how I wrote that Dr.Charnas and I usually have this back and forth commaraderie thing where we basically push each other to get our ajnswers if that is the right way to put it.... So anyways if anyone is in need of some great nurses or drs the U of MN Hospital has been great, Dr.Haines my Neurosurgeon and the drs working with him as well as Dr.Rothman and his group are very receptive and really want answers and to help.

So the actual ICP update I had the actual surgery to place the monitor on Monday under general anesthetic which was an experience since I have never been awake when they did the extubaation. Surgery was good, recovery ok - the first night I really, really wondered why on earth I was so stupid to do this as it hurt so bad and all the monitors adn stuff are a pain but each day has been a little better. I got off oxygen that night, the heart and ICP monitors are still in place as is the IV. I won't be home for ERT tomorrow so that means likeally a 2nd week of no infusion - I need to ask them about that today. Yesterday they stopped a few of my headache meds like Topamax and Diamox and today I actually have been able to 'reproduce' (funny I know) the headaches atleast mildly when lying down which is always my hard time. It is very odd but I guess typical that the headaches would not be around once I actually have the bolt in place and am in the hospital. It sounds like they are going to do the rest of the monitoring today in N-ICU and then tomorrow remove it or atleast send me upstairs to floor 6 not sure which.

I saw Karen O'Neill both yesterday and today which was great, she is sweet as can be and we are just goingto start planning an MPS Awareness type event at CHW and possibly here at the U of MN to raise awareness of the diseaes which wshould be really fun. She also sent Bill Cummings an email for me, and i'll follow up about the MPS I Pen pal program we where wanting to put together after the new year - I have just been to busy to follow up on it!

Today I talked to Barb Lyons out at Los Angeles she was on her way to Harbor - she isn't planning to go to the World Symposium in Las Vegas next month but when I go to LA with Steph Lynn her and I will get together which is just another thing I am so excited for!! I canj't wait to see her and Paula and Alla and of course Dr.D and hopefully her family!!!

So I will update when or if I know anything soon, or in the next day or two - and to Dr.Rothman, Dr.Charnas and all Neurology members on my team Thank you for your excellent care! =) To my Nurses here Thanks as well!!

Thursday, January 17, 2008 4:02 AM CST

**IMPORTANT PLEASE read entry from WEDS.
Appt. update from MN and Neurosurgery news/date here..
It was an extremely long day - a 5 hopur drive to MN then 1 hour Appt. and then 5 hour drive back but atleast the NS is/seems very nice. He is doing what is called a Parenchymal intracranial pressure monitor placement - which they shave the area, make the incision, use the drill to create the Burr hole and then insert the tubing in about 4 inches into the brain where it will be left for 48 hours. Unless they get an aswer in the first 12 hours then they will take out the monitor on weds this does not have to be done back under anesthesia so I will just be under general/intubation the one time. The Anesthesiologist will be Dr.Belani who has done alot of MPS I ariways. Dr.H 'did tell me that my Neurol. there asked him to just be the "Technician" for this surgery and procedure and that he himself would manage my care there and make decisions with the results from the Intracranial testing. I guess if the testing shows high intracranial pressyure then Dr.C will decide who best to have manage my care and if it should be Dr.H or if not him then where - perhaps locally and with what device. If it isn't high ICP then Dr.H said we (him, myself and Dr.C I guess) would go back and decide what is causing the headaches.

The reason for this slightly different, but innovative parenchymal placement of the ICP monitor is that b/c of my severe stenosis - they are afraid if they where to place the monitor farther into my brain in the ventricle draining fluid (with the placvement they are doing on monday fluid cannot be drained off) this would cause further problem with the spine and brain. He also mentioned somethings about my stenosis and pressure being different at the different levels/areas which is affecting the headaches. I hope I wrote everything correctly!

-*-*-*-*-*
Weds January 16,2008
I have really wanted to come here since Tuesday now - I need to clarify or perhaps try to apologize although more to a specific person and if he comes here I hope he will know it is for him. (I feel badly that I did something, and I wish you had felt stronger about coming directly to me to discuss whatever it is so we could have figured it out.)

I have worked so hard in the past 3 years to find my way in this MPS world, to pave my own existence and to find my own voice.. but it I think and I guess I now know that sometimes my voice does not say things in the best or most gentle way when I am talking about my experiences and I feel badly. It has never been and will never be my intention to hurt anyone, my friends, my family, my providers and I hope that most understand this. I know that I have those bad days just as every single person in the world does MPS or no MPS we all have our rough patches and I have come here time and time again sometimes to shed my tears, sometimes to vent and still other times just to share my experiences in hopes that no other person in the MPS world has to experience some of the difficult things I have - I guess I have learned in the past few months and am still learning that b/c I advocate for myself on my own but yet look so normal and also have the dis-advantage of looking so much younger than my age that I have to work extra hard and be extra careful in how I approach what may be a touchy subjects to some providers or I need to find an even better way to express myself without hurting anyone. -
I have always strived to be a gentle, caring person - it is the reason I had such a hard time with this MPS diagnosis 3 years ago b/c I gave up so many dreams I had and so many things I had worked so hard to do - I loved that I was working with people who needed help at that time in my role as a Nursing Assistant and at the same time con'ting on to pursue the Nursing profession b/c it was my dream for so long and it was hard to give up. The reason I bring that up in this is b.c it is a huge part of my I am so passionate about my own care with MPS now, I had those experiences of being one of the care-givers and I am now on the flip side being cared for. I have grown so much in 3 years and I think this febr. when I celebrate that diagnosis and my experiences with MPS these long and short 3 years I will realize even more how much I grew in that time and how much of an even better, stronger person I am. The biggest mark of how far I have come will be learning the newest research in Las vegas and bringin that back to share and then even more importantly that week after spending time in LA with friends feeling strong enough in my decisions to take time away for my self away from all of my medical things and just having fun for a week.

I want everyone to know this whether it be my friends or any one of my providers that I appareciate when you come to me, I have always been that person that people confide in and not that I am asking you to do that, just that I am asking you to trust me and to trust that I would never itnentionally do anything to place hurt on someone. If I do something to you, please tell me, I am unable to fix it if if I am unawares. I know sometimes my words are very strong and I feel unsupported but the simple truth is - I have so many of you as friends and alot of you as my providers have even become like family - the experiences I have had with you at meetings, conferences and speaking engagements and just out having fun when we have been able to meet up (for isntance when I go out of state to Appts.) has been amazing and many of you have shared so much of your time with me. Many of you have become like family, some of you (many) have adopted me as another daughter to your family. Some of you I always know I can count on to talk to and you are always there for me when I do have something on my mind that is bothering me. You are the reason I come here, I want to con't to share what I learn and to help others, my peace is now in making a difference in the MPS world and it is through each of you that I get to do this b/c you allow me to. The modicum of support, my own new found coping mechanisms over these years, my learning and your trust in me both providers and friends to share your knowledge to are what keep me in the growth and growing pattern where every person needs to be I think - myself most importantly to learn and become an hopefully even better person.

The time spent with providers and ones including above in firsat paragraph who trust me and listen to me, but also are willing to share what they feel and to share their knowledge - Thank You. I am sorry I broke trust to some level somewhere, I hope you will read this and I hope when we talk next we can figure out what to do to make our relationship whole again as dr and patient trust.

May God give us the strenght to give our best, learn from our mistakes and to always learn ot accept what we cannot change. (yes I know I re-worded this)

-----------------------------------------------------------

I do want to add an update for anyone who comes here but may not have received an email with the update or read on the forum - I have an Appt. in MN on Friday 18th with the Chair of Neurosurgery - I talked to his Nurse yesterday and the Appt. is to go over this drs opinion as well as to discuss the Intracranial pressure monitoring. I will add an update here later friday or over the weekend. This Appts. has been somewhat in the works for awhile - by that I mean my pain dr has been trying to get someone to see me since we could not get my former NS here to do anything while I was inpatient after Christmas b/c of the headaches and symptoms. She along with my local Neurologist had been workling with my Neurol. in MN to try to get the studies done there and from talking to this Matt yesterday it sounds like it is pretty well worked out.

I also received in the mail today from my commercial insurance a letter and form to fill out if I am on disability and would like to try and stay under my parents insurance under a continuation policy - so I am gathering info and going to hopefully get that faxed in tomorrow. Our LTm (lifetime max) which is one thing my Case manager and I had been pretty worried about maxing out was also increased to $2 mill. which would mean if I can get the continuation policy I would be able to hold the insurance for probably another 3-4 years. (Aldurazyme alone costs around $10-15,000 a week) I do also have medicare and medicaid which are my secondary and tertiary policies to pick up anything WEA may not cover which isn't alot but when I go out of state is some.

I want wish everyone a Happy and Blessed Day and may god Bless and Keep you in the Fold of his hands.

Erica

Monday, January 14, 2008 8:42 PM CST

Today I saw Genetics at Childrens Hospital which consists of my Genetic Counselor and Geneticists - they don't agree with what my Pain dr and Neurologist are doing basically - which is trying to get things with the drs at Johns Hopkins if Dr.Charnas doesnt get the Bolt study done by him. They also con't to argue/or push that I see my providers all at one location ie instead of going to MN or elsewhere in addition to local docs. They are taking the most recent MRI from when I was inpatient at FMLH the 27th-31st to their Radiology drs to get their opinion on what they say about the most recent findings - the FMLH drs think that there is Severe Spinal cord stenosis/compression from C2-C7 whereas on the previous reports it showed only C2-C3.

I see DAr.Bratanow tomorrow but talked to her on Saturday she called me - she had emailed Dr.Charnas finally b/c they where unable to get ahold of each other and she said she basically laid it all out in stone how bad symptoms have been and that if he wasnt going to do the testing that they where looking into other centers that had more experience with Hydro. Which is another thing I pointed out to LuAnn today - that Dr.B knows alot about Hydro she used to be a BMT dr and so seen alot of this and has alot of experience with it. Anyways Dr.B said that Charnas wrote back and said they where working on it. But she was still going to check with Dr.P my local Neurol. to see what she has found out about Hopkins in any case. I see her tomorrow and will update after.

There isn't a whole lot else to update on right now.
Love,

Erica

Saturday, January 5, 2008 10:27 PM CST

New update added see bottom of page dated Weds 9th.

Hi All,

It has been a busy few weeks both on the emdical front and on the home front - so I will try to give a shorter or condensed version without leaving out important details.

About a week before Christmas I started to have BAD Pressure Headaches - worse than they have been and just alot more symptoms like nausea, vomiting, vision problems, side and shoulder pain (from shunt) My Pain dr and I are always in close touch as we have formed a close relationship and become fast freiends I guess you would say, she was extremely worried and had me go to the local ER after a couple days of this - which they sent me home b/c the 'typical signs' where not there. (papilledema which is pressure behind the eyes and no increased ventricle size) Note: in MPS I pts. these symptoms rarely if ever show up and in my case I have never exibited either despite all of the symptoms of Hydrochepalus being there - this is usually the case b/c with MPS I pts. it is thought that the Mucopolysaccharide storage (GAG's) build up on the ventricles preventring them from expanding normally wereas in a non-MPS Hydro pt. they do not have the GAG deposition so thus their ventricles can enlarge.
-- So my symptoms became even worse to the point that I instead of going to my ERT infusion on that friday went to my Nurses house and slept I felt so badly. My Pain dr was still urging me to go to the ER at Froedert (where the Neurosurgeons including mine are) where they could admit me or at the very least do testing. That occured over the weekend right before Christmas, I did convince her that I should wait until after X-mas to go the ER and she ok'd this after a point b/c I think she realized I really, really did not want to miss the holidays.. her and I just kept in very close touch over those enxt few days and including X-mas.

Christmas turned out to be ok, X-mas Eve which is the day our Family tends to do more of the gift opening and big traditions I was pretty crummy and in a daze - X-mas Eve was better. That Thurs 2 days later I saw my dr in clinic and after still much convincing on her part I did end up going to the ER at Froedert. She had gotten in touch with a Neurol. she used to work with their who she wanted to have take me on and who had agreed and they contacted the ER people and expedited my being admitted inpatient. I ended up their 5 days.

More tomorrow have to go quickly, will finish all of the updates then! Sorry.

Weds January 9th, 2008 -
I am sorry I have not updated or I should say finiushed the earlier journal entry I started before now - I have been moving all weekend and still settling in, ultra busy all week
so thus getting to this entry now.

I ended up being in FMLH for 5 days and getting out on X-mas Eve b/c the drs could not get anything done the Neurologists on staff wanted (as did my admitting drs) testing of my shunt done but the Neurosurgeons would not do it and so I ended up basically leaving with still no answers and on Steroids to deal with the headaches.

Dr.Bratanow as well as the local new Neurologist Dr.Peltier are thinknig now that we should go to Hopkins and see the Hydro specialists there - apparently that center really specializes in Hydrocephalus and the various types and despite their not having any MPS experience my 2 drs think that maybe they will give us an good opinion that is not clouded b/c I am NOT the typical MPS I pt.

I saw Dr.Bratanow yesterday and she was going to talk to Dr.Peltier and they where going to begin the contact process to be seen there. In tht mean time Dr.B also has 2 local NS's she has a close working relationship with that she thinks would be good choices for me to be seen by on a local basis (I'll still need a local NS and we are nott going back to Froedert) so she is working on figuring out wbhich is the better one for me to see.
She also has a Psychologist friend who
used to work in her private practice and who Dr.B thinks I would hit it off wonderfully with and who would be an excelent advocate for me with my providers in addition to her and Dr.P. to vary that not only am I not depressed (which some of my other providers seem to think I am) but that I am not making these headaches up and that they are truelly not from any other cause - Dr.B., told me specifically that she herself knows I am not depressed and not making this all up - she has spent alot of time talking to me and has gotten to know me vary well and can tell I am really working hard to do everything I can to do everything I need to be. It is sooo nice to have her, she completely believes me and completely validates everything, she is AMAZING at her job!

On other news as I said above I am moved into my new Apt. if you need new contact info let me know. It is so cute and has been fun decorating - still quite a bit to do but also glad it's not last weekend anymore!!!

Now I need to start focusing on and planning for next months meeting in Las vegas and then trip to Los Angeles (staying with my friend Stephanie who is part of the Hide n Seek Foundation for Lysosomal diseasees) - hte trip to LA (actualy Long beach) is happening back to back with the LV trip so total will be gone about a week 1/2 although not sure exactly which day I am coming home yet.

I also want to begin planning a MPS Awareness Event hopefully something at CHW - as I think this would have an amazing impact! I need to call Karen O. about that this week and contact Julie Pedretti at CHW also. In addition to that want to do either just a MPS family get-together again in summer or Golf Tournament, it has just been so hectic the past few months havent had any time to concentrate on this stuff.

Next week I see genetics and on Tues Dr.B will update after and will come back and update sooner if I know anything about the Bolt/intra-cranial monitoring studies Dr.Charnas is suposed to be setting up in MN or from Dr.B.

God Bless,

Erica

Thursday, December 6, 2007 5:08 PM CST

I saw my pain dr yesterday andshe is talking about a few different options down the road but for now she took away hte Fentanyl patch (3day) and switched to Morphine 24 con't release and 15 mg IR (immediate release) for breakthrough pain. Then she also is going to start Keppra in the enxt week. She also readjusted my Lyrica dose to just at night (100mgs.)

This weekend we are doing christmas cookies on Sat. with Zander and Quinn and then sunday going shopping in Milw. - Sara, myself and Jenn. (SIL)

All for now, no word from anyone on spine stuff but will update when I know something - should talk to Charnas next week.

---------------------------------------
Thurs. Dec. 6, 2007
I saw Dr.Schwender this morning the Ortho-Spine surgeon at MN - I had saw Dr.Charnas right before this Appt. - I don't know what to think nothing is ever easy in my life - Dr.Charnas was fully reccommending surgery - decopression with fusion b/c of my symptoms - to preserve all of my functionality - so he was giving Dr.Shwender full go ahead - permission I guess.. But I saw Dr.Schwender and he is not reccommending anything - he said I would need a surgery at soe pt. a decompression with fusion and I would lose 50f my movemment in my neck bc of location of cervical spine problemms --- he said to comme back in a year unless thngs got worse - Dr.Charnas is talking to him tonight but I don't know what to think?? I already have many symptoms and he sai mmy headaches are likeally relatedto the spine problem - I see pain dr tommorrow will talk to her but confused?
--------------------------------------------------------------------------------
Sat Dec 3rd 6:56PM
I saw a new Pain Mgmt dr. yesterday - she ended up spending 3 hours with me - my infusion Nurse who I had been staying by all week went with me - this dr is GOOD!! She completely validated how I am feeling and went against everything my Geneticist and GC wanted her to do - she is going to do things her way and not have any of my other providers tell her what she should do or try. She was very happy with some of the meds my PCP had started for me, and is going to add and subtract some more meds in the coming weeks - I see her again next Friday. At this first Appt. she just took a history, talked about options, and what is going on, did her exam, told me different area where my pain is coming from (for instance what I thought was hip pain is really sachro-iliac pain apparently) She is going to do some none medicinal approaches down the road and interventions (she also has anesthesiology, Bone Marrow Transplant and PM & R board certification-training in addition to her pain management. ) This time she took away the Muscle relaxer my Geneticists pain mngmt doc started last week (Tizaindine) and also took away the Tramadol - she added in Fentanyl Patch 25mcgs. 1-2patches for every 3 days and for break through pain immediate release Morphine. Next week she is concentrating on upping my Lyrica dose (50mgs 2x's a day right now said that could go as high as 400-600mgs daily) and Klonopin at night for sleep. I'll keep updates - I am so relieved to finally have someone helping me though!!! I know when I talked to my GC after she was a little amazed that this dr. was starting all these meds and not tring alternative approiaches first - my Nurse and I are just relieved that someone gets how bad pain can be! She is also talking to my GC, to Patti Dickson (IT Study) and I think to Ortho surgeon. I also see Dr.Schwender - Ortho-Spine on Thurs.
_________________

Wednesday, November 28, 2007 11:12 PM CST

I apologize ahead of time, I just need to write and to vent - it has been a hard few weeks.

I just feel so frustrated by all we have to go through, why our drs can't try harder and work together better to help us their patients find better relief is beyond my understanding and beyond what I find acceptable. I do not understand why drs take us on as patients and then basically leave us to struggle on our own - why do my providers think that they know me and know what I am feeling? Why do they not work together and why do they feel we should have to be miserable or just feel so bad when they get to have a happy, great life? I just love the stigma my drs have and the things they say - it is like they see me as a Psychological head case instead of as a real true person with a chronic disease and resolting physical problems.
This past few weeks has been absolute hell for me, with pain that has been increasingly worse and trying to con't with having a life despite feeling so bad and my drs shrugging it off basically. Even with my having my infusion nurse (whose house I have been staying at since last friday - long story) talk to my drs and try to get them to understand and if nothing else atleast help, they do not work together and instead hemm nad haw about what to do - their best advice is to go to the ER, when we know that is such a short term 'fix' and half of the time despite all of the time we waste at ER's (i have been there 2x's in the past week and a 1/2) they tell us they don't know and offer short term relief but then not much else. With my situation alot of things are happening with my health, and none of my drs are working together to figure it out, I end up seeking out the specialists who i think are the best and have the most experience in order to find relief and help. - B/c my providers basically do not want to go outside of their 'system' and feel their is no urgency. Over the weekend my nurse ended up calling my Geneticist and my Primary dr many different times, b/c my Geneticist did not want to treat my on-going severe pain and wanted us to talk to my PCP, but my PCP wanted my Geneticist to make the decisions and find a short term resoltion to help me until I could get in to the new Pain dr. this friday. (it is another long story but my Primary dr has tried and tried to help and almost every time has ended up getting 'yelled' at by my other drs. despite her doing what she felt was best for me.) The pain issue is nothing new but has gotten ALOT worse in the past weeks b/c of something to do with the c-spine compression worsening I guess - my GC found this new Pain dr and my Nurse and I talked to the clinic about a month ago but it took several weeks to get in, then I also scheduled an Appt. with the ortho-spine surgeon in Mpls. who has experience with decompression and MPS I but that Appt. took some time and isn't until next week. In the mean time since I haven't received an IT inj. in 4 1/2 months the pain from this has steadily gotten worse and everyone is afraid to manage it. It has been so frustrating and especially lsitening to some of the things my Geneticist has said when my Nurse and I have tried talking to him. he did after much persistance on my Nurses part finally talk to his pain drs this past weekend and get a couple of the Fentanyl patch prescribed to get through this week even with that I still have big time back pain) but it just sucks that drs second guess you and don't listen and then assume such stupid things. On top of how bad that has been, there has been some disapointments with my SSDI and finding out when it will start and alot of mis-information and in addition this past week the reason I am staying at my Nuraes house is that my Aldurazyme was not shipped last week due to more mis-communication on my Genetics teams part, and so I stayed over till Sat. as she was going to do my infusin then but I woke up with an aweful cold and to top it off, on my way back from picking up my prescription for the Fentanyl on Sat. from CHW (I ended up not doing ERT all together b/c of being sick) my car died, like completely will not start - no lights, no turning over, nohing - so my Nurse came and picked me up and we where making arrangements to get it towed to my parents house (an hour away) and before we got this done my car was towed to the city pound so i couldnt get it until the week so I ended up staying a few more days at my Nurses house and we where going down to get it on Tues., - keep in mind I basically have nothing with me except for meds and clothes for one night), we went down and paid all the fees on Tues., called Triple A, etc. and it should have been towed to my parents last night - but then the towing company called us here and said I hasd to be there in order for them to get the car - which we had specifically asked if we had to be and they said NO - we signed papers releasing it so another day and I still don't have my car. My Dad got off work early today brought down a trailor to tow with and we paid another days worth of fees, did more paperwork and the pound wouldnt let us take it b/c we had to have a licensed company - fine more phone calls, more hassle - suffice to say ALOT of money later (that I don't have) my car is here at my Nurses and my Dad will be back on Sat. to get the car and to take me home - he brought me a few more things from home, but I just think i am hitting a breaking point - last week I was not able to get anything done b/c I felt so aweful, this week I canot get anything done b/c all of my things are at home and my car is not working (keep in mind it is only 4 years old so I should not have the horrible luck I have with my car). This whole car thing is minor and my dad or brother should be able to fix it I guess but it just is so hard when that is how I get everywhere. I am so tired of having to rely on other people so much, I am so tired of so many things going wrong and I am so tired of people (drs) second guessing me and thinking they know how I feel better than I do. I jsut wonder sometimes if there is an invisible black cloud that hangs over me that I can't see but that prevents me from truelly ever being able to have my life just go right?

In the next 2 days I have SSEP's (evoked potentials) as well as expecting a call form my GC, have to pick up some MRI's discs, and on Friday after ERT have the new pain dr Appt. Next week I see this Ortho-spine surgeon in Mpls. It has been a disheartening few weeks, I generally am pretty optimistic and try to find the best in any situation but it has been a little hard when at every corner something else has happened - I know I will bounce back but right now I just wish there was some easier way or atleast some good news somewhere. There are things I have to look fwd to coming up after the Holidays like a trip to Los Angeles right after I go to the World Lysosomal meeting in Febr. I guess I jsut need to put my faith in God and know that sometime, somewhere something better is bound to happen soon.

Sunday, November 18, 2007 2:55 PM CST

This week I don't have much to update, although the week after this there is alot of craziness - with Audiology one day (eek I have to tell her I lost my H.Aid?! Oops! I think it is still under warranty though. Then Thurs. 29th I have SSEP's which is Evoked Potentials where they measure nerve responses from you spinal cord to your body it can get a little painful but othre than taking 2 hours is survivable. I had that done in Oct. nad back in June in L.A. so am a little p.o'dj that my Neurologist wouljdj wanjt to doj them again but apparently they think these are just as or more important than the MRI's..... In any case my GC is talking to her NeuroRadiologists on the 26th and will call me after then I see the Orth-Surgeon in MN on Dec. 6th. I also see the new Pain Mngmt dr. on the 30th.

Yesjterday my GC and I talked, she had left me a VM on my cell earlier in the week that I missed and we emailed jbut I called her about discs - we talked about a few other things adn she mentioned how proud she was that I had gotten of most pain meds - which was nice enough but I also have enouygh pain yet adn like today not very great days.

I took my Nephew Quinn back this morningnad lo adn behold his Mom a friend of mine decided she needed to lecture and yell at me about something I have virtually no control over. GREAT.

I'll keep updated on any new hings and how things are - take care,

Erica

Read Journal History

Saturday, November 17, 2007 10:26 PM CST

Thursday, November 8, 2007 12:08 AM CST

I suspect I am not on ny physicians top list this week, rather than con't to wait I went ahead and in addition to making the Appt. with the Pain Mngmt dr for Nov. 23rd, I also made an Appt. with the Ortho-Spine dr at MN who has the most experience with Cervical spinal cord compression. That Appt. is Dec. 6th.

I have been *knock on wood* feeling a bit better this week, certainly alot better than last week - I think my PCP's idea to start the Baclofen was a amazing idea! I am taking this 2x's a day right now an will increase to 3x's shortly. I have been con'ting to take to Tramadol - but have been able to stop Fentanyl. I also con't to take Diamox.

I have been talking back and forth with Patti Dickson some this week - if you remember she is the dr in Harbor-UCLA, I really liked her and she she has ben able to help a little.

I am hoping that nothing gets in the way of this - like hte need for immediate c-spine decompression - in Jan. I am planning to go to Boston the 2nd last week of the month to stay with friends. I have not checked to see if my CM will be there then. Also in Febr. the 2nd week I will be going to Las Vegas to the World Symposium and after that headed back to Los Angeles area with my new friend Stephanie Lyn (Hide N Seek Foundation) for a couple days. I am hoping this will offer a chance to see a few friends and see her organization.

I don't really know anything else, pplease say a prayer for my Nephew that his ENT Appt. goes well on Tues. - his PCP was not willing to get them a referral despite Zander's
having 5-6 ear infections in the past year so Sara and I just went ahead and made the Appt. The NP we know in the same clinic knows Zander and recomended going to an ENT as did my infusion Nurse who is incidentally an ENT Nurse. Zander is behind in some of his developmental and motor skills and we think his not being able to hear well has alot to do with this. Will keep updated.

No word from my Neurologist, am glad I just went ahead and made the Ortho-Spine Appt. Updates to follow there to.

God Bless,

Erica

Wednesday, October 24, 2007 5:33 AM CDT

Well I am sorry I haven't updated - I have kept some of you up to date or 'in the loop' as my drs would say but I am sorry for not writing here sooner.

I did go to LA last week, - it really wasn't a great trip - my poor Mother had to put up with me, I am not sure what it is probably my headaches but I was very moody (I have been lately) and we fought alot. They did end of study testing - SSEP's, MRI's, etc. on Tuesday - on Monday they did the eye test, 6 MWT and then the Cisternal Tap in the afternoon to measure opening pressure and take off CSF fluid. My opening pressure was low - at 10 but I felt so much better that evening and for several days after, I really didn't start to get a headache until maybe Friday or Sat. again and it has slowly been coming back - I think the Diamox has been helping slow down it's return some. I just wish my drs. would get together and stop their bickering and work together as a team - for whatever reason they think that I should have all of my care locally but I chose to see drs. in MN b/c they ARE the MPS I experts and I need the experts in my care b/c it is so compicated and not very easy. My problems are not 'by the book' and not as simple as someone who does not have MPS NI who might present with Severe spinal cord compression or Hydrocephalus. I don't really think the drs here get that sometimes. I really do love some of my proviers here I just wish ALL of my providers here and in MN could work together. IF they had ALLL worked together I could probably still be IN THE IT study and already have my Headaches solved! GRR. Needles to say I am not going to be getting anymore IT - Dr.Dickson wants them to get a handle on the Headaches, she woulodn't say I couldnt ever get back into the 2nd part of the (year long) phase 2 study but she also wouldnt say yes. It frustrates me b/c those taps are the one thing that actually helped my headaches and the IT Aldurazyme HELPED my compression symptoms. Frustrating beyond belief.

Next week I see Dr.Charnas at MN - I have a list of questions for him which include: 1. checking ICP pressure in the evening or morning vs. afternoon b/c my headaches are always improved in the afternoon but very bad the other times of day.
2. Explain exactly how enough CSF can be getting around compression to shunt all of the time....
3. IS ENOUGH fluid able to get past compression and through shunt?
4. Does shunt work intermittently?
Dr.Whitley at MN told me one of their NS's thought my shunt might be 'partially effective' meaning I think that the fluid drains in the afternoon but is not able to get past the compression the other times of days. Makes sense?
I so so hope Dr.Charnas will help me get in to one of his NS's I am going to tell him I am not leaving till he gives me some answers - I am not living like this. My drs all keep saying Pain clinic which is fine that is a good thing - but they need to do something about this shunt, that in itself if it where moved would solve a great deal of issue. The compression pain is a whole nother ball of wax.

Please say prayers I am eventually able to get back into IT study - it made such a hiuge difference - the symptoms have all started coming back in the last few weeks (it has been 2 months since actual Aldurazyme was injected).

Tuesday I had my SSI disabilty Appeals - my Neuropsycologist in MN had written a letter in support for me (it was really pretty great, I read it!) and my infusion
Nurse came and spoke and we won!! My Medicare/medicaid starts immediately and so do back payments and SSI/disability payments - all will take about a month but what it means is I now have back up insurance to mine at WEA (private) and I now can moe out soon as well as go do more things - I can't wait to go shopping with Sara and with Kris! I think I may fix up my car (02 Saturn) and look into a new one i'd really like an Ion!!!! I should have a great deal of money from the back payments since it dates back to May 04' so really, really want to get some of these things like a car with less miles (I put on about 16,000miles in just a year! - my car has 130,000)

On Sunday someone ran over my cat Tiger - I have 2 cats, and I can tell Spot keeps looking for Tiger - he waits at the door (b/c Tiger used to not come home for several days at a time) - we live in a suburban, edge of town area, but people drive like crazy down our street and Tiges wandered all over the place. My Dad found him in the road - I miss him like crazy, he just had this way that drove you silly but made you love him like mad!! I'll go adopt another cat that needs a home from the humane society but I miss my tiger like crazy - this is the 3rd cat Spot has out lived. He is about 15 years old.

I saw my PCP on Monday, she firmly believes that my Headaches are from pressure and said "i'll say a prayer Dr.Charnas will help get more testing or get a NS to fix/move your shunt to a VP but if he doesn't then we'll just have to find you a new Neurosurgeon!" She is working on getting an Appt. in the Pain clinic sooner. For now she switched 2 of my meds (Neurontin, Flexeril) to lyrica and Baclofen - Lyrica helps with nerve pain and Baclofen works by interfering with something on the spinal signal and so seems like the perfect fit alos b/c of my cervical spinal cord narrowing and compression! I remember this is a drug Dr.Dickson was going to try but then my Geneticist wanted to do the flexeril. I am so glad my PCP is willing to try new things!!

This weekend is Trick or treating by us - Sat. in Waupun and Sunday in Horicon and BD. - I think Sara and I will take Zander (dragon) and Quinn (pirate) to Waupun and BD - I am going to call and see if my neice Jordan can come up with her Mom and Dad to. I also told my oldest brother he should bring Alissa my oldest neice if he was feeling better. (wisdom teeth) Should be fun!

I think that is all for now, con't prayers and will keep updates.
All love and God bless,

Erica

Thursday, October 18, 2007 10:57 AM CDT

There is so much to write I guess and I just don't have the timne or energy to put it down in words - I haven't done my usual update to anyone after this weeks visit to Harbor (Los Angeles) and I haven't really talked to to many of ym friends since the testing either. I guess one of these days I will. I see my NS today, wish me luck - I will update after thart and I guess give hte whole run down of the weeks events. For now I am going to run and hope I get atleast something good news out of this Appt. . Love nad Hugs,

Erica

Weds October 10, 2007 12:36 PM CDT

On top of everything else I have been so worried about this for all week - but my Study Dr. confirmed it last night, no more Intrathecal injections for me - I am so depressed and angry and sad. It has been 2 months since I received an Aldurazyme injection last nad all of my Compression symptoms have already re-occured so I am so scared of how long it will take before surgery absolutely needs to be done. I am holding out the smallest of hope that my stupid drs will sort my headaches out (I am not including my study dr in that stupid dr. remark) and in a month or so I could re-start injections but I don't know my Study dr. won't commit or give a time line right now. My headaces are fierce - as the last part of the study this next week she is doing a Cisternal Tap w/opening pressure but then no Intrathecal injection, Tues. I will have the MRI and SSEP's to mark the end of the phase 1 study. :cry: I don't even know when I will get results. I do know it in't a good sign my compression symptoms all returned after just a 2 month break. :cry: I am seriously beginning to not trust any of my drs. and so tired of this emotional roller-coaster, and so tired of dealing with so many things related to this alone. I just need someone here who will help nad who will actually listen to me, and then not change their mind 105 times like all of my drs have in this few months. If you recall they where so ready to put in the shunt and reservoir b/c of long term planning with the IT injections and not they don't want to do them at all, tell me how does it go from one end of the spectrum to another so very, very quickly? I just need it to be Monday morning, I wish it was already, why is it taking so lnog to get here.

---------------------------------------

Weds October 10,2007-
I really had been hoping one of these times to have a good update to write here about one of my Appts but I guess it won't be on the Appt. with the NS on I saw. It is hard to say why even but from the very first minute we didn't seem to hit it off, he was rude and I was tired so I wasn't willing to let him walk over me. He wasn't willing
to look beyond his little black box, and even in talknig to a few of my providers seemed very against trying anything they suggested. Put it this way I do not know why he agreed or said he was happy to see me, b/c he obviously did not want to see me, and did not want to take on my care. A good percent of hte Appt. was spent arguing per-se with each other b.c I am so tired of drs. thinknig they know my body better than I do , and I wasn't willnig to let him tell me he knew what was going on. In the end I told him to leave and I walked out crying. Very bad Appt.

This next week I head off to CA, it sounds like my Study dr. is trying to set up a 24 hour ventriculostomy - pressure monitoring where they put in a bolt into the ventricle of your brain and then measure your pressure for that amount of time. I don't know where she is at with talking to her Neurologist and Neurosurgeon and I don't know if I will get the Study dr (iT ) this time either. Will keep updated.

Sunday, October 7, 2007 10:21 PM
It has been a very busy week since the wedding a little over a week ago, so i haven't accomlished alot and keep getting phone calls from people asking me questions I have forgotten to answer in emails - I couldn't sleep last night so finally got caught up a bit on all of those! Or most anyways.

A week from today unless something goes on with the NS Appt. with my new neurosurgeon tomorrow (8th) and he does not want me to go I am flying out to Los Angeles on Sunday next weekend and should have testing and possible IT injection on Monday. I talked to Dr.Dickson (Patti) on Friday and she said if I am ok'd to come fly and go there she and the Neuroradiiologist will plan to do the Cisternal
tap atleast most likeally to check opening pressure and take off fluid, whether they would give the Aldurazyme is iffy yet. She did say that they might though especially b/c ALL of my symptoms from the c-spine compression have returned 110n the past few weeks and to a certain degree
more and so we know the taps/drug via injection are working. So there is alot to be decided by the NS Appt. tomorrow but it will or should all work out. Hopefully either they can schedule testing/surgery very, very quickly or Dr.Iskandar says I can fly so I can go to CA for the Study. It really sucks to have all of the symptoms some new ones back and especially my hands, my fingers are numb 95f the time and I have been slipping on stairs and things again, frustrating!

The Appt. is at 10AM - I have to bring soe records and some information (my MPS I binder will suffice) to Shona and Catie Dr.I's. NP and Nurse and not sure what will all happen. I have been so sick lately, today is really the first day I actually could g out and enjoy myself, most days I go out b/c I can't deal with laying around feeling sorry for myself, whether I go out or whether I stay home I am still going to be sick and sometimes throwing up, etc.
but I need to keep on with my life. Last week I left my Sister's house - made it to the end of her block and just barely got my car door open in time to throw up all over the road several times, very yucky. I know I really shouldnt drive in situations like that but I know enough when it is really, really bad and that was just bad. besides the vomiting just comes on sort of suddenly and I take alot of Anti-nausea (zofran)(phenergan) meds to help. So it's been along, long week or few weeks and I am so behind and have decided I need a secretary b/c my providers keep leaving me to get all these stupid records (not my CA drs but other providers - my GC is to busy right now and so I just have to do things if they are going to get done to get stuff set up) I am very fed up with tracking down records, fax machines, phone calls, referalls and the stupid, ridiculous pain mngmt clinic at Froedert but that is the way it is in my life - not a picnic! Unless I make it one and it's still never one when it comes to the daily medical crap!

Sometime soon i'll need to set up Appts. in MN - with my Neurologist, the Neuropsychologists, and to see their Opthalmologist, maybe their Orthopedic doc to. But right now gonig to get through the neurosurgery stuff and hope we
can get it all handled really, really quickly, I soo, soo cannot wait to feel better!!

I have had my oldest nephew this weekend and last weekend, I just love the things a 5 year old says and does, sometimes he wears on ym patience but most of hte time I just miss him terribly when he isn't here nad love it when I have him! So friday on my way home from ERT/clinic I decided to take a different way home and pick him up, we flew kites, bubbles, colored, candy land, cars, dinosaurs,
blocks, movie, and books, a lot of things and just so fun!!
Zander was also here on Sat. and Sara and I got Quinn and Zander to take a bath together with a bit of cajoling and it was sooo, soo adorable!!!

I do need to upload new pictures and promise to try to do atleast a few this week and more next week!! The latest pictures i'll put upare from the meeting in August at Genzyme and from the wedding - all super cute!!

Kris (my nurse and good friend) and I are looking at my schdeule for Nov. to see when IT is going to be given it sounds like if she can get off of work she'll come with, very cool - we'd have a great time I think!-- We always do!
We are also looknig at the World Lysomal Meeting in Nevada (Las Vegas) in Febr. I am pretty sure I am going to go and I think she is looking at those dates to get off to. The other thing is we want to take the bus or train up to my Appts in MN, she's never been up there and seen the campus/hospital and never met my drs there - they are THE specialists in MPS I so would be very cool for her to meet them. And last the other thing is we want to take a trip in March to Naples, FL which is on the Coast! I love traveling although makes it very hard to keep up with everything going on and getting things accomplished which is why I am always behind I guess!!!!

Last, we are rescheduling our breakfast that we where going to have this past friday, probably Nov. - depending on all our schedules, was/will be Karen, John (genzyme) LuAnn (my GC) and myself.

Not sure who i'll see in LA this time, my friends the SLawson's I think - Barb Lyons I hope, not sure is Stephanie Okey is around but Paula is moving (to San Diego) permanently so won't be in LA but this means Kris and I and Paula will all be able to take our road trip together when we are there!! I'll also see Alla and Dr.Dickson - both of whom are great and I love.

Will post an update above this journal in the next day or two on how the Appt. went!
Love,

Erica

Friday, September 28, 2007 11:27 PM CDT

Tuesday or maybe it was Weds. yeh i think it was Weds. I spoke with Dr.Guillaume - I had the chance to ask him alot of questions and just talk to him, it was nice. He is such a nice person in addition to being a good NS I wish he was going to be my dr! But good news he asked me if I would want him to contact one of his old colleagues from when he worked at UW Children's Hospital in Madison (I see specialists at Childrens Hosp. in Milw) - this Neurosurgeon at UW is a Pediatric specialist, has very extensive knowledge of Hydrocephalus and Dr.Guillaume thought very highly of him. So after we hung up Dr.Guillaume emailed me his name and info and sent the Dr. and email about me, my history and what was going on and would he see me. Dr.Iskandar emailed back very soon after and said he would be happy to see me and to take me on! I emailed him then with a few logistical questions and he replied equally as fast, both him and his NP Shona seem very, very nice! I am so thankful to Dr.G. for helping me out!!! I have an Appt. on Oct. 8th so in one week with him! LuAnn and my drs in CA are scrambling to get everything sent to him before then.

Tuesday, I went and saw one of Dr.Zuccaro's collegaus for my itching, he prescribed Phenergan and knock on wood it actually seems to help! Hope I didn't just jinx myself! I had to take aa several loooonngg day break from the Fentanyl b/c of some very bad side effects not only the itching but I was getting sore throat, losing my voice and all over body ache (to the point my muscle and bones ached, that is still going on but i restarted the fentanyl today b/c I am in so much pain and have a wedding I am standing up in over the weekend and have to be able to actually be able to make it through!

LuAnn called me Weds night, it was pretty late when she called and I was at my sister's house, but her and I ended up on the phone talking about things related to my old NS (who refused to do surgery on my shunt or even see me now) and my headaches and possible shunt surgery for the VP placement. It was a nice conversation, lots of "dialog" as my GC put it but still nice.

Today my Geneticist started me back on Diamox - I haven't been on this since before my shunt was initially placed back in June 06' but we are using it as a trial of sorts and to try and help the headaches in the mean time before I see Dr.Iskandar and while all my drs figure out what they are going to do. I hope it helps some!

I haven't been home in a few days but came home tonight to a giant box from Deb Marcellino at Genzyme - filled with giant picture frames, pictures and picture albums, so neat!! All from the meeting beginning of August.

Going to sleep now, I hope. I hope the wedding goes well tomorrow and a new life is begun for my brother and his fiancee - my friend Jenn.

God Bless,

Erica

Tuesday, September 25, 2007 4:06 AM CDT

I have new and updated pictures at the TOP of the page in the 'Rock You' album - pictures from last weeks trip in LA. Hope you like them!

Please see the next journal entry on next page for last weeks trip, and frustrations. I feel slightly bad for how 'loudly' I spoke in anger and frustration against my drs. but I also do not feel apologies are neccessary. This week I still feel frustrated but not angry just very confused about what is all going on. I have decided though that I just need to put my FULL trust in Dr.Dickson and have come to see that her slow and methodical way of deciding (and not decidng!) is b/c she is trying to make the very best decision for my care and for my future. This revelation on my part does not mean it makes it any easy on me with these headaches and resulting side effects to keep waiting b/c patience is certainly not my virtue or passion but I need to hold my self back and wait on Dr.Dickson to tell me what is going to happen. She is a good dr. and not only that but a friend of sorts and she has taken me in so I just plain need to trust her.

Dr.Dickson and Agnus Chen (another physician at Harbor on my team) are meeting with their Neurosurgeon today and I have been praying that maybe this will be the day they come
up with a final plan and we move fwd. and I get relief. I have a short list of questions for her when I talk to her and I am very nervous to hear what she will find out. Please, please pray for good news and a fast resolution and plan to move us fwd. to feeling better!!!

Also Dr.Guillaume whom some of you may remember is the Neurosurgeon in Oregon at OHSU who was going to do my VP shunt placement surgery is calling me tomorrow - he got caught up in his clinic today/tonight but was kind enough to email and say he would call today! (tues) I think it will be nice to talk to him.

I also am going to try to get in to my PMD b/c of some bad hip pain I have been having since the LP last Weds. and going to try to get ahol of Dr.Charnas (my Neurologist) I hope I can reach him once! Also I need to apparently call my NS myself, my GC and I asked my Geneticist to talk to him but he said I needed to call him! Nice, huh?

Will update soon - don't forget check out the pics above and sign my guestbook!!!

