History

Wednesday, October 8, 2008 10:27 PM CDT

I thought I had updated before now! Sorry! I tell you the days are all just running together now!

Monday we met with the school. It was ok. Not terribly impressive, but I can at least tell they are trying. I am at a loss tho as to how to help them understand something that even most drs don't. If I cannot help them to "get it" Nicholas' health may be at risk again and that terrifies me. Tomorrow is our big ARD for him and I am praying for the right words to come to me to help me explain to them just how serious this could be. I hope it goes well...

Yesterday, I went to Nicholas' school and gave the nurse a quick pump inservice. She was really attentive and asked a lot of good questions. I REALLY like this nurse, so I feel better knowing she is in charge of the pump while he is there. I hope it goes well and there are no major issues. He also got to have his school pics taken while there, so at least he did not miss that. On the way out, we stopped by his classroom and his class was at the bathroom. Our neighbors' son who is in his class came running over to give him a huge hug. Other kids were saying "Nicholas!" when they saw him. His teacher came over and hugged him and told him the whole class missed him and can't wait until he comes back. If all goes well with the ARD, he will be back FRIDAY!! YEAH!! He is so excited!

Also yesterday, I went to see the ENT about my ear. It is definitely ruptured...she saw the hole. She said what she thinks happened is that the infection got too big/bad too fast and the abx the urgent care rx me were not in my system nearly long enough to do any real good against the infection. It was basically only 12 hrs between the onset of pain and the rupture. After telling her about my recent aspiration pneumonia, she feels it may be reflux related since it is only in the one ear. Guess we need to call the GI...AGAIN. She advised me not to fly anytime soon and told me that, while the infection will go away within a week or so, the hearing in that ear (which is pretty much non-existant at the moment) may take weeks to return to what it was...or it might not come all back. UGH. So, she sent me on my merry way with a fistfull of rxs and an appt to come back in a month.

The boys all saw Immuno today. I was not impressed. We did not see our immuno. She basically stood us up to have an appt with another one of our drs and sent in another dr who is not an immuno to conduct the appts for us. I wish someone had called us to reschedule or ask if it was ok for us to see another dr. I really needed her opinions on a few things...with Daniel being on his 4th ear inf by 7 mos old, drs are starting to mention tubes and I wanted to get her thoughts on this...along with some other things. All she wanted was to get an update and send us for endless labs. Daniel got 3 pokes and only 2 of the 4 vials filled before I cut them off. Enough was enough and that poor baby screamed enough. The older boys did much better tho and Nathan only cried for abiout 30 sec. That has to be a record!! We go back in 3 mos.

Nicholas saw GI as a hospital follow up. He was not happy at all with the results of the GES...but who would have been. He set us up with a nutritionist to make sure Nicholas is gaining well and see what we can do about getting him off the pump for a bit. he was happy to hear Nicholas has been able to tolerate other foods aside from clears and encouraged us to keep trying new things as he tolerates them to allow for an easy transition if/when he can go back to oral eating. We gave him a trial off the pump this evening with frequent blood sugar checks and he LOVED it! He ran around yelling "I'M FREE!! I'M FREE!!!" and was unleashed for his bath tonight for the first time in weeks. It was nice and he held up fine for a couple of hours! He goes back in a month.

We visited with our hospital friends this afternoon before we came home. Some other friends were in the ER waiting for an admit with the SAME tummy troubles Nicholas had before his admit. I hope the drs can help them too. Our other friends were still there and staying the course and doing well. Child Life had a REALLY fun event planned for this afternoon!! They invited our boys to help out too, even tho we were not inpatient. They got to help paint canvas pictures for the children's hospital walls! They LOVED it! They took pics and video of the affair and were amazed at how long our kiddos stuck around to paint. Ours were one of the die-hard last kiddos there. Painting is one of both of their favotire things., Even Daniel got in on the action for a few minutes. It was so much fun and a much needed escape from the medical stuff we have been dealing with lately.

So, that is us in a nutshell the past few days. Tomorrow is the big ARD and I will update on that for sure. Take care and God bless!

Melody

Sunday, October 5, 2008 11:38 PM CDT

Well, I only *thought* things were going to settle down once we got discharged from the hospital! Guess I was wrong about that one!!