Love, Erica

Friday, September 21, 2007 9:04 PM CDT

I just got back frm dinner with Dr.Dickson and her family - I had a chance to talk to her some more... I feel very mixed up, down and depressed but maybe at times upbeat I don't know. B/c they not think that the pressure reading the other day was low (during the LP done below my C-spine compression) - the reading was 16, which last month at the Cisternal tap in my neck above compresssion it was 26 - well it seems many, many drs. are rethinking what they want to do, ie: go with th VP shunt, put in the reservoir and do several serial taps to see what pressure readings are, or do a 24 hour Bolt reading what this all means is even more time wasted while it has been over a month already and the headaches are getting excruciating and more and more often, not just in the mornings and at night but throughout the entire day now. And again all these drs. that are not my providers are deciding with Dr.Dickson which is so frustrating. I did call LuAnn and ask her to have Dr.Rhead talk to Dr.Sinson see if he would do the surgery b/c my damn drs. can't seem to make up their minds, they go back and forth, back and forth and in the mean time I am just left to deal. God I could scream. I hate, hate, hate this. I feel like this mess will never get over nad I will never get my life back. I never wish my problems on other people, but hell right now I wish my drs. could understand what this is like. How they can keep second guessing this isn't pressure is beyond me, this is the EXACT same it has been through each shunt revision and before each revision 3 times! Ugh. Frustrated beyond belief.
--------------------
1:18PM
I do not have much energy to write this, today or even this week my headaches are taken to a new level and this trip to CA has been filled with pain, pain, pain and lots and mlots of sleep. I wake up, shower, get ready only to la back down and sleep a few more hours. I a few my friend
Paula will be here and we will go to lunch, that will be only the second time besides going to the Hospital on Weds/Thurs. that I have left the hotel. These headaches are
BAD!
My Study dr. (Dr.Dickson) did say that she and Dr.Chen meet with a NS from UCLA who practices here at Harbor to, on Tues. and he is interested in my case. If he will do the surgery it hopefully would be soon, and hte plan would still be to place a VP shunt that is programmable with reservoir. Say a prayer he can do the surgery soon. His name is Dr.Bergsneider and he runs the Hydrocehapalus clinic.
They did not do my IT injection this time and Dr.Dickson said they would nnot do anymore until the shunt was in - they did do tests on my LP shunt - a dye study, CT scans and imaging.
Tonight I am having dinner it sounds like with Dr.Dickson and her family and not sure who else. Barb Lyons may come over tongiht after work but for sure is meeting me tomorrow and we will have brekfast/lunch and she'll take me to the airport.

Please pray we get answers soon!! Please!

Had lunch with Paula at Ruby's today very nice, will load the new pic of that soon. Please see above the new pictures I loaded on the 'rock you' album! Also please sign my guestbook or new 'corkboard!'

I talked to my Genetic Counselor a few hours ago, for a few, she is going to ask my Geneticist Dr.Rhead to contact my NS and see if that Dr. will do my surgery - LuAnn will let me know when she hears something. Also in another 2 weeks is the breakfast we have planned with Karen and John, will have to take some more pictures =) everyone knows I love pictures!!!

Headd of to dinner with Dr.DIckson and her family at 6:30 so about an hour or so. I love her family! I hope she will have some new info, I do hope.

Thursday, September 13, 2007 5:52 PM CDT

Just a short update - as most of you know the surgery was cancelled for this week. To think I could have been done by now nad headache free. Anyways though, I talked to Dr.Charnas, my Neurologist yesterday and they all want the surgery to be done here lcoally so he was going to try and get ahold of my Neurosurgeon. I hope he calls me soon with an update. I will talk to Dr.Dickson either tonight or tomorrow, maybe she will know something.

I talked to my GC today 2 different times, first about all that is going on, she has been kept in the loop but had a few questions about how the shunt would affect IT injections, how these are going and just a few questions about teh different things all the specialists where considering. The other thing we talked about was the NS here and his staff, which you'd just have to have talked to his NP before to understand but suffice to say she is not many of my providers favorite person.

It looks like we are going to meet on Oct. 5th with Karen and John, for breakfast, just a meeting of the minds between Karen, John, LuAnn and I.

Will update when I know something, please say a prayer it happens soon.

Erica

Tuesday, September 11, 2007 12:46PM

Well I am sorry I have not updated before now - yesterday I
found out that there had been alot of mis-communcation amongst the drs in Oregon, and the surgery is not on for this week. - It is cancelled. Needless to say I am pretty depressed, I guess it is to much to ask that I get a new shunt that will work better and it be done by a surgeon who
has experience and interest in my disease. Apparently the mis-communication has occured with the Geneticist their and
his not wanting to proceed until some issues are ironed out, except that my Neurosurgeon in Oregon, and my drs outside of their have no idea what the issues are and they had wanted to proceed as planned. I am very sad not to be on that plane right now, to think that surgery would have been over on Thurs. and next week we would have been abl to use the shunt reservoir for the IT inj. This would have been a double plus, not having the headaches, nausea and other symptoms anymore (finally) and having a much easier time for the IT. This just sucks completely. I dn't nkow when it is going to be rescheduled - soon I hope. =(

The Walk on Sat. was a huge success, raising about $60,000
total for MPS research! It looks like on the 4th of Oct.
Karen and John will be working a conference at the Midwest
center, and LuAnn has said she could do dinner that night, so hopefully it works out.

Please say a prayer that this surgery gets planned soon, very soon.

Sunday, September 9, 2007 8:57 AM CDT
I am sorry I have not updated now since Thurs. trip to MN- I ended up driving the 4 hours b/c it was such last munute Appts. that everything else was a little more than I was willing to pay - especially with Oregon nad CA being back to back this week and next.

The Neuropsych testing went fine, about the same as last time, with one thing being a little lower - but I was distracted and it was a 22 min. attention drill. The MRI also went fine - it did not show increased ICP but in most MPS pts. it never does - I also saw Dr.Whitley and that was ok, his feeling was it was ok to fly/travel and surgery
was needed.

Friday I was at the clinic for ERT, and I ended up spending much of the day on the phone with Dr.Dickson, Dr.Guillaume's scheduler and Dr.Whitley it was a very not great day just b/c there was so much going on and so much different communications and feelings what to do - but ultimately Dr.Guillaume still wants me to come this week I will see him on Weds. and then surgery on Thurs. These plans best not change b/c I have all arrangements made now!

Wednesday, September 5, 2007 3:16 PM CDT

Well there isn't much news - the latest I know is that Dr.Whitley has me coming to UofMN tomorrow (thurs) for some
testing. Apparently Dr.Charnas-my Neurologist who is out of
town has been in touch with Drs. Dickson and Guillaume and they would like some testing to be done including MRI of the Brain. So the schedule for Thurs. is:

9:00-NeuroPsych testing Kendra Bjoraker
1:00-Interval Appt. Chet Whitley
2:00-MRI Brain

I should be done around 3:30 and back on the road shortly after that. Other than that I haven't heard what is going to be with the Shunt placement - I heard from Angie today (she is Dr.Guillaume's scheduler) and as of yesterday he was still trying to work some things out. Monday night he emailed me in response to something I wrote him saying "II would like to take care of you as well and we will keep working on it here" Dr.Dickson also said that they would like to talk to my Neurosurgeon to see if he would see me - so I imagine that is what they are doing. COnsidering if they do the surgery next week in Oregon that is just a week
away I really do hope they let me know soon b/c I have to make the travel arrangments.

Otherwise things going on this week include the MPS Walk this weekend (www.lukieslegacy.com) and also Sunday we are
getting together with my brothers Fiancee to go dress shopping b/c my Sister and I are standin up in the wedding at the end of the month.

I will keep updates when I know something, please say a prayer I hear what is going to be soon.
Much love,

Erica

Friday, August 31, 2007 2:55 PM CDT

Just a brief update from Dr.Guillaume's
scheduler:
"We would like to tentatively schedule you for surgery on 9/13/07 but may
not be able to. He said if we can't get it done sooner, then you should
get it done locally before October. Either way, we are trying to
coordinate everything and i will let you know".

---------------------------------------
Thursday Aug, 30, 2007
Just a short update - I talked to my Insurance this morning about the trip to Oregon, and Dr.Guiollaume is part of our network and covered at 90/10 for all services including surgery. The only thing that would be needed for their end is to be notified of any in-patient stay. It looks like the soonest the surgery can be done is Oct. though so I don't know what Dr.Dickson will want to do or if Dr.Guillaume will try to work something out. I am just not sure at this moment. Will keep updates here.
---------------------------------------
Tuesday,August 28,2007 12:00AM
I think this will just be short but I talked to Dr.Dickson yesterday (my Study Dr. at UCLA) she has been brainstorming
with another of her colleagues about my options when I go to Oregon. It looks like they may have come up with an option - she is talking to Dr.Guilani the NS today and also
sent Dr.Muenzer at UNC an email seeing what his thoughts where. What they are considering and asking about is a VP shunt (ventriculoperitoneal) that has a built in reseroir - the VP shunt would be the programmable type that is adjusted with a magnetic wand of sorts - before each IT injection they would raise the pressure setting so high that it would not "work" in essence be turned off b/ fluid would not be being drained - then they would do the injection and the next morning readjust the setting back down to a # that would release fluid. Dr.Dickson said she thinks the Aldurazyme would only need a few hours to do it's therapeutic 'job'.

Last week during ERT I felt very odd, super cold and just very goofy. That night I had horrible stomach cramps but never anything as a result of it, after a few looong hours it just disapeared, th reason I am almost positive it was from the ERT is b/c another friend of mine had the same symptoms last week a few hours after his infusion and one of my other friends has had the same symptoms to. Ick.

I had my nephw Quinn this weekend, he is at that age where
he does not think he has to listen to me, lets just say we
ended up with me yelling at him alot, then later on he would do something extra sweet. He starts Kindergarten next week, he keeps saying he isn't looking fwd to it, but I think he really is!!

I have been trying to get through to the pain mngmt clinic at Froedert with know luck, I also left a message with the
nurse in the Interventional Radiology dept. b/c I want a consult there, but she never called back, grr!!!!

A week from Sat (8th) is our Walk in Oshkosh, if you are in the area please come!!

Gotta run lots of love,

Erica

Tuesday, August 21, 2007 1:11 AM CDT

Well I am home from CA - Sat. morning I woke up with a horrific headache, nausea and then to boot started throwing
up! Not a nice way to start the day, especially the day that you have to fly home!! I talked to my Dr. and she left it up to me what to do - stay another day or fly home while feeling yucky. I slept another hour or so and though I was still exhuasted decided to just try and make the flight. Of course the lines through security where loooong and took forever! I ended up sleeping almost the entire first leg of the flight - Dad told me I picked up my glass of soda and promptly fell back asleep with it in my hand, and as I was falling faster and faster asleep my cup was slowly being turned and poured into my purse by my hand! Yikes the things yah do when you are feeling yucky!

I don't have details for the Oregon trip yet, tomorrow I will sign a release so that records can get out to that NS Dr.Guhlani (sp?) and Dr.Dickson is working with his scheduling dept. to get something set up soon. She thinks my Headaches on Sat and since are from pressure - which means my pressure came back alot quicker this time usually it takes atleast a week. =( When that Dr. puts in the reservoir he will check my opening pressure and I guess we'll decide what to do about shunt.

Thurs. and Fri. we saw LA - we met up with my friend Barb Lyons on Thurs and her grandson Jake at the La Brea Tar Pits and then went for lunch - from there Dad and I went to the Santa Monica Pier and Beach. That night we had a lovely dinner with Dr.Dickson and her family.

There is more to update but I am tired and my hands hurt will update later. Will keep posted here on Oregon trip.

Much love,

Erica

Thursday, August 16, 2007 5:32 AM CDT

Thurs. August 16,2007 3:32AM
Dear friends,

I am here in sunny Los Angeles and just over 1 1/2 days out from my 3rd IT injection with Aldurazyme. This has been
a whirlwind trip not so much in that I have done alot but the information I have been muling over from my Study Dr.
- My 3rd Injection went pretty well, there is again a small raised area where the injection was given that I believe is a hematoma (I could be wrong, can't quiet remember what my Dr. said) Over all though, we decided to stay with giving Versed and Fentanyl just at increased larger doses before hand and 1/2 way through. I didn't wake up this time so that was good, the reason we tried to avoid switching to the other anesthetic Ketamine is that one of the major side effects can be raised ICP pressure in the brain - something I already have. It was a good choice to stick to the versed/Fentanyl combo as my opening pressure ended up being high (26) - and anything under 20 is considered within limits.

I have alot to update but my eyes are wuiet blurry right no so I am spending more time re-typing so I think I will be finihing this in the AM when I get up but last thing I will quick update is that Dr.Dickson has me using a Fentanyl patch this time for pain mngmt. - I am I believe either allegic to something in it or just very, very sensitive as I am very red, have rash here and there all over and itchy beyond belief. I am taking Benadryl and Zyrtec which give some relief but not nearly enough! I do have Oxycodone to switch to but ant to try to keeo using the patch as long as I can (each one last 3 days) b/c I feel so much less pain and able to do more sooner than any of the Previous times since starting IT in June. I do think I am going to have to ask if there is anything more I cn do to help the itchiness.

I am signing off here, but will be back to update about some Drs. in Oregon that my Study Dr. and Dr.Charnas (Neurol) are talking to about sending me to for a Reservoir implant in my brain and possible VP shunt. (switch from my current LP to a VP.) So that is alot there to decide but I am just waiting to hear from my Drs first and then will think on it and make a decision.

Thurs., August 15, 2007 8:36AM

Alriht I am back to begin where I left off, a little less tired so maybe this on't take me so long to type! The Drs. in Oregon are a NeuroSurgeon (Dr.Guhlani (sp?) and Dr. Robert (I can't think of his last name) who is a Metabolic Geneticist - both of these drs. it seems would ork together on my care. It sounded like from what Dr.Dickson was telling me that this might happen with in the next month - Dr.Charnas is thinknig I need a new shunt system placed - a VP (in the brain) and my old system removed. But they are unsure if they would do that right away or wait and see once the docs got in and implanted the Reservoir device (under local anethesia no less!) for the reservour surgery they go in quit aays to burrow the device into the ventricle of the brain (not sure of exact location yet on my head) and then the reservoir is placed at a locatin that can be accessed pretty easily by a NS or NeuroRadiologist for injections.

Dr.D wasn't sure yet but it sounded like once they went in to place this device if my pressure was still high then they would book and schedule for the VP imsertion - the big thing they are looking at is if a VP can be attached right to the reservoir and thn the VP turned off each time it needed accessing for IT.

Last night I finally was able it charge my phone back up and guess what - good news from my Mom - I FINALLY have an SSI Court Appeals date!
Oct. 23rd, I so hope this goes through it would help 10fold in many, many ways!!!

I also heard from my good friend Barb Lyons, she is going to call me today she said and hopefully we'll be able to go out and spend time together, I love hanging with Barb!!

I best be running but will update when there is new news!

Erica

Sunday, August 5, 2007 12:07 AM CDT

Once again I must apologize deeply for not having updated in the last week. I have been traveling again and chose to not take my laptop along.

This past week I spent several days in Boston (Cambridge across the river from Boston actually) with my Nurse and good friend Kris. Our flight arrived Weds. early afternoon where we where met by an Car service from Genzyme - Kris was duelly impressed as this was a mini limo and had my name on the placard) Neat hey? This company really does go the whole nine yards for us MPS I patients. After looking around and hanging in out hotel room Weds afternoon we went to a dinner hosted by Genzyme, very nice, fancy, good food. Here I met the Prince Family, the Checrallah/Boyce family and again saw the Madsen's. Thurs. morning began with breakfast and our Communications/survey panel at Genzyme and then lunch, tours and Kris and I went on to visit the Garment District and then to the Faneuil Hall. Thurs. night was again another dinner, which I ended up leaving early bc of being quite sick but was fun initially.
Friday morning Kris left early as CHW would not find a replacement for her at the infusion clinic and I slept a little more and then went to the Mall and the Beach with the Prince's.

This time I had the great pleasure of meeting Shannon Barrett whom is a GC with Genzyme - and she is looking into speaking gigs for me in the Boston/NY areas - very cool. She was funny, and I could tell her and Kris had a great time talknig that first night. I also saw Paula who came in for me, (my Steph was not able to be there du to being in NH on vacation) and Lora, Naomi for a short bit, Bill and Deb as well as many others whom I had a great time talking to.

Fridays flight home (midwest- which is bout the best of which airline you can get here in WI and really a good flight) was delayed quite a long time, both boarding which was behind by an hour and then an additional 1 1/2 hours sitting on the plane on the tarmac waiting to take off. I eventually did make it home though. I will update pictures as soon as I get them onto my computer, I will have to load a few from Kris, and off the Princes and the ones from Genzyme as I didnt take alot.

The only down fall really of the week is these Headaches, recall that I had a good week and a 1/2-3/4's of no headaches after my 2nd IT injection, but since then I have been dealing with nausea and headaches that are at a severe
level and vengeful. (a new made up word I know!!) I am taking alot of pain medications and nausea meds at regular intervals but I think that I need to talk to Dr.Dickson, LuAnn and Dr.Charnas about seeing a Pain Mngmt. specialist.
I am now 1/2 way through the phase 1 of the IT study and leave on the 13th (a week) for UCLA. October will be my week long testing to see if the IT has begun to help. So far I have had some improvements in lower body symptoms and headaches improve for a few weeks but then return usually 2 weeks before I go back and that 2 weeks is an up and down ride. I also have been missing ERT infusions now 2 times within the last month b/c of traveling and perhaps that does not help. Please keep this close in your thoughts that I might soon get a resolution either with moving my shunt to a VP, or with a new approach to medication/mngmt.

A great thing about this next weeks (13th-18th) trip to CA is that Stephanie Okey/Genzyme is driving down to spend the
day with me - I am very excited!! It also looks like Kris and I are going to plan a trip to Naples,FL to visit her Mom and then with Paula Ciampa/Genzyme go back with her from LA to San Diego (where Paula will be living) and from there it sounds like all 3 of us are going to go to Kris's timeshare in Sedona, AZ!!! Yey!!

I will keep updates while I am in LA this next time over the 13th and send group emails again. My Dad will fly out to stay the 15th-18th. I am going to check with a couple friends see if they can come out in Sept.

All this for now. God Bless and go in peace.
Thanks for checking in.

Erica

Wednesday, July 25, 2007 9:44 PM CDT

Dear friends,

I am sorry it has been so long since an update, my trip to MN the 13th-16th and then on CA through the 20th was good. I had a great time spending time in MN with my Case Manager (Steph) and team, Friday Karen O'Neill and Paula Ciampa who are are both Patient Care Liasons met me at the airport and we had a nice lunch at the Chocolate Factory. A little later on Paula, myself, Steph and Lora went to a MN Twins MLB game which was fun and a new experience. Sats. meeting was informational and nice, alot of great speakers and interesting topics this year.

My Study Coordinator Alla and her two kids Maia and Ivan met me at the airport on Monday afternoon and we went and had lunch and then to the wilderness place before going to my Hotel. I love her kids so it was realy great to spend that time with them!! Tues. I began the day in Bldng E-4 at Harbor and from there myself, Dr.Dickson and Alla went over the Exam building and did the Eye chart test and 6 MWT. From there we walked to the GCRC (5E Harbor) and all the admit preparations began for the Intrathecal injection. This includes more paperwork than you can imagine, lots of consent forms, the Physical exm by Dr.Dickson and Neurologic exam by Dr.Naylor. After all this is said and done the floor Nurses begin the IV, which this time took 2 different Nurses and 5 tries, ugh! I do really love the nurses on this floor though - I again had Chris as my main nurse and the other whose name I can't remember was really sweet to. This time after going down to Radiology, Dr.Dickson began giving the anesthesia through my IV, but I never responded! She said she gave a massive amount several times but my body is apparently getting used to the drugs (fentanyl and versed) and so I never fell asleep! hat really, really sucked as I was aware of what they where doing the entire time and the thing that makes this IT procedure bearable is being asleep when they do it! After the IT was completed and we where all back up on the floor, Dr.Dickson did say that the other patient (Laynie) currently in the IT study had this same response and she would use Ketamine to get me asleep this next month. There are more side effects with ketamine but we will just cross that bridge when we get there I think.

A coupls hours after the IT was over my very good friend Amy (Laynie's Mom) got the Hospital, I had flown in on Monday but b/c she had a pre-op Appt Tues morning she came in after that. Amy is from Texas so it was very sweet of her to put aside her life for a few days to come spend time with me and act as my Nurse. I am very greatful to her for doing that! After Amy left to go to the Hotel I tossed and turned most of the night sleeping in short fits
and on Weds. I got up and began roaming the hall, visiting the Nurses at the station b/c I was antsy and just needed to get up! My regular ERT infusion was done Weds. and after that was done, my good friends Sammie and Tami Slawson came up and met Amy and I - from there we braved the traffic and went into Hollywood, for which I lasted all of about 20mins! It was great to spend time with them again and I am thankful for Tami taking time out of her crazy life to bring Sammie to me so I could see them both! I am hopeful we will get to see each other again this next time in August!

My flight home ended up being on Friday and from there I spent the night at my NS Clinic Nurses house - from her house I went up to Fish day in Port Washington on Sat. and spent the day with my Family seeing my Niece!! Monday we went up to Green Bay and took my Nephew Quinn as part of his b-day to an Amusement park, that we used to go to as kids, it was great!

If you remember it took a long, long time to feel okay again after the 1st IT injection but my Dr. had also started me on a muscle relaxant and changed up my pre/post meds and the changes really seemed to help this time, as I am back to taking Oxycodone only occassionaly and otherwise just taking Tylenol with my regular Tramadol and the muscle relaxant.

This week we have been scheduling flights and hotels for my trip to Boston which is next week - that is for a Communication Panel being held on Thurs the 2nd. I will fly in on Weds. have a get-together supper that night for which Paula is coming since my Steph will be on vacation =(
and then i'll fly home late Friday afternoon. We also have been booking flights, hotel, car rental for my 3rd IT study injection in August and that will be 3 weeks from now, the 13th-18th. My IT will take place on Weds of that week and regular ERT on Thurs of that week, then fly home on Sat. I am flying in a day early to spend time with a friend of mine, Stephanie Okey is an Area Director with Genzyme and someone I have known for a few years but have never personally met so far. I am very excited to meet her!

I haven't decided about school, what I mean is whether to go full or part time, and I actually don't think I will be picking out classes until right before the semesther starts at the end of August. I do need to get working on Financial Aid and setting up meetings for when I am not traveling with the school counselors. (to finalize schedule and make Accomodations plan) One thing I am going to ask them about is how to get into the Phlebotomy program, it is a one semester program and I really want to do and have wanted to for a long time! My real overall focus will be towards special education - associate degree.

I am sure there things I am forgetting to write about here, ooh well. One thing I might ask is for all to say a prayer for my friend Amy (the same one I wrote about above) she had prophylactic bilateral mastectomies w/reconstruction this Monday (2 days ago) and is recovering
Amy had/has a condition known as LCIS which gives her a much higher risk of having breast cancer. Amy not only has adopted me as a daughter of sorts she has her own 3 children who also have MPS I (Ages 17,15,14) so a very full life. Many prayers Amy, we love you!!!

God Bless,

Erica

Sunday, July 15, 2007 1:14 AM CDT

Dear All, I write to you from here in Minneapolis, I told Miss Karen this afternoon if I took a nap I most certainly would have troubles falling asleep tonight and wouldnt you know at 5:30 I feel asleep for 3 hours till luckily Karen happened to call my cell at 8:30! It is now 1:15AM (!!!!) and I am dead to this world tired but right next to the elevator here at the Radisson and it is a noisy place!

I had the most wonderful company yesterday and today, if I did not feel 100% well then I atleast could forget about the pains/headaches/tiredness for a while and enjoy the good times. I had a great lunch at the delightful Chocolate Factory with Karen O'Neill and Paula Ciampa of Genzyme and afterwards went to a MLB MN Twins Game with My favorite company Steph Ginty and also Paula and Laura!!! Very fun night! Our Cosmos where pretty good afterwards! ;)

This morning/afternoon was the 2007 U of MN MPS meeting, always entertaining if not slightly tiring at times (from all the sitting! I do not sit still well!!!!) The speakers where impressive this year, several are my own personal Specialists and others I have met at Conferences and such. The food was great and hey thats always a plus in my opinion!

I am not sure what i'll do tomorrow, (sun) I actually fly out to LA on Monday morning, I am thinking i'd like to go to a few museums and venture around the river, it has been awhile since I have actually site seed. (I know, I know that isn't actually a word! it should be 'done site-seeing') Anyways so I think I will do that. I am craving Applebees somethig fierce right at the moment - I wish the one here at Radisson was open all night! Guess I will have breakfast there in the morning, even though what I want is really lunch!!

Monday morning my flight leaves MSP around 11:00, and I get in to LAX around 1:15, I am not positive on this as yet
but belive my Study Coordinator Alla and her 2 kids Maia and Ivan are meeting me at the airport (so that Amy can get the rental car when she gets in) and from there we may go out at about. I love her kids so am very happy about this! Tues. morning will begin the 2nd Intrathecal testing, which includes the 6mWT (a very funny experience) Eye exam, Neurologic exam (Dr.Naylor is so quiet and so thorough Ooh my lands! But he is nice!!) and then a Physical exam and lots, and lots of paper work and of course the IV start and pre-medication which will include anti-nausea and steroids this time before and after. Aftr all that is said and done we all troop (a whole mass of us!) to the Radiology Dept. to begin the Fluroscopy for Intrathecal injection into my Cisternal neck space (right below the skull - they actuall shave a small amount of hair! ugh!!) Thankfully before they do the needle insertions (2 different ones!) they give me good ole sleep meds (anesthesia) for sedation and I am out until after I am back in my regular room on GCRC!
I will spend Tues night in-patient and then on Weds morning receive my regular IV ERT infusion (I call this the over-kill week for Aldurazyme! b/c of receiving it Intrathecally and by IV!). I am hopeful that Ms. Chris will be my Nurse again - it is funny that my home Nurse at the NS clinic is Kris' and my Nurse at the GCRC is Chris! Of course to go one further my Study Dr. is 'Patti Dickson' and the Study Coord. at CHW Genetics is Patti Chico, aka 'my Patti's'! Funny, funny!

And all that know me well know that feeling good or feeling
bad I never pass up a chance to go out and about, site seeing. (I just end up sleeping ALOT when I get home or being in a hazy state!) Well this trip shall be no different, just as I have seen my very favorite people from
my Genzyme team this past few days I will see my good friends on Weds!!!! Today at the meeting I ran into a friend (and MPS II Mom) who lives in LA and she is wanting to get together with us as well. So I think on Weds, once I am released by Dr.Dickson I will be heading off with ms. Amy and my friends the Slawsons- Tami and Sammie (Sam I am)
and Kim and her husband may just well meet up with us to! A small MPS get-together hey?! =) Am I good or what at getting people togehter!?! =D I think we should have a good time, and since these are fellow MPS families they'll understand if I am in a drug induced pain state or not quite making sense b/c of whatever I happen to be taking. I am always a bit slow on the uptake as some would say so our get-together after my IT should prove to be even more interesting I am quite sure! But hey i'll be near Beverly Hills and we all know 3/4's to All to the peoples living there are in drug/something induced states and that aint normal - whereas i'll have an excuse! ;) =) HA! Perhpas on Monday or Tues. I also will get to see my favorite Barb Lyons which would be great, I love her company! Her daughter and I have been talking back and forth to and would be fun to see her! I also need to be getting in touch with Ms Stephanie Okey!

So this update is a bit more optimistic, perhps I am just overtired, but I am past the monthly PMS and so not so emotional. Now I am back to being able to look forward (literally) and see the bright future that lays there if only I keep on, keeping on. So for now my near and dearest friends thank you for stopping by, I hope you have enjoyed this most probably unusual of uodates, I will be back in a few days to write more on how things are going! To many of you, I will set up my email so that my good friend Amy who will be with me can send out a group email to you and update. Some of you will also get phone calls/VM's from Amy
or I to update. (just b/c we can!!!) =)

May God Bless you each.
Good Night and Good sleep,

Erica

Sunday, July 8, 2007 11:28 AM CDT

I should elaborate on the below, but I think it is self explanatory, just tired of feeling tired and being in pain. I have been fighting seeing a Pain Management Dr. but maybe I just need to do it, between these Headaches and neck pain it is wearing me down.

Last week I saw Genetics, I posted a new picture in the slide show of my GC and I - I actually had the picture taken for a poster I am going to be doing for the walk but decided to include it in here to. It was a good Appt. I like my GC and although there wasn't much for us to go over we had a nice visit. I think our Appts. never fall at the crucial times (decision making points) so her and I then end up sitting on the phone - what can yah do though? She is a great resource, and
just a nice person all around to work with. I guess the next big decision that will likeally come up is in a few months when I am further into the initial phase of the IT study and need to make a decision what to do (con't or surgery or what and if con't IT then at CA or in MN) I imagine that considering how I have felt so far that might be a harder decision than I ever imagined. ....

I have a week until I fly out to L.A, my good friend (and MPS I Mom) Amy Holland will actually be flying in to L.A to be with me next week for this I.T, if I am less than excited about the actual IT injection than atleast I am excited to see her! Before I fly to L.A. though I actually am flying to Mpls. (MN) for a few days, leaving on Fri. from Milwaukee. I'll miss my infusion for this week but am excited to see my team from Genzyme again - I have a lunch 'meeting' with Karen and Paula on Friday afternoon, if Steph is available she'll join us as well otherwise i'll see her maybe later on or for sure on Saturday at the MPS meeting.

The 23rd we are taking my Nephew Quinn to Bay Beach, it is an Amusement type park located by Green Bay - I haven't been there in years but looking fwd to it! The week after that I fly out to Boston for a 2 day thing (Aug. 1-2nd) at Genzyme, another chance to see Steph
and some of the others there, should be fun!

^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^^
Saturday July 7, 2007 11:18PM

Happy Birthday Quinn!! (5th)

I feel like I am desperately trying to figure my life out, something about this last trip to CA, and how it has been a rocky few weeks since has made me ..... I feel like I have been putting my life on hold since coming home, I keep expecting (hoping) to wake up and have atleast a little energy again, to have less pain and to just be less tired all of the time. I never expected to feel like I did after this last iT, it took me completely by surprise and now I am left wondering, again, did I make the right decision or should i have taken the surgery with it's own unknowns and risks and felt better sooner? I keep hoping after this next injection in a week I will feel better, less pain, and the return of some of my energy but I also sub-consciously am fearing that this next month after IT will be the same as this month and my life will still be on hold. There are so many things I have in the workings and so many things I want to be doing and instead my life is just quasi on hold and it sucks. I hate MPS right now and I hate pain, it is the most absolute horribl thing in the entire world I do think. I hate this neck pain and aching back, these headaches and nausea, the constant tiredness. It all just sucks.

Saturday, June 30, 2007 1:53 PM CDT

--Updated Schedule for July beginning of August--

Tuesday July 3rd: PT 9Am
Genetics CHW LuAnn/Dr.Rhead
Friday - ERT (every friday) meet John A.

Friday July 13th 10:30/11:00 Lunch with Karen O'Neill, Paula Ciampa - Genzyme in Mpls.

Sat. July 14th U of MN MPS Meeting

Sun/Mon 15th/16th-20th Go to CA - UCLA Harbor
--- 2nd IT infusion 7/17

Thursday August 1st and 2nd or 8th and 9th Trip to Boston
-speak at Genzyme..

August 11th Fundraiser/Awareness Event at Raceway

This week I got an email from Bill Cummings at Genzyme inviting me to come back to Boston to speak at Genzyme on a
panel again this time with teens and young adults. I am of course very excited, I had so much fun in Boston in May and
really looking fwd to going back again!!

This week I have started PT for knee/Hip as one means of 'alternative' medicine - their focus is 'deep massage of the tendon/muscle between the knee/hip joints as this IT Band is very shortened and thus causing my knee cap to be out of line. My knee caps are also smaller than normal which they said can affect the issues I am having. The massage does feel relatively good when they do it but very sore later on in the day! They did say this would eventually become less and less though with each session. -- I also have been pretty worn out from the past week and have been sleeping late atleast the few days that I could. My neck where the IT was injected is still pretty sore and a eal pain but each day does slowly, finally seem to be getting a little better. Now if the overall pain radiating down my arm from that site would improve I would be thrilled! My Clinical Study Dr. at UCLA
and Genetics team here have collaborated together and prescribed a Muscle relaxant which I take when I am able.

Otherwise I haven't been getting alot done this week, I am a little behind on the fundraiser/awareness campainging and f/up with people about sponsorships (eek) so hopefully my energy will be better this next week and I will have more time! Time is definetly of the essence it seems!!!

All for now, more soon.

Tuesday, June 26, 2007 8:52 PM CDT

I have been home from Los Angeles for a couple days now, I actually slept from about 12am to 1pm on Monday, and except waking up briefly a time or two slept right through!
I think my body was just purely exhausted. I have been con'ting to have some pain at the injection site where the Intrathechal was given in my neck and surrounding area, along with mild headache and nausea today seems to be worse
than yesterday even. I have been emailing with Dr.Dickson, the Study Director and she thought (as did I) that maybe it
was from all the hustle of traveling, and hte pain would improve but to let her know. For right now I am taking Tylenol and Tramadol (ultram) and then Darvocet along with Zofran for Nausea. I really didn't think the pain would last this long, I think this is a bit dscouraging that I still have pain almost a week later I do hope it gets better fairly quickly. After all it is only 3 or so weeks till I return to UCLA for the 2nd Injection!

The next few weeks promise to be a little chaotic some days
Thurs. I have Pulmonary and Physical Tx. Appts. and on Fri.
ERT infusion and after Audiology. Next week in addition to
watching my Nephew Zander I have Appts. on Tues. (Genetics)
and PT on Thurs., with ERT on Friday. The following week I
go to MN for the MPS meeting 7/14 but on 7/13 have a lunch meeting with Karen O'Neill and Paula Ciampa of Genzyme and Laren's friend Kristin. Shortly after that I should be heading back to UCLA.

Yesterday I developed the pictures that I have from this past week which indlude Dr.Dickson, and Alla, Barb Lyons and Jake, Daphne (one of Dr.D's little girls) and Ivan and Maia (Alla's kids). I will try to post them here soon, they
are very cute and nice pictures!

Please say a prayer this pain eases, I need to go lay down right now I have taken an extra pain med and hope it kicks in soon. I hope!

Erica

Sunday, June 24, 2007 1:28 AM CDT

Copied from the group email I sent out today, update on the
IT and past days.

Some of you are wondering if I fell off of the face of the earth after Weds., I apologize! I went in-patient after repeat testing on Weds morning, and about 2:30 CA time, we went down to Radiology from Research center. From there they prepped and =( shaved at the base of my skull and then put me under, after which I didn't wake up till I was back in the room upstairs. I actually had no side effects like seizures, headaches, etc. just some slow breathing (gave oxygen) and some pain. I actually even was able to eat after I woke up more, which Barb brought me food from 'outside' an other than that pain at the sight I stayed awake talking and all till 9:30 ishwhen Barb left. Thurs. Dr.D. came back and prescribed stronger meds as the night was rough, and Barb did the floor infusion in-service and they got that under way. And then in addition to having them as visitors and the others I made friends with, Paula at Genzyme flew in from Boston on Weds and came to visit on Thurs!!! Very nice! Her and I are already planning a trip for Aug. or so when she is living in the area permanently and she will drive down again

I'll go back to how it was in a minute but I got out of Harbor Thurs. real late afternoon and went home with Barb Lyons whom a few of you know. (she is a doll and my new CA Mom I think!) ;) I spent the night there and this morning was not in a good way, so they where already iffy on my flying home today (fri) so she 1st got me taken care of then started her calls to Patti Dickson and Alla for flight. So thus I spent the afternoon not on a plane (or 2!) but back at Harbor on the GCRC under going scans and Neuro Exams by 5-6 different specialists! ugh!! After that I did get to be released again and Dr.Dickson and her staff started me on Prednisone (for those of you who don't know the whole story closely Prednis. is what my Genetics team and Dr.D. took me off of before ERT infusions a few weeks before coming to CA for this!) So I am on 50mgs of Predn. for 5 days and then 30mgs for 3 days b/c they think there is some drug induced Inflammation/meningitis type problem going on and along with bleeding/blood clot at the site. The Prednisone is in addition to 650mgs of Ibuprofen, 250mgs of Tylenol, 20mgs of Oxycodone and 8 mgs of Zofran (all prescription) that Dr.Dickson started me on and that we are taking like clockwork b/c the pain is very, very aweful in those locations from my shoulder blades/neck up and then into Head. The plan is the future IT's to keep them in-patient, but in addition to the Anesthesia narcotics begin these regimen before I even go in-patient and they will have the prescriptions waiting in out-patient pharmacy ahead of time each time for me. Tonight since I was just starting to feel a little better I spent the night with Dr.Dickson and her family and we are planning to head to Redondo Beach to the Fair on Sat. with her kids. (5 and 1 1/2) I can't wait to show pictures!

So in all it was not a bad experience, many neat visitors and nice surprises and even when in pain they take care of you like royalty so you still feel human. I think this may be the last update from CA, for this trip - I return in a month, for which they are working on schedule now. A friend of mine from Tx may fly in to stay with me then, and another friend at Genzyme will be around as well as Barb then. Before that trip i'll head to MN, where i'll talk to Dr.Charnas him and Dr.Dickson are tentatively talking about the future plan and he has 'taken on' the role as my primary dr. for handling this compression, and coordinating with her. I think although it is a bit more complicated to have another set of drs. here in CA, so far it is working out ok and all teams have really worked well to try and make things go smooth. Is interesting! Harder even to believe that the 1st one is over, and I am recovering!!!

Much love,

Erica

Tuesday, June 19, 2007 11:20 PM CDT

I have been keeping many of you updated by 'group' email and some phone calls or through the Forum but though for any one I may have missed I would update here.

Today I completed the MRIs of Brain and Spine and after went over and met the GCRC/Hospital staff and on to SSEP testing. During the 2nd phase of testing Dr.Dickson was speaking with the NeuroRadiologist they where looking over the MRI films from this time and from back in Febr. and there has been some progression, more Cord flattening, no Spinal Cord signal (which means lack of fluid I think) so although those are bad things they explain my progression of symptoms and I do qualify for the Intrathecal Study.

Tomorrow (weds) I do repeat 6Minute Walk test, Labs, Physical Exam and then we head over to the General Clinical
Research center at the Harbor where we begin in-patient. From there they'll start the IV, hook up a million monitors, and go on down to Radiology where they do the IT under Fluroscopy. Before they do the actual IT procedure where as most know they inject the Aldurazyme into my Cervical (neck) space just below the Skull base they will measure the opening pressure which will give us the first truelly accurate reading of what my ICP is, and an idea of what they might need to do with the Shunt, etc. (which Dr.D and the Neurol. here and my Neurologist at MN are thinking maybe the Shunt being that it is located at the Lumbar area is causing the Symptoms and Compression to accelerate faster than it otherwise would. So anyways after
that they will inject the Intrhathecal Aldurazyme.

I'll spend Weds night in-patient in the GCRC at Harbor and Thurs will receive my regular ERT infusion (which I otherwise normally receive on Fri's.) and Paula Ciampa (who is a PCL with Genzyme and flying in from Boston on Weds to stay till Fri!!!) is coming to meet me, which I am very excited for! I had not realized it, but apparently her and I have met before in AZ at the MPS Conference at the time I met Naomi! So anyways she is coming in for me, and Barb Lyons is also coming and I am going with her for the day - it seems that I will spend all of Thurs and night
with her but I am not sure of that, it is just what the Study people have told me Barb told them. Not sure! I jsut know Barb asked me yesterday if I would like spend Thurs with her, which of course will be nice!!! (she is amazing!!)

So that is the update, I am not sure when I will get here to update again, but hopefully someone can send a group email for me.

Lots and lots of love to all my family, friends and providers!!

Erica

Monday, June 18, 2007 6:26 PM CDT

Just a short update to say I am in LA! My flight arrived about 4pm yesterday (sun) and Dr.Dickson met me at the airport. From there we went to Manhattan beach shopping and then to Hermosa Beach where we had supper and a little more shopping!! (fun!) Today was the consent, Physical and Neurologic exams, 6MWT and Eye test which went well enough.
Right now I am sitting in the Clinical Lab Managers office
before we head back to the Hotel and then on to Supper at the Cheesecake factory. (Myself, Dr.D., Merry, and Barb L.)
More tomorrow!

Saturday, June 9, 2007 6:01 PM CDT

Yesterday my good friend Amy Donaldson lost her fight to Cancer - may you rest freel of your earthly pain and know we will all do all we can to be there for your family as they experience the loss of a wonderful Mother and Wife and friend. Amy know that you touched so many of us and I am so fortunate to have known you and met you and had your friendship. My sadness is profound as your life here on earth was so short and yet you managed to do so much good, we have all lost a great person. We will be there to watch
over your sons as they grow and to help your husband with the struggles we all know will be there. We will fight even
harder for better answers and a cure for MPS so that your sweet son may grow, and the battle for cancer although a fierce one will not stop at our loss of you. I wish all of you could have known my friend Amy, she was sweet, funny
and the kind of friend we all only hope to have.

I don't know what to write, there are updates to be written
but right now seems not the time to right them here, come back in another day and they will be here but for now, just
reflect on the greatness of this life we have and if you are not already reach out and touch another persons life, in memory of my friend Amy.

Saturday, June 2, 2007 1:17 PM CDT

Schedule - June 2007
6/14 Final Appt. Dr.Brosig
6/15 ERT and Audiologist
6/17 Fly to CA

6/18 Consent with Dr.Dickson
P.Exam(s) Dr.D and Neurologist
6MWT and Eye
6/19 MRI Brain and Spine
Somatosensory Evoked Potentials (SSEP)
6/20 Admitted to UCLA Harbor
6MWT/Eye repeated
PE's repeated Dr.D and Neurol.
Intrathecal Injection (cisternal tap)
6/21 Released

Kathleen, (who is probably reading this!!) is sending the pictures from the days in Boston, they should be here any day! Yippee!!

I also have been burying in doing Walk things working with Bill and Karen on Genzyme's involvement and also this next week speaking with a woman who has a walk of her own which is in multiplie states for Colon Cancer Awareness! This woman is helping us with anything she can on our walk also.
I also spoke with Karen on Friday and she is looking for some info for me from the Registry meeting as well as gathering other infor. to add. She is so, so sweet! I am so
lucky to be able to work with a company like Genzyme, all of the employees there I have come to know have become like
friends as well which has been very great to.

I also have been working burying myself in the applications
process for the AmeriCorps program which you may know is the US vs. of Peace Corps and people are able to apply in any state and area and many offer pretty decent benefits and great opportunities! I have to talk to my GC yet and get her take but I am applying to multiple sites as I really want to get into this and be able to go out adn do something that is unique and making a difference while I would still be able to do my ERT and go to school at whatever location I was in. Right now I have applied to Milwa. locations, (2) 3 in Boston area and looking into the
MN and maybe OK areas as well. As lnog as the program offers living expenses, I can still be a student on the side and allows the person to have outside things going on
I think I can make my crazy life work, especially ERT. This
is a big reason for applying to big cities. Right now I also am going to talk to my Study Coord. who said she would
answer any quest. I have as she served 2 terms doing this
program also. If I am able to still be a student then I can
keep my Insur. and the program also offers Insur. I don't know what that would do with the SSI/SSDI stuff or even neccessarily with the CA study but I am determined to do something I tired of putting my life on hold. This is a way
to make a small income, make a difference and still be able to be a student. Hopefully my GC will get back to me soon also.

Today, my brother, boy am I steamin mad and if it werent a capitol crime I would kill him with my own bare hands I do think. You see for months he has been saying he wanted to go to CA for the week with me, fine, great really would be fun. This past week the SC booked the Airline tickets, they
are expensive for them, even with 1 stop I seen the price. And they are non refundable, non tranferable...... HE TELLS MY PARENTS HE CAN"T GET OFF WORK!!!!! IS there any particular reason he waited till now? So do you blame me for being p.o.'ed???? So of course my parents think it is as easy as the Study Coord. changing the ticket to my Dad's name, which as you read above, NONTRANSFERRABLE, they totally don't get that concept so now we are arguing
and I am frustrated. I will just fly out alone and go solo, maybe one of my friends can pick me up at the hotel. Grr I am so angry, and I hate being angry, I do not like being an angry kind of person. I need to move, then I will get along better with my parents. Please AmeriCorps hire me!!!!!!

I don't know what is going to happen with the Talk we where wanting to give at Childrens the day before our Walk as usual no one in Genetic is being particularly helpful and they do not seem to want to consider the idea. It is so not about PR but about helping out patients and educating Physicians and stff, don't know why they don't get that! Grr what a week in some ways!!!!! No one wonder I dye my hair I will be grey by the time I am 30!

More applications to do, talk soon. Thanks for checking in and sorry this is all a vent session!!!!

Erica

Ooh yes, we are slowly moving fwd on school things for fall and will meet with counselor again on 25th for classes and ADA accomod. counselor July 19th to begin IEP planning. IF by some chance I could get into a Americorps
program starting in the next few months I would abolutely do it and put off school another year here. I would enroll whereever I relocated to. Otherwise several of the program begin in Jan 08. PS: I have to figure out some financial things also for school yey yippe. not, lol.

Monday, May 28, 2007 5:28 PM CDT

Weds. May 31st MPTC Campus 9:15 meeting
June 5th. Appt. Dr.Zuccaro (change to 6th?) Audiologist 6/6
June 17th Fly to CA (UCLA Harbor)
6/18 Consent and PE Dr.Dickson, Appt. Opthalm.(?)
6/19 MRI's (Brain/Spine) Neuro Exam, SSEP testing, 6MWT
6/20 Admitted to Harbor begin IT - spend the night.
6/21 Released if not complications
6/22 Lunch with Stephanie Okey and/or Barb Lyons (??)
6/23 Fly Home

I don't know what to write about really, this past week has
been somewhat of a 'slow' one and I have been a little down
many days. I see my PCP next week and actually going to have lunch with Barb Pfeiefer whose little girl is in the process of being dx'd. right after I have the Appt. Anyways
I am seeing PCP for headaches but suppose it is time to call my NS also? My GC thinks that maybe the Headaches are related to allergies or sinuses somewhat to, which they could be but I also know that there is pressure going on, it is a very distinct yucky feeling.

I have been out in the garden(s) planting some lately which
is nice, relaxing although it gives me a sure backache! I am looking fwd to as more flowers start to come up and as I
plant more. Also we have opened our pool up for the summer and hopfully it will warm up again very quickly! I really don't have anything planned exciting for awhile, going to see if some of my friends want to go out or see a movie or something and maybe if my Nephew and his Mom want to go to the Zoo as well. I desperately need it to be Fall! Summer is my favorite time of year but I cannot wait to be busy again with school and classes. Other than the CA study once a month all that there really is going on this summer is a few meetings in June and July (hoping to drive down to Chicago June 8th for the weekend) and planning for the Fundraisers in August and Sept.

I am pretty sure once MCFI is moved into their nmew location I will try to get a job part time with them as I have already talked to the Nurses who run the Peds. Special Needs program and am hoping to stop in and meet the kids, see the place and talk more about possibly working there this Thurs. I don't know if I would go their, but if they hired me I would! I think that would be atleast a couple mnths down the line, although I am not sure. In any case I need something else to do this summer so I think I am going to try to get a job in the Garden center at one of the local Stores for the summer months.

Slowly but surely I am adding new pictures to my site, and will con't to do so until I get all of the ones from Boston loaded. =) Thank you for checking in and con'ting to stop by!

Erica

P.S. We are (Bill at Genzyme and I) working to set up a Join the Search Event at Children's Hospital Sept. 7th, the day before our Walk in Oshkosh. We hope to bring in the
JTS displays, have a talk or several and just educate physicians and staff. Karen O'Neill whom I will be speaking with at the Walk would also be there from Genzyme.

Wednesday, May 23, 2007 9:07 PM CDT

I was wrong about the new dates for the CA Intrathecal Study (my bad) these have been pushed back from the 1st week of June to the 3rd week so 17th-23rd.

17th - Aaron and I fly in to LA
Mon. 18th - Consent with Dr.Dickson
Physical Exams Dr.D and Opthalm.
Tues. 19th - MRI (Brain and Spine)
Somatosensory Evoked Potentials
6MWT (I think)
Neurologist
Weds. 20th - Intrathecal ERT injection Harbor Hospital
Spend that night in-patient
Thurs. 21st - released back to Hotel
Fri. 22nd - Lunch with Stephanie Okey (?)
Sat. 23rd - Fly Home

July 3rd - Genetics Appts. (Plan) LuAnn/Dr.Rhead
July 14th - U of MN/Genzyme MPS Family Meeting

Today I spoke with Bill Cummings (US Marketing Aldurazyme, Genzyme) and we came up with some more ideas for the Walk in Oshkosh Sept. 8th. Bill is also talking to Mary Yuska and Karen O'Neill about bringing in the 'Join the Search' Display and having a presentation/talk given on this at Children's Hospital on Friday the 7th. How cool would this be? We will con't to work on these things and the walk and will talk again soon. I will see Karen, Mary and Steph Ginty in July in Mpls. at the MPS meeting.

I have hit a slight 'road block' with my transcripts from LTC where I was a student and am awaiting word from my Cousnelors at DVR and MPTC, we also have a meeting next Weds. at the school. I really am looking fwd to being a student again in the Fall - I know it will be somewhat tricky with doing this and Appts. and Intrathecal Study, ERT and all else that goes on but going back to school is so important to me and I am so looking fwd to it.

My Dad is in negotiations right now with the School Districts Union/Insurance, right now we have WEA which is expensive but phenomenal in what they cover. If BDUSD switches Insurance we will go to Dean Health Plan which has a $5 million cap but I will still be able to be covered
b/c of making under a certain amount and going back to school.

This Monday the local Paper ran an interview that was done of myself on Friday after I got home from ERT, Terri Pederson did a really great job! You can go to www.citizenol.com and search keyword Erica Thiel The article really is very good.

Otherwise this week has been slow, catching up on sleep. I saw my Psychologist on Monday which is one of the last times I will see her before she goes to her new clinic. I'll miss her but I guess we have made alot of progress in the 2 1/2 years I saw her and it is okay to move on. I rescheduled my Appt. with Audiology, and actually got the contact info for Dr.O'Grady today so I sent her an email and also gave her contact info to my Dr. My new Appt. will be in 2 weeks. I also see Dr.Zuccaro that same week for Headaches. Not sure what to do about these yet, maybe she will have some ideas and I should talk to my GC one of these days.

I did update a few pictures from last week, and will upload the rest soon and when I get the others. I can't believe it's been a week since I was in Boston!

I better run. Lots of love, and thaks for coming by to read.

Erica

Saturday, May 19, 2007 1:55 PM CDT

INT'l MPS AWARENESS DAY at GENZYME CENTER May 15th, 2007 was GREAT! There is so much to tell about my week in Boston
and funny stories and just tons and tons it could take me all day! The panel which was myself, Denise and Barbara speaking to Genzyme Center and broadcast int'ly was a outstanding success it seems and all through the week people came up to me at Genzyme and thanked us, which was rewarding although really it was just a very fun experience
for me and I am so thankful to Genzyme for all they did for
us. I will upload my pictures when I get a chance, and Simone and Marie who are with Global Aldurazyme Marketing are also going to be sending me their pictures of all of us. After the Panel we had lunch with abunch of Genzyme people and THEN we went back up to Steph's floor to ring the GONG! OMG, this was so funny, I hit it so hard I must have just about jumped out of my skin it was sooooo loud! Funny! I cannot wait to see pictures!!! After this we went to the Allston Plant and toured which was great and Jenn S. came with me, a neat experience. From their I went back to genzyme center and spent more time with Marie, Kathleen,
Bill and Naomi talking before Marie and I and Pablo went to the Beach (THE OCEAN!!!) I have lots of pics from this and shells and driftwood and funny, funny stories from this to as all had told us NOT to go to Revere Beach!

That was all Tues. I actually arrived Monday into Boston early afternoon and after getting to Hotel (beautfiul retro
mixed with funk mixed with old style!) went to my first day at Genzyme and ran into Marie right away for the taping. The video which I will get a copy of soon to took only about 3 hours total I think and consisted of lots of questions answered in front of the camera of myself and then footage t/out Genzyme. Guess who I got to seee, yeh for the first of many times anyways in the week - My Steph!! Ahh Hugs and kisses all around!!!!! After the video we where off to Supper at Dante's which genzyme people now have a new "favorite" expression for the restaurant thanks to me, lol. "Not Normal" cause I had a hard time figuring out what to eat, Steph and I shared lots of things!!! I loved, loved, loved spending time with
everybody nad catching up with Steph - bliss, pure, bliss!

Genzyme of course did so much for us, between the site seeing they took us on, and the Ocean and Suppers and lunches and the Panel nad Video to, they also got us a beautiful book all on Boston sites and for which many of them signed for me. And also many different Genzyme gifts to me. =) (pens, coffee cup, bag, m&m's. Expressions of Hope cubic block (stress reliever), etc.

Weds. I left Hotel Marlow and went to Jenn Siedman's house which is in Wellesley (home of the famous Ivy League girls school) and spent the night there with her and Ben and her Hubby Stuart and her kids which was a blast! Thurs. we did some site seeing in Boston on the way to Cambridge back to Genzyme Center to have lunch with Steph Ginty and Naomi O'Brien and after I spent the afternoon with Simone and meetig other Genzyme people which was great. I flew home about 6 eastern time and got in to Milw. about 7:30-8 Central time.

Friday was back to ERT and no pre-meds except Zyrtec and it went well! The fundraiser at H.S. went well, without me
as I couldn't be there for obvious reasons and then drug delay.

Last Friday I had talked to LuAnn, my GC for quite awhile about my HA's and symptoms and fiuring out what to do, I have to say I didn't connect with many people on getting Appts. set up but was busy to. I spoke with LuAnn briefly this Frday and she is going to call me Monday. On Monday I
have Appts. in Dr.Brosig, with Kim Jones, my Audiologist and at MCFI to as well as Dr.Patti Dickson from UCLA Harbor is going to be calling me. (this per Alla who I spoke with on Friday over the phone) Our IT start dates have been pushed back again but just by a week to June 8th-17th. depending on which days I want to go or if I want to go the entire week and get it all done.

This next week I will be back into routine I guess but also
connecting with Bill again at Genzyme regarding some ideas they have and also our Fundraisers this fall which he and Karen O'Neill are helping with.

This is all think will get pics up soon. - THANK you SO much to Steph, Kathleen, Bill, Marie, Faraz, Naomi, Simone,
Pablo, Sara and all who spent time with us while in Boston - I can't wait to come back!!!!

See you in July Steph! (((Hugs))
All my love, to all,

Erica

Monday, May 7, 2007 9:22 PM CDT

Well I don't know what to write? This may come back to bite
me but I am frustrated with my Genetics team as they need to talk to the Study Dr. at CA (Dr.Dickson) b/c she has some questions for them but they have not been calling her back.

I saw Endocrine last week, and just got the new scrpt from Dr.Maas today - she is increasing the Thyroid dose up by 25mcgs. to 75mcgs. I also saw my Audiologist last week and
hearing tests (which she is going to repeat in 2 weeks) showed small improvement in my left ear and bigger improvement in my right ear! If tests show these same improvements next test then no need to get the right hearing aid!

A week from now I will be in Boston, my flight from Milw. is at 9:55 and I get in at 1:04! Genzyme booked the Hotel Marlow which looks so fancy! Tues. is the Video interview in the morning and panel from 12-1. I'll stay at the Hotel through Weds. and then stay with my friend Jenn and her family till Thurs late afternoon! It sounds like I get to go to Fenway Park (red sox) and the Aquarium with her sons class!!

Remember that next Tues. is National MPS Awareness Day which is why I am speaking at Genzyme - and the High school
here is holding a Fundraiser throughout the week for this! I will hopefully get home from ERT early enough next Fri to
be there atlest the one day/lunch periods. * Also August 11th we are holding the Fundraiser at the Raceway for MPS Awareness locally.

Otherwise I have been doing ok, the Pressure Headaches from my Fluid not getting to or around the compression or the Shunt not working right (maybe both) has been getting worse but I do not want to do anything as I am hoping I can wait till the week I go to CA for IT. (if my Dr./providers ever talk to Dr.Dickson anyways...) I am crossing my fingers I can wait that long and I get into the
actual study as my pre-meds are a question now. Ugh tehe headaches and shunt! The other thing has been hip/knee pain which has been worse since I started walking more.

The meeting in MN for MPS is July 14th this year. I am goin to take Alexis's Mom Barb with me this year. I first met her and Lexi and family this weekend they are so great!
I also am going to go in to the day care Pediatric center where Lexi goes and apply (I met the Nurses) as they said I should stop in soon.

I talked to LuAnn on Friday (my GC) and no word from the other patient they contacted to see if would talk to me. I hope this other girl will, please I hope! I know there is something else her and I talked about that I was going to update but I cannot remember what it was! Oh well.

I'll try to update again soon, or atleast after I get home from Boston next week.
Love,

Erica

Thursday April 26th, 2007 1:07PM CDT

I am home from MN and NeuroPsych testing which went well. Improvements actually even on some of the testing! How exciting and I do not need to repeat those for 4 months. it was kind of late by the time I settled at the Hotel Monday so I just chilled out and after Appt. in the AM and a nap in the afternoon I went nad met Kindall Nelson nad her daughter Lauren (Hurlers) which was nice.

Weds. I met Karen O'Neill (Genzyme) for
lunch which was really great and I love her! She is working with me on a Fundaraiser at the local Raceway and also planng to come to the Walk/run we are holding this Fall! I'll put up the new picture of her and I soon.

It looks like the new dates forb the CA
Study will be the 1st week of June and Dr.Dickson is talking to my GC. She is concerned of the Headaches I am having but we both hope the will not get worse
before June so that I can wait until then to do MRI's and she'll check pressures. (ICP)

Today I am at Children's Hospital - this AM I met with my Pschologist whom I like alot and trust. She is leaving to go to a new position at CHW in the Heart center which I know she will be very geat at, i'll just miss her! I'll probably only see her 2 more times. I also this afternoon have the Appts. in Cardio with Dr.Earing, we'll see how that goes. I also met with Patti Chico
just briefly a little while ago before Dr.Dickson (the other Patti!) called.

I'll keep all updated on all of the changes and new things should be an interesting if not always somewhat liveally few weeks! My genetics Appt. has been changed to May 30th. although Patti C. is meeting me at the
NS clinic tomorrow for UGAG Study testing. Is all for this day!

---------------------------------------
-Monday, April 23, 2007 4:12PM
I only have a very few minutes before I need to leave to fly to MN but wanted to update a few things.

Today I found out that I have been invited to come out to Boston (MA) May 15th for MPS Awareness Day and speak with 2 other pts. in front of Genzyme personnel! Afterwards I will do a Video interview with myself and Marie Muniz who works in Marketing there is a good friend. I am VERY excited to see Boston and even more excited to see Steph again (!!!) as well as Naomi and Marie!

That of course means pushing back the start date again for the CA IT Study but I really do not want to pass up this opportunity! Hopefully the Study can start that following week? This also means I should be home for my Sister's B-day. On that front Dr.Dickson and Alla V. are awaiting word from the UCLA IRB on their decision and what information they need yet.

Weds. last week I did the Cardiolite Stress test for this weeks Cardiology Appt. not the best test, but not the worst either. They do pET scans before hand and then inject dye and have you walk/jog on a treadmill and follow all of your heart patterns and then after do more PET scans
to see the heart and heart muscles response to exercise. I think I did alright, I know my Heart was working over time before I even started walking (faster than should have been
rate) but ooh well, what can yeh do? I already take Nadolol
for it..

Sat. night I heard from a Mom whom I had found an articl on her daughter at the church where my Neice was baptized, I had thought the little girl looked way to much like she had MPS... well it turns out the Geneticist they are going to that is one of the tests he has run and they are waiting on! The Mom just was excited to talk and I gave her alot of information on Drs., etc. as well as she is bringing Miss Alexis down to the ERT infusion clinic on Fri
to meet Dr.Rhead, my Geneticist. You wouldn't believe how much Alexis looks like the other Hurlers children, it is astounding!

Friday night on my way to somewhere I got a call back from my GC, we talked for quite awhile, apparently they have someone they are waiting to hear back from "a young adult women" that they hope will agree to talk to me! We also talked about schedules and ERT, IT Study, the current Study
I am in, school and what I am doing for exercise. It was a nice conversation filled with alot!

Tomorrow I have the NeuroPsych testing with Kendra, and tonight possibly having supper once I get to Mpls. with a friend and another MPS family. Tomorrow the testing and supper with yet another family, and Weds. I am meeting Karen O'Neill (Genzyme Patient Care Liasion) for Lunch! Thurs. is Appts. with Dr.Brosig and then Echo and Appt. with my Cardiologist and then spending the night at Kris's. Friday is ERT and Alexis and Barb coming to the clinic.

I have to run more later, running behind, oops!

Erica

Monday, April 9, 2007 3:05 PM CDT

An update before I head over to Zander's house in a little while. I don't know that there is much to update on but i'll give the latest I guess.

I did talk to Dr.Dickson last Monday and her Study Coord. Alla in the days since then and their IRB (Internal Review Board) meets this Tues. and should have an answer if they are approving the Addendum to Consent form/Study or need more information written in. (this is regarding Dr.Dickson injecting the Aldurazyme via my Neck and associated risks) They are also working on the schedule and as long as they get IRB approval the 1st injection would occur the week of April 29th. after 2 days of testing, results and consent. Unless I decide to not participate or my Genetics team feel that I should come home in between testing and first IT. (in that case I would then fly back to CA within a few weeks). I hven't really decided anything, and will wait until I talk to my GC this next week.

In the mean time I am changing around a few Appts. nad also
my NeuroPsychologists at the U of MN would like to have me do repeat NP testing right before I go to CA. Today I talked to my Study Coord. for quite a bit which was nice, just an unbiased mind to run ideas and thoguhts through and she gave me some ideas of things to do in order to make a decision. I also saw my Psychologist for the 1st time in about a month - our schedules have jsut not matched up well
which is ok, we meet when I can. Anyways it turns out she is leaving her position in July and so I need to find a new Therapist which stinks. I hate making new relationships in this type of case b/c that person gets to
know everything about you...... I am thinknig of maybe just seeing a Psychol. only for Relaxation training and BioFeedback and will talk to LuAnn and Steph as I always do and otherwise just talk to friends on decisons, I am jut not sure I have the energy to get to know someone and explain all about me right now with everything else going on.

Other than NeuroPscyh testing upcoming tests/Appts. include
UGAG Study, (if I do IT) Cardiology (including Cardiolite test, and Echo and Holter monitor), Endocrine 3 month, and Audiology 6 month.

Weds I meet with School Counselor and Accomodations person which I hope goes well I am looking fwd to getting more information and starting planning. Otherwise I have just been doing stuff with Zander the days I watch him and spending time with Quinn and Jamie K.

A wonderful friend of mine runs an Non-profit Foundation (Leslie Jurado) in honor of her daughter Izzy who has MPS III and Leslie has been kind enough to help with travel expenses to MN this next time - both Gas and Hotel. I am so relieved and so joyed to have such good friend like her! (and Tracy to!) B/c I am not working other than watching my Nephew I really have no income right now except for a small amount each week and so money is so tight it is stinky... I can't wait until the day when SSI is approved for that back up Insurance and just so I will not struggle to buy my medications
each month and other costs that I have. I hate living week to week like that!

last week my Case Manager Steph was able to finally get things all settled out with my Insurance - the Aldurazyme will now be coming out of my Lifetime max (major medical vs. pharmacy benefit) and co-pay every 2 weeks/month is up - grr! I am so indebted to Steph for being such an awesome and persistent CM and an even better friend!!

MARK THIS DATE _ SEPT. 9TH. Lukies's Legacy a Walk for MPS/ML in Oshkosh, WI. More details soon - but this will be a phenomenal event and I hope all will consider coming!!! Jill is working hard on this and I am helping with sponsors, etc.

I am not sure there is really anything else to "report" maybe next week will be more news once I talk to my GC.. who knows.
Sending lots of love to all,

Erica

Monday, April 2, 2007 7:37 PM CDT

Well I know I am some weeks behind on updating here so I will try my best to get all up to speed. .....

Last week I fell down the stairs at my Sister's house probably due to a combintion of things but needless to say was not feeling to great for several days. On Sunday 3/25
we had the MPS Family get-together which was really very nice, just relaxing and good to see families and meet many whom I had talked to but never met in person yet. On

Monday I flew out to the U of MN, to see Dr.Charnas my Neurologist for a 6month Fup on Hydrocephalus and this memory medicine as well but we also had him look at my past and recent MRI films. He believes there is Significant Narrowing and compression and reccomended the IT Study at UCLA CA.
.. On Thurs I met with Genetics and spent 2 hours talking to my GC and just discussing what to do as well a couple other questions she had researched for me. Their concensus (or atleast my GC) was that we would wait and see if the Principal Investigator Dr.Dickson felt I was a good candidate to come try out and if so do that. From there I could decide if I wanted to con't on that avenue (requires 4 injections over 4 months on Aldurazyme Enzyme Replcement into the Spine. My GC felt that atleast if I were in the Study at UCLA they would be keeping a very close on the Compression and symptoms and watching to make sure it was not getting worse. *(the hope with IT injections in this study is to reduce the Compression in the C-Spine thus avoiding the need for De-compression surgery which is so incredibly risky in MPS patients. )*
****** My Geneticist brought up my weekly ERT infusions and the fact that although these arent written on the drug label to be given this way we could try going to bi-weekly infusions at double the dose over a longer time to help with IV sticks. (the doses that I would get in 2 weeks would be doubled together and given in 1 week with 1 week off) The thing is that there is no scientific data backing that this method will even work and the 1/2 life of Alurazyme in the body is something like 4-6hrs. meaning
that I would be going a significant amount of days more with no Enzyme to break down/slow down the disease progression. I talkd to Dr.Dickson about this today and she said she wouldn't reccomend it as their is no data and
she doesn't believe it would be as effective. My Dr. told me also something to this affect so what on earth am I supposed to think? Yes, it would be nice to reduce how often I go to the clinic especially if I am doing this IT once a month in CA as I will still have Appts. in Milw. to some weeks but tehn again is it worth messing with something that we have tuned to working well for me and not to mention remember all the struggles we have had in the past with just staying at a 4 hour infusion... Imagine
what would ensue if I had to go over 8 hours? I am not saying I won't try it - just trying to write it out and ask for any, very unknown! My GC did say we could try it and if it didn't work or I didn't like it she wouldn't give up my Spot at the clinic on the other week so maybe I should? Aagh!

There are so many, many decisions and things today.....
The other thing is we are looking into pursuing Alternative Medicine approaches to Pain Mngmt and daily life like Biofeedback and Relaxation and Suplements. I don't want to change up a bazilion things all at once but so many different things want to look at!..

This afternoon I talkd to Dr.Patti Dickson on the phone, and she and her team of Drs. believe I fit the criteria to come out for the Initial Study testing and have a good chance of meetng the entry points. Alla Victoroff who is her Study Coord. will start working on the dates for all this, is something like 4 days of grueling tests. I am relieved to atleast be able to go do the testing not that I want to but that it means I have a reasonable chance at getting in and possibly avoiding Surgery. (again no one knows that it will help but that is why it is a study and we can hope and pray!)

She and her team are writing in a special section to the release form and have approval from the Hospital IRB to use a untested (in humans with Aldurazyme anyways) method of injecting the IT Aldurazymw Drug. This is a less straight forward method and risks may be higher (seizures, affecting breathing, etc) b/c the Lumbar Puncture would instead be done injecting into my Neck at/above the Chord compression using Fluroscopy. (which is intensive amounts of Radiation but shows real time pictures of where the needle is at.) So those are huge risks and uncertainties
but the Surgery to decompress is probably even riskier. One thing is that this approach injecting right at the sight of Compression might be more effective at treating the Brain and Neurologic stuff but that isn't known either.

Then there is the question of my Shunt location being in the Lumbar spine which is why we cannot do the IT injections there. My Geneticist thought I would be excluded
because the drug would just leak into my Shunt and stomach vs. entering the Spine/Brain but then Dr.Dickson told me today about their plan to use the alternative approach and having gotten approval. Dr.Charnas (my Neurol), Dr.Dickson and her Neurologist all wonder though if I am still having high Intracranial pressure (ICP) and the Shunt isn't able to get an adequate sense of how much fluid it really needs to drain. (b/c of compression there is little to none Cerebral Spinal fluid flow through the compressed areas.) So they would be measuring CSF pressure at each injection and if it was high and did not improve with IT then the Shunt would likeally need to be revised/ completely replaced once the Study was done.

Aah and if this all isn't enough my Insurance - I got a letter from WEA last week saying that they had made a mistake on how the ALdurazyme drug was billed through them and it should have been coming out of my Lifetime Max. My Case Manager told me today she had talked to them a week and a 1/2 ago just didn't want to worry me before she left on vacation and WEA said they would not go back and take all of that money (Aldurazyme at $10,000 a week would have brought me very, very close to the Million $ max.) b/c it was their error. But starting the 28th Aldurazyme was going to be going from my Pharmacy benefit to Medical deduction. Thankfully I have the most wonderful Case Manager (nd friend) in the world and she checks LTM's and tracks them every 4 months so WEA had to admit their error and eat their costs. At this new way of Az being billed my LTM will be used up in Approx. 1 1/2 years (right now have about $735,000 left) which will bring me right around to the end of 08' and my being 25. The school district my parents work for is entering into negotiations with WEA again this summer (this per WEA telling Steph) and BDUSD may opt to up the LTM to $2Million
which would be hreat b/c then I would be able to use the Cobra extension of 36months at teh end of 08' and that would be about right for using up the 2nd Million dollars. (about 3 years to use a million dollars can you imagine???? crazy!) If WEA and BDUSD don't up the LTM to $2mil. then I would have to go onto the State High risk Insurance Pool and that would be good for a 1 time only $1million - once that was maxed I would never be able to go back to it again... We also should find out/have the SSI Appeal within the next few months, I think I should contact my Lawyer and see if he has any idea time line.

Does it sound like things have been just plain nutty in the MPS part of my world lately???? THEY HAVE!!!!!!!! Thank Heavens for next week I meet with School Counselors (I am excited!) even though I know things are going to be soo soo crazy with this Study in CA once a month and upcoming yearly Appts/tests, and everything for school and then starting school and just finding balance in every day
to avoid thinknig of this all. One thing is my Sister starts her new job tomorrow and I am watching my Nephew during the week from 5-9:30 for her and also for atleast the enxt 3 Sats. (she will only have to work 1 Sat. a month but during training has crazy, insane hours!)

So I am sure this is long, long, long and Thank you so much if you have made it this far! Please remember I value all of your support and those of you who help me are truelly My earthly Angels.

May God Bless you on this Special Holy Week - For I know that my Redeemer Lives.

Erica

Monday, March 19, 2007 4:32 PM CDT

Honestly isn't alot to update on this week I guess the major thing has been Home infusions and straightening that
out all last week. THANK YOU LuAnn and Steph and Margaret Mary for helping me all throught last week! As you know from either talking to me or reading here I have had alot of problems with Home Health and so on Monday my GC contacted me to say there might be an opening at the NS clinic again and she was looking into this for us + would let me know ASAP. While waiting to hear back from her (and praying the entire time for a spot to open back up!) I talked to Margaret Mary at TCI and Steph at Genzyme and we all where in the middle of talking to HH co. and trying to get things straightened out and make sure there where going
to be nurses every week. Well the Co. said they would ahe 2 back-up ones and initially they where training this past Fri. but then instead they where going to come this week (which this is something they have been saying for months now) but Thank Heavens my GC wrote back on Weds and said there was a spot open again and I could start right away on Fri. + to bring my meds and from then on she would take care of ordering it. I'm not sure the HH co. was thrilled
with our decision but you know what I don't really care as I hated HH and I was so tired of everything they pulled. Maybe someday it will be the right decision but right now it isn't working well.

I meet with the College Guidance Counselor, Voc. Counselor and ADA/Accomodations Coord. April 11th about Fall Sems. and starting to plan - which I am very excited about! I think I have settled on a 2 year Associate Degree 'Instructional Assistant' program which train to work in Special Education.

This weekend is the MPS/ML Family get-together in Milwaukee
right now we have about 6-8 families coming I believe which
is great!

On Monday I go up to the U of MN to see Dr.Charnas (Neurol)
for 6 month Appt. and also for him to look over the MRI films and give us his opinio on what we should do. --- Then the following Thurs. the 29th I see my Genetic Counselor and Geneticist at CHW to talk to them about what our next step might be for this Cord Compression and basically if I should see Dr.Schwender in MN (Orthop. Surgeon) or try for the Intrathecal Study at UCLA-CA. I really have no idea what Dr.Charnas or my Genetics team will reccomend I know that LuAnn will likeally lean towards whichever Dr.Charnas supports. At this same Appt. LuAnn is going to answer a bunch of questions I have about mutations and enzyme levels and how it all works, which as crazy as it sounds I am looking fwd to b/c I think it is so fascinating but yet completely don't get it!

I'm very sure there is something I am forgetting to write about but unsure what it is so will come back later if I remember!
Thanks for stopping by, remember to sign the guestbook!

Erica

Monday, March 12, 2007 10:28 PM CDT

Well I am home more than a couple days and still just getting to this update so I apologize for those of you who are wondering how the Conference was! I will include in this blog a summary of what the different Specialists I talked to said but first let me say I had such a great time seeing some of you again and meetings others for the first time! Our Next Conference is Summer 2008 in Vancouver, B.C. which seems so far away in more ways than one! Remember 3/25 is the WI Area MPS/ML Family Get-together!!

I flew into D.C. around 4:30 on Sunday last week and after an "ok" flight was so glad to be at the Hotel finally! Not to long after I was there I see Patty and Jesse T., Jenn S., Carolynn, Danny and her Mom, Barbie as well as Laurie T. and many others!! After supper and hanging out with Patty, Jenn and Jesse and then Carolynn in her room we had our MPS Breakout sessions which are always my favorite part! Here I talked to Kendra (my NeuroPsychologist) as well as Amy H., Denise, Ana Pozuelo and her Husband and after Klane White. <-- He is Awesome! he is an MPS I Father
but also an Peds. Orthopedic Surgeon and reviewed the MRI films (on disc) for us and talked for quite a while in addition to doing a cursory neurologic exam. He has answered so many of my questions and just done way more than ever expected! Monday I saw lots more families including meeting Amy (Owen's Mom) and family and attended many of the morning and afternoon meetings featuring guest speakers from the MPS Medical research side to the FDA to Eductl. IDEA updates, all interesting and I learned alot of things and information which I can use. Monday evening was the Awards banquet where I sat with some of my friends and also 3 Reprs. from Shire which was actually really interesting. (the Medical Director, a Regional business manager and a Sales/Patient Rep. ) and after had the chance to finally meet Faraz Alli (Genzyme( who I have always heard alot about from Marie Muniz and Denise - he seems like the coolest guy and funny to talk to! This also happened to be my B-day (24!!) and so after dress up clothes and a bunch of us hung out in the Lounge of the bar at Hotel and just had a good time. Tues A.M. was off to Capitol Hill for Lobbying and Lunch and here I had yet more chance to meet others I hadn't met before including Barb Lyons from BioMarin!!! I had such a good time and time sure flew talking to her about anything and everything
infusion related and non-medical related to! If you see my Collage of photos right before this Blog you will see add'tl new pictures from the Conference including one of Barb and I, Denise and I, Dr.White and I, and some of my friends. Tues. afternoon we where going to head out site-seeing at the Zoo and Aquarium but Carolynn and I just decided it was to cold so we chilled and took it easy at the Hotel the rest of the day instead. Weds. the NIH part of the Conference began where speakers, doctor's researchers, etc. from literally all over the world studying the MPS diseases presented the newest information on what is currently being studied/done. There is so much promise down the road for these diseases and I believe if there is never a cure then atleast even better strides are going to be made and are already being made. One of the hottest topics is Neurologic which is 1 of 2 hot button areas for me so I found that extremely fascinating! Another thing that is being studied, and I forget the exact wording but combining two different approaches to treating the Disease for better outcomes (ex: ERT and Intrathecal or ERT and CBT, etc.). I LOVE that there are so many Doctors and researchers working so hard on these diseases, this was such an awesome experience! Weds night I flew out of D.C. and into Atlanta on about a 2-3 hour delay (weather, mechanical, no gate) and low and behold missed my connecting flight back to Milw. which definetly was the suckiest experience!

It was very funny, any one who knows me well knows there is always someway to get ahold of me, well Tues. Carolynn and I where hanging out with little Danny (4 yrs old, so you can imagine very, very outgoing and very loud!) =) in her room and he had my phone and was playing with it when my GC called so we managed to get it away from him that I could see who was calling and answer it - well he was running around that room full speed ahead just being so silly, I thought I was surely going to pee my pants (and I thought Carolynn was going to to!) while I was trying to talk to my Genetic Counselor - one of those memorable and to funny moments! (and I am not sure how LuAnn heard anything I said!!!) Well then my GC was teasing me about "no bunny hopping'" and so later on carolynn and I went out to get something to eat and hit the Drug store and this was a hot topic of conversation not in a bad way just b/c it was cute and funny!

Below is the Summary I gave to my Doctors, I will update some other day on the hassles with HH, just put it this way
I am frustrated!

Much Love,

Erica

Summary: Opinions of Doctor's National MPS/NIH Conference

C1-2 to C4 Compression, Tx options: Intrathecal ERT (UCLA) or Posterior De-compression

Klane White, M.D. :
Reccomends Dr. James Schwender, Twin Cities Spine Center Oprthopedic Surgeon 612. 775. 6275
His opinion is that it is "early in the progress" of the Compression - although very little CSF flow at that area C1-2 to C4, given the symptoms he believes we should look at treating this sooner rather than later, although we have a time frame of months before damage could get to the point of becoming irreversible and/or even worse outcome. Window of opportunity to serious complications likeally short, given the nature of the onset of symptoms and progressive nature of MPS storage. "New" leg pain, (which Dr.Franco (Sleep Medicine) believed was related to Neurologic progression from the MPS vs. Restless Leg ) likeally related to C-Compression. Believes a Treatment/decision shouldn't be held off long, we have likeally a window of months to work within to prevent long-term damage. His reccomendation is Posterior De-Compression but since there is no damage to the spine itself a Fusion or instrumentation is not likeally to be needed. Dr.Charnas, Neurologist will determine what tests he wants ordered/done before we go fwd. on any decision, and see him the 26th of March. Not sure when he'll want me to see Dr.Schwender, maybe the same week, to save a trip. Dr.White's other thing he mentioned, although he said was less concerning to him was a 'Disc Bulge' in the lower Spine area, which he thought should be watched. He does not believe the Intrathecal ERT would benefit the Compression problem, b/c of it being both Boney and GAG/Mucopolysaccharide storage. The Orthopedic. Dr he recommends has a lot of experience with MPS patients.
Contact info: Klane White, M.D.
Seattle Children's Hospital
P: 206.987.5678
F: 206. 987. 3852 klane@mpssociety.org

Patricia Dickson, M.D. :
Lead Study Doctor Intrathecal Enzyme replacement looking at treating Mild to moderate spinal cord compression at UCLA - Harbor. This study would require 4 days of initial testing before being admitted into the IT trial and then a 1x a month trip to UCLA for Intrathecal ERT under sedation after a complete physical, and neurologic exams and testing (usually 2-3 days). Initial testing at 1 month and 4 months includes 6 minute walk test and MRI/MRA's. Extension study for 1 year of additional treatments just approved. By Irb and FDA. 2 patients in the U.S currently enrolled, the 1 patient I know has seen improvements in her physical symptoms although not on actual testing/films thus far. She is sending copies of release forms to Drs. (genetics, study coord. and Neurologist as well as NeuroSurgeon.) to include copies of NS Neurologic reports, Surgery reports, Genetic studies, MRI film(s) and Enzyme levels.

Grant Sinson, M.D. :
Believes there is some degree of narrowing in all people and this does not need to be treated, would not treat this given no damage to spine. Fup with him any Shunt problems.

Thursday, March 1, 2007 12:10 AM CST

Some of you already know about this from my posting on the MPS Forum and/or talking to you via email/phone but yesterday on top of finishing up the Endocrine Labs for Dr.Maas I also completed Spine MRI's at CHW for my Genetic team and Neurologist. Within 2 hours of the test and a 1/2 hour of being home my GC had already called and said she had the results and there where "abnormalities" could we talk. I never mind talking to her, but wow, I never like those words either! Apparently I now have "significant" narrowing in my Neck (Cervical Stenosis) which from what I understand there is some le-way where it is "a safe narrowing" and then there is "to much narrowing".. So while I do not have alot of clinical symptoms yet, and she did not think it has affected my spine, LuAnn does not know until we talk to my NS and other Drs if we have to do something Interventional like Surgery ( =( ) or watch it closely. I do not want either, I do not want to deal with tihs, can I have a pity party right now? Grr, I HATE, HATE. HATE MPS yesterday, today and Always! I will get through this it just seems like how many things else can be affected for heavens sakes I AM SUPPOSED TO BE A MILD FORM! I DO NOT know anyone with my form, my age or younger or even close to my age who has Hydrcephalus, a Shunt, Cervical Spine problems, Endocrine problems and the other things! NONE!!!!!! DID I SAY I DO NOT LIKE THIS YET?? Right now my GC has me on all kinds of restrictions, not to do anything physical, no exercise other than some walking, nothing over my head, no gymnastics type movements, etc. I do not know if this means I cannot carry my Nephew around, =( He is around 30pds I guess I have to ask, I will be so sad. =(

I did get results from my Endo and her treatment plan. The ACTH Cortisol levels are within normal and do not need to be treated and other Hormones are also in level, it is just the Thyroid that is below the low normal range again. I am now on 50mcgs of Syntroid for Life.. and F/up in 3 months. I was frustrated at first that this would be more meds when I had just gotten rid of two, but my GC pointed out yesterday that she does not see these as meds at all but simply as what they are, hormones replacing what my body needs which is a better take!

3 days until I leave for D.C., I do not know about my Luggage but I guess I can do car side drop of and ask for assistance if LuAnn wants me to get it with taking my Luggage to where I will be picked up and dropped off both times. I guess I need to call her huh?, I just hate to bug her again I know she is so busy! ** I talked to her on Thurs. and as long as I only do Luggage on wheels and do not carry anything on my shoulder or over my shoulder am fine.****

4 days until I turn 24 - Monday March 5, Yippeee!!! What a way to celebrate knowing about this newest stuff, but ooh well it will still be a Fabulous new 24th year I think! =)

Dr.Klane White is meeting me after the MPS I Breakout session on Sunday evening to look at my MRI disc to give us suggestions. I will also talk to and see my Neurologist while in D.C. Their advice will be greatly appreciated for both my sake and to take and give to my GC/Geneticist and NS later in the week. I also Fup with my own NS on Tuhrs once I return and LuAnn was sending him the report, info.
I do have to admit from what I am reading cervical neck involvement is scary business in MPS patients and it does not seem it is something they often mess around with. Dr.Kakkis thought I would be a candidate for the IT trial and I suppose we will know a little more after the films are reviewed by the MPS Drs. and by my own NS within this week. To decide if that is a option or if Surgery is needed right away or if we can even just wait and watch.

Will update later, sending all my love. Wish me luck on these newest things!!!!

Erica

Friday, February 23, 2007 6:31 PM CST

Last week Thurs after my Appt and meeting with my Clinical Study Coord. Patti I went and spent the night at Kris's house who is also the Nurse at the infusion clinic. Was fun!! Friday I went to the clinic and I seen a few of the guys including my favorite one!!! To cool! It was not at all a good IV day though it took us till the 7th try and then we ended about 45mins early b/c that IV blew and it just wasn't worth retarting. Needless to say I finally called Mark Hunter who is the guy that heads up the IV Access team at FMLH. He had a lot of amazing good info. and I gave his number to my GC and asked her if she and my Geneticist could please talk to him. After I came home and later in the day I spoke to LuAnn (my GC) for over 30mins which was a really long talk and especially b/c I had just been at an Appt. with her 4 days earlier! She is completely a nice person though and so was a nice talk and
we got some things straightened out as much as possible for now atleast. Not Port/FMLH stuff but other infusion things and she is also helping to locate a Person in her clinic who will also have a Metabolic Genetic Condition and be about my age who i could talk to!!!!

Just today I made the final arrangements for the Hotel and picked a date and time for the MPS Family get-together (March 25) email me for details. I have to pick the menu and possibly an activity in addition but it looks like there will be 3-5 families coming from out of state and 4-7 families coming from In-state! Wow!!!

I saw the Endocrine Dr. Dr.Diana Maas at FMLH this Weds (2/21) neither my GC or I really thought we'd get much out of this Appt. but she amazingly must have done a little homework on MPS and also really feels that there are 2 different things she can treat to help improve how I feel. She showed me a past Brain MRI I had an how my Pituitary gland is shaped and then explained how it should be which was prety interesting actually! She wanted to get these Labs and ACTH test for Cortisol level (Hypo-Pituitarism) before I left for D.C. March 4th so she has me coming back in next Weds morning (fasting Labs) and said she would let me know the results over the phone by Thurs (Fri lastest!!!) and get Medicines started right away! The other medicine I think she is planning to start is for Tyroid (Under-active). Drs never speed things up and solely for the sake of getting a patient feeling better ESPECIALLY when they just met you!!!! Yup, i like her already - I see her in May again.

A week and a 1/2 till my B-DAY!!! 24! Steph Ginty today told me I am getting "old" lol to funny! Of course then my friend Jamie is 7 months younger than me (Quinn's Mom) told me I was going to be old to! ;) Can't wait to D.C. Trip!!!!!

When I get home from D.C. have (as always?) Multiple Appts. in the weeks ahead including School, Neurologist in MN, NeuroSurgeon, Cardiology tests, Audiology, PCP Physical, Psychologist, Study Coord. etc. Crazyness!

I feel like I am forgetting something but in any case All for now! Much Love to ya'll!!!