We took Daniel in to the new ped and it turns out he had an ear infection we did not know about. That would explain the frequent night wakings, the nursing issues, and the general crabbiness and crankiness he has been having. Within 3 doses of the abx, he was looking and acting so much better, so I can only assume he was feeling a lot better.

Things with school are at a standstill right now. We are scheduled to meet with them tomorrow morning to go over changes that need to be made for Nicholas to return to school. Dr K would prefer we wait to send him back until we have the ARD to make the necessary changes, but that may be the end of the week. She faxed us over a school excuse note for all next week, but the teacher in me is hating for him to miss all this instruction. UGH. I hope we can start getting some of these issues resolved tomorrow.

In the mean time, he continues to try new foods and has done well with some of them. Others have given him trouble and we will wait to try them again later when his stomach can tolerate them better. He has worked up to a small variety of things and has been doing wonderfully making good food choices and staying away from the things he knows will not be good for his stomach. He is starting to ask hard questions tho...like if he can eat his birthday cake on his birthday and if he will be able to have any of his Halloween candy. It hurts me that I cannot give him an answer, but he has been a real trooper throughout and has accepted it in stride with little complaint.

Nicholas got to go to TaeKwonDo yesterday. His academy is so awesome! They did not even bat an eyelash when we called to let them know what was going on and the new circumstances with his feeds and pump. They said just have him wear it over his uniform and come on back! The kids were naturally curious why he was wearing a backpack and the instructor told them "because I said so". Then the child said to the instructor..."cool...I wish I could wear one too!". If he only knew... ;o) Nicholas had so much fun and is eager to go back. I am glad he has no idea that he missed his graduate belt test while he was in the hospital.

This morning, I got to be the one with all the excitement. I woke up really early this morning with excrutiating ear pain. I somehow managed to wait until urgent care opened and made it in to be dx with an ear infection. No big surprise. The numbing drops did not touch the pain...it was killing me! Then, after piggybacking Tylenol and rx 800mg Motrin, I finally got some relief. Turns out it was not the painkillers after all but that I ruptured my ear drum. UGH. It no longer is killing me, but I am sure hoping the draining stops soon and I did not suffer any hearing loss or permanent damage as a result.

I guess that is about all for the time being. I will keep you posted on the school situation. Thanks for checking in on us. Take care and God bless!!!

Melody

Thursday, October 2, 2008 0:10 AM CDT

Sorry for not posting an update sooner. Yes, we are FINALLY home! We were discharged Tuesday around noon...just in time to drive home and stop by the school to pick up Nathan. Boy was he excited to see me standing there in the doorway. I am sure he realized we were home. He ran down the list of all the family members and asked where they were and I got to answer HOME for everyone!! Since then, we have done a LOT of sleeping. Daniel went to the ped today and we found out he has an ear infection in addition to the crud he caught at the hospital, poor baby. I hope he is feeling back to himself soon and the abx the ped put him on kicks this infection quickly.

Nicholas is doing as well as can be expected. He is struggling with being surrounded by all this food he cannot eat. He keeps asking can he eat this or that and I have to keep telling him no, that his tummy is not ready for a cookie or fruit snacks or crackers just yet. We are just doing trial and error with "liquidy" foods (yogurt, thicker soups, applesauce, ice cream, cream of wheat mixed thin, pudding, etc). So far he has tried applesauce and pureed chicken noodle soup and done well. Other food items have given him a stomachache, so we are going slowly.

The school is giving us some issues about needing to call an emergency ARD to make changes to his IEP to reflect the new eating situation as well as making sure the other things in the IEP that were not being done will be done now. Dr K does not want us sending him back until this is done and has written him a letter outlining more specifically what he can and cannot do at school, as well as laying out some ways the school can accomodate his needs better than they were before. The school is dragging their feet about this and not wanting to schedule this before he is scheduled to return on Mon. I am not sure what to do as I need some reassurance from them before I can feel comfortable sending him back after all that has happened the past 2 weeks. If they cannot make accomodations to keep him safe there, I will need to make other academic arrangements for him. Please pray we can get this worked out somehow for Nicholas' sake.

Thanks again for all your thoughts and prayers. They were so very much appreciated. Take care and God bless!

Melody

Monday, September 29, 2008 10:09 PM CDT

Today had to have been the longest day we have spent in this hospital stay. They were hoping we would be going back for our Gastric Emptying Scan (GES) at 7, so after a very long night (which included sugar checks at 12, 4, and 6, vitals at 12 and 6, and stopping feeds and hooking up to the IV at 2) I was up, dressed, and ready at 6:45. Well, then the waiting began. Finally at 11:30 they told us to be ready in an hour, so we went to play with our friend from downstairs. Meanwhile, I still had not eaten breakfast or showered for fear of not being ready to go when they came to get us. Then, we waited and waited some more. At 1:30, they said 30 more minutes. At this point, I snuck a granola bar when Nicholas was watching a show as I was starving. So more waiting...and waiting...and waiting. FINALLY they came to get us at 2:45. At this point, I still had not showered and I begged Nicholas to let me have something to eat. I got to eat 1/2 a bag of pretzels and a pack of Twix we had laying around in here.

The Child Life girls (who are SO awesome!) went down with us as the one was training the other and wanted to show how this test worked with a child who was not going to be freaking out. Nicholas has been through 4 of these already and knows the ropes well. You eat or drink something with a radioactive tracer in it. Nicholas got eggs with the tracer and a slice of bread and OJ to bulk it up. Then you lay down on a table with this machine inches above you and below you and stay totally still for 90 min. Sounds easy, right? Nope...they did not even have a TV in there. Luckily, we knew this beforehand and our friend from downstairs loaned us her portable DVD player as she was not using it. What a lifesaver!!! Then, you can watch your stomach and intestines glow on a screen while the radioactive tracer moves through the GI tract in the food. At then end of 90 min, they can measure how much has left the stomach and how much is still in it...and calculate how long it is taking the food to empty out.

So, normal empty time for a solid meal is 90 minutes for the stomach to empty halfway. Are you ready for Nicholas' half emptying time??? Ok...brace yourselves. The time it takes for Nicholas' stomach to empty halfway is 370 min. Yes...three HUNDRED seventy minutes. Basically that means the food goes in and sits...and sits...and sits...for HOURS. No wonder the poor kid is bloating, cramping, nauseated, and complaining of pain. I think I would too if the food just sat in my belly for ages and didn't leave when it should! This test was also done on higher doses of meds to help the motility move food through faster AND including a previous surgery he had to help improve the motility time as well. The last 2 scans he had of these were 218 min and 181 min...so this one is much much worse. We will get more information tomorrow, but this is what they found just from the preliminary report. We expected it to be bad, but we were surprised that it was this bad.

Since we did not get back to the room after the test until about 5, the team made the decision to keep us overnight for observation to make sure he did not have any problems after the test. Within a few minutes of returning, the meal he had at the test (that he was THRILLED to eat as it was the first *real* food he has had in over a week)was already causing him to bloat and cramp. Hooking him up to the venting tube helped with that and he fell asleep.

Now, the hope is to go home early tomorrow. PLEASE PLEASE PLEASE let us go home tomorrow!!! Thanks for checking in on us. We sure appreciate all the prayers and thoughts and all the encouraging messages left in the guestbook. It sure is nice to read that there are people out there who are pulling for us. Take care and God bless!

Melody

Sunday, September 28, 2008 10:31 PM CDT

Well, another boring day in the hospital. I will be SO glad when we get to go home!!

Today, Nicholas ate little more than his cup of jello for each meal and one popsicle. No bloating today, but we did have one episode of belly pain that was bad enough for him to ask to be vented. When I went to hook him up, the red jello he had at breakfast had forced its way up into the g-port of his tube. When we hooked up the venting tube, we got a lot of air out and red jello forced its way out into the venting tube. After waiting over an hour, I finally asked the nurses what to do with it since it was not going back in with gravity like it usually does and they said not to force it back in, but to just dump it. We had to measure it to subtract from his Ins and Outs and come to find out that the jello came out of his belly exactly like it went in! It had not liquified at all, but was still in little solid pieces just like you would expect chewed up jello to be. UGH. When the team rounded later, they said that almost solidifies the theory that his stomach is not working well at all and the gastric emptying study he is having tomorrow is not likely to look good.

GI came by today on rounds and we also saw the team. Nothing really earth shattering was discussed...just more of the same ole same ole. Our favorite day nurse Anna was on today but she was charge nurse. Honestly, we hardly saw Nicholas' actual nurse since Anna hung around our pod and took care of him herself for the most part. She even stayed late after her shift to check Nicholas' IV since he has to be hooked up in order to fast without his sugars tanking. Since it has not been touched at all since 12 am Thursday and has been in since a week ago Saturday, we thought for sure it was a goner...but she checked herself and said it was still good! Thank goodness we did not have to start another one just for a few hours! She is just the best! She will be back on Tuesday and said she hopes we are still here so she can have us again. As much as we like her as our nurse, I hope we are long gone by then!!! We also saw his favorite night nurse Patti from downstairs tonight and she was glad to see Nicholas looking so much better than when we were down on her floor last weekend.