Erica

Wednesday, February 14, 2007 8:30 PM CST

I copied this from a post I put on the MPS Forum (www.mpsforum.com) but thought I would update since surgery and on a few Appts. yesterday. After about 5 days of low pressure headaches almost all the time, these have decreased to less than every day and no high prssure headaches at all!!! YIPPEE!! A good sign I do think.... I went to NeuroSurg. yesterday and they took out the stitches from my side and back, I don't really know anymore from surgery yet why it went "bad" again but will see my Dr. in a month and we'll talk then. For now I am just happy the stitches are out and I can shower w/out covering them! I saw Genetics after the NS Appt. and this one to went well - pretty much only good or positiv news, I mean we kind of know now where I fall in the MPS I spectrum and that still kind of "shocks" me but I also know when I am keeping things fairly stable over 3 months times that it is good.... MY GC is working on a short list of things we need to do before May including Spinal MRI's for the Study and Dr.Charnas and they are worried about how pale I am (I guess more than normal) I also hav lost weight again but that has been recent and we thnk related to surgery and this recovery. Nausea is a bad, bad thing! It has not been good, and the NP at NS actually just prescribed a Zofran medication to take which helps. One of my other Drs. also thinks that the leg problems and symptoms I am having with this are "Neurologic progression" so although I never, ever want to hear that again I guess we will do these studies to figure things out. This next week I see the Endocrine Doc. so that may or may not help explain how extremely tired I am but if it does or doesn't I guess it's just the way God wants it.. Otherwise I just have Cardiology Appt(s). coming up in April and May - he is doing some funky test to look at Arteries and Heart function with Dye and the other fabulous tests like Echo. Otherwise just Neurology in MN, Audiology here and maybe 1 other thing coming up.

The thing we are as always going back and forth on is my Hands, when to do the dang Carpal Tunnel surgery - my GC though needs to do hers so we decided we should do them together at teh same time give our Doc a run for his money. Kidding of course but I seriously don't know when i'll do it though, not in any hurry to do surgery again.... Right now I think I might have a Sinus infection so really need to go see my Dr. but one of these days. MPS is crazy as you all know! Tomorrow night I am spending the night at the NS clinic Nurse House, I love her
to pieces and miss the guys at the clinic so can't wait to spend time with her! Friday before we start my infusion Patti my Study Coord. is meeting us at the NS clinic to do the UGAG Labs. Otherwise not to much going on..

Love you all,

Erica

Saturday, February 10, 2007 12:19 AM CST

This might have been the longest week in a loong time, I can deal with not working b/c I have tons of other things going on when I am feeling well and not recovering from surgery but this week I cannot do anything and haven't had much energy either. My Headaches are pretty well gone, the High pressure is right on out the window and low pressure usually occurs only in the mornings so that I am thankful for. I do go to Neurosurgery on Monday and get my stitches out from this last revision a week and a 1/2 ago so I will have to celebrate freedom or something then! After this I do not need to return to NS till March 8th as long as things go well!!! I also see genetics on Monday at 2:00 - my genetic counselor first and then both her and my geneticist after. I have so many questions for both this time actually - I always feel a little uncomfortable talking to my dr. but need to ask them both some things in addtion to just my GC.

This coming Thurs. I also have an Appt. back in the same clinic building as Genetics with my Psychologist at Children's Hospital - this will be the first time I have seen her since before surgery to. I am a little apprehensive b/c I have not found a new job yet, but much else to do when I can actually do it....... Thurs. ngiht I am spending with my NS clinic Nurse at her ohouse, which is absolutely gorgeous and Friday have to do my infusion (ERT) at the clinic to use of my drug there. That will be the last time I will be bck at that clinic for atleast a few month it seems. =( My Study Research Coord. is also meeting us on friday to do study UGAG Lab draws.

I have been thinking of asking my Genzyme CM Steph if we could look closer into if I could possibly do my infusions up at teh local hospital in town, if we could get special permission from my insurance. I like the company that comes out here and the nurse but just can't get used to all the work that goes into HH and drug ahipment/arrangement/etc. It is something I will talk to her about on Monday and talk to my GC about.

Family news, my Nephew Quinn and Mom (his) came to visit me while I was in the Hospital this last time, I think he didn't really know what to make of it, but was fascinated and the Nurses loved his Dinosaurs! My Zander is 8 months
this last week, no crawling or sitting on his own yet, but he does move himself in circles and also roll all over the place. We have his beloved puppy dog Bear this weekend.

I know this is a little late but Thank you to my Family who came to visit while in Hospital and who called! Also Thank you to my providers and friends who called as well!

Take care, I love you all,

Erica

Monday, February 5, 2007 5:44 PM CST

Hi All, I know this is so behind I am sorry!! I guess i'll just jump right in with the news that I had the Shunt revision on Thurs. (2/1) and my NS replaced 2 parts of the shunt as one part of the tubing was "sticky" and not flowing/draining right. I stayed in the Hospital till Sat.
and came home on pretty heavy duty pain meds which just knock me out tired but I can atlest be up for a little while at a time.

No other news really, beginning to work on things related to school and going back. My Genetic Couns. called me on Fri. at the Hospital and she had some exciting news about someone she "knows" who is starting up their own business and who she will put my name in for. Yey!!!

No other new job news which is stinky I hope to find a different job soon - right now i'd have to be off anyways for a few weeks but still when I am feeling better..... In March is the D.C. Scientific Conference and also beginning to plan a small get together for WI MPS families.

Next week I see my NeuroSurgeon for Post-op to get stitches removed and also a Genetics Appt. on Monday.

Will try to updte sooner this time. Love you,

Erica

Tuesday, January 23, 2007 12:42 AM CST

It is funny how one day you can feel happy and despite and despite road blocks still feel upbeat but then another day you feel a little under or blue bc of all that goes on...

After much indecision on my part and talking to many of my providers who are the closest ones I opted to get the shunt revision over with and am going in next week to have it done. I will go in on Weds th 31st and probably be home Friday afternoon. I am a little nerved about this surgery given how bad Anesthesiology was last revision back in July but will keep my faith with God.

I talked to my Genetic Counselor last Friday and she was faxing in the info for the Endocrinology Appt. with a Dr.Maas at FMLH. Should hear on that Appt. sometime soon.

I lost my job this last week it is a long story and I ma not up to writing about it right now but it seems unfair and I am now back to looking for employment part time.

I have been keeping busy otherwise alot of time t my Sister's and Nephews with them and just catching up on things that need to be done. I also have Appts. coming up othr thn the revision and this weekend Sara and I are headed to the Museums. In another month right during my Birthdy I head to Washington D.C. for the MPS Society Conference and also planning a small MPS get-together here in Wi.

Will post details on Surgery when I know more.
Much love,

Erica

Tuesday, January 16, 2007 11:03 AM CST

Wow! Can you believe it is already 3 weeks into the New Year!? AND this is the first update I am writing for 07' Sorry!!

The biggest news is the same that it has been since Nov. now - what to do about this danged shunt!?? I did see my NeuroSurg. last Thurs. and the decision is mine when to do surgery to check out the shunt function (by taking each piece apat and checking flow) and changing the Valve to a lower pressure. I am hating this decision, I know I need to decide pretty quick if I am going to do the surgery sooner rather than later (next few weeks vs. when I get home from D.C. in March). There's just no good choice, last night I was ready to just do it now, my side and head hurt ALL afternoon/night and today I feel "ok". Aargh! I guess I need to talk to my GC, I know she's busy but I hope
she gets back to me soon.

Sara and I and Zander have been doing different things, sometimes as simple as cooking at her house and watching a movie to this weekend we're going to a Hockey Game and nxt week 27th we are going to Milw. to Museums and Shopping. Zander is getting as big as can be, and babbling away which
is sooo cute!!! He is also rolling over all over the place and trying to sit up. His newest fascination is the Dog who
tends to steer clear!

No word on the Endocrine Appt. I really don't know whats up
with that exept I do know for sure that LuAnn's bigger concern was this Shunt. (which makes me think she'd want me
to get the surgery over) I hopefully will hear frrom her this week. No decision on my part about my hands either, I
know I need to atleast go back and see my Hand Surgeon but..

Work is going good, and my HR person at WEAP is looking into job placements with other homes to pick up hours. Which makes this surgery decision even more complicated even though I think i'd probably only have to miss 2 or so weeks.... B/c of the nature of what I do which is mostly low key interaction.

ERT is going fine, I miss Kris like crazy but she actually might meet Sara and I and Zander next week! I hope!! We also are planning to have lunch together the 1st of Febr. which is a day I have an Appt. later in the afternoon at CHW. This week both of the other Nurses from Maxxim are coming with Amy to train (or this is what they told me yesterday) and Joe and Karin are also coming as well, I guess just to meet me. Will be a busy infusion! IV's are as they always are.. this last week 1 stick but the entire month before that 3 or more tries!! Ugh.

Better run, things to do. maybe sleep?! =D Love ya all.

Erica

Friday, December 29, 2006 2:03 PM CST

I am not much in the mood to refelct on 2006 but as it quickly draws to a end I thought i'd write a few comments and how I am doing. I pray that 07' brings happiness and health, energy and acceptance on my part. For all others, my friends, my family, my caregivers I wish peace, love and happiness to you in this New Year.

I have often wondered in the last few weeks when does this MPS stuff truelly get easier and when do I finally begin to accept it? I sometimes think I have and wham somthing comes up or I begin to feel much more lousy and it makes me sad. I have thought so much about what 07' holds, will there be new or novel answers to MPS I, will I be approved for SSI so I can move onto my own place? Will I feel better
and feel like I am making a difference? I don't know these answers but I always hope so, I always come back to this hope, that if I do not give up, if I trudge fwd one day I might feel better and actually be able to live a real true life again. I do not know much how to explain this feeling but when it takes so long to get answers or explanations and to get someone to believe you it gets frustrating and is hard to even want to keep pushing. Even
though I am and always will be the stubborn, pushy type right now I just want more than anything to feel better and to have an understanding of why things are the way they are. I know that my Father in Heaven is using me for a purpose that only he knows and I trust him with my whole being but it gets frustrating to, when things are always so up and down and all over the place.

I know that 06' has been a roller coaster year, worse even then I think almost the year I was diagnosed, I am somewhere in between MPS and it being an integral part of every single aspect of my life no matter how I try to separate it and my life which I enjoy but wish I had more energy to be free and care-free. I know that my Drs. or atleast some of them if they read this might think this is a depressive state but it is simply me trying to come to terms yet with my life.. I know my Drs. think I can live an every-day normal life and that I am well enough to hold down a full time job and go to school or whatever and these are all things I work towards and will grab hold of but I wish they understood what it felt like to walk in my body.. to feel so tired and even when you have slept all night and taken a nap, to hurt and to just want to feel good again. I would never wish my MPS problems on anyone but I do wish they truelly could understand what a day or week in my life is really like. I think I have had a really hard time lately, b/c I strive again and again for answers which seem so evasive and not forth-coming and so many things have happened that don't seem fair. There have been lots of good moments in 2006 from the trips I have taken and experiences, to my job which although my energy is so low I work only very part time right now which is sad b/c I struggle to pay for everything and my car which is needing alot of repairs that where caused from the accident. There are so many things I want and I work for them but I don't ever seem to be able to have enough money to go around despite my good pay. I am so blessed to have my newest Nephew Zander and Neice Jordan whose births where this year as well as to see my Quinn and Alissa so I apprecite what I do have and wouldn't give it up I just wish there was some way to feel better, so I could work more, so I could be freer to do more.

I don't know wht 07' will bring, my GC today said one we get this Shunt stuff straightened out then before I know it I would go 10 yrs without any problems and I do hope. I just want this one to work and to help. I have no idea about IV problems, they are so against a more permanent option and go back and forth on this but I know that I have to do something... I just don' know what that something is.. I think if these providers went through tough IV sticks every single week then despite the risks they would consider options. Endocrine is also another thing but will be on hold for another week or so till my GC returns the 8th. And all I ask is to feel better? It doesn't seem a very bad wish to me??

I love you all and want to wish you the Best 2007 Year ever! I hope to see each of you soon.

Erica

Tuesday, December 19, 2006 3:16 PM CST

Less than a week until Christmas is here, how exciting right? I truelly am excited just very down about other things going on. I know I have updated this since I had the Cisternogram and LP done on Friday so I apologize. I had these tests done at FMLH, they did the LP in radiology
and took me down to Nuclear Med. for the Scans. The dye took alot longer than expected to flow through the Shunt so although it dd makes it's way into my brain and spinal cord and into the Shunt this is where it started to get very slow. I ended up being at FMLH for 6 hours on Friday for repeated scans and today heard from my NeuroSurgeon that the Shunt is working but he wants to see me in 3 weeks to see how I am doing. I guess at that time if I am having problems yet we'll go the next step, when I had saw him last week he told me that the Shunt might also not be draining enough CSF fluid and could be why I am getting headaches and fatigue and things again. That result was disapointing in and of itself b/c all I want is to feel good again...

Then on my way home from FMLH Friday I asked my NorthShore clinic Nurse if I could pick up some meds from her there since we where not going to do my infusion. Less than a block from the clinic as I was coming through the intersection another lady ran her light and hit my car which is completely un-driveable now. Kris met me at the accident site, and helped with getting my car towed to the clinic (by a towing company) and sticking by me when I was talking to the police. The officers let me go first and did not give me a ticket but said probably the Insurance co. would have to deal with the logistics of the accident. Kris took me home for the night, which I was and am very thankful to her for, she is such a kind and sweet person and she did much more than she really had to. When my Dad and brother where able to come down the next morning she took me to meet them at the clinic where my car was. Dad called the Insurance co. yesterday and we got my side of it taken care of, the great news is that other lady is saying that I pulled out and blaming hte accident on me which I know wasn't teh case. Her Insurance company is refusing to pay, my Insurance comp. is doing there invest. but even said to me 'how could you have made it across 3 other lanes of traffic without getting hit? If you pulled out", they do not believe her story. I do not know when I will get my car back but not this week which is really disapointing. I am frustrated that any time I hve caused an accident and if it has been my fault I have admitted it so why does this lay have to lie?

Right now I just need a break from my life, about the only things looking up are my job and being with my Sister and Zander. I hope and pray that the New year brings great blessings. I know I need to put my faith in God he has a plan for me and I just need to trust him.

Thanks for stopping by to check-in, as always sign my guestbook. Wishing all a Merry Christmas!

Erica

Thursday Dec. 14, 2006 3:08pm

I saw my NeuroSurgeon yesterday and had the CT scans which went good and looked alright. He thinks that either
my Shunt is malfunctioning (not working properly) or is not draining enough ICP fluid to give me relief from the High pressure. His plan is to do a Shunt flow study (doing an Lumbar puncture and then injecting dye to watch it flow) tomorrow at 10am at Froedert in Nuclear Med. which will tell us more of the problem. They have me NPO after midnight tonight so I don't know what this means, I hope it doesn't mean that if the Shunt is malfunctioning that they are going to do surgery right away. =( Tentatively have it planned to meet my Nurse at the clinic after Flow Study and she will do my ERT infusion. Could be a long day!
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Monday Dec. 11, 2006 8:42pm
What a week, what a week! All I have to say is these last 2
weeks w/out my Genetic Counselor I would have seriously strangled someone! All I want is to do my own life thing, work, be well, have fun... AND this last 3 weeks have been
crazy, the last 2 Insane-ish! Since the last Journal update LuAnn tried calling my PCP w/ no luck about the Shunt and Thyroid things.. She talked to Dr.Rhead and he to thinks it is my Shunt that is giving me grief with these Headaches, side pain and LuAnn also thinks the increased, relentless fatigue may be related as well. They got a phone conference with Dr.Sinson my NS last Thurs. and he is having me come in this week Weds. for CT scans and to see him. Please pray for answers and something simple enough to "fix". I want to feel better and feel good soon just not surgery or anything please!!!

Once The NS stuff is figured out and I am feeling better and we know if my Shunt is also causing my increased fatigue then we may or may not proceed to Endocrinology at FMLH. This is something I am talking to my PCP about at Tues. Appt. w/her. If we do see Endo then LuAnn has the name of a Dr. there and will make the Appt. for me, once she returns in Jan. ( =( ) and I guess we'll figure that out. When does it ever end!?!? The Endo would be s/t my Cardiologist thinks might be useful b/c of Abnormal test results related to Thyroid Function and possible pituitary
insufficency.

This week OTHER (yes, can you believe it other than Appts.
I get to do something else, hahahahah!!!) I am going to the clinic at NS for my Az infusion. I am really thinking
about going down to Chicago and is much easier to leave straight from there then it is to do my infusion at home and leave from here. I can't wait to see Kris!!! I miss her like CRAZY, crazy! My Az. infusions here have otherwise con'td alright, I do really like Amy, the Nurse, she is very nice and we generall have a nice time talking and "hanging out". I just despise all the set up and things that go along with Home infusions! IV's are not going well at all again, the week before it was 6x's and this week 3 which stinks. Hopefully atleast the new Patch (Synera) will help decrease what I feel!

I have added a new picture slide show thing to my site which I hope everyone likes. I will add new pics. in the next days when I get a chance, right now it is just the few I have on my laptop. Please, if you ARE coming here
SIGN MY GUESTBOOK!!! =)

2 weeks till Christmas! I have about 3/4 of my presents bought and most wrapped and under my tree. The rest, my Mom, Sister, Grandma, and a few small ones i'll probably get this week or next. I am very in the Holiday Spirit this year, I love Christmas!! A little down about all this medical junk, b/c I got like what a month or 2 of feeling pretty good before the Shunt went to crap or Headaches returned and the Cold weather makes my body hurt and well suffice my body is falling apart!!! But that isn't going to get me down all the way around, I still get to go to work at a job I love, see my Nephews all the time and my Neice should be home for Christmas!!!

All for now, this is long already. Had to add a little good
to the down news! P.S. this weekend we baked and decorated cookies, I cannot wait to get the pictures back of my Nephew - totally priceless! That kid was coated I think from Head-to-Toe in sprinkles!!!!

Much love, and to each of you my MPS friends sending wishes for God to surround you this Holiday with beautiful and wonderful surprises.

Monday, December 4, 2006 3:15 PM CST

I don't know but life never seems to take a slow moment or atleast for me? The little boy I work with today is sick so I have time to get a bunch of things done and thought i'd do this to. Our powe is out for the last few hours, but get this a scheduled outage!!! Who does that in the middle of winter?? Anyways though medical news what I don't want to update about the most b/c I am a little disapointed.

MY GC emailed me on Thurs. last week apparently my Cardiologist had called her right before Thanksgiving about my Lab results from the Appt. with him awhile back. He wants me to see an Endocrinologist and asked LuAnn to help set me upwith this. She talked to the docs at CHW and found out who they reccomended at FMLH and told me once I talk to Dr.Z. she will go ahead and set up an Appt. for me. I guess from what LuAnn told me on the phone it is under-active Thyroid problems and possibly caused from the Hydrocephalus. She did say they think by treating the thyroid problem it might help my fatigue!!!!!! I'll keep updated on this. / LuAnn also got a script written for a new Patch that works both by numbing the area it is applied to and heating it so I should get to try that this week or next. It is expensive but she did order it so that it will be like 10 weeks at a time.

I saw Dr.Sinson's (NeuroSurgeon) NP last week about my side
pain but she was pretty adamant that it could not be from the Shunt or that my Headaches also where not related. Frustrating! I am calling Dr.Zuccaro my PCP in a little bit to see if I can get in to her sooner than next week, LuAnn also thought she would be able to help. I love when providers think they know how we feel?!?! I love mine that are kind and caring and really the ones who are not really need lessons in it!

This weeks Home infusion did not go all that well, I mean it went alright but the IV's where pretty bad - my Nurse here Amy felt pretty bad I could tell. It took 6 tries this
time, how nutty is that! When do you say is enough and just go se a Surgeon on youre own?

I better go before my battery dies. Hope our power comes back soon!!! I know my friend Kris her power has been out for several days already!
Much love, and Holiday Cheer!!!!

Erica

Saturday, November 25, 2006 10:56 PM CST

I am a little behind for an update - so I will try to get out out tonight.

I had my 3rd Home infusion yesterday which went fine - I like Amy the Nurse... but I still hate doing these infusions at home and really, really miss the clinic. I can say the break from the clinic having any discussions with my Geneticist has been nice, but I really really miss seeing Kris and going there. I don't like the added "work" of Home infusions, they are no easier for me, and I miss Kris. I hav gotten into several arguments with my Dd about this, but ultimately I don't know what to do.
I think after winter I will end up going back to the clinic though... it is my body and my decision and I am gonig to do what makes me happier. I do like Amy, I really do I ju8st miss the clinic to much and it is easier to go there. Then I can leave the medical stuff there and
go about my life for the 3/4 of the rest of the week. It is my body right?

I have been having more and more side pain the last few weeks happening more often and headaches and back pain to which make me a little frustrated but I am hoping to talk to my PCP or NS office this week. I see my Hand Surgeon at FMLH on Monday right after work I guess to make decisions about that. I am supposed to see Dr.Zuccaro on Weds. but want to try to reschedule for Tues. so I don't miss work on Weds. I really need to see her but don't want to miss work.

I got an email from my GC earlier last week - she had been out of her office for just over a week prior to that but hopefully she will call/email me this week with some idea about the ERT access options and what the next step will be.

Thurs was Thanksgiving which I had Zander and Quinn here all day so was quite fun. Quinn spent that night and was here all day yesterday running full steam ahead, even during my ERT and after I did a little shopping and went and watched Zander for the night. Today we went shopping all day and so tongiht I am quite worn out. I really am looking fwd to Christmas this year but can't wait for winter to be over.

I will try to update after my Hand Appt. and if I see/talk to Dr.Z. and my NS's office. I really do want to feel better again.

Love and Many Hugs,

Erica

P.S. my ERT dose went up by 5ml yesterday again. So hopefully that helps wit energy.

Friday, November 10, 2006 6:33 PM CST

A Little bit behind on updates for All the Appts, hey? Sorry!! I did have the U-GAG Study labs lst Thurs. followed by Sleep Med Pulmonary , and then on to EMG/NCV testing and finally an Appt. with my Psychologist at CHW.
All these actually went well, the Labs where as usual, Sleep Med went fine - nochanges. EMG's where about what we thought I guess and Psych was fine.

This past Tuesday, I had the Genetics Appt. at Children's
with LuAnn and Dr.Rhead which was later in the afternoon.
The Appt. went really well, atleast talking to LuAnn, I don't have to much to say to my Dr. these days, I just don't know what to talk to him about anymore. It's not like it really seems like he listens and we can never just have a conversation between the two of us, like if I ask him something he involves everyone else. It was really nice to talk to my GC though, all of my med stuff is doing really well and although there are things we have to figure out and little things going on yet, she thinks I am doing great and at a turning point. That was the best thing I could have asked to here! We also talked about my work, I about how I am not sure that this is exactly what I should be doing, even though I love it.. just b/c it often reminds me so much of the MPS Sanfillipo kids I love and wishing I could help them and feeling at a loss. My GC did think that I have changed in so many good ways since I have started with WEAP and that if this isn't the life long career I want (to move up with them) then I definetly am in the right area of work and that it suits me so well and my personality. She is the first person I have gotten to talk to about burnout and detachment and involvement and finding a work that you really fit into.

The other thing we talked about, after my Dr. left was why I got so quiet. Not somthing I used to do but like I wrote about when you don't feel like you are being listened to then it is hard to find trust with a person anymore. Her and I had joked around and laughed and just mixed what had to be done with a little funny stuff in between, and while we waited for him after we finished our inital reports she sat in and talked to me for another 15mins about this and that. I don't think it is a conscius thing but I just knew when he was coming in and kind of "shut down", so she wanted to know why that was, why I wasn't sticking up for myself and telling him my thoughts. I think maybe now that I won't see him every week maybe the distance will help. Right now I don't have much to say to him. I will work with
LuAnn, atleast she listens and tries to help me find things
to help when the options that I have are not doing great.

Right now the big thing going on medical wise is when i'll do surgery for my hands again, it has been 4 years since the 1st CTS releases. We do hope that when I do surgery the symptoms will stay away longer since being on ERT. The other thing is the Port and.or what else might be an option. It is alot of going back and forth between my 3 Drs. (genetics, Crdio and NSg) all seem to be against it and Genetics especially so b/c of the risk of infection if I where to get one is higher problems wise. I asked them now if we can consider low-dose Antibiotics and/or cream when accessing the site. Otherwise Dr.R had said somethings
about IV lines that can be placed for a little while at a time - alhough now he does not want to do that either. Once LuAnn gets back from being gone the 20th she may have heard from the GS office as she is trying to get me an Appt. there. Atleast even though she spend so much time talking to me about it and her fears, and I agreed if it where really not an option I would try to learn to live with that she is going to still see what a GS says.

They are also looking at upping the dose of Aldurazyme again - I had started in May 05' at 60ml but lost about 20pds so went down to 50ml and now since I have went back up by about 10pds looking to see what the right dose it. (Probably 11 vials)

My Nephews are doing good, I haven't seen Quinn since that weekend of Halloween, I hope Jamie will let me take him soon. I seen Zander last night and earlier this week, he gets bigger everytime! I have new pics of both to put up.

I started HH infusions this week again with a company out of Madison, Maxxim they have 1 Primary Nurse who will come most eveyr week (Amy) and 2 back-up Nurses Jack and Liz. All are youngish but older than me and seem nice!! It didn't seem like we where really going to get to start earlier this week as know one outside of TCi had updated my GC or Case Manager at Genzyme but we did get things straightened out and Margaret Mary happened to call me while I was talknig to LuAnn so they squared away and Steph talked to Carol. Todays tx went well, we started real early and where done by Noon.
Margaret Mary called shortly after to check in and I also talked to Kris, my clinic infusion Nurse. All where happy as am I. I miss the clinic and the guys and the fun there but like this company atleast.

I better run have all kinds of paperwork I have to catch up on, yuck! And some forms to fill out, double yuck. Will keep updates when I know more.
Love,

Erica

Tuesday, October 31, 2006 9:30 PM CST

Thursday Nov. 2, 2006 - Appointments CHW and FMLH -
8:00 Study Labs CHW Patti
9:00 Sleep Medicine Pulm. Clinic
10:00 NeuroPhysiology Clinic EMG testing
11:00 CHW CDC Clinic Psychologist

Tuesday Nov. 7, 2006 - Appointment Children's Hosp.
3:00 Genetics LuAnn W. (GC) Dr.Rhead.

If you wouldn't mind keep me in your thoughts for some good
news from these Appts. some answers and some good news would really make a big difference. I have still be so tired lately even the days when I don't go to work till a little later in the morning ( thus can sleep in) so either it is the Aldurazyme dose or something with the Sleep stuff. I also need to call my ENT in the next day or two (along with 20millin other people!) since it has been over 2 weeks that we tried the nasal spray but it didnt help my cold/morning ha symptoms. After much discussion with my GC and Geneticist (mostly by email this time!) they are going to really get me a consult to talk to a General Surgeon. It took some talking on my part again even though it was somethin they had agreed to previously. (long story)
I don't know when that Appt. will be but I see my Genetics team on Tues. afternoon anyways.

Ironically I had rescheduled the Genetics Appt. to be in the afternoon on Tues. but b/c of the kiddo I work with going to school 4 days a week now my and the other Therapist schedule is changing and I just had to get next Tues. completely off! (usually I work Tues. AM's) I got news the other day that i'll get a small raise in another month and then in 4 months can apply for an ALT position at my job which has more rsponsibility and another raise.

I have no results from my Lab work form Cardiology 3 weeks or so ago nor do I have the results from the NeuroPsch testing in MN yet. Dr.Charnas also has to send us his report so that my GC can order the MRi.
I also got news on Friday that Universal Peds. in Milw. whom Kris my infusion Nurse also works for signed a contract with my Insurance, Genzyme and TheraCom to do my Infusions weekly here. (good or bad going back to HH I still don't know, but atleast they are a good company from what Kris says!) Anyways Kris will be the back-up Nurse and will also train whomever comes out here but it is a matter of finding a Nurse I guess right now? (or so it seems, no one seems to be on the same page quite yet we just know that they are the company).

That is all for now. Much love and many, many hugs.

Erica

Wednesday, October 25, 2006 11:35 AM CDT

I owe this update a little late though I guess?!? Last weeks Appts. in Mpls went well, although the day was long and I was soo glad to come back home. The night before I had dinner with Kristin and the Sheridan's (Michael, 13 has MPS IH post BMT) which was alot of fun! Weds morning was bright and early, the MRi itself lasted about 45mins so
I watched a movie and tried to relax (yeh right!). After that Kate took myself, and Cheryl back to the PWB bldng where we did many hours of NeuroPsych testing until the early-mid afternoon. After that it was off to see Dr.Charnas which wasn't alot new either, I don't know most of the rsults of the NP testing yet. From what they told me I haven't done that well on the Memory part of the NP tests but we are keeping the Aricept as it does seem to help short term memory.. He is inclding in his report to my
Drs. here at home to do an MRI as he is concerned about some chnges I guess in rlfexes (or something like that) so I guess that will be sometime in the future, ooh joys. Otherwise they are working on the Intrathecal Enzyme Repl. Study and he said that he thinks that would get FDA approval within a few months.. maybe be the time I see him again in April? This would hopefully treat the High pressure (Communicating Hydro) as well as any compression issues and/or help Brain issues. Atleast that is the hope. If you are interested in knowing more about it, go to my friends webpage www.caringbridge.org/visit/hollandkids - Laynie is currently enrolled in a Clinical Study at UCLA looking at
this very issue.

Otherwise I am on my own at work now, which is very nice, it is just nice to work at my own pace and Danny and I seem
to have a good time. I like working with him and it is fun
to be helping a kiddo.

Still no word from my Genetic Counselor, I think she may be
out of the office the end of this week.. but I guess if nothing else I do see her and my Geneticist in the office in 2 weeks.. I will wait and see if she calls or writes me
back though, hopefully!
We are still waiting word from TCi about HH, I think my GC is ordering my dru 2 weeks at a time for now, we just don't know where things stand. I know Steph is working on it and who knows if we'll ever get to go back to HH? I guess I like seeing Kris so who knows maybe it is better to
just stay at the clinic? I think my GC thinks that it is less of a Headache this way and I almost am agreeing, but I
guess will leave it up to whatever Steph is able to do. I just hope she doesn't get frustrated with always working on
things for me! She really is such a great person!!

Since LuAnn is ordering the Aldurazyme 2 weeks at a time, and had been ordering it week to week for a little bit there I am not sure now how TCI is billing me for the co-pay.. from what we new and Steph had found out way back when was that we would just get billed 1x a month for the co-pay vs. everytime it was ordered but when I have been tracking it on WEA/Caremark's online pharmacy tool it says we have been charged a $20copay 4x's in Oct. which is not good! I do have a call in and hoping it is really just a once a month cost...

I am off now to watch my Nephew, Zander, we spent the afternoon/night together last night - he is a doll but boy does he get unhappy smetimes! He is very hard to figure out what he needs/wants? We think he is either growing or cutting teeth. (that would make anyone unhappy???) But otherwise he really is a joy to play with and the smiles are priceless!

Always and Forever,

Erica

Saturday, October 21, 2006 8:23 PM CDT

Yesterday was #75 for infusions - I didn't even realize it until today either. Guess it was like any other ERT day - no HH company found yet and I still dont have a Port. :frown: I guess some of the things i've noticed are better endurance (most days) and some other mild improvements like in physical features but I know to that
if nothing else most things are kind of staying the same. And their is the hope of the Intrathecal ERT treatments Study at MN within a few months so that may stop progression of some other problems.
My infusion nurse is looking at Gen. Surg. for me to see, who does the most Port surgeries - if I don't hear from my GC by the time I get those names then i'll call and schedule the consult. In a few weeks I see Genetics for the 3month Study/6 month Appt. Fups so will also have an MRI done at that time per Dr.Charnas in MN, he wants to check for Cord Compression due to some things he saw while I was there this past week. Will also make our decision about the Port at this time most likeally. I should hear this week on the rest of the NeuroPsych testing from MN this last week to.

Friday, October 13, 2006 6:37 PM CDT

Lots to update, between my Appts. earlier this week with Dr.Zuccaro and Dr.Earing (PCP and Cardiologist) and my car
and work and then infusion stuff.

My PCP started a new Anti-depressant med that also works to control pain at the Nerve level, which will be awesome!!
It doesn't help weight problems/appetite like the Remeron did but if I could maintain the weight I am at now for a while then I think it will be as my GC says "back at the Fighting weight" and this mgiht mean my body isn't going to
go through anymore crazy significant weight losses again soon! This med does cause Nuasea at first (per my PCP) and my Pharmacist told me today since it works by speeding up all sorts of different things most of the time it causes weight loss at least for the first month. And I thought I was getting ahead!

I saw my Cardio on Tuesday night, he didn't have much new news but he did let me out of the tests for this time and we'll just do them ALL in May again (the 1yr). No one had repeated the tests he did in May which showed all the abnormal Thyroid levels so he re-did those that night but said if those levels came back normal this time then my tiredness (which has been worse) is probably b/c of the multiple differnent physical problems, (heart lung, joint, the Shunt thing, infusions, etc.) and there probably wasn't much he or anyone could do that would help me to feel better. =(

I have been talking to my Genzyme Case Manager Steph Ginty just a few times this week but we got to talk on the phone today (a friday thing) which was really neat, she is just the coolest person to talk to and always cheers me up, even
when I am not down! I talked to LuAnn, who is my GC a few times as well, just about various things - I see her in
clinic in 3 weeks. (6month/3month Genetics) No word from her on a GS consult yet, I wonder why?!

This week I fly up to the U of MN (Midwest this time) on Tuesday for clinic Appts. all day on Weds. They start at 8am (MRI) and then NP testing right after with Kendra (I think) at 1:30 I get the test results and from there at 2:00 I see Dr.Charnas. My flight home is at 6:30 that same night. This is all part of the Study looking at how the MPS I Brain function using the High Resonance Functional Imaging MRI and NeuroPsychological testing.

Otherwise just little things, I switched the team that I am
on at work so although I have my 30hours of training completeled I am doubling with another Therapist for next week yet. This little boy is 5, adorable and although less "High functioning" is very good at alot of the things he does. I wrecked the front of my car on my way home from the Appts. earlier this week - I hit a raccoon that had run out in front of me, so although I could do nothing to avoid it, my bumper really needs to be replaced now. Grr! I've had this car for not even a month!!! There is something else little that has went on this week but I will have to come back and update if I think of it.

Take care and Much Love,

Erica

Sunday, October 8, 2006 2:46 PM CDT

Hey All, I know many of you live surrounded by stress so you know how I feel this week - CRAZY!!! How much can or is 1 person really supposed to take? I know that God truelly does only give us what we can handle I just feel very overwhelmed by what is all changing and going on and on top of it really not feel good the last 3 weeks or so. On top of everything our Insurance went up from no deductible under Level 1 providers and 2 Tier drug Costs ($0 and $5) to a $250 deductible for Level 1 providers and
3 Tier Drug costs at $0, 5 and $20. This means my out of pocket expense a month for all of the 10 different meds that I take daily and then as pre-meds/ERT will be over $70 a month. I make decent money at my job but I only work very part-time due to everything else that I have going on. What a stress!

I am trying to get my Primary Dr., Genetics Counselor and Geneticist to have a Team mtng. so we can all make some sort of plan for the future and figure out a better way to
make things work. It really seems like no one is on the same page. My Genzyme Case Manager is going to get in touch with TCI this week, if they haven't made nay progress
on an HH company, then she told me she would go ahead and using the list of HH companies from my Insurance contact them to see if there was any she could find who would be willing to do my infusions. If that would fail then I think we would look more closely at doing ERT at the local
Hospital if this could be done, Dr.Haase who is the Internal Med. Doc. I know here in town has a partner whom she believes would be willing to take on my care. Like everything else it will be a while before we know anything.

I haven't heard anything from LuAnn about the General Surgeon, I talked to her on Friday - to let her know what little update I knew from Steph, but she did not say anything about finding a GS yet. When I see Dr.Earing, my Cardiologist on Tuesday I will also talk to him make sure he is willing to write in his report what he thinks about a Port (we have talked about this several times in the past). I really hope Dr.Earing can help us find some answers to why I feel so tired, I so don't want anything else to be wrong - I DON'T think I could deal with it right
now but I DO want to feel better!!!!!

Will update after those Appts. and/or when I hear anything from my Genetic Counselor or Genzyme C.M, Steph.

Much Love,

Erica

Sunday, October 1, 2006 2:52 PM CDT

Time to add a new journal, another busy week full of the usual up and downs of my life, but I think I can hardly complain. After much debate I went back to the clinic for this weeks ERT infusion, it looks like i'll end up there for a few weeks or until a new HH company can be found and trained by TheraCom. The infusion went so smoothly and what a difference, it felt great to have it go good! I don't know what my feelings are about doing HH infusions here at home or doing them at the clinic, which one is the right decision anymore, but I guess for now it is week to week. Even though my parents want the infusions here at home (which makes me want to stay at the clinic) it is my decision, my Genetic Counselor, Geneticist and Nurse have all told me it is my body and I can tell them when I have had enough if HH infusions do start back up and do not go well again.

I had been thinking about this alot especially since the fiasco with the Nurse and infusions here but several of the parents I know very well wrote letters on my behalf stating the reasons that their kids have Ports and how they have helped, etc. Well I guess I knew this probably wasn't something that would go all that well with my Geneticist but I also knew that as long as I talked to him after the fact that it really couldn't hurt anything more, b/c, well I am just his pt. trying to push for what I think is right for me.

So I talked to Dr.Rhead on Friday and we talked for almost 20 minutes, we settled on the consenus that neither of us was going to make a decision or he wasn't going to veto the idea of a Port completely till we had all of the info in specific to my body/risks. He asked me to talk to my GC, so I called LuAnn friday afternoon and she is going to try to get names for me and find the best person for me to see to talk to about this and just see what the risks are from a General Surgeon stand-point to discuss options. Then I will talk to my Cardiologist, Dr.Earing, when I see him at CHW next week and ask him to include in his clinic note what he thinks the risks are vs. the benefits to my specific case and especially to my valves given the disease what he thinks.
I'll also do the same with my NeuroSurgeon, Dr.Sinson and ask him to write a report listing the risks if there are any addt'l ones given that I already have the LP Shunt and if in his opinion a Port poses any additl. problems. After we get all 3 of these Drs. letters, Dr.Rhead and LuAnn
will look at them and I guess we'll talk. It doesn't mean i'll get a Port right now just means that we know what the risks are to my health and if it is an option, when I truelly run out of options as I am hitting more and more barriers.

I see Dr.Earing a week from Tuesday, I hope he can help me figure out what the extreme tiredness is from and why I feel so crappy - not only do I have this but also the heart rhythm problems which come and go but very frequently regardless of the Nadolol which I still take daily.

Work is going good, I hope I finish training this week, I could really use to be on my own, it would sure be nice!!! Right now my schedule is Mon-Weds. mornings with our Team Mtngs. Weds. afternoon - their is still so much to learn but I think I am starting
to really get the hang of it!!

I better run, God's Blessings, Remember he is a Good and Gracious God - Believe and he will be good unto you!

~Erica ~

Monday, September 25, 2006 2:25 PM CDT

I know I should try to update more often - sorry, just very tired lately. I get up really early for work weekdays which is nice but I end up napping most of the late afternoon anyways.
This last week I had a Sinus infection which I just finished Zithromax for, it helped the fever part but I still have some parts of the "cold" hanging on, which brings me back to why I always hate this time of year!
The kiddo I work with is sick also so everytime he coughs I generally am in direct contact which does not help, but the job is still good and I like it. I go out on my own after Tues. or Weds. this week so I am happy about that. I am having a really hard time remembering all of the data and knowing
exactly when to use what, which makes me a little flustered and very frustrated, not to mention I feel horrible b/c I feel like I should know this stuff. I hate absolutely hate this
disease when it comes to times like this why does it have to affect even these aspects of my life like memory and reason?

Last week did not go any better with Home Health and ERT, after about 4 or 5 unsuccessful tries at the IV we got ahold of Margaret Mary who is the Nurse I am working with at TCI now - the Supervisor did not want to come out here so get this, I had to go up to to the Outpatient dept. of our Hospital to get the IV started then Hep-locked! Needless the infusion took FOREVER and a not very sweet day
so it was around 6:30-7 before I left the house for the weekend. Steph and Margaret Mary and Carol are all working
at finding a new HH company - I guess for this week I am back at NS clinic.
=(

I called Dr.Z a little bit ago to see about switching off of this Remeron, I think either this or the Headaches are making me extremely irritable, oddly not towards other people really but in situations like at home.... I hope she will switch it up. *we are leaving the med alone for a few more weeks but I have a message into the Nurse Pract. at my NS office.
I still don't know when my Appts. are in MN, I haven't heard anything - I am hoping they are the week of Oct. 1st so I can go and visit the new MPS I family in Sauk Prairie on my way home and go to the walk/run in Eagan, MN on Sat.
Regardless of the date of the Appts. I am talking with the family about when we can all meet. Neat!!

I picked up my glasses on Friday night, they are cute, definetly different but still cute! My one eye is definetly so much weaker than the other which is a very weird thing with these glasses and how they are! I see the new Opthal. on Thurs. (Dr.Koenig) who is in place of my Doc that left the
clinic.

Guess otherwise things are okay just going through the everyday things and trying to figure out how I can do everything I want when I am tired. I hate this disease, even more I hate to let it stop me from doing anything I want to do...

Many, many Hugs!!!

Erica

Monday, September 18, 2006 3:59 PM CDT

I should have updated sooner since alot of new things went
on since last Thurs. night when I updated last but here it is anyways. I went and picked out glasses today - new ones that is, thanks to the 'Pennies for Nichol' organization and Family I am able to purchase some new equipment including these glasses. They are pretty pink, the plastic type - so it will be an adjustment to having the glasses which are much stronger in my right eye but will be helpful since I am terribly night blind and squint at everything. This Weds, I am picking up a new car that I am buying - it is a 2002 Saturn I know your thinking where does someone in her situation come up with the money but I have it.

I started Home infusions on Friday, I must confess for those of you I haven't talked to since then I don't really like doing ERT at home.. I like having home be completely separate from the medical part of everyday life, I mean I know I deal with MPS everyday and I love all of you friends
so very much I just like having space between when I go to ERT and then I come home and don't worry about anything to do with ERT until the following week. I love all of my friends and I am lucky that this sucky disorder has brought
me alot of awesome opportunities it's just that it's hard to have ERT and my life in one place.
Home infusions the first week didn't go well at all, it was a disaster to say the least between having the wrong IV
tubing to not being able to get an IV and then the IV blew
and the pump wasn't working right and we finally ended up
quitting after getting just 1/2 of the weeks dose of Enzyme.

Something I hadn't thought of, I wonder why, if I can get a
Nurse to teach me how to start IV's then why wouldn't I be able to do my own treatments? I really wonder, have to run that past Steph.

I found out from Steph on Friday when we where talking that
she is giving up WI in her territories but she asked her manager if she could keep me on her team and her manager said yes!! I sure love Steph!

This weekend Sara, Zander, Jeremy and I went to Wautoma to some grass drags - which is racing their snowmobiles on grass! It was soo windy and a loong day but fun and both did well. - Sara placed 3rd! Now that I am making better money i'd really like to find a sled this winter and go snowmobiling with them, I love the thrill and I have a helmet here already of Aaron's. (he has another of his own)

My Headaches are really bad yesterday and today, they had been semi-okay but today and yesterday are horrible again, I also have had a fever since atleast Thurs. so I guess I should be calling my NS's office but I am dragging my feet
b/c I don't want anything to be wrong with this Shunt, I just want to go to work (which I con't to love!!!) and go out and do my own things, not worry about MPS or Headaches. =(
Will see how the next few days are I guess. Will keep updates here on this situation.

Many hugs, much love and God's blessings,

Erica

Tuesday, September 12, 2006 2:03 PM CDT

I started my new "job" yesterday - the only reason I write it like that is because it hardly seems like work I really like it so far. I know it was only the 1st day but I think once I pick up on how to log data and the routine that I will really enjoy this work.