Stooling was interesting to say the least. He is finally passing the barium from the fundogram on Friday. We discivered it took 10 flushes (5 without and 5 with the shower rinser thingy on the back of the toilet) to get the first one to all unstick from the toilet bowl and flush down. The second time took about 7 flushes with the rinser thingy to all flush down. Who knew barium made stool so super sticky??? It was definitely a site to see us all leaning over the toilet watching to see if the stool was all gone or not!!

So, the rest of the day today we hung around, played with our little Mito friend downstairs and our friends from down the street who also have a little guy with Mito here in the PICU. It was a lot of fun. Doug took Nathan and Daniel to my nephew's birthday party this afternoon. Daniel was SO excited to get out of the hospital and see the light of day for a change! He apparently worked the crowd at the party pretty well and Nathan had a good time as well. Nicholas and I stayed back and took a nap since we had nobody crying to wake us up. It was nice to be able to focus all my attention on Nicholas for a change as he has had to share it with Daniel all this time (and Daniel is usually louder and more demanding and wins most of the time).

This evening Doug took Nathan home early as he was so tired and Nicholas hopefully has a test early in the day. We are hoping that maybe we can go home once this test is done. Fingers crossed.

That is about it. Thanks to all who have come to check up on us. We sure appreciate it. Take care and God bless!!!

Melody

Saturday, September 27, 2008 10:48 PM CDT

Well, I tell you...leave it to my child to be the one to throw in some excitement on the weekend when nobody is here!

The day started out fine and we saw our team this morning. He had gotten to have clear liquids for dinner and breakfast and done well. All of a sudden, Nicholas was on the floor complaining of just having liquid diarrhea. When Doug was finished cleaning him up, the belly pain had returned and so had the bloating, but not as bad as before. The nurse measured his belly and called the dr on call. This was not anyone on our team, but whoever was on call for them had no idea about Nicholas at all except what she saw in his chart. Her suggestion was to take him off the pump for an hour and see if that helped. We unhooked him at around noon...the same time he had a scheduled sugar check. It came back fine at 120. Within 30 min, he was saying his belly felt better and then a couple of min later, he yelled that he was dizzy. I made him lie down and we checked his sugars again...this time they were only 51! It had dropped from 120 to 51 in under 40 min. Not good. He immediately went back on the pump and within a hour, his sugars were fine again.

Lunch came and shortly afterwards, the bloating returned. This time, he was huge!! 21.5 in around the waist!! This time he said he had no pain along with it, but was very obviously distended and his belly was taught as well. The nurse was concerned and called the on-call dr again and asked for her to come see him this time. Before she came, he had a bowel movement that was explosive, but normal stool. He was extremely gassy and had lost half an inch of distention by the time he was done. His belly went back to being soft as well. When the dr came by, he was feeling fine and he was still bloated, but had a soft belly and had no areas of pain when she did an exam. She said her only recommendation was to take it easy on the solids for the time being because if the bloating continued we may have to take away the clear liquids. Well, this would be a devastating blow to lose eating right after he just got it back, so we made sure he took it easy on what he ate orally the rest of the day (not that he had a lot to begin with tho) and he seemed to do ok. I am hoping it is just an adjustment period to have something in his belly again after a week with nothing.

The sugar issues raises a concern with me about this test he is supposed to have on Mon. From what I remember about doing this test in the past, it is a fasting test. If his sugars tanked so much after less than an hour off the pump, I am afraid to think about what may happen after an entire night off of it. I am hoping they will hook him back up to the IV D5 overnight to avoid sugar fluctuations from fasting. This is something I will have to bring up with the team tomorrow. I was not thrilled with Dr B and the team's recommendation to stay 24 hrs on the pump, but now I am glad we did not try to go to 20 hrs with a break...that break might have been serious with respect to his sugar levels.