Later this week i'll start Home infusions the Nurses are coming out tomorrow (Weds) afternoon to fill out paperwork and make sure they know how to get to my House and then Friday is D-day. The Aldurazyme and supplies will arrive tomorrow morning (also Weds) i'll be at work but Mom can sign for this. I haven't talkd to my Genetic Counselor since last week so I am unsure if I need anything from her but I assume she would let me know.

I saw Dr.Brosig yesterday (my Psychologist) which went well
it seems like we should be able to get back to a more normal schedule again. I have been pretty happy the last week with the different new things so it was nice to have so many new things to talk about and a positive overall session! We are going to meet on a every other week schedule.

I am awaiting to hear on Appts. with Dr.Charnas and Dr.Bjoraker (Kendra) in MN, I was originally headed there
next week but asked to do these the 1st week of Oct. I haven'y done the NeuroPsych testing for this year yet which
will also tell us how the Aricept is working I guess. I seen Dr.Charnas in July but haven't really talked to him since either.

Our Insurance is changing a little bit probably the 1st of Oct. our out of pocket expense will go up from $0 for in-plan providers (is different for me than for my parents whom we see) but we'll have to pay the 1st $500 then will be full coverage again. Also our med costs is going up from I think $ 0 and $5.00 to $5 and $10.00 for generic and
brand-name. Yikes!

I sold my car the other day so am looking for something new
not brand-new or anything just a different more reliable beast I guess. Dad found one we are thinking about a Saturn
which would be awesome! Cross your fingers!!

I better run, I know this hasn't been the most exciting update, sorry! Not to much new going on which might be a good thing for once!!!
Much Love and Many Hugs,

Erica

Thursday, September 7, 2006 6:43 PM CDT

It's been awhile since I updated (last week) so I thought I
should write about all of the new things that have happened
this week - I also put up new pictures of my Nephew in the photo album.

Beginning of the week I found out I got the job with the WI Early Autism Project - which I have been on cloud 9 since! The hours are great, + i'll have every Fri-Sun. off
and pay is really good to. I start the training tomorrow at
the main office in Brookfield after ERT and then sometime next week will begin working in the families home.

I also have pretty much everything set to begin Home infusions so that should start next Friday. Steph (Genzyme)
has everything approved for this with my Insurance, I have
another person at TheraCom who takes care of shipping the drug and supplies every week and when I talked to LuAnn (GC) the other day she was sending in the paperwork and making sure everything was set on her end for infusions. I'll meet with the Home Health Co. next week, fill out paperwork, meet my Nurse(s) and then Friday things should roll.

Just for a little treat to myself I went and had my hair cut today, my stylist is moving away for school =( but she
gave me the names of who I should go to from now on. I love getting my hair cut, very fun to play with and style my own ways afterwards! I haven't had to much time to think
lately, but I am feeling alright atleast the last few days
which is nice. I haven't done any Appts. for the last 2 weeks which is amazing and good (!!) but this next week will see my Psychologist and as well as my Audiologist.

OMGosh - I went to Chicago over the weekend (labor day) and
stayed with my friends there who live in a suburb of Chicago. - I had totally alot of fun, we did alot of differnt things like going downtown, eating in some of the restaurants that are very popular, going out to Navy Pier (so pretty and fun!!!). Sunday night I did what I should never do again - I don't drink very often but I can hold my
own to just about anyone including most guys, well I definetly did on Sunday night! I don't think I have ever thrown up on someone before nor have I have ever plain thrown up that much! I didn't have a hang over on Monday but man that was a pretty long night after drinking!!! Suffice to say I am partied out for a while now!

Thanks for checking in, better sign off. Much love and many Hugs,

Erica

Sunday, August 27, 2006 11:41 PM CDT

Do you ever stand amidst a group or with friends and still feel terribly alone?

I know I am running from my life and trying to forget what I feel each and every day but I am overwhelmed so completely and totally that I don't know what else to do.. I have been spending almost every day with my Sister, whom is my best friend. It isnt that I really feel different around her, it's more just that I am not reminded so much of how different my life is than other peoples when I am with her.. when we are together she just accepts whatever way I feel that day and we have fun. When I am here I face
reality, I am surrounded by the little things that will always remind me I am not the same... I know I shouldn't let the MPS define it doesn't normally but lately it is also hard to get away from it, hard when I hardly ever feel good and when I am struggling so hard to define myself.
I don't feel like I belong in any right place right now, I want so badly to feel good and be "care free" like many of my friends but I can't get away from the every day things about me tht are different. I want to run away from the pain and feelings and sadness and hide in a make believe world where none of this is true and my worries are more of the every day little things that I used to face. ..... I wnat to stop second guessing myself, I want to not worry what others think, I just want so many things right now and I am trying so hard, so really hard to get them even when as now I don't always know how.

Awhile ago, a year ago, even a month ago I didn't feel like
this, I felt much better about things.. I wish I could say the same was still true, right now I just feel
very overwhelmed. I wish for the thousandth time I had someone to help me figure this stuff all out, manage the MPS stuff I mean so I could not worry about it. I know I need to do alot of accepting about things like feeling bad and accepting about my life not being the same as other peoples I am around I just have to figure it out first, I mean this acceptance thing.

I keep sitting here trying to type out what I feel without
sounding like a basket case and I think I am no where near
figuring out how to explain it so before I go on and on more I better just sign off.

So many thanks for being my friend,

Erica

Thursday, August 24, 2006 2:46 PM CDT

Hey, I had a long update all typed out here and then I lost
it when my computer froze so i'll try to update some.

I saw my NeuroSurgeon today, I don't usually mind going there other than the fact it means being in a Drs. office..
but it went fine, nothing major or new to write about. I am
free from any further follow up unless something new would come up or headaches would get worse. I of course can call if I have any problems. Kris whom is the Secretary and who I really like asked me to call her occassionaly so i'll do that also, she is super sweet so no issue and not hard to do!

My Sister and I are considering taking a Real Estate pre-licensing class that is being held by one of the bigger Companies, Sara found out the class is held on the West side of Madison and is 4 nights a week from 5:30-9:30. Otherwise I also have an interview in Brookfield on Weds. this is for a Company to work with children with autism. I should be hearing real soon from the Couns. at DVR to set-up our next Appt. go over test results and such.

Otherwise there isn't alot new going on just mainly keeping busy spending alot of time with Sara and Zander and my Nephew Quinn and his Mom Jamie.

I have really had a rough week trying to work through my emotions and deal with the fact that I feel pretty burned out in dealing with Medical stuff and not feeling very good. I am not so sure right at this moment I want to do any of this, even though I have no choice. I am real tired of these Headaches, right now I think I might have something like a Sinus Infection just by the way my ears and head feel, these combined with the up and down pressure headaches sure make me run down tired and crappy feeling! No fun.
I doubt I would ever do this and I am unsure why I am even writing this here but lately I have been wondering what would happen if I just dumped all of my providers and got new ones all across the board? I mean in every single speciality.... I doubt I would but it sure seems appealing
maybe they would all work together better and I would feel like I wasn't the only one pushing for answers and always pushing to get things done? It's not even that I don't like my Drs., I do most of them alot, I just wish there was one person who would help me set up Appts., coordinate things and talk to my Drs. for me when I can't get answers!! Aaagghhh!

Going to bed now, thanks for checking in. I love you guys,
your the best friends and family I could ask for!!!
Love, Hugs and God Bless,

Erica

Wednesday, August 23, 2006 12:38 AM CDT

So do you ever wonder when things get a little less crazy??
I think I do like every single day..

Tomorrow I see my NeuroSurgeon, and next week I should be headed back to work which I am super excited for, not b/c I
like work only b/c I am to bored otherwise!! My manager is
putting me on the schedule I don't really see any reason my NS won't let me go back to work. I also have a job interview Thurs. afternoon in Brookfield, it sounds like i'd either be working in Juneau or Watertown and is working with Kids with Autism on Behavior Modif. I think it
might be a great learning experience and rewarding.

There is also a pre-license Real Estate class being offered
through Stark Realty starting in Sept. that I am thinking about taking. There is a small cost to this b/c of books, etc. but I just wonder if it isn't something that would be
interesting to look into. In addition to these I also put in applications for 2 other jobs.

I haven't really been putting alot of thought into medical
stuff these days, I guess I am focusing alot more on things
I want to accomplish and just having some fun but this week has been quite miserable for Headaches. They haven't exactly ever been a peach since the Shunt revision but atleast where pretty tolerable... this week I think they are trying to be front and center!!! I am taking Zofran daily which helps 110% with Nausea so then I can function and eat and go about whatever I want to do. I am not sure what I would do w/out that med!

I think this is about all I know, will try to update soon after my NS Appt. Take care all my sweet and kind friends!!
Love,

Erica

Tuesday, August 15, 2006 12:13 AM CDT

I haven't put nay new pics up in a while but I spent all day on Sunday with my friends the Nagy Family (Chicago) at
State Fair!!! We had so much fun, I feel so blessed to have such great friends like I do. I have been so busy the last few days I haven't seen my Sister or Zander, I think I am going through withdrawals, you would think it has been more like a month since I saw them but I miss em!!
I found out on Sunday from my oldest brother that my Neice Alissa was moving to Michigan yesterday, she is the sweetest, cutest little girl I miss her so much already.

Tomorrow (Weds) I see my PMD hopefully she will have time to help me figure out some different things most of all I guess is the weight loss issue and trying to figure that out but then there is pain issues which have been acting up
the last few weeks again so I hope she can help me figure something out with this. I think I jinxed myself when I told my GC this has been really mild all summer!!! Aagh! The other thing is I really want to switch the Anti-depressant, I hate Effexor and if I am going to be on something I think it might as well be something I could notice a difference on all of the time. I really do feel very good mood wise most of the time but there are always times when I struggle or things get overwhelming.

My Case Manager Steph (Genzyme) is working on Home Infusion approval with TCI (who supplies Aldurazyme) and my Insurance, about this time my Insurance will probably change but still it is exciting to think about when I do decide to do it. I am going to ask my PMD about possibly getting a Port as well. I don't see my NeuroSurgeon again till next Thurs., I feel pretty darn good as far as how I have healed from Surgery 3 weeks ago it is just these
darn Headaches that have persisted. Some days I feel great and no headaches but the majority of the time this time around I am still dealing with what feels like pressure. Stinky! Maybe there is a simple answer and whatever the case I really hope this time around maybe it is a Programmable type Shunt Valve maybe it just needs tweaking.
Who ever knows though!!

Yesterday I did the DVR testing which actually was fun, I did really well on some parts and struggled on other but I did remember most of how to do Math things which are my biggest struggle always. I did above the 50% percentile all the way around on testing though but word recognition was my high point. We did alot of talking and looked at diferent Job areas so I do have ideas now and need to go see if I can sign up for a class or two for the Sems. I am excited, I didn't realize how much I missed working on this kind of stuff.

Off to figure out what to do with the day, take care all of
my friends who come "visit" me here, I will talk to you soon.
Much love and hugs,

Erica

Wednesday, August 9, 2006 12:30 AM CDT

Not to much new going on, I talked to my Neuro team at the U of MN last friday the Appts. I had next week are rescheduled for later in Sept. This means it will be another month till I get to meet my friends going through BMT there anfd hopefully not doing the NeuroPsych testing right now won't affect school things if I do take classes..

Sara and I and Zander spent most of the weekend together which is always fun, it's fun to just do aimless things and
be together! This coming weekend one of my friends from Chicago are coming down, we are going to go to the State Fair on Sunday which I am so excited for. This is the family who had offered to come pick me up at the Hospital this last time around when I had the Shunt revision on thatTues. and was supposed to fly to the Conference in KY. I so had wished that week I felt better so I could have atleast driven down with them, not only do I miss them I missed all of my friends!!!!

Nothings really new happening with the Headaches, I did get
stitches out on Monday which went fine enough. I still don't feel that great or even remotely close to how good I
felt after the 1st surgery, and it has been 2 weeks now so I am really, really hoping this gets better soon. I just want to be Headache free already!!!!

I'll update pics when I get them developed both from Zander's Baptism a week ago Sunday and then from this weekend at the Fair.
Will write again soon, Love,

Erica

Thursday, August 3, 2006 1:49 PM CDT

To God be the Glory, great things he has done.

I thought I would write a update about how my Appts. went yesterday and a few things that are new. I met with Theresa
who has been my Clinical Research Coord. right away in the morning, she introduced me to Patty who will be the new lead on the studies. She seems nice so hopefully this will con't to go well. I will definetly miss Theresa, we haven't
talked as much over the last few months but she is still a very sweet person who will offer anything she can do to help.

After all of the Study Labs I met withLuAnn my GC early on in the morning, she to is has always been very kind and helpful so although I just didn't have to much to say yesterday we talked some and she just offered any help she can give if I need it. She is helping me to coordinate different Appts. though to work on the same days which is so helpful. My Geneticist brought in 2 Med students so these are another 2 doctors who will know about the LSD's disorder and MPS specifically which is great. Patti to is going on to Med School so will be fun to work with her in the mean time. Other than con'ting to need to recover from the last surgery which seems soo slow this time there really isn't alot new since May. LuAnn is very much encouraging food and supplement drinks to help with the con't weight loss so I guess that and goal things are my things to work on in the next few months.

I have con'td to keep in touch with Kim my Audiologist who went to a new clinic/Doctor practice but I met with the new
Audiologist yesterday for hearing Aid fitting and adjustment. She does seem nice and like she will try to help which is always a good thing with this MPS stuff but Kim has also extended the offer that she would be happy to con't my care at the new location. I am not sure what I will do but I may see the new Doctor and decide which seems to compliment my care/future better. I do really like
Kim though!!!

Only 4 more days till I get these stitches out from early last week I am a little more enrvous this time they seem to
be healing into the incision a little to well. I hope I
get the same Nurse I had the first time around to remove them she was talkative and gentle. Right now the last few
days *knock on wood* I have had less nausea and what Headaches I do have now seem stable at the least. I will update after Monday how things go and if anything new.

For now i'll leave off, please con't prayers for my friends and especially Dylan Lashay during her transplant.
Alot of love and Hugs from me.

Erica

Monday, July 31, 2006 12:59 AM CDT

Not to much new to say around here - except I absolutely hate this recovery this time - atleast I am as my friend Amy says "a more knowledgeable Shunt patient" but I sure don't have much of how good I felt last month after the 1st surgery! I guess as Steph says I need to try to learn to rest more during the day and think less but I just want to feel better NOW!!!
We had my Nephew Zander's baptism on Sunday the 30th - was very warm in Church but a very pretty service and Zander was such a good little boy throughout! He likes his snuggling and being held for sure!!! Congrats to my Sister
Sara and her Husband on this wonderful day!
Today I am headed down to the Voc'tl Assesm. job center - I
am off the wait list so hopefully this will be an avenue for getting bak into school (with help from my NeuroPsychl)
and finding a new job that is better on my body and less taxing.
Weds I see my Genetic Couns. whom i've talked to since May but haven't seen since then - this is alot for our every 3
month uGAG Study things but alot of times she'll take time throughout or after to talk and help me figure things out - which I really need help with this time around. In May I was goping through a different struggle whether or not to do a Shunt placement but now that I made that hard decision
and have been through 2 different Surgeries I struggle with
how to adjust my life to this and a slower recovery time. Which is really, really hard for me.
I better run and dash to my car - our weather is sooo hot and muggy and sticky!! - gotto try to get places on time! ;)
Keeping you my friends in my thoguhts through each of your different struggles and joys. Remember through God all things are good and all things are possible.

Love and Hugs,

Erica

Saturday, July 29, 2006 1:56 AM CDT

For some reason my Friend and Case Manager Steph Ginty always reminds me that I should update here! I come anc check the guestbook often and try to make little changes here and there but don't often sit and write. Today her and
I where talking and she said something about coming on to my site to see if their was an update from surgery on Tues.
since she hadn't heard from me yet.

I don't know what else to say except that Surgery sucked from start to finish this time around - the Anesth. doc was
really not nice and from what the Nurses told me he tried 2
different times to Intubate with a normal sized tube when it is clear as day written all over and I told him that I have problems with breathing tubes and small airway. The 3rd time a differnt Doc. finally got a smallr Lightoscope in but he said they still had difficulty - I can tell! My one Recovery Nurse whom I also had last time told me "make sure you let anyone know from now on you have a small, difficult airway - this was ridiculous!" I ended up on Oxygen and in recovery for over 5 hours. Miserable.

The Shunt revision itself went good my NS said - he found the problem right away (a blockage) and only had to reopen 2 of the incisions to replace those parts. I am soooo soo
glad to be out of that Hospital, I wish I had someplace I could run away to but atleast I am not there.

Since I missed the Conference =( I am going to see about using my plane ticket to go down to Amy and Steve Holland's
wlk/run in Oct. and then stay with them. I hope so, I love the Hollands so much - Amy is like my 2nd Mom and a total
angel many times over. This weekend is Zander's baptism - he is getting so BIG! I will try post pictures next week!
He was "talking" while Steph and I where on the phone today so she may be the 1st of my friends to hear my sweetie "babble"!

I will keep posting updates and especially about this recovery and anything new. I see Genetics on Weds., so although it is always nice to see Theresa and LuAnn, I don't really want to go. Afterwards I go see my Audiologist and the following week I get all of my stitches
removed from my back and side incisions.

I Love you guys,

Erica

Friday, July 21, 2006 10:58 PM CDT

Monday July 24th - Audiology, Jody 10:30

Tuesday July 25th - Shunt Revision Surgery Dr.Sinson, time?
Thurs 7/26 - 7/30 National MPS Society 22nd Conference KY

I apologize ahead of time if this ends up being a down and out update - I did not expect when I woke up this morning that I would end the day feeling so down. I had to go in to see Dr.Sinson my NeuroSurg. this afternoon after ERT and
did not get good news. Less than 6 weeks out from the LP Shunt Surgery and I am headed back in for a revision next week already. He isn't sure if this will be a partial revision or a full out shunt replacement but everything is being set up for Tues. this next week. =(

I am scared about this I can't believe this is happening, I
had just gotten back to work, was driving and up until a week or so ago was feeling great. I don't know what to think accept "why me?" when I did everything I was supposed
to.. I have my flight booked and hotel reservations set for Weds-Sun. for the KY Conference and my friend Jenn was going to meet me at the Cincci Airport but instead i'll be stuck at Froedert which sucks. This is the one time this year I would be able to see most of my friends from all over together so I feel so let down and sad. It's hard to think about this surgery as being a necessary good thing when I just want to be boarding a plane and going to have fun instead. Have I ever said I hated this MPS disease?

My friends the Hollands found out this week their daughter
Laynie isn't able to participate in the IT trail out in CA
for her cord compression so I feel badly for that loss for them. Also keep in prayer my friend Devin and her daughter
Dylan as they begin the BMT road.

Will try to write again soon, Much love,

Erica

Tuesday, July 18, 2006 10:44 PM CDT

Hello All, I hope each of you is doing well and many of you I cannot wait to see in a weeks time!

Doing okay here, the usual good and bad days but thankfully
other than some Headaches and Nausea and bad stomach/side and now some back pain am doing well on other fronts. I know that the pain issues probably makes it seem like "how can she say it is well" but I just consider all else that could go on to and I can say it!!! I am 4 weeks out from surgery now and went back to work this last Sat., today I had to call in b/c of the pain but hopefully this will get much better really soon. I am supposed to call my NP at the NSg's office on Monday if this isn't better but honestly I am not sure how to hold that long as this is really, really aweful - makes it hard to sleep, walk and do
acitivies.

My 2 Nephews have been here the past couple days (Quinn was
here last week most days to) which has been really good, both are so cute and Zander is picking up quite the personality of his own! Quinn who is 4 loves to be in the pool now, he has went from sitting on the ladder always to
going around the pool by hanging on to the side. In 2 weeks
is Zander's Baptism, Sara has asked me to be a sponsor I feel bad though - I am at the KY Conference until that Sun.
afternoon and still not sure if i'll be able to change my flight.

I planted a few more flowers earlier tonight in my garden so it is becoming quite pretty. My faorite are the different Lily's but in close 2nd is the Moonflowers and then the big Bush with purple things hanging off. My neice
Alissa helped me plant the original flowers and Quinn helped me plant the others today. The other day while I was
working out there I had a pretty purple Butterfly winding around me which was very beautiful.

I leave for Kentucky early on Weds. next week, my friend Jenn is picking me up at Cinci Airport from there we'll go across the river to KY and our hotel for the week. It will
be so good to be surrounded by my 2nd family for all of those days so I am very excited. I get the chance to meet many new friends in person and see others that I haven't seen since AZ and in the case of Amy Holland and the kids I haven't seen since last June(05'). My friend Naomi who is a Case Manager with Genzyme for another territory is getting to come to the Conf. so that is very cool to! I met her through Steph last year so will be great to visit
again! We have kept in touch but is still fun to see people in person to!

Amazingly no real Appt. coming up atleast for right now, I see my Psychologist on Thurs. - it has been over a month since we last met so that should be "busy". On Monday I meet my new Audiologist who took over for Kim and I guess
get fitted with my new Hearing Aid. The week after I have a NeuroSurgery Appt. Fup to reeval Headache/symptoms, see if there is more we are going to do (he as talked about a
Radiology test where they inject die via Lumbar Puncture to track the flow through the LP Shunt and evaluate if it is working properly.) The other thing is they have talked
about possibly revising the Shunt Valve so that it drains
more fluid. (yuck) That same morning before the NS Appt.
I see LuAnn my Genetics COuns. and Dr.Rhead in the clinic
for our 3 month Appt. which is always pretty busy. I am sure by the end of the 2 Appt. I will be worn out and ready
to not see another Dr. again for a long time!

I guess that is about all that is new - not to much going on. Check out my photo album for new pictures if you like.

Much Love and God's Peace,

Erica

**Please con't to keep my friend Devin and her daughter in
your prayers as they journey through the BMT journey and pray that things go well. The actual date is 28th for Dylan
to receive the new Bone Marrow. ***

**Please also keep my friends the Hollands in your prayers,
thir youngest daughter (Laynie, 14) is beginning the Intra-
thecal Study this week and is very scared. This is one amazing family (Amy is much like a 2nd Mother to me) so I pray this is a smooth journey that reverses the Cord Compression in sweet Laynie.

Sunday, July 9, 2006 2:05 AM CDT

I should be going to bed right now as it is really late but thought I would give an 'I'm home from MN' update and how things are going.

I first seen my NeuroSurgeon on Weds to see if he would even let me drive up to Mpls. as I actully am not supposed to be driving till after Monday but I got the okay! Things are doing pretty well - having some pain issues in my stomach/abdmonen and side that I am not sure what it is - I
thought it was just from healing but dang it really hurts! Otherwise pretty good, appetite has plateud but still is more improved then it was before the Shunt surgery. No weight gain yet almost 3 weeks out - but I also see Dr.Zuccaro on Tues. for this sort of thing. Headaches are really good - I do get the occassional ones yet but definetly NOTHING like before surgery and seeming to be adjusting to the lower pressure which is a huge relief. The SOB is still there which bites and I don't know what it
is from I had really, really hoped this was caused from the
Hydro. I need to contact my Pulmn and find out what our next step is or if we'll give it some more time. The best part of my Appt. with Dr.Sinson NSg on Weds. was the simple fact that we where talking about adjusting to life living with a shunt and basically going on just as if it wasn't there. I don't have any set Fup Appts with him for right now which although I liked my doc he is very nice and
smart and all but that felt so awesome beyond belief to walk away! His secretary Kris, came out - and I told her that I hoped we'd only talk in the case of good news updates and gave her a huge hug.

Thursday I made my ever adventurous trip to big ole' MN to see good Ole' Doc. Charnas. was a good Appt. but I knew it
would be! I knew I would get to walk away from that Appt. knowing I wasn't going to be waiting for symptoms to come back this time but instead I was already on my way to recovering!!! I loved that when he said 'You showed us!' How cool for a Doc to say something like that - when they don't always think sometimes is what it is but are willing to work out the answer and find the solution anyway. No changes in meds right now except to decrease one of my Headache meds and eventually hopefully our plan is to be off of it completely in August! I see Kendra Bjoraker (NeuroPsychologist) and DrCharnas again in August and then we'll do testing make a decision about memory stuff and whether to up the dose on the drug I take or what to do with it.

Todays meeting went well - was pretty fun a ton more familis than last year at just the MPS I mtng for sure! I met the nicest family from Nebraska and look for to seeing
them again in KY!!! I did not get the chance to meet my friend Devin Fryer and her family this time - there plane actually arrives into Mpls. Sunday around 10:30am. and I drove back to WI earleir today(Sat.). I did get the chance
to talk to her on the phone though, we are gonig to keep in
touch and will meet in August which I am very excited for!!

Please keep Devin's daughter Dylan in your prayers I will post an webpage when this is available but they are just beginning the BMT process at the U of MN in the next weeks.

I am home tonight writing this - glad to be home I missed my family even though I was only gone for 2 days but it almost seemed more like 3 weeks. I got to see Quinn and Zander (and my Sis) right away when I got home and that was awesome.

Good Night and God's Peace,

Erica

Wednesday, June 28, 2006 7:11 PM CDT

Just an update on life Post Surgery (LP Shunt) -
-Other than having this obsurd feeling that the distal end of the LP Shunt is moving freely about in my stomach I am feeling more optimistic since the surgery and feeling much better with Headaches. I should maybe not be so cautious but knock on wood I feel really good other than some shortness of breath and nausea here and there. I am eating much more and actually craving food - that is something I actually missed! This may sound weird but I am climbing out of the post-surgery funk and finding myself in my old mood which is happier and less "snappy". A really good thing.

The incisions seem to be doing alright the stitches come out tomorrow (thurs) I think, or atleast that is when I see Steph Christian-Lobley the NP for a wound check. I talked to her the other day over the phone and she
is going to check out the cathether tubing + possibly have a Shunt X-ray series run to make sure nothing has b/come disconnected. I see Dr.Sinson in a month forthe Surgery-Hospital F/up so I think I will have a break in between these 2 Appts., unless something would come up.

Otherwise I have been trying to get myself back into the grind of my life, it has been a little harder than usual though.. Maybe I am to hard on myself I guess in some ways I really was very sick last week so I hadn't the chance to do things and am recovering now from the insult and adjusting, but yet.. Sometimes when I am busier or more "scheduled" than I am better about getting things done if that makes sense. There is alot I still need to take care of for upcoming things but instead I find myself aimlessly going from one task to another, crossing things of my list but not relly always getting the very important thing done or making a decision that really needs to be made soon enough. Half the time I don't even know what decisions I need to make this week though aggh I just know I am restless and bored and ready to jump back into life atleast a little bit more.

Struggling to decide what I should do next week I am pretty
set on all the plans to go to Mpls. but yet I sort of want to stay home and go another time. I love the MPS meetings there but yet I don't want to spend what little money I have while off of work when the Conference is coming up.. I
know I can reschedule my Appt. w/Dr.Charnas he wouldn't mind a bit or I could keep that and come home the same day but I just don't know what I should do. I really want to see Kris my infusion nurse and if I go to Mpls. then it will be another 2 weeks till I see her to so that is another down factor. (I missed ERT last week due to being in the Hospital and Kris is gone this week). I should probably go and have fun but I don't know I guess i'll decide for sure by the end of the week. I haven't said anything to anyone though yet.

I guess that is it for now, for today it is happy Headache day. Hopefully the same goes for tomorrow and all days after!!!

Much Love and God's Blessings,

Erica

Sunday, June 25, 2006 10:51 AM CDT

I made it I made it I made it I made it!! yey Yey Yay! =0)
I don't even know where to begin to update this jounral and
guess most of you either talk to me or read the Forum so you've gotten updates but i'll try!

Surgery for the LumbarPeritoneal Shunt was on Monday the 19th - I met my Anesthesia Docs Dr. Lauer and Dr. Schneider
and went over the game plan for intubation. Both where very
nice so that was a relief!!! Some of my friends have had horrible experiences so I was a little nervous! We went to
Surgery about 1:30 and after that it's was lights out, sleepy time for me! I just remember coming back to the Recovery/Pre-op room and getting a *Star pt.* (!!) cut-out and the oxygen and pain meds and otherwise going to Neuro-ICU. My NS Doc. came and talked to me, told me that the pressure was elevated even under General Anesthethetic, (generally if you are under anesthesia your ICP pressures are lower) told me a little about what he had done and reassured me it went well. That night was pretty hard I liked my Nurses just couldnt sleep very well.

Tuesday had a CT scan for my Pulmonologist and went to the Acute Neuro Surgery floor where I got to meet lots more new
Nurses and staff. (as you all know though Hospitals sure don't like letting you sleep!) Weds got all the IV's and Cathether removed + should have been home. .. Dad and I made it as far as CHW before I started throwing up and getting sick so they wanted us back in the NS clinic. From there symptoms like vomiting kept up so back to the NS floor I went (ooh what a joy) to be readmitted. Nothing like a welcome homecoming or something?! IV fluids, meds, etc. where restarted and tests where run. Thurs was just pretty much getting better and them deciding what was wrong + whether or not I could go home. My NS Doc Dr.Sinson
thinks it is b/c of my body adjusting to the new lower body
ICP pressure and having to take time to adjust. Makes sense
since after all if your pressure is High for a long time then you do surgery and lower it you are going to probably need time to adjust to a different "setting". Many of my MPS friends have said there kids went through the same things with the Shunt surgeries.

Friday I got sprung and home I came and I actually made it!
I could walk about 20feet and then have to sit down but atleast I was out!!! I am SOOOO incredibly sore from back to front but good to be home. Sat just took it easy hung out with my Sister at her house with my Nephew watching movies and talking + reading which was nice. Today is early
but waiting for the morning Headache to subside and hoping that the vomiting I had this morning doesn't come back!

I think that is all hopefully it will be good things from here on out with improvements I could use good things!!!!!
Thanks for coming by to check in, Love you all lots!

God Bless,
Erica

Sunday, June 18, 2006 11:24 PM CDT

Just a quick journal tonight but tomorrow is the day - I cn't relly believe it is the night before! I am starving right now and thinking I should eat something quick before midnight but nothing looks good. Go figure!

Dad is taking me to the Hospital (Froedert) - we have to be
there at 10am with surgery at 12 I think. My NeuroSurgeon is Dr.Sinson so wish good prayers for my Surgeon, Anesthesiologist, Nurses and all staff!!
The Shunt model and type is Integra and placed in the Lumbarperitoneal space which means no monkeying with my brains, yippee!! (sorry bad humor)

Many of my friends have already asked for and are calling Dad on his cell phone for an update: the # is: 920.210.5118
Otherwise i'll be at Froedert until Weds so can always call
my cell: 920.382.3049 or just plain leave a message here!

Was a good weekend - I had my Nephew Quinn from Saturday till Sunday night and Mom + Dad watched him while I went to work. Friday night Quinn, his Mom and I went to see a movie and hung out. A very busy weekend sure makes for no time to think which is good!! Baby Zander's doing really well sleeps so much but is so cuddly and cute!!!

Well I guess this is all for tonight though - will try to update when I get home in a ew days. Am sure i'll be bored out of my mind while off of work!

Love and Hugs,

Erica

Tuesday, June 13, 2006 7:24 PM CDT

Thursday June 15, Appts. at Children's Hospital Milwaukee.
- 9:00 Dr. Brosig Psychologist
- 10:00 Pulmonologist Dr. Julie Biller
Friday 6/16
- ERT at NorthShore Dr.Rhead/Kris
Monday June 19th Froedtert Lutheran Hospital Milwaukee
--> LumbarPeritoneal Shunt Surgery Dr.Sinson

Happy Birthday Dad!!! Last week my Nephew Zander was born on 6/7 exactly one month before my Nephew Quinn turns 4! I have pics set to put up in here but haven't been able to resize them yet! Zander was born to my Sister Sara and her Husband Jeremy at 5:06pm weighing 6pds with dark black curly hair.. He is a doll to be sure!!!

My Dad's Birthday is the 15th so I hope he has a wonderful day am trying to think of something special I could do, my parents do so many wonderful things for me!!! Hope you have the best day Dad!

After much careful thought and a whole lot of talking to a whole lot of different people from my family and friends to my providers I made the decision to go with a Shunt after all. I really wish I knew that the I.T enzyme approach would help and that my symptoms werent seeming to get worse
steadily but I feel like this is the best thing to do given the circumstances. This is atleast one thing I can try to get a handle on with symptoms and hopefully by helping the headaches this will help the symptoms like nausea, some vomiting, weight loss, etc. I sure do hate making these kinds of decisions though and look fwd to the surgery being
over I know i'll be completely out but I am not at all looking fwd to the breathing tube!

Unfortunately do to the timing of the surgery i'll miss going to Chicago =( but thankfully the Mpls meeting is in another few weeks (July 8th) and KY Conference is just a month away!!!! My friends the Nagy who I stay with in Chicago will be in KY thankfully so I very much look fwd to seeing them there! I so cannot wait to see everyone else as well and meet many new faces to! AMY HOLLAND you had better be there girl I don't care if we are stuck in a hotel room together if you are not feeling good we will be there together, I haven't seen you since last June and I MISS YOU!!!!!! =0)

I am afraid I have no inspiring words to share today. I of course am surrounded by lovely people who share their kindness every day, I just am feeling without the words to
express their special and unique trades.

Please continue to keep my friends and their families in your thoughts and prayers as each goes through there own good and difficult times. Pray that each may find joy and happiness in what might be todays sadness and sorrow.

Love,

Erica

Sunday, June 4, 2006 1:31 AM CDT

I have been oweing this update for many weeks now all these things that are going on considered anyways! Steph if you are reading this then I finally did it, I updated again!!

Tonight was great all of us kids where home and Mom and Dad except my Bro Jami but my Neice Alissa was here and so was Jeremy and Sara and well the Baby still riding along tummy side. I went in late to work today whatever it is Kris my Nurse gave me yesterday right after I laid down (when we shut off ERT early) "knocked my socks off" I never woke up from 7:30 last night till 12noon today. Everyone else put the pool up and tonight Lissy and I planted all of my Lilys and Begonia and Moonflowers in my Garden. I took the Neighbor girl and we went to DQ which is always fun, just nice for us to hang out.

Last week I missed ERT due to going to MN with Dad to see Dr.Charnas my Neurologist for a LP (lumbar puncture to remove spinal fluid) so yesterday I was back but only made it about 3 hour of the 4 hours and started to feel sick - Nausea and Headache was worse, really red. So I asked to stop (I have never ever asked to stop ERT and NOT con't it atleast later!) and Kris thought it should be to. She is a very sweet Nurse so I am atleast glad if I have to feel that crappy then she is there to help./ In another of the 'seem to be occuring alot lately' theme my Dad and Sis' drove down and picked me up. The week before Dad took me to the ER, to the U of MN to my Neuro Dr.Charnas and this week I have asked Sara to come with me to see my NeuroSurgeon.

My Neurologist was setting up a Intrathecal Enzyme Repl. using Aldurazyme (same as I get every week by IV) to be given via Spinal Tap/LP protocol with a Dr.Patti Dickson at UCLA but to be done at MN to treat the High Pressure and Headaches as a sort of experimental approach but we also had agreed if these Headaches came back sooner than we both hoped (this last LP he took off more ICP fluid which is what is causing the Headaches and also created a bigger hole for fluid to hopefully keep leaking out off) we would have to go with a Shunt placement Surgery. Not high on my list of things I want to do nor do I really know how I will manage this with my crazy life I am a little freaked out.

I guess as my friend Marie M. put it I sort of have to prioritize Health > over Plans. =( I know it is true and I
know the Headaches the worse they get with each day the more problems I will have and the less i'll be able to do until something is done again but I am stubborn and I hate that medical stuff is getting in the way of what I have been looking fwd to for months. =( I want to work and NOT miss work and go have fun those weekends I have get aways planned and I want to have spending money and YES I want to feel better WHICH IS most important to me I just wish it was as easy as the LP's where I feel better immediately and
hop of the table and am good to go vs. a surgery where all is unknown to me. How do I do that? I am not good at unknowns as all of you know.. The scary part is days like today when I'd feel good I think clear, when my Head hurt I had so many issues it's bad which can't be good. Things that go beyond all that the Aricept really does help with Memory wise...

I seen Dr.Earing, Cardiologist a few weeks ago didn't really pass his "magic" Stress Exercise breathing test- Endurance is down but Echo shows Heart valves are stable at early Mod. and that is a good thing. He is one of my Fave. Docs so I love making him happy, (hahah) the St.Ex. Br. test we both decided may have to do with the Headaches situation so we'll let it go and see how I do once this is all situated and worked out. His other concern is any of my other Drs. concerns and that is weight loss but I eat what I can and atleast am trying to drink atleast a couple shakes a day to make up for what I can' always keep down or eat alot of. (makes me nauseated when I have these headaches) So he of course threatens me but he's a good Doc and we'll check in from time to time and unless things go down hill and ok till atleast Nov.

My AUdiologist and I are working together on my Hearing aides so unless LuAnn would say she doesn't think they are going to do any good those should be ordered and set within a few weeks. Kim and I are just doing paperwork right now and Insurance work, the fun stuff! (yeh right) But they are actuall pretty cute hearing aides and very invisible. I like Kim and she has been extremely nice to work with and so easy to be in touch with!

Sara and Jeremy and the Baby are doing good - could be any day now or could be her actual due date the 22nd-23rd of June. But we are all thrilled and waiting for Baby "Hey-you" to make an appearance to the world!! I think this will be a tremendously good thing for me and I don't mean that to sound stuck up, I jsut mean to have the Baby to take care of and help with and watch!!! I love him to pieces already and he is my Best Friends (my sis) little boy which is even better! I am so excited for Sara and Jeremy and just hope I can be a good Aunt and a help to Sara when she needs it!!!!

I don't know that there is alot I have covered but as usual I have managed to write alot so I wonder what I am forgetting.... I guess I will just wrap up with asking for prayers I haven't ever had to make such a big decision or atleast one that took so much thought about my own body while also con'ting to do everything else when I can. I never want to give up my Independence and don't want to be seen as a sad or angry or bitter person I am just struggling right now and know that even if I can't figure out how I can handle it all, God already has a plan and is handling it for me, I just need to put my trust in him more fully. It is these words I keep repeating to myself these days. 'Trust in the Lord and You shall Safe.. His words are like a Lamp to my Feet and a Light to my Path'.. I think of my Grandpa P whom my Family says I am most like and I know that he would have known what to do, so I am just praying I am making the right decisions. I believe that God will bring me through and I con't to talk and write and do things to try to find my peace.

I end with your words Steph; once again:
"I find taking one day at a time, embracing that day, the good and bad and learning what you can from all you have experienced in that 24hrs is the way I approach life." ' Life is a Journey with Bends and Turns and sometimes we just don't know what to say and sometimes there just isn't anything anyone can say.'

And on a final note I end with words from my Study Coord. Theresa, who I have long thought of as a friend despite our not being able to talk to often these days:
~ She wrote me last summer something about Christopher Reeves and his becoming President of a Club he hadn't asked to become a part of but despite the amazing work he did towards helping those in similar situations as himself he still needed to have other goals and accomplishments in his life. She went on to write "I know this isn't quite the same as your situation, but I think there is some similarity. She told me things about being a person outside of my MPS I, that by helping others and setting examples I could be leading and inspiring others but I also should try to find my future that was not dominated by this disease. Her thoughts where that I might find peace with my situation if I saw myself as me but not in the picture of this overall disease.

I know Theresa's words like Stephs have had a profound impact on me both initally and everytime I come back and re-read them but I feel like this summer these words are my mantra - I may have to do these things I don't want to this summer but I am trying to take it day to day and not give up the hopes that I may be able to go and do atleast some of what I have had planned. I also am looking at "if I feel better, well then I can do more so then I can pick up a class or two especially if I get this new job". It doesnt make it all better but I just want everyone to know the trooper is sailing on and the winds are blowin' fair each and every day' there may be some rain and some clouds and more of the the days I go knowhere in between right now but I will eventually reach my desination and with all of your support, my family and God's good grace I will keep truckin' along.

Thank You to each of you who are the very special ones in my life you are amazing friends, family, providers maybe one, all or some of both. For Steph, Theresa I love you for being here and thank you for always letting me borrow your words even months later.

May all Good winds blow Kind Gentle and Fair..

Erica

Saturday, May 13, 2006 4:54 PM CDT

5/15 - Cheryl Brosig CHW 11:00 Psych
5/17 - Dr. Cheryl Zuccaro, PMD 3:00
5/17 Con't - 4:45 Dr. Michael Earing CHW Heart Center,(Echo, EKG, Exercise Stress Test w/Breathing Funcs.)
~ ~ ~ ~
Something my wise friend (and Case Manager) Steph Ginty once told me last Summer.. "Be angry if you are angry, Be sad if you are Sad, but when you are Happy be sure and make the most of it" ** I again have come back to this when I have been struggling through a couple of bad days and feeling down. I am not sure there is really any one reason why I feel down I think it is some to do with that alot of different things. Seeing olds friends coming home from College, Graduating the year I should have been, having plans, knowing what they want to be or do and having no seeming bigger worry than little things, things that I am envious of I think.
~On another hand I think I have been down because I am so used to taking care of myself when I have the time but I am now waiting for other people to set up and schedule and awaiting word when the test will be, the dates of the Appts and so on to see if I have to re-arrange any other Appts. to fit together or work with my job schedule and so I can make sure things just work. Not a big deal, I am jsut used to doing these things myself and not used to waiting.. ** I love that I have my Genetic Counselor and Neurologist to help me set up and arrange things - b/c they are taking time to research the best providers and places they believe to have these things done at it is just hard to wait.

I think a bigger part of the last few days was on Monday I had not only the 4th 3 month uGAG Study Visit but also the 2nd 6 month visit - and our 1 yr. Anniversary basically of being on Aldurazyme and looking back. We have made progress in some areas and in other areas have regressed and in still other areas stayed stable. I think we (my Genetics Couns. and I) both have learned alot about this form of MPS I in the last year, some of our biggest hopes haven't been answered but then there are things that have brought us so much closer when I've struggled or fought for something and she has been there to back me up. Overall I think it has been a learning process and even if I haven't gotten the thing I really, really wanted out of the last year of infusions I am so thankful for what the ALdurazyme has given me and for what improvements I do have already.
** I love that I have known LuAnn for over 14 months, she has become like a mentor through this struggle, I see her as my GC but also as a friend and she has helped me when I can't always fight it alone.

I see Dr.Brosig this week and although I dont usually have any one thing I go into an APpt. with that i'd like to talk about. Usually it is more of a whatever comes to mind or is on our minds that day but this week I have a few things and one is: "Making good decisions with Imperfect Information"
- Something I probably drove my GC nuts about on Monday asking her for advice on what to do and how to make a decision about my Drs. and not hurting 1 provider if I saw another, etc. The big thing is that she thinks that since my LP #'s are so "on the line" not "black or white", "cut or dry" that I may get differing opinions from different providers and ultimately I will have to decide what is the
right decision for me in what to do to treat these Pressure Headache issues and where to receive treatment when I do make a decision whatever the decisin may be. She said she knows I wouldn't even be considering such a drastic measure as seeing a NeuroSurgeon or Shunting if my H/A's werent severe and affecting my QOL but I still feel extremely unsure!

I am realy glad she is that good at reading me and knows just what I think and can tell when I am troubled I just think this is something that will probably trouble me until I feel better one way or another and talk to my PMD about things, and to LuAnn and Dr.Charnas again.

In the mean time one of the few things that has remained a really good distraction for me oddly enough b/c my boss pisses me off s work, I think b/c I get immersed in other peoples lives and don't have so much time to think about my own. Even when I am doing treatment or at Appts. or just away from home I am much better than if I am just alone. When I am alone I relect and that makes me sad or down or frustrated and then I get either angry or sad or something. This week does seem like i should be busier than
last week - b/c other than Mon. I had alot of spare time in between work and being with friends. This week I have Appts. and work, hanging out with friends other than on Weds. and Thurs. which are: Appts. all afternoon. Thurs is work, church and baking something for 2 B-days on Fri. and my 1 yr Anniv. of ERT. Fri. is ERT, then Baby shopping and doing something fun with friends. (so what if i may be 1/2 out of it!)

I know this has been a pretty boring update so hang in there this is always as my friend Denise says "A wild ride"
next time I may have something truelly exciting to tell and you'll want to check in! ;0) I'll try atleast!!

Wishing Peace, Love and God's Blessings upon You.

Erica

Saturday, May 6, 2006 3:41 PM CDT

Hey All, I think it has been only a week or so since I updated so tat doesnt seem so bad for my record! I guess it has been a busy week between a couple of different Appts. on different
days and work and other things. I can't believe it but only 6 more weeks now until Sara - my Sister is due with Baby Boy Bailey! They haven't picked out a name yet but is between Austin and Ethan right now I think from last word!

**May 13th is the NHS Sponsored Fundraiser to Benefit the National MPS Society - Wal-Mart 10-2 Car Wash suggested donation is $5.00. **

It doesn't seem hardly possible but we have passed the 6 month mark of Tyler Kent Deskins becoming an ^MPS Angel^ my prayers are continously with my Good friend Jenn and her
Family through the good days and the bad.

We are also coming up on the 1 yr mark of my Cousin JJ's death May 20th - this also marks my 1st ERT infusion and my
1 yr mark (54 weeks then!) but the day will seem much more sad than joyous I think. Perhaps since Infusion falls the day before this sad day that we lost one of our favorite and closest cousins I will be able to cheer reaching the 1yr Anniv. of ERt.. It will be a good thing and a sad thing for sure!

I met with my new ENT Doc just this last Tues. - Dr.Linda
Dindzans and my new Audiologist Kim. This was a good Appt. with mixed results I suppose! I am not really sure what to do yet but have to talk to a few of my providers (have talked some to my Family and to other MPS Friends who
have had similar) about geting Hearing Aids to help with the Mixed Hearing loss that I do have already. I guess to preserve what I do have left, ugh!

I have come to the conclusion to never ask what more can possibly go haywire with this body but it's alot lately!

I met with Theresa my CLinical Study Coord. on Thurs. b4 having the LP in Radiology (LuAnn my GC had set this up for
me per Dr.Charnas) it is always nice to see her - we had an
issue with something with the schedule so poor Theresa had to do alot of calling around but we got it done! She has been working to get the uGAG results for some time now and is working with BioMarin directly now but her and LuAnn had also thought of maybe doing a separate Lab locally to get Urine GAG results for atleast now to let us know how things are doing or changing since last May. Seems like alot of work for her but I think she is very sweet and patient so she just wants to help.

The LP itself went fine, my Psychologist actually met me over there since we where originally going to meet for an Appt. I dn't really have a problem with the LP's I think it hurts much worse the days after the test than during but
she was stil la nice distraction. I do have to say I really
think she was looking a little pale at times but Dr.B was a
champ and we just kept chatting. =) The Opening Pressure which is what Dr.Charnas really looks at was 19 and right at what it was in MN when he did the test (was 20 then) and I have felt pretty darn great since Thurs. albeit the backache and other things but no Headache! I can't keep up with this appetite but I won't complain either! ;0) These
are both the same things he was looking to see if we would get so now it is basically the decision of whether or not to Shunt and what he thinks we should do. Plus in the long or short run i'll have to decide where to have it done. - In MN or Here if we do go that route. I await to hear from Dr.C though about his opinion and Appts (Him + NS) so of course we all know that means the waiting. I will in the mean time pray this H/A remains free for a long while yet!

I have also been talknig to Dr.Patti Dickson at UCLA she is
conducting a Study on Intrathecal ERT and Cord Compression but is interested in working with Dr.Charnas and my NeuroPsych Drs. in a attempt to see if the 2ndary goal of her trial Reduced ICP pressure (hydro) might benefit my situation! This is something Dr.Charnas would do but she would work with him and if so this ERT which is Aldurazyme given by LP every month or so might put off the need for a Shunt or might reduce pressure or help with the Memory and
Cognitive Problems I have! (for which I am on the Aricept)

It is alot that has been going on on those fronts but I see
my Genetics team on Monday 5/8 and will post an update from
that Appt. of anything I learn frm there about all of the stuff I wrote about.

I hope all of you my greatest friends, family and those providers who come here are doing well and Thank You so much for Checking in! Love and Hugs,

Erica

Wednesday, April 26, 2006 2:52 PM CDT

Appts: Dr.Dindzan ENT 5/2/06 8:40
Physical Medicine Rehab - Dr.Borca 5/2 11:10
uGAG Study Labs # 4 - Theresa CHW 10:00
Psychologist - Dr.Brosig 5/4 11:00
5/8 - Genetics uGAG/6month Fup 12:30 LuAnn/Dr.Rhead

Hi All, sorry it has taken me so long to update again things have con'td to be pretty crazy here and when I am not busy it means I am probably sleeping! Last week I seen Dr.Charnas my Neurologist at the U of MN on Thurs. for Fup on the Headache stuff and just general re-evaling the situation. What he has decided is best is to have a repeat LP done but here in WI vs. in MN so that we can see if we get the same results as the 1st test when done by someone else. His thoughts where that if the test results are the same then we'd go to a NeuroSurgeon and discuss options but
he thinks that a Shunt is going to have to be done. I am to
say the least a bit scared about that, I mean a Shunt? My head --> yikes! But on the flip side I am so tired of feeling yucky and tired and nauseated that if this will help than do whatever yah gotta do. My goal is just to feel
as good as I did that week of the 1st LP and Dr.Charnas + others think this can be done.

On other things have been working alot --> yesterday when I
called in sick my Boss gave me this big lecture about responsibility and putting my job b4 Appts., and them counting on me, etc., etc., which was horrible. I was so upset by this and just in tears. I rarely call in sick and the last time was probably Jan. and 2nd I really was sick I
felt just aweful. It's like he doesnt get a clue at all and
even though I make less than an ideal amount working there
and work my butt off when I am there, + pick up shifts, stay extra, help out, whatever and this is what I get from him? I am sorry but what an Ass. The job search con'ts I would love nothing more than to leave that place, I hate him with a vengeance right now and can't believe he could be so callous.

I talked to Laurie from the MPS Society yesterday, I was able to get a Scholarship to KY!! I cannot wait only 3 more months and I get to see all my friends again!! My friend Jenn lives in KY so she is picking me up at the Airport which is extra cool! **We have a date for the NHS 4 MPS Fundraiser - May 13th at Wal-Mart 11-3 a car wash to benefit the MPS Society that I have been helping with. I am excited for this weekend, very, truelly excited to do something to give back!

I guess I better be off now, gonig to plant some MoonFlower seeds in Memory of Kaity Bug Wellman - I hope these plant well and grow and grow I would love to see these each and every day and think of all of our ANgels in Heaven "Dancing in the Wind".

All my Love, thanks for con'ting to check in.

Erica

Monday, April 10, 2006 10:27 PM CDT

I am finding it hard to find anything to like about having or dealing with MPS righht now.. I feel so aweful lately between these Headaches and the Joint pain and being so run down from all of these colds to being so tired and having no energy that no matter the good things rigt now I just don't want to have or deal with it.
I'd like to be able to be able to go to a job and come home
and still have energy to do things. I'd like to have a little bit of money and not always owe my parents for once. I'd like to have days off to have fun and not to just go to Appts. or ERT and not have to worry about scheduling things, arranging this or that and making sure this thing and that gets done. I think I just want to feel good and have sme fun for once, it's hard to go and have fun when you don't feel good. And equally hard to focus and do all of the things that need to be done and focus on good things (even though I do) and have some fun when you just feel crummy 99f the time.

There really isn't anything new to report here I haven't had the energy to do anything, spending some time with my Sister but other than that my other friends around here are
kind of on "hold" b/c I just really no matter what might be
thought can't go out when I can barely stay on my feet my knees hurt so bad and my Head just pounds. My Primary Dr. started a drug last week called Diamox for Commun. Hydrocephalus this will be to see if we can achieve the awesome same results I had after the LP (lumbar puncture) and although the Med. can take up to a month to kick in, I am crossing my fingers once I get up to the 500mgs 2x's a day that this will kick in more-so and i'll notice more of a difference and start to feel better. I am supposed to Fup
with Dr.Charnas next week also. I am going to call my Physical Med. Rehab Dr. and see if she can put in that referral to the Pain Mngmt Dr. she talked about and also see if we can move up our Appt. <-> I had rescheduled from last week b/c of feeling really sick due to cold # 901.

I have a call in about a Receptionist job today for a Hair Salon so have my fingers crossed that i'll here back from that. I think it would be fun and to mostly off of my feet which would help. Waiting to hear about some lawyer business to see if they'll take my case it would really be a big stress relief if I could get both of these things! Between this and waiting to hear from my C.M. in Juneau and
trying to ignore everything that my Dad says and not let it urt my feelings I feel like a rocket that is about ready to go off so could really use some good news.

I guess that is all for now but thanks for checking in and thanks for listening to me while I vented some.
Lots of Love,

Erica
http://www.jointhesearch.org/AboutTheCampaign/Erica.asp

Tuesday, April 4, 2006 10:19 AM CDT

I have been meaning to update this for sometime now..Sara and I did go to the U of MN last week and I had the Spinal Tap (ie: LP) done and felt great afterwards!! Dr.Charnas thinks the results are within normal but didn't give much explanation then why I felt so much better. We flew home the same day which went great - slept the entire way and boy was I tired! I saw Dr.Zuccaro on Weds and talked to her some more about the results she mentioned something called Normal Pressure Hydro (NPH) I am not sure now if that was Dr.C's thinking or hers but it seemed logical at the time. We where just crossing our fingers and saying prayers that Headaches wouldn't come back and going to take
things from there when/if they did. I felt so much releif t/o the week, clearer thinking, incerased appetite, not so tired or run down, (other than this dang cold I have again)
and Headache free which was amazing. If I had any doubts that my H/A's where pressure related somehow I don't really
anymore!!! By Sunday the Headaches where starting to come
back though and last night I felt truelly miserable, nausea, headache, pressure, sleeping upright, etc. A bit bummed about this comnig back already I really thought the relief would last longer - I was amazed to begin with that I felt so good after the LP, considering most people feel real yucky and low pressure headaches, etc! I do see Dr.Z.
today and Dr.Charnas in a few weeks. =(

I have been busy with work and other things and one might think that stress could play a part but actually I am loving it. I love being busy, having lots to do and having just a little bit of time to sit down and update friends or write here. Sara and I are hanging out when we can and the Baby is doing well. About 12 weeks to go for 'Baby Mr' so we are all excited for that!! This means a Baby Shower to plan, yippeee!!! Am also going to be working on plans for an MPS/ML meeting here in WI and plans for my parents 25th Wedding Anniversary next fall. Still doing school stuff and waiting to hear on some of those things, as well
as SSI. Lots to do and thankfully although I get a little
absentminded (alot??) =D with these headaches atleast it keeps me distracted. Have to also start Therapy again haven't been going since Febr. just has been nuts and alot of different circumstances playing into it. (trying to do just one day of Appts. a week, and ERT rest normal stuff
so tat requires alot of balancing and also working and money and all that)

Thanks for checking in though - keep watch some friends of mine are heling me update the pictures and my friend Alicia
is re-doing my photo album for me!!! Thanks Alicia!!! Jenn
and Andrea and Gabbi and I are all talking COnference plans
which is exciting to - we might share hotels or atleast come in on the same day and spend a night at Jenn's House!!
How Cool!! (can't wait for July!!) =0)

Well Thanks for checking in - things are doing alright. Love you guys and you sure are the best!!!! Love,

Erica

Monday, March 27, 2006 2:22 PM CST

I am getting very terrible at writing these updates but I have a few minutes here so I made myself sit down and write before I take off to do the next thing! Not to much is new these days - mostly just lots of the same old same but alot to keep me busy! I have been working most days not long hours but just enough to keep me busy and keeps me distracted! I haven't had any Appts. in the last few weeks other than the 1 in MN with Dr.Charnas 2 weeks ago so that has been a nice break for sure! Tomorrow (28th) I
fly back to MN though with my Sister for the LP test at Fairview Children's so I have been pretty nervous about that to say the least!! I am actually feeling more calm about it today but will definetly look fwd to when we are talking results and making decisions about what we'll do for the enxt step.. if it is High Pressure or whatever the cause. I know Dr.C and Kendra think that the headaches are very likeally High pressure related so I will be glad to have this test done, out of the way and to have my Sis' there when we talk. I haven't heard from Kendra now but she has said she would sit with me during the actual test
so I am really hoping she will be abl to be there!!! I trust Dr.Charnas completely and love his personality just since my Sis' has said she won't come in for the LP (can anyone blame her, lol! I don't want to come in for it either!!!!!!) It will be nice to have someone to get me through it even if it is a fairly quick thing..

Weds I see my PCP for the Weight Management Stuff make our next decision about what we'll do next - we had talked about some differnt options a couple of weeks ago but wanted to wait until I was done with this latest round of Antibiotics. My weight has been stable for a couple of weeks now I just have no appetite and I think all would like it to be a bit more stable or increased or have a better handle on it. We'll see. =) ERT is going good, we are still doing 4 hour to 4hr 15min infusions and that works perfectly for no reactions and overall how I feel. At that rate it also doesnt aggravate my headaches which is the best part! I usually sleep during the infusion for about 3-4 hours and boy am I out of it a storm could blow over and I would have no clue!

I haven't talked to my Study Coordinator or Genetics COuns.
lately but we do have a 3 month Appt. coming up again in May, (where does time go?) But hopefully i'll talk to them soon both are a wealth of information and I sure have lots of things on my mind these days to ask the two of them! I am going to talk to Steph Ginty and Mary Yuska about planning an MPS meeting here in WI for all of the MPS disorders though - Dr. Rhead said he would be interested so
maybe just maybe we can make it happen!!! That would be fun to plan! There are meetings in Chicago and MN coming up in June and July which I am looking fwd to as well as the Conference but something local would be neat to host!

I guess I better sign off and go get something done lots to do before tomorrow and I still have to work tonight!! I will update when I get home.

Thinking of all my friends and family and keeping you in my
thoughts for good things for those of you who are gonig through difficult decisions right now.

Love Always,

Erica

Saturday, March 18, 2006 1:53 PM CST

Hi All, so it has been a while since i've updated here I know I send emails to some and update on the Forum here and
there but I have been really bad about my Journals here! I don't even know where I last left off I think last month! So March has con't to be a busy month I was working alot the first two weeks and dealing with the remaining bits of a infection that just wouldn't get out! I don't think I had alot of Appts. which was nice just maybe with my PCP, Dietician and Physical Med. Rehab Doc. (PMRD) I have been missing alot of Appts with my Psychologists due to not feeling well, the weather, work schedule and this last week
getting stuck in Chicago b/c of a train delay downtown. I have been doing alright though, I think I am so busy and have alot of projects going on that I don't neccessarily have alot of time to think so much.. And of course I have a ton of support and great friends to talk to if or when things do get tough so that helps..I con't to write Steph atleast a couple times a week and I think sometimes she is my biggest help, she just listens with a non-assuming ear and if she has advice shares it others wise just cyber listens. Alot of times we just chat back and forth about life things.

My Primary Doctor is working out a plan for the weight manangement stuff, it does seem these last few weeks that atleast my weight has been stable within a 2-3pd range atleast. But I think her goal for us is to increase my appetite which in turn may help with things like overall health and maybe ability to fight colds (immune system) and
energy. She has talked about switching the Anti-depressant to something that will cause increased appetite but the one
I am on now can I guess be a bear to get off and she is talking about a medicine that is often used I think called Megase but we meet in a week or 2 and will talk more.Right now I am finishing the last day or so of the 2nd stronger Antibiotics.

This past Tuesday as many know I flew up to St.Paul, MN via
a privte Org. called 'Angel Flights' which was very cool, I
loved my Pilot and the Airplane was very awesome. They had just a ton of snow in MN vs here where we had NONE (!!) but
we made it safely although my Drs. there didn't expect me to come apparently b/c of this. I met with my NeuroPsychologist Kendra Bjoraker and we talked about some
school things and some suggestions she had after looking at
some paperwork I had with me given the IEP i'll need. She sat in for part of the Appt. with my Neurologist (whom she
works with) and then I met with Dr.Charnas. I had met him informally at several meetings before but this is the first time we worked together in office w/me as his patient.

It was a good Appt. they are as I kind of knew from Kendra and Dr.Shapiro starting a memory medication that they are studying and are also doing a LP to check for High Pressure Hydrocephalus on the 28th. I am generally feeling a little bit freaked out about this test not the results as I think/know Dr.Charnas knows what he is doing and like him very much + it will be good to have answers.. I am jsut scared of the actual needle and test!!! I can do sedation if I want so that is something I may just be leaning towards but I just don't really know yet, maybe I will just take a relaxant type drug that he talked about.. Kendra has also said she can be there so that will help to have her sit by!

I am reading a really good book by Anne Patchett called 'The Patron Saint of Liars' I haven't gotten very far and haven't had time to read in the past few days but it really is good. Otherwise jsut doing things for school
and fun things like work (haha) but there is the June MPS meetings in Chicago and MN coming up and perhaps a visit to my Cuz which will be fun if we get it worked in!! Then July is the Conference in KY and seeing most of my friends including the Hollands and Miss Amy!!!! Yippee!!

Remember if you haven't 'JOIN THE SEARCH!!!' for MPS/ML Patients if you are one of my Providers!!!!

Love and Hugs and Always Keep the Faith - Spring is a Time for New things and New times! God Bless!

Erica

Monday, February 27, 2006 0:21 AM CST

I had a wonderful Appt. on Thurs with my Genetics team and as well with my Psychologist. Fridays ERT went pretty smoothly also - I seem to be getting fevers about 1/2 way through treatments these days but then they disappear by the end so I won't complain! I had a chance to talk to LuAnn a long while both about weight loss stuff and too about things going on in my life that she is very willing to try to help me with. She is very supportive and a great listener so I am hopefuly we will be able to get back in touch this week so that I can work out a few details with her about some things, i'd really like to be able to sit back down with her for maybe 15-20mins in a few weeks go over a few things that are coming up for Appts. the results
I mean but we will see! She also said she would be happy to
talk to my parents - explain some Insurance things (SSI) and coping type things that she has seen in other parents to them and just try to help Dad with whatever he is dealing with. This in turn with hopefully help get some of it off my back and help all of our stress levels. I really do love my Dad and I know he loves me and we all mean well, but it's hard when I picture my life as one thing and he as another...

I talked to Steph Ginty on Friday - I sure love her, but I do wish she lived closer, that lady is one smart woman full of a mothers knowledge but way to far away! How come Boston and WI have to be so far away??? ;0) Probably b/c she'd feel like she had a 4th kid instead of just her own 3
and I talk her ear off enough I suppose! ~ Amy is my Amy, still as supportive as ever, there to talk when I need her and funny as ever, gotta love her plant stories!!!!! (inside joke, lol) and I am me, jsut way to busy and way to tired. I am working so much this week and still looking for the job thing, cross my fingers I find something, golly
I want out so bad!! I like what I do in some senses but I need to get off my feet and better pay... I so badly wish I
could support myself and move out, I wish oh how I wish I could have my own place these days.. love my parents but wish for my own place..

Well keep all of my friends in your thoughts, I am truelly surrounded by the best group of people anyone could ask for - between my friends and family and providers I am lucky, if I have this disorder and have to feel like this then atleast I get to have the people I do around me for support! ~I LOVE YOU GUYS ALL!!~

LET'S KICK MPS! LOVE,

Erica

PS: This is the Article/Essay I wrote for the Debut of the New Program through the Pharma Drug Co. and the MPS Soc. to
create and raise Awareness among communities and Physicians
so I hope all will enjoy! www.jointhesearch.org

Join The Search / Why Erica Counts: / MPS Society

I count because I am paving a way for parents to understand how their children feel on a day-to-day basis with MPS. I count because I am a voice to MPS. I count because I am a person and not just MPS, and I am a living, free spirit who can become anything she wants to be! I count because I AM Erica Thiel, and I AM NOT defined by my MPS disease!

Favorite Hobbies:
My favorite hobbies include writing and expressing my feelings to others in the hopes of making a difference to the world in some small way, either to fellow MPS patients and families or to some small aspect of our population, and just expressing my feelings in a way that allows me to feel better and keep on living freely without letting MPS become who I am. My very favorite hobbies include talking on the phone, shopping, reading, going to movies with friends and just relaxing!

Proudest Accomplishment:
My proudest accomplishment has been learning to advocate and stick up for myself in the past few years. I used to be a very shy, not outspoken person and believed that if a doctor said something was right then that was the way it really was. I now have learned that I am my own best advocate and that it is okay to speak up for myself, to be my own voice and to stick up for what I believe is right when it comes to my own body and mind. My other proud accomplishments have been my pursuing writing further and speaking. I have done a few things over the past year that have included speaking in front of a group of physicians, patients and parents which I was really proud of and also just recently attended a Lobby Day in Washington to advocate for needs for adult patients with Congenital Heart Disease.

MPS Heroes:
Stephanie Ginty (Genzyme Case Manager), LuAnn Weik (GC) and Theresa Kump (Study Coordinator) because they have always allowed me to express myself whether I am having a bad day or a good day, a great experience or a tough time. These three have always supported me in every step I have taken. When I have wavered into a path that seemed unsafe, they have set me straight again and always, always most importantly listened and guided me. They have been my providers throughout all of this, but more importantly they have been more like friends and mothers guiding and protecting each step and thing I’ve done. / My Sister Sara is also my hero because she has been my best friend throughout my life and has been a supporter of me throughout my trials and all of the times when I have just needed a friend to talk to. She has listened to me, hasn’t treated me any differently and, best of all, I am still her little sister so we argue and fight and go about our life just as if I didn’t have this silly thing called MPS. She is my very best friend and one of my MPS heroes because I know that even when I doubted that there really was something underlying going on, before I got the diagnosis, she was there to push me along and was a voice to say that something just wasn’t right.

Words of Wisdom:
Something that Steph and LuAnn told me and that I have learned myself over the past year. “One day at a time, one week at a time is the best way to take things and especially when you are going through a new diagnosis. Sometimes you just don’t know if you can face another tomorrow but you can always take today for what it is worth and make the very best of all that you have now.” Steph told me, “Remember, life is a journey with a past, present and future but for now all we have is today.” And I firmly believe we should try to make the very best we can with each day we are given!

Personal Diagnosis Experience:
I was diagnosed at the age of 21 yrs of age, just 2 weeks shy of my 22nd Birthday. I spent many years seeing many specialists including dozens of doctors in dozens of different areas of medicine. I had always seen a pediatric cardiologist for Congenital Heart Valve disease but throughout the years, this also progressed into what we now know is the secondary MPS valve disease. I saw a rheumatologist, several neurologists, hand specialists, cardiologists, my primary doctors, internists, orthopedists and a genetics team before I ended up just kind of giving up that there was any one defining diagnosis.

I just ended up switching cardiologists due to worsening heart problems at the age of 21, when the new doctor suspected, again, that something wasn’t quite right. He ran further testing which showed restrictive lung disease along with an abnormal chest wall shape and tachycardia and heart valve disease. I had previously had carpal tunnel releases, and tonsils and adenoids removed, as well as hernia repair. After seeing a pulmonary specialist who ran additional testing, I was referred to the new genetics team at the Children’s Hospital, where I then underwent the testing for MPS diseases.

My ACHD cardiologist had explained to me what he thought was going on but that he wasn’t sure what type it was or even if there was a treatment available, just that he had seen the symptoms before in other patients while he had been in his training. About a month later I received the call from my then genetics counselor that the test results had come back positive and I did, in fact, have MPS I - Scheie syndrome. A week later I met up with the genetics team again and met my new genetics counselor and geneticist and learned all about this weird sounding disease and what we would have to do in order to set up treatment.

About 3 months later I started enzyme replacement therapy (ERT) and now I am here, 9 months later. Although ERT hasn’t been all smooth sailing, I am appreciative to have the treatment and am very grateful to my genetics team for all they have done for me. They have been wonderful and even despite some disagreements and occasional stresses, we work well together and make a good team I think. ERT is a blessing and the diagnosis, although hard to take and accept, has really been such a gift. It has brought so many wonderful things, opportunities and people into my life! I often struggle daily with many challenges and symptoms which sometimes seem unbearable but I also wonder if I could ever give up the great new friends and opportunities I’ve made and had.

The Join the Search campaign is made possible by support from BioMarin Pharmaceutical Inc., Genzyme Corporation and Shire Human Genetic Therapies.

Wednesday, February 15, 2006 3:00 PM CST

** 2/18 - break in update, will update next week again but Genetics Appt. rescheduled for 2/23 at 8:30am***
___________________________________2/15
So it's been a while since i've given a real update - last week I was in Washington - for the ACHA Lobby Day, what an truelly amazing experience! I have never done such a cool thing and felt like I have truelly made a difference on such a "large" level..I mean I think I have done a quite a few things in the past year since being dx'd but this was the most amazing of all!!! My Cardiologist and I where there to represent WI and talk to our Senators and we also had the opportunity to speak to Reps from Illinois including to 1 Senator, 1 Congresswoman and 1 Congressman which was very exciting. We where lobbying to raise awareness for Adults with Congenital Heart disease and the need for funding and better awarenss as well as Funding to start a ACHD Registry to better track adults over time. I also had a little bt of time to see the sites which where awesome and meet some new friends, including my rooomate Faye whom I loved!! It was really hard coming home as I loved my freedom there but I am settling back into the routine here and going about doing new things and new adventures.

I have been able to escape from Doctors Appt. all last week
except for ERT which was such a GREAT break I can't even begin to explain it! Tomorrow (thurs 2/16) I have a Genetics Appt. F/up and Appt. with my Psychologist but I think I can go in very open minded and relaxed b/c I just feel good after having that mental health break from Doctors! Hopefully the Genetics Appt. will go well, my Study Coord. is unable to come b/c she has another Appt. she has to be at which is disapointing as it is lways fun to see her but I know I can always call or email her anytime I need anything. And it will be good to see my GC, we hvn't talked in probably a month and I kind of miss her,
I wish our email worked! Crossing my fingers that the snow stays away tonight and into tomorrow atleast until I get to what I need done there!! Friday is again ERT but it has been going pretty well other than some slight probs with the IV's but it is very nice not worrying about rate problems and jsut being at ease with my Dr.

This past weekend I ended up with a Flu Bug which was nasty as all can be, I couldnt keep anything down and had a real bad fever, chills, headache, etc. Monday I finally ended up calling my PMD and she said to go to the ER where they after many hours ended up donig lots of Lab work and running in Fluids for Dehydration and Anti-Nausea meds but
I did get to come home after 6 or 7 hours and quite an experience. I have found a 4th drug that I am allergic to,
I think it is a given that any time I get IV meds I react these days!UGH!!!! But now thankfully a few days later other than a Headache I am feeling pretty better. The fever finally broke after the Fluids and can keep my Meds and Foods down!

Next week I am talknig before the NHS students at BDHS about a FUndraiser Tiffany (their leader) is wanting to set up so I am both excited and nervous about that. I think it will be fun to talk before a group closer to my own age but nerve wracking bc they are my own age range! The Fundraiser would be for MPS and for next Fall or late Summer so that should be really fun to work on!!!

Well I am off to get something done - should probably get ready for work or write up a list of questions for tomorrow or something but this is more fun! (lots to talk about if you don't update for a long time!)

Hugs and Love from me to all of my Friends, Family and Care
Providers!!!

~*~Erica~*~

***PS: Genetics Appt. rescheduled due to Snow Storm scheduled for 2/23/06****

Sunday, February 12, 2006 9:23 PM CST

Appointment Schedule - back to Life~
~Tuesday 2/14 Physical Medicine Specialist Dr.Heidi Borca
******12pm: Dietician Wendy Balister

-----Thursday 2/16 Genetics Fup w/LuAnn Weik, CGC, Dr.Rhead at CHW
**11am: Psychologist Dr.Brosig

Friday 2/17 ERT infusion #36 (?)
****************************

Wednesday, January 25, 2006 10:10 PM CST

Next Weeks !Crazy! Schedule so If You CAN'T Find me...Cell!

Monday: Work and try to get to the Y to Swim/Work out
Tues: Dr.Borca - Physical Medicine Rehab Specialist, New Dr
@ Columbia St.Mary's Mequon. - all morning. / Night - work

Weds: Meet Study Coordinator initial 3 month Labs
9 AM: 3 Month Genetics Appt. LuAnn and Dr.Rhead till???
~right after, LuAnn and I planning to just talk,ques,etc.
~Meet with Theresa again to complete rest of Study Labs Reports.
~Appt. w/Dr.Brosig sometime in the Day ~Psychologist, aka my sanity (?!)

Thurs: Meet ^Angel^ Flights Pilot at Dodge Cty Airport fly to U of MN for Neurology Appt. with Dr.Charnas. c headaches and memory probs. (small plane, eeek!!)

Friday: ERT w/ Dr.Trepane
~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~
All that said, my life has been chaotice lately!!! One of my good friends just asked me today, "why do you have so many Appts? We have alot of Drs. to, but don't have Appts. all the time.." LOL! Had to laugh at that!! ~ I think it has just been a bad momnth for Appts. really alot has fallen in January/Febr. and all at crazy times it seems. Plus we are trying to cordinate many things, and get a better handle or better assess some of the problems like the Pain Mngmt and where can we go with it, or what are the options besides heavier Meds? So yes, too many Appts. but my Dr. and GC are also pretty good at knowing when/if I am getting to overwhelmed and kind enough to ask my opinion whether or not I want to see certain specialists or when I want things done... And of course many Appts. just happen to have been the 3 or 6 month F/ups these 2 months and it all makes for ALOT when balancing between work, life and staying well enough to function..

~Last week I seen my Psychologist for the 1st time again in over a month and a 1/2 - usually meet up pretty often but bc of being sick, the holidays and other Appts. things where a little messied up! =0) Was definetly good to see/talk to her, a HUGE load off my shoulders jsut to be able to talk to her about things that I know she will listen about and not only try to help but not be directly involved in the way family/friends nearby are... Shed a few tears at that Appt. which I hate to do, but still felt better once I was at the end of the Appt. ~

Thurs. night I called my GC and talked to her for quite a while as well, we email back and forth on and off but not like we used to, something is messed up that doesn't allow her to reply to my email. So that was also an added relief to talk to her, we go from serious subjects to joking around to things we might need to do, just anything or everything. Sometimes one or another of us is a little busy (she is ALWAYS hectically busy (!!) but still totally an Angel) or maybe crabby but we seem to always cheer each other up atleast it seems that way once we get off the phone! She is so supportive and caring I still wonder how I was so lucky to have her as a part of my team!?!? But I won't complain.. =0)

Work is going okay, really hard on my body, and I hate to say this but my pain level, joint stiffness seems only to have gotten worse in the past month not better as I had hoped. :( But I am waiting to hear from someone Dad talked to her Sister about a possible Job and also starting a Warm
Water Program at the local Y.- My sister Sara has even said she wants to do this, so it will be twice as fun with her!! I was teasing LuAnn on the phone last week about their being alot of "older" people and she was like, that'l
be no problem for you, they'll all love you, you'll just have 20 grandmothers and 20 more mothers! (i already have about a 1/2 a dozen of each, lol!!) She was like I'm not sure you'll get the same returns on the friendship but I think you'll like the class. =) I think it will jsut be good to do something that is non-weight bearing but good for my joints and warm water sounds GREAT!!! (only about 10degrees here, lol!)

D.C. trip is coming up fast - if I make it through all of the craziness next week then I am home free and D.C. (and Patty!!) here I come! Have all of the info so far and am so
excited! Booking my flight finally tonight, and have "met" my roomate. She is 28 and from Idaho and born with SHone Syndrome which is a form of CHD. We seem to have alot in common and are both excited. =)

I guess this is all the news from my end..for now.. please keep 2 of my friends in your prayers and one of the cutest
little boys I know as well.
~Andrew Jump (MPS II) is in the Hospital complications.
~ Jenny Klein (ML II) like one of my little sisters
she is battling pneumonia and very sick.
~ Mike Bernhardt (Fabry) Infusion Clinic Buddy but in Hosp.
complications :(

Thank You for continuing to check in on how I am doing and for caring - your love and support means the world! Love,
~ Erica

Saturday, January 14, 2006 3:33 PM CST

This is a journal entry that has been a while in coming...

The New Year rolled off without much of a difference from 2005, but non-the-less I mae it through 2005 and this is a new year with new opportunity..

Just 2 weeks into the New Year and I do have some exciting things coming up already, some things out of the blue but non-the-less that I am very appreciative of and excited to do!!

~Last week my Cardiologist Dr.Earing sent me an email with the details of an ACHA (Adult Cong. Heart As.)Patient Lobby Day being held Febr. 8, in Washington, D.C for patients, families and providers to have the chance to speak to their states represenatives about the ACHA and funding a Registry to meet the unmet needs of our patient population.
~
Dr.E., asked if I would consider going and speaking on behalf on WI ACH pts., ~apparently my big mouth must get me somewhere ~ =0) and speaking. So a month from now i'll be on Capitol Hill doing something I never dreamed i'd get the chance to do, but have always thought would be awesome!!!

This week I had some Appts., in Pulmonary, the Restrictive Lung
Disease remains stable which must be good.. atleast it's not getting worse! Those breathing test(s) sure where ALOT more difficult this time than ever b4 though! Dr.Biller, talked
me into a Flu shot and then a Pneumonia shot to beat the band, such fun! (HAHA) No changes in those meds, and fup in 6 months as long as stays well.

Weight loss has been the big issue so far in 06' although it has been on-gonig for sometime now. I have lost over all about 24 pds since May (not trying) and 9 of those pds have
been since Nov. Dr.Z had me see a Dietician this week, so we have a "plan of action" and see how that works, hopefully by tracking what I eat and purposefully trying to add in more calories I can atleast keep from losing more weight. Alot of the problem is the meds I take, several of them cause decreased appetite, nausea and other fun Side effects, but the Topamax especially can cause weight loss. So i'll see how this goes and trying to surround my non-hungry body with lots and lots of food to hopefully "trick my mind", otherwise i'll just plain be eating even when i'm not hungry which bites! :(

Joint pain remains the other constant, the Docs just don't know what to do with it though.. between my upper spine/neck and my knees/hips i'd swear I live in a 89yrs olds body vrs. my own most days. I wish of anything most, that there was something that could help this, the pain is so extreme and affects so much of my life, I hate it and I hate how much it changes things. I could deal with the rest but why does this have to be so bad???????

Please con't to keep my friends in your thoughts and prayers.. My friend Jenn, her son Tyler who became an MPS ^Angel^ Nov. 1st, 2005, celebrated his 2nd Birthday yesterday and his 1st B-day as an ^Angel^. Please keep Jenn and Kent in your prayers. Please also keep my many other friends in your prayers as we all struggle through the ups and downs of living with MPS.

If you are able Please consider becoming a Member of the National MPS Society - www.mpssociety.org - a great organization.
~ ~ Please also visit ~ www.MPSFORUM.com ~ ~ ~

Friday, December 30, 2005 2:22 AM CST

A last entry before the New Year.. just some reflections on people i've met, friends i've made and some of the changes and experiences i've been through...

2006 definetly snuck up out of the blue, it really seems like just yesterday that I was still trying to find answers to my increasing medical symptoms and life was still what I
call "simple". Alot has definetly went on but even when I want to go back and re-write this chapter of life, I know I wouldnt ever change the people i've met and friends i've made!!
~ I haven't really come to 'terms' with MPS as a "reality" yet but I think it is still a relief to have an answer and even to be lucky enough to have a treatment..Sometimes the ERT is the hardest part about this diagnosis, b/c it's like being thrown in the water with your head just barely above water while trying to find your way through a maze.. the treatment helps and it's effects hopefully are good (!!)..but then there's still that voice inside a person's head that wonders "well if i'm doing this treatment every week then why do my knees still hurt so bad, it hurts to walk and why do I have these headaches still and joint pain, why are some things worse, not better?" .. But I also realize God has a purpose behind every trial he settles before a person and always does everything for a reason..

I am very lucky I think that I get along very well with my medical team - I think we work together pretty well, I mean there's been some bumps in the road but overall I can ask questions or if I need help I can get it. I really like my Specialists and my Primary MD/Genetics Team is very good to. ~Maybe a few to many Appts some weeks and alot of meds to take on a daily basis that is even more complicated on Thurs/Fri., but overall Dr.Zuccaro, LuAnn and Theresa really help me balance things with who I should see, tests, Study stuff and just Support.

~All are very easy to talk to, have the patience of saints and whenever I have had a bad day dealing with MPS or Drs or Life since the dx and meeting LuAnn and Theresa, they have spent much time giving me kind advice and advocating on my behalf.
~ Even through the tough times of setting up ERT, and then these last few months they have con't to help.

I con't to see my Psychologist, Dr.Brosig, which works very nicely, I just started seeing her last Winter and must admit I never thought something like talking to a Therapist could be so helpful. It helps that she has a sense of humor and is willing to look at all sides of a situation - in some cases she has worked with my Genetics Couns. on ERT stuff. I of course just met all besides Dr.Z in 05'. ~ I also started seeing a NeuroPsychologist at the U of MN this past Summer which was amazingly very helpful - I sure never knew there was a reason for some of my quirks! (hard to explain here)

I've also met Steph through Genzyme - she is the Case Manager for our area who helps us with getting our Insurance to approve these Enzyme treatments and talk about 1 sweet Lady!! Shes funny and nice, but yet also very helpful when I have had a rough time and just basically always offers any help she can give. Through Steph I have also met Naomi just last month - and she too is very nice, and fun to talk to. =)

My friends, what would I do without my friends... Amy and the Hollands, Sammie and Tammie, Jenn and Angel Tyler, Andrea and Katiebug, Helen and Family, Chels and Kris, Jenny and Terri, Gabbi and family, Leslie, Sheila, the MPS Society Staff, Denise, MY FAMILY, Marie and so many others I have met - you guys are the Best and sure have helped me in so many ways!! 2005 might be remembered years from now as finally being diagnosed with something but it sure wouldnt have been the same without you guys and I sure am glad we all got to meet!!!

There is no words enough to say for my
family except that they are Wonderful and although we have our times - as do all families, through illness or not, they have helped me through all this change both good and not as wonderful..

Dr.Weinhaus, thanks for just con'ting to be a friend, it's been so MANY years since we've been a Dr/pt. team together but your still the best and great to talk to-and a huge help this
past year in all that has went on..

And last I have had the wonderful opportunities to "meet" my Cousin Mark, to attend the AZ MPS Society COnference in Nov., the U of MN MPS I Pt. mtng in June and also have had the opportunity to learn much about my disorder, meet many new faces and even discover a few new hobbies. (like writing!) =)

My Hopes for 2006 are that Drs con't to be Blessed with the Wonderful knowledge they have that allows them to work towards contd research towards ALL the MPS and ML disorders! May there be Great strides towards Treatments and even better therapies for ALL of these Disorders and better undestanding and knowledge for ALL Doctors and people alike!

My thoughts are with all of my friends this Season and Always who are traveling the days without a beloved Child. God holds these children in his Heavenly Home, but may you find peace and comfort and know we are thinking of you.

Wishing you Love, Hope, Joy and Happiness,
~~In MPS we are together~~
Love, Erica

Tuesday, December 27, 2005 0:51 AM CST

Hope all had a VERY MERRY CHRISTMAS spent with your family!
--------
My siblings and neice and nephew where home on Christmas Eve morning and we kept up our families tradition of opening our gifts to each other. We had a lovely time and I
got all of the gifts I was hoping for including a digital camera and cell phone.

Please con't to keep my Fellow MPS Friends in your thoughts
and if you have the chance visit my friend Katelyn VanGhle
who is in the Hospital being treated for GVHD of the Gut secondary to CBT. Her web site is
www.caringbridge.org/visit/katelynvanghle

ERT infusion con't to go well - this past week was infusion
# 29 and week #3 that we didn't have to restart the IV at all and had no problems whatsoever!! I slept most of this
infusion, which is actually pretty normal for me, but the Prednisone makes it really hard to fall asleep on Thurs nights.

I have been having some tough times as far as struggling with feeling kind of yucky and dealing with MPS as a reality. This past week I guess it kind of hit me hard that instead of knowing exactly what I wanted to do with my life and having a plan I now kind of take things day to day and a week at a time. I guess i've felt kind of lost and a little bit sad for some of the losses but I also realize that this isn't neccessarily the end of my life. I mean ALOT of things have changed that I wanted to do and I am no longer certain of many things but I also have met many wonderful people, made tons of great friends and some of the opportunities like the AZ Conference and writing the
"Ask Erica" column are awesome!!

I guess thats all - always remember that each day is a blessing and no matter how tough one day is, tomorrow offers a new and brighter possiblity! -

Thanks for con'ting to check in!!! Love and Hugs,

Erica

Wednesday, December 21, 2005 2:57 PM CST

Not much News to Reports from my Home except a few new Appts and a REMINDER to remember the REAL reason for this Holiday Season. Please keep in thought my close friends who have lost children this year and who will face the usual Happiness of this Holiday apart from their beloved ^^Angel^^ Sweeties. ~~~

Take a moment to visit a few friends of mine:
Angel ^^Tyler Deskins^^www.caringbridge.org/ky/tylerdeskins
(his mommy is one of my closest friends)
Katelynn MPS IH- www.caringbridge.org/visit/katelynnvanghle

Sammie Slawson MPS IHS- www.caringbridge.org/ca/slawson
Jenny Klein ML III - www.caringbridge.org/mi/jennifer
~~~~~~~~~~~

These are just a few of my many close friends who are with me in the daily fight against MPS/ML and we all hold each other up during times when things are rough.
```

Last week I had Appts in Opthalmology(Eye) and Sleep Clinic
at FMLH and an Appt with my Psychologist at CHW which all went very well. The guy who did my Eye-vision exam at FMLH
his name was ANgel(!!) which I thought was really neat and he was nice kinda cute ;0) I do need glasses now and actually have them hear just need to get a new script and actually wear them now!! lol. My Appt with Dr.Franco in Sleep/Pulmonary went well, although this was just for the SLeep stuff i'll actually see Dr.Biller at CHW for the RLD/Lung stuff and PFT testing in a few weeks. We didn't change any of my sleep meds so i'll remain on the Requip and Neurontin b4 bed and Niferex (iron/mlti vit) with my afternoon meds. I see both of those Drs. in 6 months unless
something should come up b4 then. Psychol. went fine, talked about ERT but moved on to some other things to just basically life stuff.

Went to see the Kirch's over the weekend which was fun although my car kind of had some problems on the way home. Sunday kind of turned in to a really bad day. I was upset and frustrated and tired all of my emotions finally came out. -------------------
I do have to get ahold of my Genetics Counselor this
week and talk to her about somethings that are going on, unfortunately our email doesnt work back and forth to each other which was our best way of "talking" back and forth so
it is much harder for us to chat now. :( I think it will help also when I can talk to LuAnn, as some of the things going on are things that I think she will know what to do about.