Other than that, it was a quiet day. Hospitals on the weekend usually are. The boys' grandma came up and had a nice visit and Nicholas worked on all his homework he missed last week. Hopefully my mom can take it in to school Monday and pick up the work for the upcoming week to make sure we do not get too behind since I am not sure when we will be able to go home. Right now, we are hoping for late Monday or sometime on Tuesday. The biggest event of the day by far was Doug meeting a LifeFlight paramedic in the dining room at dinner tonight and getting an invitation from him to come up to the helipad with the boys to see the LifeFlight helicopters. The boys were THRILLED! They got to sit inside and get pics and we posed for a family pic in front of the helicopter. Once I figure out how to get them off the camera, I will definitely post them! I tell you tho...that helipad is interesting. It is on the roof about 12 floors up and there are NO railings at all! You can walk right to the edge and if you are not careful, down you go. I was watching the bigger boys like a hawk to make sure they stayed well away from the edge!

Guess that is all for today. Hopefully we will get to see drs who know Nicholas during team rounds tomorrow so we can get some questions answered from what was going on today. I just want everything sorted so we will be able to take him home with no concerns.

Thanks again to everyone who follows our sweet little boys. We sure appreciate all the good thoughts and prayers. Take care and God bless!

Melody

Friday, September 26, 2008 10:15 PM CDT

Well, we had a day full of questions today.

Nicholas' 6 am blood sugar check came as a surprise. It was 69, when it has usually been 85-99. Nobody knows why...and he was on feeds at the time. The rest, thankfully, have been fine.

We have heard conflicting things from GI, nutrition, and our team of drs regarding the goal feed rate and if we need free water or not and if we get a break from the pump or not. It did not make a difference to us, but we just needed someone to make a decision and stick with it. Finally we got a final answer. 55 ml/hr as the goal rate and stay on the pump 24 hrs a day to make sure we have no blood sugar fluctuations. No additional free water as we opted to let him try clear liquids again. So far, so good. He had a few sips of beef broth, a popsicle, and some jello...and LOVED EVERY BITE!! He was SO excited to eat again!

He had a Fundogram done. This is a test to make sure his fundoplication (reflux surgery) was working properly. They use barium, but did not fill his stomach quite enough to be conclusive...no reflux was seen with the amount they put in. He also had his j-port tested to see if he had any flow back into the stomach. There appeared to be a partial backflow...but that was not a surprise as we already knew he had intestinal bile backing up into his stomach from before anyways.

Now, GI wants to do a gastric emptying scan, but by the time they decided this, there was no time left to order one today. They do not do these on the weekend, so we have to wait until Monday. We will be in until at least then to make sure we get this one last test done. This test will determine how well his stomach is working (or not working) by determining how quickly his stomach empties any food that is put into it into the intestines. We already knew his was slow, but this will tell us how much slower, if any. I am SO bummed about having to stay, but will do whatever is best for him to make sure we are ok to go home and do not have to return. So, we are in for at least 3 more days...UGH.

In between all this, we had Doug and Nathan up here with us today. It was nice to spend the day as a family. The boys all played in the playroom with one of our Mito friends and had a great time and then went down to his room to play some more. We also got to meet a couple of people I have met online who were in for an appt with Dr K in clinic today. It was nice to put faces with names!

Weekends in the hospital are always long and boring as nothing happens and time seems to drag. Hopefully Monday will come quickly and we will be home later that day. I am trying to stay optomistic.

I talked to the asst. principal at Nicholas' school today. We need to call another ARD meeting if we can before Nicholas returns to make sure the new changes his dr wants will be implemented. Dr K feels we need to be in regular contact with the aide assigned to Nicholas to make sure we get a report on how he is doing. She agrees that we cannot let this happen again. I hope the school will be supportive and assist us with all the changes that need to happen. I want him to be in school and be happy, but not at the expense of his health or level of function.

Thanks for all the continued prayers and support. We appreciate it more than you know. And, if anyone wants to come by for a visit, please be sure to stop by! We are in room 1035 at Memorial Hermann Children's Hospital. Take care and God bless!!

Melody

Thursday, September 25, 2008 11:39 PM CDT

Well, today started off on a really bad note. My poor mom called me in the process of trying to convince Nathan to go to school. He was going back and forth between wanting to go and not wanting to go. She thought maybe talking to me would convince him to go. Well, that was a big mistake. He was so sad and said in the most quiveriest voice imaginable "I want mama daddy" and started sobbing hysterically. Then, I started bawling because it upset me and my heart was broken in a million pieces. My mom dropped him off and then SHE started crying too. Doug called right then, so I have my mom on one phone crying into my one ear and Doug on the other phone in my other ear and I am bawling to him. Then, Dr K walks into the middle of the whole sorry affair. We quickly decided that Doug would use his lunch break to pick Nathan up from school (where he did just fine and had a good time, I might add) and bring him up to the hospital before going back to work. So, I had all 3 boys up here this afternoon. We later realized that most likely the reason for his hysteria was that he was afraid that daddy had left him and then Nana was leaving him too (at school). Poor guy. :o(

Poor Dr K...she must think I am an emotional wreck or something as she has seen me cry twice now this week. We talked about the school letter she wrote for Nicholas and about changes that needed to be made to it. We decided he will likely go back on a shortened day and then try to work up to a full day with a rest period if we can. Nicholas woke up choking a couple of times last night, sounding like he was refluxing. He described it to her and she is worried...especially since his cough worsened since yesterday. She asked GI to come by and see us because of this. She saw my scope results and was not thrilled. She says she worries about the stress I am under and the effect it is having on me. Other than that, she is pleaced overall with the progress we are making. We just upped his pump feeds to 55 and seem to be doing well, aside from one episode of diarrhea. Not sure what that is...I am hoping it is not a sign of intolerance. It could be due to Miralax, tho he only had a tiny dose (a fraction of the usual dose he gets). Who knows.

GI came by and we discussed options as far as testing for the reflux episode last night. So far, only a KUB (abdominal xray) was ordered to check the placement of the J port wire. No word back on that yet. He will let us know tomorrow what else we may or may not be doing.

Nicholas and I went with Daniel to the playroom this morning and had a good time doing crafts. He made 2 name signs for his room door "so the drs will know where to find me". Once Nathan came, they laid in bed together and watched 101 Dalmations and then all 3 boys laid down and took a nap. I just sat and enjoyed the peace and quiet.

This evening we had a nice visit from my sister and then Doug took Nathan home. Daniel decided to throw a screaming fit that could have woken the dead and likely woke more than half the hospital. He was so overtired there was no consoling him. He finally fell asleep. Nicholas' breathing treatment was delayed by a code, so he just got to sleep as well.

I hope we are getting close to the end. We are hoping for a Saturday discharge if all goes well. Guess we will see! Thanks for all the good thoughts for us. We truly appreciate them. Take care and God bless!

Melody

Wednesday, September 24, 2008 11:41 PM CDT

Well, another day in here and more progress made. We are currently running at 30 ml/hr and he is tolerating it ok. He is not asking as much about food anymore...I am so glad. Poor boy. He is happy now that he has a new chewy tube to gnaw on and he is getting his need to chew on things satisfied now.

We met with nutrition today and he will be going home on maintenence feeds that are 8 oz of free water too little...so now we worry about finding a way to squeeze 8 oz more into him. We were going to go home on 24 hr feeds at 55 ml/hr, but will now be trying to go to 65 ml/hr to try to give him 4 hrs off the pump at the recommendation of the nutritionist. I am thrilled with this as 24 hr feeds sounded really hard.

Nicholas and our other litte mito friend got to see a poppet show tonight put on by Child Life. He had a great time. It was a welcome distraction that he looked forward to all day. Meanwhile a friend of mine brought dinner and goodies to me and our mito friends here so we ate while our kiddos who could not eat were distracted. It was so nice to visit and have some adult girl time. Thanks so much, Joy!!

He woke this morning running a slight fever and had a barky croupy sounding cough. So did Daniel. We are all overtired and run down. After upping breathing treatments and adding in Xopenex, he is sounding a lot better.

Dr K came by again and sat with us. She was not happy to see him looking puny again. Between the fever and getting a bath (that wore him out!) he was wiped out. She was also concerned about me, saying she was more worried about me today than him. I am hoping today is "hump day" for this stay and we will be on the downside of things soon. She wrote a new letter for school, so I hope this one will help. No more PE for him and we will be shortening his day for a while to get him adjusted. He is likely to not be able to return early next week due to him needing to rest at home. We will need to try to call an emergency ARD to make some accomodations to his IEP before he can return.