My Cuz Mark and I have been chatting back and forth for a couple weeks now, he seems just like my Grandpa and actually I think him and I probably have alot in common too
but anyways he is a really good listener and has great advice. He wrote me back yesterday and at the end of his email, he wrote in "oh by the way, when you are feeling better and up to it, I have some frequent flyer miles so you can come out and visit!" WOW!!!! How exciting is that
and not to mention how nice is that of him and his family to offer!!!!!

Well if I do not get to updaing this b4 I wish you ALL a VERY Happy and Merry CHristmas which I hope is filled with lots of memories and time spent with family and friends! I know I cannot wait to be with my family and for Christmas Eve, I think if I would like to go to the Candle Light Service this year. (usually go to the 5 or 7 kids service)

My thoughts are with Each of you who are going through this Holiday Season without your Dear Child, my prayers are with you.

God's Blessings and Merry Christmas - Love,

Erica

Monday, December 12, 2005 1:01 AM CST

This weekend has marked another sad loss.. to many in to short of a time. :( Mike Bernhardt called me on Saturday and told me the sad news that his brother David had passed away on Friday. David was 44 years old, he and his brother received ERT infusions every other week for Fabry's disease
at the clinic where I receive my ALdurayzyme infusions and I had fondly come to know them as "the brothers". Mike and David where sweet and kind, and truly nice guys and we will definetly miss having David at infusions on his Fridays. Services are Tues. evening in Brookfield for which I will attend, I hope I can provide some kind of support and comfort to Mike and his family.

Fridays ALdurazyme infusion went well - I woke up 3 hours late, I have been feeling very exausted this week and must have either not set my alarm clock right or accidentally shut it off b/c I woke up only when my Nurse called to check where I was. - Dr.Rhead gave the okay to come anyways and despite the infusion starting 3 hours late we managed to finish by 3:30 with only 1 IV stick for once and no bumps in the road along the way! I give kuddos to my Nurse for being such a great person and for being so cool and to my Dr. for letting me come and receive my ERT despite the back-up and am happy that it went well. I came
home and went to sleep around 7:00 and stayed asleep till around 1:00 a.m and then back to bed around 2 until 11:00.

Steph, if your reading this, are you proud of me?? =) I went to sleep for once after ERT!! =) Although I have been so tired this week sleep is about all I feel like doing!

Today we decorated Christmas cookies and my Twin, was outdoors decorating the house and yard. We have been having
snow on an off all week so everyone in my family of course loves sledding (snowmobiles) so they have had those out around the yard as well.

This week I have many Appts going on, I see my PMD on Tues.
for a variety of things, basically to check in but will ask
about Pain Mngmt given the increasing problems in the cold and also about setting up the Care COnference btwn LuAnn, Dr.Rhead, myself and Dr.Zuccaro. Thurs., I see the Eye Dr at FMLH and am kind of nervous about that b/c I know I have
definetly had changes since my last visit and I also see Dr.Franco in Sleep/Pulmonary Clinic. (which may be a good thing) After those I will see Dr.Brosig which is good, I like talking to her and it helps to be able to make a "plan" for each week and for ERT and basically for anything.

I talked to LuAnn on Friday and not sure how we will proceed about the infusion rate stuff, but she does still support me and wanting to stay at 4 hours. She said to me that some pts just handle Aldurazyme better at a little bit longer and if that is what works for me that is what we
should do. She also said we would make sure the other Drs. in Genetics knew that this was the infusion rate that works
for us, as they will be covering for Dr.Rhead while he is on Vacation in the enxt few weeks. She really is great and boy I don't know what i'd do without some of my friends and care providers!!! =)

I am chatting into the wee early morning hours with Sammie and Andrea on YIM and although I should be going to bed I can sleep in semi-long tomorrow b4 I have to go to work. I talked to Jenny Klein a little bit earlier this evening and her Mom, Terri, was so kind to help me with a project I am doing for the girls for Christmas, I am very excited about this and cannot wait for her package to arrive. I will see Helen this weekend so I can give her, her gift then and our plans for Jenny's are still up in the air for after the Holidays but I will end up mailing out some of the girls gifts. Hopefully I will get to see Jenny and Terri in person though!!!

All for now, This entry was Long enough I think?!?!

Thanks for stopping by and checking in on the life of me and living with MPS - although my life is about much more than just MPS, these days it is a big part of who I am and has given me many pleasant things atleast including great friends and opportunities.

Love n Hugs,

Erica

Tuesday, December 6, 2005 4:38 PM CST

19 Days till Christmas!!!~~~~~10 Days till Shopping with
Helen!!

PLEASE KEEP ALL OF MY MPS FAMILY and FRIENDS in YOUR PRAYERS during this Holiday Season and especially those who have lost CHildren to the MPS Fight. Please say an extra prayer tonight for my Friend Sammie Slawson as she leaves Home (Cali) for another round at St.Judes in TN., and that her Drs. and her Mom are able to make the right choices for Sammie's fight against Cancer. Sammie is a brave kiddo nad fighting against both MPS I and Cancer!

MY WISH CAME TRUE, Steph and Mary where able to come out to
the NS Clinic on Friday and we got to spend some time together! I love Steph and it was also great to see Mary! There was anothr guy with them Greg, who also happened to be very cute! ;) It was an otherwise rocky kind of day at ERT - the whole Infusion Rate and IV things continue to be a problem and although I can deal with the IV's for now, it
would be SOO NICE to just be able to stay at the length of Infusion that I am comfortable with! /^o^/ I hardly think it's unreasonable to ask for this one thing and for my Dr. to be able to agree.. and let things be..

But thats that and will write again soon. Thanks for checking in!!! Love and Hugs,

Erica

PS: If you are able please consider making a donation to the NATIONAL MPS SOCIETY (www.mpssociety.org) and help fund
Research for these Diseases and/or a donation to help fund
the MPS Society work.

Tuesday, November 29, 2005 1:09 AM CST

I talked to Steph Ginty today and although she wasn't sure yet - it sounds like her and Mary Yuska may be
able to make it to the NS clinic Friday
which I am very hopeful for!!! I would
so LOVE to see Steph and Mary!

Marie Muniz called me today as well and
I got the chance to talk to her for awhile on the phone to - very cool, must be my lucky day. =) She also has much info for me and is working to gather it all and fwd it to me.

This week it is back to normal ERT schedule - although we are going to slow it down just by 15mins to see if that might help with the tiredness - this means it will be about a 4hour 15min infusion but that beats being SOO TIRED for the entire next few days!!!! - And Marie is fwd'ing that info to me..

It is also my 1st week back to having to see any Drs since b4 the AZ COnfernce - This week I see Dr.Brosig and also since I haven't been to the Hospital since then I have to fill just about all of my 18 million meds. which will take a days work, lol!

Well thanks for checking in... =0)
Erica

Saturday, November 26, 2005 2:21 AM CST

I was just re-reading old everyone's guestbook entries and Thank You All so much for thinking of me and for always being here for me, every one of you, my friends, family, providers, forum family, etc help me in ways you cannot even imagine and even the smallest words or things mean the
most!

Today, I think was WI first actual SNowstorm - I think we have gotten atleast 3 to 4 inches of snow and it's probably still coming down. I like the snow but hate teh cold!! I think it hates me too! =) We ventured out shopping for quite awhile today to Watertown, Jefferson and
Johnson Creek and boy where the roads slick on the way back!

I didn't have ERT today - as there was no Dr. to cover the clinic, but maybe thats a good thing, my little car was all
over the road yesterday just in the wind and I have a really hard time seeing when it is dark to boot! That might
have been a bad, bad situation! It means that next i'll be super exhausted again from missing so much lately and i'll
feel like a zombie for a few days but I guess thats just the way these things go. I did ask Kris (my nurse) if we can try slowing it down just for this one week to see if that helps the tiredness at all, it really does seem to be
only when I miss weeks now or when the rate is faster than
4 hours. A week ago the ALdurazyme took about 4hrs 15mns to get done.

Steph Ginty and I believe Mary Yuska fly in on Friday (as far as I know so far!!) and Steph wasn't 100ositive yet
but said they where going to try to come to the clinic Friday which is Great!!! I so cannot wait to see Steph again, instead of calling her i'll get to see her in person! Maybe i'll have to call Naomi instead! :)AAh only a week!!!! yippee, yippee. yippee!! ANy of you reading this are thinking boy that girl either leads a boring life or she adores that Steph, but you gotta
understand Steph is like the coolest person to talk to and easy as pie to kid around with!!!

I talked to Marie Muniz earlier in the week - she was on vacation but she had given me her cell number and she called me back which was super cool! She is sending me some
info and I am also going to ask her if she can help me with
something else with the Enzyme. I like her to, it must be a
reuirement to work at Genzyme, b/c Steph, Marie and Naomi are all super nice!

I think but am not sure, that LuAnn, my GC is back in her office this next week so I am hopeful I can get ahold of her, hoping we can set up that meeting between her and I and Dr.Z and Dr.Rhead sometime. I also need to ask her about scheduling the Febr Appt. since it has to be 1st week
in Febr. Anyways though.

THanksgiving was good here, although it was sort of divided
neither of my older brothers came home which stinks b/c it's stupid, my neice's 6th Birthday is the 26th and we never get to see her and I don't know its just nice when all of us are together. I mean my parents and sis and twin and I and my grandma had a good time and Mom makes great food so it was good - we where all happy tht we could be together atleast.

I hope everyones Thanksgiving was good and that ya'll are ready for the craziest time of year! Just remeber the true
meaning behind Christmas and never forget what it's really about!! Love Always, for now,

Erica

Tuesday, November 15, 2005 4:33 AM CST

2005 National MPS Society Conference!! 2005 MPS Society!!!!!

I got to meet soo many people where shall I even start?? I never did make it to my Resort room for hours and hours after getting there from the Airport b/c I seen Chels and Helen and Sammie and Leslie and Jenny right away and we all met up! Then I met Tami and hehe made her figure out who I was but she is very smart! Sammie's brother is pretty cute!
I also got to meet Gabbi adn Rei and Nikk and Ronni and her family and the Beg's and Terri Klein, Jenn Deskns, Andrea Van Ghle, Denise Dengel, Patti, Alicia, Steve (again) Barb,
Kris, Adam, Joan, etc! To, to many!!

Stayed up way to long way to many night and am wayyy to exhausted tehse days and sore to beat the band but it was just to much fun! Friday we all went to the Zoo and Friday was the Adult MPS/ML dinner which 10 of us atended which was
fun and then I met back up with my friends. I took a ton of pics which will post as many here as possible very soon!!

I loved meeting Denise Dengel and so many people commented that we could practically be sisters! She is amazing and I loved talking to her and also have pics of us together!

Sat was anotehr sleepless day/night but sure was too much fun!! I did get to meet Steph Ginty's friend Naomi Sullivan
and got pics of us together - she is very pretty and very, very nice! I also met Marie Muniz who works in Marketing at Genzyme, she is 3 years older than I and we talked and talked into the wee hours of Sunday morning - these are all more friends that I now have from Genzyme that I can't wait to see again!! I loved Marie and Naomi!

Kris Klneke asked me if I would run the 'Our Kids Party' on
Sat night which was amazing and fun and energizing and hilarious and tiring, exhausting, butexhilirating all at once! I missed hearing Dr.Neufeld speak but Marie is also going to send em some of her papers! I am so thankful that Kris trusted me to 'host' that party and thankful to the kids that we all had a great time!

I did not get to see Amy Holland this year which makes me so
sad but I will of course still keep in cloooseee touch with
her and cannot wait until the next possible moment when I can possibly see them again! Thinking, thinking!

About 2 or 3 more weeks till Steph Ginty comes to WI I cannot wait, I love her and it has been since June since I have seen her so I am determined to see her and to get pics
of us together this time! She is amazing and nice and a great friend to talk to.

I hope that Marie and I can talk again soon, I cannot wait
to see her again I love her. I know it will be way to long again b4 I see Naomi but atleast we can keep in touch!! =)

Hats off to all my friends fopr making an amazing and memorable weekend and thank you for all the pictures and wonderful amazing memories!!! 9 more months till Cinnci/KY!!

Erica

Thursday, November 10, 2005 0:10 AM CST

10 Hours till we get to the Conference!! The MPS Forum is already really quiet and I am soo excited!!! I am already exhausted b/c I haven't been sleeping that great this week and will get no more than 1 or 2 hours of sleep tonight on the way to Chicago Midway Airport but thats ok cause I am on vacation!!!!!

I have my bags packed, everything is set, meds are in my bag, clothes, makeup, toothbrush, etc. and laptop as well as various other things and even movies for the kiddos in the day care. I'll miss ERT this weel but that's jsut fine with me, I consider that a mini vacation just in itself!

Thurs we check in and register and the opening banquet as well as Syndrome breakout sessions and then free time to spend as we like (swimming i think!!!) and Friday morning is more of the breakout sessions including Dr Emil Kakkis, Dr.Vern Tolo and Dr.Charnas so I am definetly going to those and the afternoon is a cookout at the Phoenix Zoo and
then Friday night is the dinner for us Adults with MPS/ML and ou parents/others not going to the other 2 dinners can
do anything they please.

The kiddos also have a sleepover that my friends want me to come to. (can you just see getting NO sleep on this trip??) and Sat is more breakout sessions and free time, in the afternoon b/c I am not all that interested in the sessions going on i'll work in the day care which should actually be a good time. And of course Sat. night is the Closing Ceremony/Banquet. There is a shopping mall near by
and many other things which many of us are already making
plans to go see and do, I think this will be so much fun and the sun will be great! I am definetly way excited!

More later or when we return!!!!!

Saturday, November 5, 2005 11:51 PM CST

IN TRIBUTE TO MY FRIENDS AND PROVIDERS

Please keep in your prayers my friends Jenn and Kent Deskins and keep them in your thoughts for they are going through the very near recent loss of their precious son Tyler.

^^Tyler Kent Deskins^^
Jan. 13, 2004 - Nov. 1, 2005
^^ Heaven's Dancing Child ^^ ^^

I only hope that I can be half the friend to Jenn that she has been to me and support her during this truly difficult time.

Released a single purple balloon today in memory of Tyler, and although it was such a sad moment I also knew it repressented far more than just a balloon,but more Tyler being free from his earthly pain, and dancing free with his ^^Angels Wings^^.

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

All of my MANY Appts went well on Weds., it was a whirlwind kind of day with one Appt right after another, pretty much straight until 2:00. I was able to get many things done though and
several tests completed and out of the way for Genetics.

I started the morning with Theresa, we did the yucky, ducky lab work, but I guess if I have to do it, than I am glad that atleast she is there to talk
to!! This week was especially really nice to be able to talk as she is a natural at listening and just helping during tough times with great emotional support and as always offered a kind shoulder to lean on.

I feel so lucky that I met her and honestly I do not know what I would have done these last few months if I had not had Theresa's support and often her kind encouragement to keep me going. She I don't think could realize it, as I have probably not ever told her (till I send her to read this) but she is a very important part of my treatment process to me, not so much even treatment just it is very healing to be able to express myself and to know I can call or email her if I have a problem or am feeling sad
or upset about something. She realy is a very kind person and one of the most easily likeable people I could ask to want to know or talk to.

After labs though I then went to Genetics and met up with LuAnn whom is also a very sweet soul and we went over everything that has (or hasn't!) changed in the last 3 months and spent alot of time talking about the headaches and Enzyme rate and she encouraged me to express myself to Dr.Rhead and tell him what I wanted b/c it is what was right for my body. So she brought up the topic to him and then when they came back he
expressed his thought that my problems are anxiety or depresson and it was SO HARD to get him to believe me that I am much more than just a nervous wreck.

He really feels the rate troubles and increased related headaches are in 'my imagination' and this is a major culprit (i don't deny there is anxiety but come on who wouldn't have anxiety when faced with these kinds of decisions and especially with how I feel physically???) so we debated back and forth good naturedly but albeit heatedly for a good long while until he gave in (finally!)With some help from LuAnn we where able to convince him that I am just trying to lead a normal life outside of ERT and not let it define who I am. I do not have these
increased probs b/c of anxiety due to a
faster rate b/c I am "anxious" it doesn't matter that my symptoms aren't neccessarily "in the books" b/c they still alter my life and cause me alot of trouble!!!!

We did finally settle on and agreed that in a few weeks (3 ERT's, 4 weeks) we would probably try going up by 10mins but of course that is if the headaches are doing better on the Topamax.

I don't know I just have to say I am comfortable with that and happy that LuAnn is so supportive of me and she even told Dr.Brosig and Theresa I did an A-Okay job of standing up for myself!!! (i know silly me but right now these things matter to me!!)

I am seeing Dr.Zuccaro this week for a physical so I am going to ask her about if she might be willing to meet with me and Dr. Rhead, LuAnn and Theresa. (Theresa may not be my Study coordinator for long, but I have my fingers crossed that if she has the time or is able she will stay on with me, I love working with her) and since this IS a long term gig, the Enzyme treatment it seems like it would be a good deal if they could all meet and compare notes plus get to know each other and kind of share each others thoughts for where they want to go with things, since all are involved in my primary care. (in different ways, both enzyme and primary/health related)

I know that LuAnn thought it would be a good idea if I had Dr.Z come down to the N.S infusion clinic and sit down with me and Dr.R about the H/A's and ERT so I think with some time, patience and careful planning I could make this work. (crossing fingers)*

I talked to Steph yesterday and although she isn't able to come to the AZ Conference :( which is so sad b/c I miss her terribly (but i am glad she is almost always only a phone call or email away!!!!) she told me is coming to WI for a Fabry pt. meeting in WI next month and is going to try to fly in on Fri so she can come to the clinic!!! How awesome, ooh I hope, I hope, I hope!!! She is so fun to talk to and always always know how to cheer me up or make me laugh!! I haven't
seen her since June in MN. She does have one of her fellow co-workers who is going to AZ on the look-out for me though which I think is so funny!!! :) OOh Steph I am going to make you read this journal so you should be laughing right now!!!

Sorry everyone else!!!!!!!!!!!!!!!
4 more days!!!!!!

Will post a complete Arizone MPS Confernce journal and picture webpage update when we get home next week and will bring home lots of pictures to send to my friends and providers.

Thanks for hanging in with me here and reading this. Thanks for waiting for my long over due update! Remember keep all our MPS families in your prayers and especially Kent and Jenn!

Always and Love, All from me~just for Now,
Erica

Tuesday, November 1, 2005 10:22 PM CST

I know I am long over due for an update and one will come soon I promise for my MPS/ML family and for all my friends and those who stop by to read about my life.

I know it has been weeks since I have updated, I guess life has a way of keeping me busy and sitracted but believe me I have ment to come here and journal more often. I have a Genetics Appt. on Weds Nov. 2, 05' so I promise to return here soon after that and add an update on both that Appt and my Appt that was had on Monday the 31st with Dr.Zuccaro.

I feel confident the Appt on Weds will go well but right now dealing with my own MPS stuff is the very last thing I want to have to face. I hate MPS right now and I hate the fact that I get to move fwd, go to my Appts., go the Conference in AZ next week, and receive ERT when so mny others have become Angels this year from there battles with MPS.

My Heart is heavy and sad today, the tears are endless and the night seems long, my dear buddy ^^Tyler Kent Deskins^^ lost his fight to MPS IH this morning at 6:00 a.m at Cinnci Children's Hospital due to complications from EB and
GVHD 2ndary to his otherwise successful BMT.

His Mom Jenn Deskins has been a close friend of mine since day of 1 of dx and Tyler and I where about a month apart in
our MPS I diagnoses. Jenn will always be a best friend to me and to offer support to her and Kent there website for Tyler is: www.caringbridge.org/ky/tylerdeskins

I sign off with the thought that although I know it's not true, ' life just isn't fair'.

Wednesday, October 12, 2005 0:10 AM CDT

I have this goal unofficially set for myself in the last few months that I won't take more into advance or think on things more than 1 or 2 days ahead of time. (such as ERT) This is typically so that I can just focus on making the most of the time when I do feel well and have the energy when I am not feeling so well to just get through that down period.

Some times I feel like I meet this goal and think that I do pretty well at balancing my self between both the medical things and my otherwise life. Othertimes, like this past few days I find myself worrying in excess about what is uncontrollable to me and/or just thinking alot about the next ERT and/or what is happening that I can't really control.

One thing that I have noticed is that although during the "rough" times I probably drive all of my care providers nuts and feel nuts to my own self, when I do get through the worst of that particular point I feel renewed or more refocused on cont'ing to fight this crazy thing called 'my inner self' and it's determination to let MPS define me. After I struggle I tend to feel like I have an even stronger sense of determination to get through the following "milder" rough days and then to the better "freer" good days.

My hope is the "crisis" pts become fewer and more time between and that by experiencing each problem point, all the different struggles that I faced emotionally or physically (and hopefully tackled atleast fairly well) will help me get past the struggle point of the "new" (or not so new..) part of the dx and I can hopefully come out stronger or atleast a little wiser. -->to better live life>

I con't to believe that MPS isn't who I am, but just a part of me as the whole person and I guess the working goal is to accept that I have it but not let it define me.

---> ~No one said it was easy though did they! <----

~ I believe my inspiration of the week will come from the circle of people around me who stand by me (even when I might be driving us all nuts). And also for anyone (like me) needing a little 'show how' along the way, from a boy named Mattie J.T. Stepanek who despite a short lifetime of struggles and losses con't to find joy and wonderment with life! ~

Love and Hugs to my friends and all who stand beside me (or behind me holding me up somedays!!) Thanks for always reading on. <-----

Tuesday, October 4, 2005 11:58 PM CDT

Nothing to new is going on around here - just alot of the same old same. It is Tuesday night (weds morning) as I type
this and not much going on. I asked out the guy I really liked and we are going to go out Friday night. I do have ERT that day so am crossing my fingers that it will be okay
and that Dr.Rhead will let me stay at my same rate. I really hope that he will not force me to increase as I am scared of feeling worse again and already have a very small reserve of energy for the rest of the day and week ahead. I do not want the headaches to return with a vengeance and also do not want to feel worse. I really wish
that I could make the decision about my treatment - LuAnn had told me this was one area of my care I could control so why do I now feel like I am controlless? She has been extremely busy these days as well so I cannot really talk to her about my concerns. I hate this worry every week!!!

I may be headed to the U of MN on Tues/Weds of this coming week. I do have Appts scheduled for Monday but asked if they could be moved to Weds instead. I will be seeing Neurology, Audiology, Ortho-Hand, Opthalmology and maybe 1
other thing I am forgetting. (not sure) I have not heard from Kendra nor Dr.Shapiro either so I am really unsure about all of it. If you are reading this please say a prayer that I might be able to keep my ERT at a level I feel confortable with and that I will soon beable to talk to my GC about my concerns. Sometimes this life is SO HARD!

All in Good Love, Erica

Friday, September 30, 2005 8:52 PM CDT

I can't even begin to really believe it but today was my 19th Aldurazyme infusion which means net week is #20! Amazing, I jsut can't believe it has been 4 months already since I started! ERT continues to go pretty well - other than the IV's which are always a hassle or headache it seems like I am noticing small changes to my body. Most of the time I can handle my pain better and my joints seem to be loosened up some, my overall manageability of it all seems to have impoved.

Dr.Rhead wants us to increase the infusion rate enxt from from 10-20-40-60-80 to 20-40-80 which I am really nervous about. I couldn't sleep very well in clinic today and I have just felt kind of restless and agitated all day. I think to that it is coming up on the week b4 my period and I always seems to have a harder time dealing with stress and management.

Tonight I am feeling tired and really fatigued, I had been feeling so much better for the last month or so what with sleeping in clinic that this is a coplete shock to my mood and coping reserves. I feel let down like I had gotten through the worst of it and like I could deal with not feeling 100% as long as I felt 95% ok on Fridays, but today I feel about 75% instead. I know it may just be today that I feel this way but I really jsut want to be a normal 22yr old and be out having fun or something instead of getting ready to go to bed at 9:00 after having slept for atleast a 2 hour nap today. Grr. I hate how MPS gives so much - like new, great friends, support,and a sort of inner knowledge, but then in the next breath it takes away little things like feeling good, having energy and feeling like I am doing ok. I want to get off this rollercoaster and just be free for a while to not worry or stress over things and I want to be able to forget about next week and
the infusion and just think about today and tomorrow at most.

I hate it, I love it but I really just want something to be
better, I don't want to hurt and I don't want to worry, I just want someone else to make all the decision now for a little while so that I can just be free from stress and it's clutching binds.

All from the heart of a tired girl,

Erica

Thursday, September 22, 2005 2:53 PM CDT

I guess it is due time I update here although not much going on. I have been working alot lately and although I don't work more than 5-6 hour shifts that still drains my energy level (or lack of!) and so I don't get alot done. My
only days off this week where Monday and then Friday. And imagine parts of Monday where at the Dr and Friday is of course ERT. OOH WELL! I did spend some fun time with my sister Sunday night and then Weds night and we are also going to go out Friday night. We have worked together every other day to except Weds. :) Very cool! ERT is going well, some of the same troubles but I am trying to look at the bright side of it and also not focus is on the medical sides of things so much. I feel bad for how many questions I ask and really think I must drive people nuts sometime! (eek!) I hope I don't though. I also found out today I didn't make it on the BOD elections but maybe next year. I have been feeling kind of down today so was a bummer to find out but just trying to stay positive. All for now,

Erica

Friday, September 16, 2005 5:38 PM CDT

Sorry for not updating sooner, it's not even that I have
been totally busym just tired and distracted I guess! Things are going alright, jsut trying to accept that the IV
stuff is forever and ever and ever and that forever is a long, long time..I think that if I just take it day to day
and infusion to infusion vs thinking of it all, all the time I will be alright. I missed last week infusion so this week was 1st one back and strangely enough I don't feel all completely zonked out tonight, although I did sleep quite a bit of the time today in clinic. I was basically just happy to see my Nurse though, she is great!

We ended up having Dr.Rhead place the IV today, poor Mary tried and tried and tried (she is a sweetheart and so kind)
So Dr.Rhead tried several times and by about his 4th or 5th
attempt and 9th or 10th attempt total they got an IV in and
set. Even Dr.Rhead was apologizing that they had to try so
many times in order to get it in. He commented that my skin
is unusually tough and my veins move around/disapear alot!
I wasn't bull-crapping on that one, I know the IV's are
hard!!!! Well as it turns out that IV ran the entire infusion but was infiltrated w/out any of us knowing (the
machine didn't alarm, and my arm wasn't real sore) so my entire lower arm is HUGE like the end of a Bat! (Slowly absorbing, sooo slooowwlly) (not pretty!!)

Otherwise should be getting the Fall copy of 'Courage' soon
with my article and new colum,n in (yeah i love reading the
newsletter/mag!) and should be getting Kendra's report in which her and Dr.Shapiro are also including the CD of pictures! :) I habven't heard word on those MN Appts. but maybe soon. Have a ton of Appts. on Monday, ugh back to back to back in Milwaukee. Most of them I won't mind though
I see Dr.Cronin (Rehum) and my Psychol. whom I both like and the new Pain Mnmgt Psych as well as Hand Surgeon.

But enough from me, this seems pretty mumbo, jumbo boring..
Love, Erica

Sunday, September 4, 2005 12:25 AM CDT

Well as last week was a pretty busy and somewhat hectic week this week seems like it will be less hectic but also lots going on. Ufortunately mostly just work but hopefully it will go fast.

The Appts. with LuAnn, and all went really well on Thurs. I
really sometimes have to believe myself somewhat lucky to have this great support. I had an enjoyable meeting with LuAnn and we came up with some prelim. ideas for checking into the headaches. I am going to talk to Dr.Zuccaro and she has left a message for Dr.Brosig. Her and Jean are also
going to tuch base on a few things including the Voc'tl Assesm. info that they had talked about. I will see her in Nov. (the 2nd) for the 3 month Genetics-Study F/up. It is a
bit early but b/c of AZ and her schedule. The Appt. with Dr.Franco also went well - she is super nice and very helpful. She has started me on 2 new meds - one is for Anemia (a prescription Iron pill) and the other is for the RLS-Requip. I aso met with Jean Hudlett and she gave me some info she found and also helped me with the SSI Appeal
form. They all told me to feel free to contact them with any questions or concerns.

Fridays ERT was not all that exciting - b/c I SLEPT THE ENTIRE time except maybe an hour. I did meet a very nice, loveally new nurse that will be helping Mary out. Kris is
all for a Port to and it seems she will hlep me fight for one. I already love her and of course Mary is already very,
very cool and my favorite!! ----- We had so many problems with the IV's on Friday, they infiltrated and blew the vein
3x's in a matter of 4 or so hours! Can you see why we all think I need a Port? They are tellin me that if we don't do
someting soon they will have to start using veins in my feet, and that will stink. I have left messages for LuAnn and for Theresa and will F/up on this throughout the week. I need everyone I can get to help me fight. Mary and Kris and Dr.Bick too it seems all have said they will help.

I got an email from Kendra on Friday (Hope and Amy believe that Kendra and Dr.Shapiro have taken me under their wings and are helping me) she is looking into Hotels and RMH for me and also scheduling Appts. for me. How nice is that cnsidering I know she is always swamped???

Can you fell the love in the air?? :) lol. :) Well all from me. Blessings to all. Love Always,

Erica

Wednesday, August 31, 2005 11:26 PM CDT

Monday August 29th 2005:

NeuroPsychologist: Dr.Kendra Bjoraker, Dr. Elsa Shapiro 9:00 @ U of MN
Cardiologist: Dr. Liz Braunlin 3:30 U of MN

Thursday Sept 1st:

Meet with LuAnn Weik, GC @ 9:00 at CHW Genetics
10:00 - FMLH Sleep Clinic Eval Dr.Rose Franco
10:30-11:00ish meet with Jean Hudlett Social Worker CHW

It has been a bit of a busy week but I am happy and it seems that I am feeling well emotionally. I find that I am
happiest when I am out and about or even at work. The Appts. on Monday at the U went really well and I really like those Drs. Kendra and Elsa con't to help me a great deal and are constantly looking into ways to help and guide me with all of the MPS and especially memory related
things. I met the Cardiologist there and also liked her. She knows Dr.Earing and that is great. We both think highly of his skill. I then went to LaCrosse and stayed with the Kirch family. What can I say except so completely nice nad kind and welcoming - I love this family so much already. I am so lucky for all of the nice people I have met.

Tomorrow - Thurs I am meeting with my Genetics Couns.
to talk to her about a few things including the Headaches
and how to better manage them. I will fill her in on the earlier in the week Appts. as well. LuAnn is very nice and I am hopeful we can come up with some good things. I think she will help me and it is nice to have her support. After
that I meet with the Sleep Specialist Dr.Franco and then Jean Hudlett the S.W whom I met at the last Genetics Appt. a few weeks ago is meeting with me to answer some questions
I have. This is so nice of her, I am thankful to have her.

Friday of course is ERT, I have been trying to not think of
it as I am both nervous and as always lately not really looking fwd to it. I hate feeling so yucky afterwards. Steph Ginty and I have been keeping in touch via email and
hte occasional phone call and I always look fwd to her emails. We may be far away and not know each other very long but we already have discovered many similarities and
things we hve in common. Her emails are always humorous and
I cna always count on atleast smiling when talking to her.

Sara and I worked together tonight - we all had a pretty darn good time. We where laughing and joking around the entire night and even chasing each other with squirt bottles. I made like a bunch of messes but no one gets mad at me and that is so nice! I never mean to do these things on purpose so when we can all laugh and they tease me it is
all okay. Always good for a good laugh really!

Well I have to run and see if I cna get my email to let me
send out messages, it isn't cooperating at the moment and I
want to write! I do love my new laptop thoguh!!!

Lots of love,

Erica

Wednesday, August 24, 2005 10:42 AM CDT

I am sorry I have not updated sooner - there really is no new news on my edge of the line and I just have been taking
it easy. Trying to not do to much but still trying to get things accomplished. I have been trying to keep up on the Forum and have visited a few new webpages.

I spent last night working on SSI Appeals stuff and am jsut about ready to do the online parts of it. I am still not sure if I am going to ask the SW to help me, but I am thinking maybe it couldn't hurt and maybe she would be willing. I haven't hear back from her or my GC on the Vocational Assesment stuff and I am kind of anxious to see what it will entail. I also have not heard anything about the IV stuff, study info or Appt stuff.

I seen my Psychologist on Monday and we talked about seeing
the other new Pain Psych and about if I should just transition to seeing this new one. I am not sure and I have
a few weeks to think about it. My Psych is out of the office for a couple of weeks and I don't see the new one until Mid-Sept. I am just not sure what I will do, I like mine now and I feel comfortable talking to her. Yes, we have made a ton of progress and I feel like I am coping and
handling some of the disease process things better, but it
is still hard to start all over talking to someone else about how you feel and all.

This next week I am going to the U of MN for a couple of Appts. and I am hoping I can get ahold of Hope Madsen as I
would love to see her and her family. I miss Bubbles and their cat too! I will be curious to see what we come up with out of the NeuroPsych Appt. I think it will be good and helpful, but always curious to see.

Well all for now, talk soon.

Erica

Thursday, August 18, 2005 1:31 PM CDT

This is long and not how I would normally post an update, but I think this email that I sent to a very favorite person touches nicely on how I am feeling and how I feel after my Appts this week. I feel like a thousand pund boulder has been taken off my shoulders and I feel like I am free to share and to be me. I have learned alot in just this week and lifelong worth of information and insight in just short months. I am glad that I have the support that I
do.

------------------------
Thank you so much for writing, your emails mean the world to me and although I have been doing beter, it still helps to talk to someone who listens but who is also on the medical side of things. (if that makes any sense) I had a whole bunch of Appts yesterday and all went fine, I am happy with them. I seen the Pain Clinic for the 1st time and although we are keeping my pain meds the same we are going to work on some other aspects of coping with it. (PT, to learn exercises, and Pain Psychologist) and we will reasses in a few months time.

The Genetics Appt went fine, and although I was not thrilled with my Genetics Dr. I spent alot of time with my Genetics Counselor (whom I think actually fights for me, listens better) and I got every single one of my questions and concerns addressed. We talked about the Headaches, which we are going to closely monitor, I will keep a diary for this pt. and if they become worse I am
to let her know ASAP so that she may decide what we should do. She thinks they are more like a Migraine than MPS related. (which would be a good thing) Although my Dr wasn't too concerned about them, she is and that helps to know that she is there for me, and that she does think they are happenning just to often. I also talked to her about the Port and guess what? When I explained all
of the difficulties we where having with the IV's she said she will talk to my Dr., and although she isn't exactly for one, she thinks that something needs to be done in the face of that. I know that at this pt if anyone can do it, it will be LuAnn and I am thrilled that she understands. (Can you tell I am just happy from the results of this Appt?)

She and the Social Worker also supported my decision to appeal for the SSI and are also going to help me with some Vocational Rehab (finding jobs that will
work, in face of this disorder) and I will be excited to hear from them on that. I think it is something that will help and like e/t else I am thrilled that they realize what effects this does/will have on the future and want to
help. It is great. Let's see she had also wanted to talk in person about the MPS Society involvement and so I explained my desire. And you know what I realized when I talked to her? B/c I had explained that since I am unable to work as a Nursing Assistant I miss that challenge, the reward and the helping feeling, it suddenly (seriously) hit me that being involved with the Society is/ has already given me back some of that sense of accomplishement that I had been missing greatly and my spirits are up b/c I am helping someone else.

I told her that being involved didn't have anything to with the MPS for me, which really, honestly it doesn't, it just offers a reward of being helpful and being creative and in the running yes it does help me with the MPS but I
think more indirectly and that is good. I think it is good to learn along the way, but ultimately it isn't about me, and wanting it to be all about me, it's definetly not (which after explaining all this she thoguht it was great too just as long as it doesn't consume me) it's jsut that sense of having a purpose that I don't get from my job anymore. I woke up today and althoguh I
have to go to work, you know what? I am going to start searching for a different job (she suggested a office setting would be better, less on my feet which may help pain, which may help other things, like a cycle. And I am
excited to have a mission and to have something to go for. (new job) Although I have been happier lately, this is the first day I have woken up realize I am learning to appreciate the small successes (like a really good Appt) and looking fwd. Although I am only looking a few days ahead, which I think is better, I also in the back of my mind am looking at the things that I want to do, ask and accomplish in the next week and then after that, the week after. I know I will have the valleys and hills yet, but right now I am going to appreciate the happiness I have and feel. There is just so much that went on at this appt. but every single thing about it (well except maybe Dr.Rhead, but he really is there for the medical parts, which is fine some people just don't have the ability to really be able to understand the emotional sides) was
geared at helping me manage the MPS, get the right help, and just anything I needed. Each time I see LuAnn I have a little bit more respect for her, and if there is anyone I would choose to see every 3 months I think it would be her, b/c each time I walk away even more amazed and respectful of her abitlities. She really wants to help and I am thankful for that.

Anyways so thanks for listening it justs feel so great to have this and to even be able to write a really positive (i think) email to everyone I care for and appreciate who are involved. I could never repay e/t everyone does for me, but I sure will try some day!
Love,

Erica

Thursday, August 11, 2005 1:13 PM CDT

Monday Aug 15, 2005 - 10:00 Meet Research Coordinator for Study-Lab work.
11:00 - Psychologist CHW
11:50 - FMLH Hand Center - Dr.Matloub/Diane

Tuesday Aug 16, 2005 - 10:00 Methacholine challenge PFT 4 Asthma CHW
1:00 - Pain Management Clinic FMLH
3:30 - Genetics Clinic LuAnn Weik/Dr.Rhead 3 mth Study F/up

Hi All, Sorry I have not updated in awhile. Haven't been feeling that great this week either from the heart monitor removal or from headaches, stomach nausea. Not a wonderful mix I might add!!! Well, I found out on Friday I was approved for hte Laptop and in fact just ordered it today.
I was going to go with a Dell, but was having osme problems so I ordered a Compag Presario notebook instead. I am excited about this.

I haven't been up to much this week just had the monitor removed on Monday so feeling sore and the only things I have done are sleep, read and work! I am hoooked on a new author LuAnne Rice, she writes mysteries and they are really good! As you can see above I have muchos Appts next week. I can't believe it has already been 3 months since the last Genetics Appt, my how time flies! This also means that I am on like week 12 or 13 of ERT and last week after we slowed the infusion rate back down I felt much better and no reaction! 2 more weeks till I am going to the U of MN, I am excited. I really love my Drs here, but I
also really am touched by the caring of one of the Drs. I have met there. It is a good thing I think to have great support all over.

Well all for now, love and hugs, Erica

Friday, August 5, 2005 2:12 PM CDT

Almost done with ERT (#12 I think) and feeling ok, albeit a little tired - benadryl tired though. And I took a nap last night which was a good thing since I woke up today at 5 AM
in pain, so I couldn't sleep. It's been an alright day though, kind of slow, kind of boring but ok. I talked to Steph Ginty on the phone, she is so nice. My friend Kara came with me today, she likes coming so thats cool!

I guess nothing else much going on, it really had been a totally laid back week and I really haven't done anything. I
should find out tonight about the laptop and I am excited about that. Also Kris thinks that the booklet idea will go through and I am hoping it will! It would be fun!!

Larry Kirch is going to get ahold of me on Monday about planning some sort of WI MPS/ML get together maybe for Fall,
which also would be great. I've only met 1 WI family and talked to him so that'd be great.

Otherwise I really am just taking it day by day and trying not to get ahead of myself or off what I can handle. I've been setting small goals and those are good too, something to build up on.

Thanks for coming by and reading this!!

Erica

Wednesday, August 3, 2005 10:33 AM CDT

Well, I don't know, (my favorite new phrase it seems is "I just don't know" I think I am some where in between happy and a little sad. I don't know why sad, I guess just b/c of trying to cope and deal and live with the fact that all of this might never be perfect. It scares me really that is so little know about MPS I and especially 'Scheie' form.
But at the same time I am looking fwd to trying new things and on Friday I get to meet a new guy with Fabry. I have no
idea how old he is or anything, but it's always fun to meet
a new person. I guess I am mildly excited to for other things. I don't know what exactly but I won't complain with feeling a little better on that end.

Saturday, July 30, 2005 5:17 PM CDT

I finished off ERT # 11 yesterday and all seemed to go well. I am con'ting to have a big problem with being overly tired the last 2 weeks. I usually don't feel to badly tired until maybe a 1/2 hour before to a 1/2 hour after ERT is over and then for the entire next few days. I slept for about a 1/2 hour in clinic and then as soon as I got home, I slept from 4:00pm until 9:30 this morning. The only reason I got up was to go to work which dragged on by. I have been con'ting to wake up with headaches and today felt some form of difficulty breathing. Very weird. I hope it doesnt stay like this long term, for a while I was doing good.

I sent in all of the forms for the laptop to hte MPS Society and may have an answer as soon as
the end of next week, which is exciting. I talked to Kendra Bjoraker for almost a 1/2 hour yesterday and that was nice, she is very, very nice and totally helpful.

Otherwise I haven't done alot of anything yesterday or today, just sleep and then wanting to sleep. This coming week I have several appts including EMG's, I guess it is good that I have these since my hands are totally bothersome but this is one test that really hurts and I think it will not be great. I will be happy when it is over.

I will keep all posted if anything comes out of this being tired and feeling yucky from the breathing and headache stuff.
All my Love,

Erica

Saturday, July 23, 2005 5:00 PM CDT

Thanks everyone for keeping me in your thoughts and prayers
it always helps knowing that I always have someone to talk to and even people who understand! I just love that many of
you get it. Those of you who come to support me who aren't directly affected by MPS keep me real and are always willing to share your kind words and great wisdom which is awesome and I really, really love all of you.

It sounds like instead of meeting with my Genetic Counselor
in the next few weeks we are going to plan on adding this in as one of the things we want to do at the next Appt in Aug. She is gonig to help me pla, organize and just in general deal with handling all of the Appts, tests, and medical stuff. Basically she is just going to help me to deal with all of the responsibility and figure out a way we
can communicate or that she can help me with all this.

It has been so hard realizing that I am basically coping with knowing that I am different than I was even in Febr and that I have left behind the person I used to be. I don't mean that as a bad thing just that it has been hard and that I realize now that my life will be lived differently than I had thought or expected then. My parents
support that what I want now is to be involved as possible with the MPS Society but my Psychologist whom I really do like is incouraging me to also live a normal life outside of MPS.

I know that I am Erica and not MPS but I still find it helps to be involved and that I do really enjoy this. My very best friends (besides my sister) are in-or through the MPS Society and right now I love it. Since I have to have this aweful, complicated disorder than I am very glad to have met so many wonderful people.

I hope through my column or through my writing (which I hope to con't to do and maybe eventually expand to try to write something bigger, maybe??) but I don't know really where to start or who to send it to... I wonder I think I am going to look into this and ask around.

So although I am having some days of feeling up and some days of feeling down and have many reservations about all of this, I think maybe possibly that I have made it through the worst of this episode of deep depression (or whatever it was) and am resurfacing as myself. I know I still am a little quieter and take a little more to laugh, but I think each day is a new day and I can only try to look up.

Thanks for being great friends. Love,

Erica

Wednesday, July 20, 2005 0:37 AM CDT

Even amongst still feeling pretty sad and really, really tired (I haven't slept in for like a week!) I was excited, I had the chance to talk to my very favorite Amy Holland (ok, you ALL are my favorites, I just love each of you for different reasons and you each help me in your own individual ways) tonight and we must have talked for over an hour! We talked about everything from Camp, to dealing with all of this and wanting to scream, to just plain laughing, b/c that girl is funny! Seriously though even with feeling down these last few weeks I am so happy I got to talk to everyone I did. All of the lovely emails giving support and advice, the lovely phone conversations (with Amy, Kendra) and even just knowing that I have so much support helps, Alot.

Mom and I spent some time together tonight, that was super nice and some things we talked about really made me think. I guess I am not very supportive of myself and although I love helping anyone I can, I also need to be supportive of myself and believe in what I want. She just wants me to be
happy,and not because it is something, someone else wants of me, but because it is what I want. Does that make sense? When Mom said this to me, I was amazed and yet I completely got what she ment. She also told me that if what I want is to be involved and to help others I should do it, but only if it is what makes me happy. And you know what? Being a part does make me happy, I don't always know how to make myself feel better but I do love helping others and maybe I can make a difference for someone else and indirectly help myself. I hope I can.

Amy and I talked so honestly tonight about all of these emotions (after I had talked to Mom) and I felt like, although I don't exactly know how to explain my feelings, I also don't always have too. Some times people just want to help, to listen and to give a shoulder to lean on. I think each day is a learning experience and we must take each day, the good and the bad and learn from it. In a very sweet email I received a few weeks ago from my Genzyme CM she told me "sometimes people don't know what to say, sometimes there isn't anything anyone can say. When you are sad, be sad, when you are angry be angry, but when you are happy then be sure and enjoy it" It isn't until now, tonight, that I realize exactly what she is saying and that I appreciate even more her kind words and thoughts.

I was thinking earlier today, I would be lost if I didnt know the MPS Society and if I hadn't found my friends, what would I ever have done without modern day technology? It is email and phone and writing that allow expression and
that allow support, camaraderie, friendhsips and in the case of MPS, when there are so few of us, it allows a chance to share knowledge, help one another and have family all over the world!

Just by being able to sit down to make a phone call or type out an email or find new support I can connect with anyone at anytime. I can write any number of my team of friends, support and medical providers when I need help and know that someone will always help me. Although I hate that MPS is going to affect aeverything I do, I also know that it will bring about alot of great opportunities. I just really do hope it isn't always this rocky of a road. Strength through Strife.

Thank you so much for all of you who read this, remember you will always matter to me and that we ALL always hope we can make a difference.

Love, Erica

Monday, July 18, 2005 9:49 PM CDT

I got the sweetest call this morning from the NeuroPysch Dr. I have gotten to know from the U of MN - I was originally going to go there this week for some Appts but due to everything being so crazy in my life with feeling down and all, I asked to reschedule. Well anyways she called me this morning just to see how I was and to say she was worried. She must have spent atleast a half an hour on the phone with me and gave me a bunch of reccomendations. She also asked if I would want her to talk to my Therapist, which was kind. She knows WAY more about all MPS stuff than I do. I was so touched by her taking the time to call and to talk to me, that always helps!
(She is one of my the easiest people I know to talk to, she really gets it and understands/cares about how I feel)

I just talked to Leslie (a friend from the Forum) tonight and she asked me if I would mind if she nominated me for the
Board of Directors of the MPS Society and had talked to Kris Klenke (who is on the BOD) to make sure I would qualify. How sweet is that? Even if I didnt make it, to know I have the great friends and that they think I could make a difference is awesome!

After talking to my Psychologist, my Genetics team, Kendra and another MPS Mom/friend who all thought it was best to go back to a Psychiatrist for a different trial of a med, I called again and actually was able to get ahold of my own Dr! He called me back tonight and is seeing me Thurs before I see Dr.Brosig. A good thing.

I still have alot of emotion up/down swings going on and honestly sometimes it is very scary so I am happy that I have that set and that I got the chance to talk to Kendra. Atleast I don't feel like I am not normal anymore and that what I have been feeling doesnt make me crazy. (she says it doesnt, so I must be ok)

I hate this struggle and I hate that it is always going to be there, I hate that every decision I make in life might be affected by or bring up these down emotions just b/c of a 3 letter complicated disease, but I am hopeful I will eventually get to accepting it and that it will get easier.

I just wish it didnt affect my ability to do so many things and I wish I could believe in myself. I have so many wishes, but I guess I must take each day one step at a time right? Whether right or wrong my decisions affect me and I can only try to look forward.

As hard as it is, I am thankful for each and everyone one of you reading this and I know I am never alone in my struggles.

Love and God Bless,

Erica

Saturday, July 16, 2005 9:09 AM CDT

It is Sat and it has been another of those crazy weeks, as evidenced by my very down journal entries and to my friends
who have been supporting me, trying to help me feel better.

I seen my Psychologist on Thurs and even though for the first time ever I cried in front of her, it was also sort of therapeutic and it always feels good to talk to her. I seen my Pulm and although that Appt took forever and a day it did go well and we switched up to another sleep med to try. B/c of that Appt going so far over, I didnt end up getting to have lunch with Treance though, which wasnt cool
I was so looking fwd to that! But we are going to try for this week.

Yesterday I had my 9th ERT infusion and all cont's to go well. I always like seeing Mary and the guys so it is sad that Mary is gonig to be on vacation for the next 4 weeks. I really like Monica our other nurse but no one beats Mary.

I spent last night at my sister's which was fun, we just watched a movie and hung out and I got to see my kitty. I am still pretty down and unfortunately this time is taking ALOT longer to feel happier, it is all really getting to me
and I especially don't really know how to talk about it. I can write my feelings here and I can email with my MPS friends but ultimately I just wish there where someone right close by that I could talk to and be-friend who was going through some of the same feelings and hurt that I feel. I really, really, love my friends and family, but I also could so use someone close by who understands some of the struggles of coping and I think I need to find that. Something for me to think about..

I guess I better get going to take a shower, I really hope one of these days I can post a happpier note. Thanks for stoppig by!!

Always Love,

Erica

Wednesday, July 13, 2005 10:53 PM CDT

Not that I ever would but I feel like I could just jump off of a bridge and it wouldnt matter - I am so sad and angry and tired and frustrated with my life and with trying
to remain positive when in fact inside I am strugglnig so much to cope. I just want to be normal, but right now normal
seems like the elusive, unattainable vision at the end of a
long, tumultous journey that I will quite possibly never get
too.

Inside I hurt so badly it feels like it could kill me, it hurts to even walk, it hurts to do anything - there is no relief and I just don't know how to get better. I try to find help, but everyone is so busy and I just have to go this alone. What an impossible, horrible, aweful struggle. I cry so much lately, I don't feel like I should be able to
cry ever, anymore for along time. My eyes are puffy, I feel like my smile is fake and fleeting and above all I can't get
past this hurt b/c it seems like there is nothing. Am I doing this all for nothing? Why do I even bother to get out
of bed? Why do I have to care so much and why do I have to
feel like this?

I am so angry, angry, angry I could scream.... what good would that do for me though? nothing... how and why and when
does this get any better?

sadly feeling down,

Erica

Tuesday, July 12, 2005 5:17 PM CDT

I've got NEWS!!! Laurie from the MPS Society called me yesterday and I got a scholarship to the 2005 AZ Conference! I am so excited.

I think I have finished off the questions and answers for the Fall Edition of the 'Courage' newsletter - which is cool but at the same time it was fun to write..

I did a probably bad thing today - I skipped both of my Drs
Appts and left messages to reschedule. I feel bad but at the same time I don't think that with the kind of morning I
had I could of made it through those Appts. I talked to
Julie Leighton on the phone for over an hour last night - her daughter Christina had MPS I - Julie is SO NICE!

Otherwise nothing new here - still gonig back and forth between feeling happy and feeling down, so I know I really
should have went to the one Appt for sure today, so it is pretty much my fault that I even feel down low. My kitty
Salem is sitting underneath my chair right now, what a funny furball he is and so adorable and feisty.

Have 2 other Appts and ERT left this week and 2 days off work, how exciting..lol.. not! I am having lunch with Trenace on Thurs after the Appts I think that will be pretty cool.

All for now, Love,

Erica

Saturday, July 9, 2005 5:24 PM CDT

Tues. July 12, 2005 - 2 Appts at FMLH (Psychiatrist, Gyn)
Thurs. 7/14 - Dr.Brosig - CHW 10:00,
Dr.Biller (Pulm) CHW 11:30, Lunch w/Trenace (ERT nurse)

I have been so bad about keeping up this journal, I am soo sorry! Each day I have thought I really need to come on here and talk about this week! I have been feeling happier the past few days, (knock on wood) ERT went well and I love
my infusion nurse Mary, My GC is working on a referral for me to a Pain clinic and overall I just got a few days break
from Appts which was great! (other than ERT) I have started
a new project for the MPS Society newsletter (Q & A called "Ask Erica") and have ques. from my fellow MPS'ers on the Forum and have one answered already! Which I will post on here soon. My MPS friends and family have rallied around me when I was feeling so down and it was so great to
know that I have friends who understand how I am feeling and that even appreciate when I talk about it! B/c indirectly it helps the parents to help their kids. How cool!

I think I may be having my first "side effect" from ERT, I
have felt extremely itchy the last few days and especially today a day after ERT. it is crazy, I am going to have to ask Theresa or LuAnn on Monday about it. Maybe there is something I can do for it! I was thinking of calling today but I don't want to have my Geneticist paged, let them have
a weekend off and let me be away from them. (Not that I don't like em' just a break is good)

Theresa sent me some way cool info she wrote for the Study
protocol and my binder is quickly filling up with info on MPS I and stuff from providers.

I am sure going to miss my Amy Holland this week, she is gone at Camp with Laynie (Amy is a cabin counselor!) and won't be back till next Sat. But I hope she has fun and gets to relax some. I talked to Leslie Philips on the phone
last night which was way cool, she is very nice and I can't
wait to meet her and the rest of my friends from the Forum and from the MPS Society.

Next week the 20th I think I am going back to the U of MN
to f/up on the NeuroPsych testing - which will be good, Kendra is very, very nice and I will see a few others which
will be ok. I wouldnt mind running into Dustin (who works with her) he is very, very cute!

All for now, will try to update again soon. Thanks for reading!
Love,

Erica

Tuesday, July 5, 2005 12:50 AM CDT

Although I am not really feeling super better today either pain wise or emotion wise I am trying to be optimistic. I hate being sad, and although I am I don't want other people
to be able to notice it so easily. I will have to put on an
appearance until I really do feel better. I know that sounds dumb, just be honest with people, but I don't know..

Otherwise nothing to special this week - just hanging aroun
doing the basic of basic things and otherwise trying to take it easy.

My friend Jenn who's son just had a BMT for MPS IH last month is moving out - they have an aprt and are staying there for the rest of the time they have to be in Ohio, how
exciting for her and Tyler!!

I received an email from Dr. Emil Kakkis today who was the original investigator/researcher into Aldurazyme - he sure seems like a nice guy! I am going to be going to MN in 2 weeks to visit my friends, so I am going to meet with Dr.Kendra Bjorakcer to go over my results from the Q.O.L. and neuropsych eval. She is very nice so that will be ok. I can't wait to see Hope and family also!

Otherwise I just need a vacation from my life, it is too crazy these days! I wish I just had more control and that it didn't have me so down. All for now,

Love, Erica

Sunday, July 3, 2005 11:49 PM CDT

I wish that I could write that I am having a better last few
days or even a better day to day but that would not really
be the truth. I feel like a giant disapointment right now and like I just am irresponsible and stupid. I feel like everyday lately is a bad day and like I will never get to be
happy. I hate MPS and I hate how I feel. I am tired of hurting and tired of being a burden to my family, and of them having to help me. I just hate my life right now.

I want to wake up on July 4th and be somebody else, I don't want to exist as Erica and I don't want to hurt anymore or be afraid of the tomorrows.

I had ERT #7 on Friday and although I haven't had any side
effects or adverse reactions to the Aldurazyme I am sad and
frustrated with having so many problems with the IV's and I
wish I could make it so my Dr understood my side of having
to deal with numerous IV pokes and bruised, hurting hands for the entire week after, only to face it all over again each week. Why does it not matter how I feel and how it is to me??? Isn't it my body that has to be stuck so many aweful times?? I wish I could stick him for every time I get stuck each week. I really like this Dr all the rest of the time, but I am very angry and upset about this refusal to consider my side...

I just don't know, I am tired of hurting, I am tired of Drs
and I am tired of MPS running my life. I want to have some
control and right now I don't feel like I do.

Sorry this is so depressing, just very frustrated with life right now. I know I am soo lucky to have so much great family and friends, but I still feel alone right now.

Erica

Thursday, June 30, 2005 10:52 PM CDT

I honestly don't think I have had this bad of a week for a long, long time.. atleast certainly not since being diagnosed. Which seems weird to me it seems like I would have had a tougher time then vs now... I don't know..

I had the chance to have as my Mom called it "A Marathon" phone conversation with my wonderful "2nd mother" Amy Holland today. I always feel better after I talk to her and
I sure hope she gets some relief from feeling bad and from having to defend poor Laynie and how she feels. I don't think Drs always get how we feel or really understand that we as the patients and parents know what is best some of the times too... Please say a prayer for them!

I have been feeling pretty badly phydicslly this week, very
achy, super tired, adn extremely depressed with not noticing
any difference and in fact feeling worse even though I am into my 7th week on the ERT. I have also been having some new symptoms, including a slight audible or noticeable decrease in hearing ability (I struggle more to hear the same) if that makes sense. I also have had terrible vision in regards to adjusting from light to dark and cannot tell you how many doors I have walked into this week alone. I look like I beat myself up between the IV bruises and bruises from walking into things. I have also been having some slight headaches, very weird. Why all of this all of a
sudden??

Otherwise Fri I am off to have my 7th infusion of Enzyme and to hope that I can feel better soon, I sure am tired of strugglng like this and off feeling badly. I don't want to be sad, I just want to be happy again. Why am I mourning the self I used to be, it is soo hard to understand it..

All for the night, Love,

Erica

Tuesday, June 28, 2005 7:56 PM CDT

Yesterday was another one of those days where I went between
feeling extremely sad and kind of angry. Angry and sad not at the world but at MPS and having to deal with it and the many changes it has brought into my life since Febr. By last
night I was in tears and my poor family was speechless, sometimes though I don't always know how to explain it to anyone else. I did call Hope Madsen (she is a dear) and talked to her for quite a while which always helps. I had emailed Kendra and Steph (NeuroPsych,Genzyme) and woke up this morning to wonderfullly uplifting messages from them which helped - their perspectives where nice and really nice that both are willing to try to help! I guess I must be
lucky in some ways if not in the way of health then in the
fact of everyone who has and does help me!

Today I still feel kind of down but not so much as yesterday
more or less just very tired today and that too sort of gets
me down. I guess I was hoping by now that I would be noticing some sort of difference from the ERT and I haven't
yet, so it makes it all that much harder to deal with. This and the fact that I am a bit leery of the IV's this week after last weeks struggles to get one to stay. Grr!

I did talk to Barb Wedehase and they have come up with 2 things so that I can stay involved with the Society since I am done with the essay and also the article write up from the meeting. I will talk to her via phone next week to see
if we can make the ideas work and to get info.

For now I just try to deal with the symptoms I have and cross my fingers that 2 weeks comes quickly enough so that I
can go back to seeing my Psychologist, I think talking to her again will help too. She is soo nice. Also LuAnn my GC is going to look into some options for us for dealing with some of the symptoms. Like a Pain clinic or something. I am
so tired of Appts but I guess since LuAnn is always so helpful setting things up it will be ok. I think I just need
to plan another vacation awayy from my life, maybe in late July..

All from my end. Love, Erica

Tuesday, June 28, 2005 7:56 PM CDT

Sunday, June 26, 2005 11:33 AM CDT

Here is a copy of the recent article I wrote for the MPS Society Newsletter 'Courage' - The excerpt I have below is what I read ffrom for the MPS I Patient meeting that was hosted by the U of MN and Genzyme on June 18th. The entire article will be appearing in the Fall issue of Courage.

-----
I have only been diagnosed with MPS I since February since that time I have balanced classes, appointments, a part time job and some resemblance of a “life” if you call spending way too much time in doctors’ offices and clinics a life. I’ve never been the typical so-called party girl but I also didn’t expect or want to be a frequenter of doctors’
offices and IV sticks. This is, in fact, way more than I bargained for.

I recently started ERT, and although this is like a new lease on life, it has also been the toughest time for me yet with the diagnosis. All along I unconsciously thought that someone was going to tell me there was a mistake, but with the start of ERT
it was like that is it, no doctor is going to tell me such a thing. I imagine this is what it is like to have an ice cold bucket of water dumped onto you, except for me the “shock” is still there and showing no signs of drying up.

I always remember something one of my favorite doctors told me, “all of these things are just words, it is important to remember your life is what you will make of it.” I have repeated these words so many times there ought to be a hole in that part of my memory. These words have become like a mantra, as a reminder that I am Erica, and not just a disease. The kind comments like these from everyone involved, give me something to hold on to and keep afloat.

Not in a million years did I ever think my medical problems where this serious or imagine the impact they would have upon my life. I’m not sure how I could have handled all this without the great support I have received. I know, too, that if I had the choice I wouldn’t change any of my specialists, as half the battle is finding a group of providers whom one can talk to, who will listen and in whom you can place trust. I have found all of these things and more; heck, I figure anyone who can put up with a million questions from me ought to be considered for sainthood!

I have often wondered in the last few months how I could have made it so many years without further symptoms or worse side effects from the enzyme deficiency. It took many, many specialists, multiple years and probably a lot of patience and many a moments of self doubt, but I finally made it, here today. I wonder, though, does this mean I have arrived? I think that there was some purpose for all of these struggles that I don’t
understand but what I do know is, I was very lucky to end up with an answer and even a treatment. Even if it takes some getting used to!

I know that I have many struggles to face yet and probably a lot of days where I won’t feel very great probably feeling sad, angry and upset. I do think however that the treatment will allow many more days of enjoyment and happiness all around. Right now I will enjoy what I have and not take life for granted.

Friday, June 24, 2005 11:24 PM CDT

Finished up ERT # 6 today and although there was no reaction
and no adverse side effects we had one heck of a time trying
to get the veins to work as needed. The first initial IV placement took 1 or 2 tries (have forgotten already) and this one stayed in place for about an hour and then infiltrated (in lay terms the vein blew, hand swells up) so
my poor nurse Mary had to restart the IV, except for the life of us we could not get a needle to stay in or the veins
to not roll. It took us (Mary, Trenace) 9 or 10x's to get an
IV placed that would work, got it all placed, taped up and
Aldurazyme running in again..with myself sitting between my
2 wonderful nurses doing absolutely nothing more than shifting in my chair.. about an hour or two later the 2nd IV
blew and once again began infiltrating. Not Cool! My hands look like pin cushions and ought to be brightly colored to black and blue by tomorrow, but it isn't even myself I cared about, I felt so bad for them!!! They are 2 kind!

..........this doesn't mean I don't still wish for a Port...
......I do!....even if for times like today when my veins are being impossible... I don't mind the IV, that is just a
lot of time spent uselessly for myself, the nurses and the Dr. when we could all get out sooner than 6 1/2 hours!!

I am sad Trenace is leaving us after this week, that girl is
just to funny she could be like my big sister, thats what it
feels like! :)

Steve Holland talked to Mary Yuska at Genzyme it sounds like
I may be able to get reimbursed for my trip to MN last week
which is really, really cool! He was so nice to do that for me!! I really like Mary and Steph too so another reason to
talk to them! :) although I can usually come up with one reason or another to call or email Steph to chat.

I watched the movie Simon Birch tonight, what a awesome
movie, I can't believe I waited this long too watch it! What is totally cool on top of the fact that the movie is both heartwarming and heartbreaking is that the actor Ian Michael Smith has an MPS disorder - Morquio Sydrome... that is cool and inspiring!

Love and Hugs, till next time, Erica

Wednesday, June 22, 2005 9:01 PM CDT

I do not know how to adequately explain how I feel, but today was my first full day back in WI and I really miss being around people who understand and friends who just get
it. By that I mean they understand when you are tired and why and I can just be "free" to be me with or without the
MPS. It is hard to be back where everyone else is normal and
where I constantly have to explain how I feel and why I feel
that way. It's not that MPS is my entire life - it's not at
all, it's just comforting to be around others familiar with it and who are going through the same things I am. I loved
Maddie, Spencer, Laynie and Fran - wish we where all in each
others backyards!

I hope to be able to go back to MN atleast once before classes start up in Fall. I really miss the Madsens and Hollands. I wish we all lived much closer!! If I do go back this summer I will also have to make a trip to see some of
hte Drs and especially Kendra - I don't know why but she is
just the nicest Dr I have met in a really, really long time,
and very helpful. Having talked to her the other day, I somehow feel better about some of my struggles and more comfortable with starting classes again this fall, I think the things her and I worked on might help. I liked school
before but now maybe it will be even better and less of a struggle.

LuAnn, my GC sent me a couple of reply emails last night, when she returns to the office in 2 weeks she wants to look
into other options for treating and dealing with the pain issues. Like a Pain team and/or Rehab. She said basically b/c it does not seem that my Rheum thinks she is the right MD for my problems. Partly b/c of the GAG/Anti-Body study I
will formally see her and Dr.Rhead every 3 months. To review
and evaluate symptoms, ERT and/or changes.

Thanks Jenn for adding new pics for me from the meeting! This week is ERT # 6! REmember if you are reading this, please sign my guestbook!!!

Love, Erica

Tuesday, June 21, 2005 1:00 AM CDT

I had such a blast this weekend - this is/was my first chance to attend a Genzyme sponsored MPS I Patient meeting and it happened to be in MN with the U of MN staff.What a wonderful group of people!!

On Friday I met the Madsens whom I adore - they are sooo nice and so fun to be around and talk to! I also met the Holland's who are also exceptionally nice! Amy and Steve are great and know so much about MPS I! And I finally got the chance to meet Spencer, Maddie and Laynie. I think Maddie and I will stay the closest.

I also got to meet Dr.Whitely, a great guy. And have my ERT
infusion with Fran - neat! I also met Dr.Elsa Shapiro and Dr.Kendra Bjoraker both of whom are also just exceptionally
nice. The meeting was awesome, even my speech/talk went faitly well it seems. I guess it was a hit. Maybe I can get
involved with the MPS Society in some form this way, it would definetly be cool. I will See what Kendra and the Holland's come up with. I also got to meet Stephanie Ginty
and Mary Yuska - Genzyme, this was awesome. I like Steph just as much in person as I thought I would. She is very neat.

I met with Kendra today (stayed in WI an extra day due to being tired and weather) did all kinds of survey and Q.O.L
information testing for a study on effects of MPS I and ERT in cognitive ability. (general idea I think) She also then spent the time helping me understand the results, going over things she can do to help me as far as school and just talking. (I led her on a wild goose chase to find my car, anyone who will walk along to help just has to be a dear!)

Now it is back to the grind, work and life and MPS-ERT and
figuring out every crazy thing I need to get accomplished that I have put off and odds and ends. (run on sentence I know)(too tired to correct) I do have new pics to add from
the MPS meeting on Sat - that Dr.Shapiro sent me. Once I figure out how to transfer them I will to this website.

Thanks for keeping up on my mostly dull life.

Wednesday, June 15, 2005 3:12 PM CDT

Fri June 17th - Aldurazyme ERT infusion with Fran Madsen
Sat June 18th - Genzyme MPS I Patient Meeting 9-1

Only 2 more days till I leave for MN I am so excited!!! I am meeting the Madsen family Fri morning and picking up Fran - we are going to our treatment together and I get to
spend the entire day with her!! I am excited of course! I think Amy Holland is also going to try to sneak down and gab with us. I promise to take pictures and add them when I get back.. I just cannot believe it is this close!! Yippee!

Erica

Sunday, June 12, 2005 0:45 AM CDT

Monday June 13 - Appt Hand Specialist-FMLH
Friday June 17 - Leaving for MN - Possible ERT imfusion
Saturday June 18 - Genzyme MPS 1 Patient Meeting (!!!!)

Only another week until the Genzyme MPS Society Patient meeting in MN, I am so excited!! I am meeting Hope and Fran Madsen + Amy Holland and Family Fri and everyone else Sat.
at teh meeting. This is also my chance to meet Stephanie Ginty, which will be neat too, she is a really great person
and very easy to talk to + very helpful! I am sitting on the
patient panel for this meeting and they have also asked me to read my essay that I wrote for 'Courage' the MPS Society
newsletter! scary but neat...

Amy, Hope and I are planning a dinner for their families and
myself and any one else we can talk into coming, probably Sat night. (yey!!!) I should find out Monday when I talk to
Stephanie if my Ins wil give approval to do my ERT at the U of MN, but if they do cover it, then Fran and I are going to
be doing our ERT at the same time Friday!! (as it stands right now) yippee, skippee!

ERT 4 went well on Friday, no side effects and they went back to running it slower (a mixup the past 2 weeks) and I wasnt so tired - I took a nap during the infusion, but although feeling tired Friday night I still was able to go out with my sister. Was fun for a change! I am afraid of how I will feel in a few weeks when they speed up hte infusions again. I just don't know though.

Okay, anyways I am chatting with my brother and his g/f so forgot my train of thought. Will write more tomorrow, when I find out more info. All for now! - E

Saturday, June 4, 2005 11:19 PM CDT

Tues June 7 - Rheumatologist at FMLH 12:45
Thurs June 9 - Dr.Brosig at 10:00 CHW
Fri June 10 - Aldurazyme ERT Infusion 8:30

Yesterday I received my 3rd ERT infusion and I think all continues to go well with that - was about a 4 hour infusion and 5.5 hrs total at the clinic. I got home at about 3:00 and after replying to a few emails (about next week) went to bed at 4:30 until 7:30. The went and got a few groceries and did stuff around the house till 12, slept until 2:30 today.

I hate that it makes me so tired and that I sleep pretty much all of Sat, it is hard to think that it may be like this forever. I don't even mind (well too much) getting the ERT b/c I know it will help and too slow the process of build up of GAG's but I hate the thought of always feeling bad the day after. That part really worries me.

My Genzyme Case Manager is helping me to figure out if I can recieve my infusion at the U of MN Fri June 17th - the clinic here will not be open that day anyways so I would have to miss it
if I don't go there - but unsure about the copay costs.

I will be there that weekend for the Patient Meeting which I am so
EXCITED for! I can't wait to meet Amy and Hope and Stephanie and all the others!! Dr.Elsa Shapiro and Dr.Whitley have asked me to sit on a Pt panel for that meeting which should be both fun and nerve wracking.. it is going to be webcast, yikes!

I seen Dr.Brosig on Thurs. which was probably good, it has just been a up and down kind of few weeks with everything going on and I just sometimes need someone to talk too.. For
which her "help" is always good. Ultimately, basically she just helped me to see what everyone else too has been tellin me and that was just that it is okay to struggle sometimes and to need help.. and too that being honest with myself and others that I trust is probably better than "hiding" from what I really feel..

I just don't always know, all of these things scare me sometimes and sometimes i'd just like to run from what I feel and know and pretend it just isn't happening. It's great to have the dx and the treatment but at the same time it is soo much to get used too and it just has hit me lately like a ton of bricks..

Thursday, June 2, 2005 1:14 AM CDT

Thurs June 2, 2005 - Psychologist 2:00, Psychiatrist 3:00
Friday June 3, 2005 - Aldurazyme infusion

I seen my Cardiologist today (the great Dr.Earing) and all went well - it is always a nice change to go "visit" him as even when the tests stink (sometimes literally) he always treats me like a kid sister (at least it feels like it, this is how my brothers pick on me too) and we get to give each other a hard time the entire appt. When I say "kid sister" though keep in mind too that he gets to play Dr and I have to be the obedient patient, hahaha. Not that I ever cooperate, who else is there to keep Drs like him in line?? ;) He is a good guy and actually kind of the one who put pieces together and suspected MPS as the cause of my problems.

Seriously though, the Echocardiogram looked fine, no change and the Stress Test was fine, no shocking surprises there. Which is good b/c last fall when I ran it that is when they
found the Lung Disease. I am free of those tests for another year, yippeee!!

We had my cousins funeral tonight, talk about a kid well known, wow! It was held at the funeral home and the parking lot, side street and all down the side of the Hwy where packed with cars, there where so many people there!! It was sad, I know that there was some reason God took him so young,
but it is still hard to know that we will never go cruisin on his motorcycle or just talk/have fun. He was well known and well liked and died just way to young..

Tuesday, May 31, 2005 1:04 AM CDT

Mon May 30 - Memorial Day!
Weds June 1 - Cardiologist Appt 3:00 (Stress test, Echo) CHW
My Cousins Funeral @ 7:00 to sad..
Thurs June 2 - Psychologist Appt 2:00 CHW
Fri June 3 - ERT (if staffing goes)

I guess I didn't really give a very good update about ERT yet did I? I was very down Sat so hard to write about #2 since that is what had me down! I think it went well, n/t
major - I was able to get out of the clinic an hour earlier though.. and I did meet those really nice guys I think I talked about Fri.

I am feeling happier today - I spent the entire afternoon till I went to work outside and it was gorgeous - very un-WI like weather for Memorial Day! Being outside really helped me feel better I hope this feeling I had will last a
LONG while! I think I had enough sadness and hard time
dealing to last a long, long time...I know that I have alot to take care of and I think these things cann will all help
to deal in the long run.

Otherwise there isn't anything new, I will update after Weds Cardiology Appt - although it will be pretty late by the time I get home from my cousins funeral. (:()

My cat has been giving me evil glares and keeps wandering from here to my room, trying to tell me it's time to go to sleep. (this while my other cat prowls the neighborhood tonight!) so I better oblige, before the cat starts getting evil... :)

PS: this is strangely therapeutic, almost better than writing in my journal... how odd.. imagine anyone can read this!

Saturday, May 28, 2005 11:19 AM CDT

I fell asleep last night at 6:30 and woke up this morning at 8:30 - but in between I had this crazy dream about trying to make peace with an old friend of mine (who by the way i'm not even fighting with!) so I was talking to my
friend Sheila this morning and we came to the conclusion that I wasn't really trying to make up with this friend, but I was trying to come to terms with and make peace with my body and how hard I have been taking this lately with feeling down... why did it come in the form of a dream??

I should be well rested today, but like last week too am still very tired. I too forgot to take my sleep disorder meds before falling asleep last night so that isn't helping
any today..

Again today I have been feeling really down, I think it has
to do with being a little disappointed with not feeling any different physically from the ERT. I know consciously it will take a while, but subconsciously I think my psyche
and my body are hoping for change sooner and maybe this is why I feel so down.. I really have to try to get some things taken care of this week.. there are so many things I
put off this week and so many people I need to talk too and
calls that I need to make but instead i'd like to run away
just for awhile... these are things that I just can't get off my mind and thus my mind is almost constantly working like a mile a minute... Aagghh!! Why are these last few weeks so impossibly hard???

Friday, May 27, 2005 5:20 PM CDT

I just got home from my 2nd treatment a little while ago - I met the nicest people today - 2 brothers who make me realize how much I have and how much I have to be thankful
for. They are Fabry patients - and older one was about my parents age and the other a little bit older. They will be there every other week so I will get to know them quite well and we did exchange addresses. I think I have made 2 new friends today.

The treatment itself went well the only thing that bummed me was it took them 6 tries to get in an IV that worked and didn't cause my vein to blow. That hurt! I kept chatting and talking to the nurse throughout but I so wish in my heart that my Drs would let me get a Port a Cath put
in.. but what am I too do, it's not worth getting down about and I already have enough that has been getting me down.

Otherwise I am a little sad tonight about the circumstances for these 2 brothers I met today - they have such an incredible will to live - I admire them... I wish there was a way I could help people, I guess all I can do is be a friend and be willing to be a friend.

All my thoughts for right now. ~

Thursday, May 26, 2005 10:02 AM CDT

Thurs May 26 - Diane (Hand Ther) 8:00, Dr.Matloub 9:00 (canceled see note)
Fri May 27 - 2nd Aldurazyme Infusion
Weds June 1st - OT 1:00, Dr.Earing (Cardio) 3:00, Cousins funeral - 7:00
Thurs June 2nd - Psychologist Appt 2:00

I cannot believe how crazy these weeks can get, I swear life
is just completely crazy sometimes!!! I am updating from the
Hospital in between Appts, I was to see the Hand Specialist
this morning but he was out sick so I seen the Hand Therapist and now idling time until I head for the next and final Appt for today.

Tomorrow (as far as I know) I am set to receive the 2nd ERT infusion - which is good (in a long boring kind of way) as this will bring us that much closer to feeling better physically in a few months - I hope! I have been con't to put it off but I need to get ahold of both my GC, for ques.
I have on what or who I should see in regards to a possible Anti-Depressant (one of these days I will actually do it..)
And sometime between now and the 13th have to get a bunch of records sent.

Otherwise nothing new, I spent the afternoon with my Grandma yesterday which was nice - I love spending time with her!!! Probably go to church tonght, I have Sunday off of work but I like the week night service - quieter I think.
Otherwise I'd like to get my nephew on Sunday or one day next week - Quinn is always a light too sometimes dreary days!! ~ All for now in my ever boring life... ~

Tuesday, May 24, 2005 3:09 AM CDT

Tues May 24 - OT 10:30-11:45, Rheumatologist 12:45 FMLH
Weds May 25 - Off (All things!)
Thurs May 26 - Hand Specialist 8:00,9:00 FMLH
Fri May 27 - Aldurazyme infusion 8:30 CHW Northshore

Tonight I fell asleep quite early which is unusual for me, but also a good thing! I woke up this morning to update and then headed back for a few more hours.

Nothing new here, I see my Rheum at 12:45 and praying that she will agree to re-adjust my meds or something - today was by far the most aweful day I have had as far as the pain, everything hurt horribly. I maybe spent more time in the bath/shower than anything else (j/k) but felt like it!

The pain was especially bad in my shoulders, hands and hips and I just don't know. (seems like I don't really understand any of this all the time lately!!)

I haven't figured out what too do about the Anti-Depressant issue, but have many reccomendations, basically now a matter of talking to the right person. I feel like I should talk to my GC first, maybe later in the week, I really don't know what to do..

I will update again after my Appt today
hopefully something will go good there, I coud use good news! You would think the ERT would con't to have me excited but have to go day to day basically at this point to cope and deal with all this stuff which really stinks.

I do have to figure out what to do for next week with my Cardiology Appt and my Counsin's funeral as they may collide, unsure about that at this moment too, that is such a sad, sad situation, I wonder why God does this? I know he has a reason, but it is really hard to even understand right now or reason with...

Monday, May 23, 2005 0:54 AM CDT

Tues May 24 - OT 10:30-11:45, Rheumatologist - 12:45 FMLH
Weds May 25 - OFF (work and Appts!)
Thurs May 26 - Hand Spec. 8:00 FMLH, Eye Institute - 10:30(?)
Fri 26 - Aldurazyme Infusion
Sun 28 - OFF (work!!)
Weds 6/1 - Cardiologist 3:00 CHW,
Cousin's Funeral - 7:00 :(
Thurs 6/2 - Psych 2:00 CDC @ CHW
Fri 6/3 - Infusion?

Just when I don't think it's possible to feel any worse the
pain kicks in - it just amazes me how differently I feel now
compared to even a few months ago. It's like I had alot of health problems even a year ago (worsening I guess) but just
the last few months have been increasingly bad. The most Aweful of all is the pain, it is like it just persists and resists.. ~ I am thankful I have found pretty wonderful providers to help with these things ~ but on an emotional level it is sometimes really hard to deal with.

This past week when I seen my Psychologist - she always keeps up on "how my mood is" - but even she can tell a difference when it is at it's worst - suggested again to reconsider an Anti-Depressent, for which I was and have been resistant. But now given how I have felt emotionally lately,
and given that my GC too has mentioned this, I think it is okay to do something, or I guess if there is ever a right time to need to take something it is probably right now. I guess I still resist the idea of a medication, but too know it can't really hurt and it
might even help... I can only hope....

Several of my MPS friends have tried to help me with this too, both by reassuring me it isn't trying to take an easy way out and that there isn't anything to be afraid of... it's still just hard.. it's like I should be able to control how I really feel... I don't know though, really just don't know..

What I do know is I hate how all of these things make me feel, and I hate how I feel emotionally.. I hate the up's and downs of dealing with this all..

Sunday, May 22, 2005 0:36 AM CDT

Thursday, May 19, 2005 2:29 PM CDT

One day until I begin infusions, this should be exciting but it has kind of been a crazy, sorta down, kinda week and
just taking things day to day for now. Went to have the eye pics taken this morning of my corneas but no could do, they sceduled the actual pics for next week instead. I seen
my Psychologist this morning - was a good visit - although probably not our best - as far as talking goes. We are still considering an anti-depressent - but for now just going to keep a close eye on how my mood is. I completed all of the labs for the ERT and ahve those out of the way 4
another 3months. I walked over with the Research Coordintr.
was a nice talk with her, she seems nice, and easy to talk
to.

Will be sure and update after treatment tomorrow or Sat!!!!
All for now, Erica

Tuesday, May 17, 2005 2:37 PM CDT

I don't even know how long it has been since I updated but I know alot has gone on since. I had the Genetics appt last
Weds which I thought went really well.. my mom and sister came with me but I was still able to talk to my GC in private for about an hour alone. Just going over things I had done so far and things that i'll need to do yet. Plus
some of my questions. I have signed to participate in a study that meausres the levels of anti-body vs GAG's in the
blood and urine and met the research coordinator who will take care of most of the paperwork having to do with this.
I seen my Pulmonologist on Thurs and I kind of have been feeling pretty down since then. I guess because the appt didn't really go as well as I had hoped and I am still on the same meds. I shouldn't let this bum me out but too it really has gotten to me in the last few days all of these appts and tests and trying to educate and advocate for MPS and teach some of my Drs and the PT/OT people. I see my Psychologist this week and hopefully she will be able to come up with a way to help me deal or just get me to talk and to see that I am doing okay. I know that I don't always
have to wake up happy but it sitll stinks when so many days
in a row I feel down all b/c of one bad appt. A good thing is that I may be starting ERT on Friday as long as all goes
well these next few days!!! That too should help cheer me up.

Love and Hugs,
Erica

Friday, May 6, 2005 0:22 AM CDT

Today was my last official class for Economics and you know
what? I think I am actually going to miss all of the itneraction and cameraderie that went around studying for these tests. Too me this is a fascinating topic. Not so much that i'd want a job in it, but practical instead. This leaves only 1 assignment and 1 class to finish up the sems. Interstingly after class I sat and talked to my Instructor for quite a long time just about the sems and e/t that has/is going on in my life and summer plans (or lack of right now)

Next week is a busy week, what with having school (not classes) tues/weds and OT/PT tues for 2 hours and Weds the
Genetics appt. (Both apprehensive and interested for this)
Thurs i'll talk to my Psychologist and then walk over to the Hospital for PFT's and appt with my Lung Dr. hopefully
no change in lung fuction since Febr - could use some good
news already.. Have to finish forms for the Camp in MN that is held the end of summer, forms due in 2 weeks. Not
sure how this would work around the infusions though or what I would need to do.. especially given that by then
treatment should surely be going (i hope) and atleast for the first year or so I guess they don't want pts missing the infusions.. hmm hmm.. another question to ask..

Tuesday, May 3, 2005 11:58 PM CDT

The count down has begun! 2 classes left for Economics and 1 (well really 2 but going to miss next weds class) left for Human Growth Development. A total of 1 week to go until summer break! Will finish up my other class(s), with 1 potentially carrying into summer. I talked to my Genzyme Case Manager today and felt better after hanging up with her. Also talked to Amy Holland,6 weeks until I get to meet them and Stephanie in MN!
In the long run, hope that all of these things going on won't affect my patient relationship with my Genetics team, I want us to all be able to work well together as a team, as I think this is very important when it comes to treating/managing MPS.
All for tonight.

Tuesday, May 3, 2005 1:08 AM CDT
Has been a busy day here, alot of stress over the weekend about the entire situation with getting ERT set up, but feeling slightly better now. Have talked to Amy Holland today and she is always a source of good support and advice.
Steve and Amy have fwd all of my contact info to the head Genzyme staff and to there own case manager. I just received an email from their Stephanie and it seems that they have already talked to my C.M, Stephanie and all are working to get something set up!

I don't know what I would have done without the treatment support Genzyme offers, my Case Manager has been a godsend when it comes to answering my millions of questions and giving advice. It is amazing all of the great people I have met and talked to already in the last few months..

Hopefully talk to my GC this week, I hope that all con't to
go well from here on out. And that her and I can work well
together - she has answered alot of ques to from me and helped me - so hopefully differences aside.

Have this week and next of classes left and then we are done for the semester, exciting, but what will I do with my time?? Received the results of my last Econ exam friday and scored a 27/29 which was really well for this exam, our
instructor told us it was the hardest one of the sems.

Have no appt this week!!!!! (really exciting!!) 2 weeks of PT/OT left, atleast for the time being and I think 4 appts next week. Of course they all fell on the last week of classes, but this is life.. :) Will see Genetics, (my sister is going with me) my Psychologist, and my Lung Dr
for F/U and it seems lots of PFT tests weds/thurs of next week.

All for now, thanks again Jenn for your help here! Sign my
guestbook!!

Saturday, April 30, 2005 11:36pm
My first try, the other one I just wrote got deleted. Please come back often to learn about myself and my disease,
my life, my family, my friends and my Drs and progress towards receiving treatment.

Without the wonderful support of the MPS Society and my friends on the MPS Forum I am not certain what I would have done. I owe an especially big hug to my friends Amy, Gabbi,
Hope, Sheila, Jenn and many others. You cannot begin to imagine how much your emails and phone conversations with me help.

Thank you for becoming my second family and a great support system. Amy, thank you for becoming a 2nd surrogate mom to me, for advocating for me and for teaching me how to become persistent. Your lessons will do me well, with fighting for myself and for standing up for my own good.

Saturday, April 30, 2005 11:36pm

Thank you for visiting my webpage, created and updated by myself and my good friend Jenn Deskins. I am 22 and have officially been diagnosed now with MPS-1 Scheie Syndrome for 2 months.
(see web pages for further info on MPS disorders and Scheie Syndrome)
I am a college student - and although unsure of my major - I have always wanted to work with children and have based my class selections on this desire to work with kids in a medically oriented field.

It has been just 2 short-long months
since I began the fight to receive ERT, and with much patience by my Genetics Counselor and Genzyme Patient Adv/Case Manager have learned a quite a bit about
this disease in such a very short time.

Although I am 22, I see most of my med. providers at the Children's Hospital,
including my Genetics team, Cardiologist, Pulmonologist and even a Peds. Psychologist. I see some others at
Froedert Memorial Lutheran Hospital adjacent to CHW.

My Cardio/Pulm were the one's to pursue further answers after they first met me and with their persistance finally received an answer to my many health problems. It took so many years, and so many Drs, but finally I landed in the hands of a wonderful group of providers - that too are always
willing to answer one more question, and
one mre thought from me. I think I could
become the Question Queen of WI - and hold the title for a record sum of time!

As you will read in my journal - I am in
the midst of figuring out when I will start Enzyme replacement, and where I will receive the infusions. Have a bit of a struggle going on right now to figure thise ever important info out.

This has been a hard part of the diagnosis - losing control over my life
and these kinds of situations as there is basically not one thing I can do, but
sit back and trust that everyone is doing their best to advocate for me.

The support I have received this far from my family, friends and my medical providers has been awesome, and I hope that in the struggles ahead I can always
stay strong, positive and kind.
Read ny journal for more insights into
my life and how I came to be dx's with MPS 1.

Click here to go back to the main page.
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