I am worried about Nathan though. Things are not going so well with him according to my mom. He is so sad and keeps telling her "I don't understand...what is taking daddy so long to get me? Why isn't he here yet?" and she said he cried for almost 30 min before bed tonight. His chin quivers and he tears up every time she mentions one of us. So, Doug is leaving work early tomorrow, going to get him to bring him up here for a while, sleeping with him at home tomorrow night, and then taking Fri as his off Fri and coming up here. He will likely have to work his way out of a hole for hours, but we cannot leave Nathan like that. Mom says he knows we are all together and not with him and I bet he feels like we left him. I am just heartbroken. Tomorrow I will see him again...I miss him.

Thanks for the good thoughts for our family. Take care and God bless.

Melody

Tuesday, September 23, 2008 9:18 PM CDT

So, today sure started off quite interestingly. Nicholas had to get his blood sugar checked at 3 am. Yes...3 am. Blech. To make matters worse, the machine was not working...so, the nurse and the PCA had a huge discussion over the machine (loudly I might add) with all the lights in the room blazing. It took them 20 long and loud minutes to sort it out and then the PCA decided she might as well do the 4 am vitals while she was there and had to check the blood pressure several times since he runs so low at night. All his vitals do (breathing and heart rates and blood pressures...even his temperature). This is because his body is conserving energy any way it can and does not need to run optimally while asleep...so it saves what it can and gets by with the bare minimum. What would be alarming vitals for the average person are actually normal for Mito kids while asleep. By the time this 30+ minute ordeal was over, Daniel was wide awake and crying and the PCA commented to me "not much rest, huh?"...Ummm...YA THINK?!?!?!? What a way to start the day...

So, I think I have had less than 10 hrs of sleep in the past 3 mights...*sigh*

Anyways...today we started feeds at noon. 5 ml/hr went well and we are currently at a rate of 10 ml/hr and doing well with that! YEAH!! As soon as feeds were started and our team rounded, we got sent upstairs. YEAH again! We were in the IMU (intermediate care unit) which is the unit that is less care than the ICU but more care than the regular floor. Thus, the reason we were constantly monitored for blood pressure, temp, heart rate, oxygen saturations, and respirations per minute. We have a standby monitor up here, but no need to use it. And, we have orders to do NOTHING to Nicholas while he is sleeping...no vitals, sugar checks...nothing! YEAH!! Maybe now we can actually get some sleep!

Dr K rounded with the GI for the first part of her visit. We talked quite a bit about oral feeds and have decided that if he reaches his goal rate for feeds with no major problems, we MAY opt to stay another day and let him try to eat while inpatient and see what happens. By that time, his stomach will have had a week with nothing in it and may tolerate little bits of food. YEAH!! At this point, he has mostly stopped asking to eat and only had one episode of tears about eating tonight. He has been chewing everything in sight tho...the edge of the emesis (puke) basin, the clip on his pump packpack, the flap on his hospital bracelet...he was even biting his own skin. The drs said he is trying to fill the oral void left by not being able to eat...he just wants to chew as if he is eating.

Once GI left, Dr K sat down with Nicholas and had a talk. He told her how tired he was when school was over and we talked about our options. I am not sure what we will decide to do, but something has to change. This cannot happen again. she was glad to see him looking perkier today and told him he had scared them pretty bad yesterday. She also gave him a stuffed monkey and ordered him out of bed and to play with one of our other little Mito friends here in the playroom as Nicholas has not felt up to getting out of bed since Sunday. We did go and play and the awesome Child Life ladies in there hooked Daniel up with some toys for the room when we left. Now maybe he won't be so bored anymore. YEAH!!!

This hospitsl is COLD...freezing, sub-zero type of cold. Daniel has been refluxy and wakes up cold in spite of his blanket sleeper. All three of us staying here now have runny noses, scratchy throats, and the boys have a cough as well. Just what we needed.

Nathan is faring much better. He is styaing with my parents until we are discharged to keep him as scheduled as we can. He was getting so run down from the running around and going from here to there and hack again. He did well today, but was super quiet. I am sure he is exhausted and he will now be able to catch up. I cannot wait until we are out of here and I can see him again!

Guess that is all for today. Tomorrow we are staying the course we are on and hoping to continue tolerating feeds well. Thanks for all the continued prayers and offers of support. We truly appreciate them. Take care and God bless!

Melody

Monday, September 22, 2008 9:51 PM CDT

Sorry for not posting an update sooner. Today, nothing went as planned and I barely had a minute to sit down.

They came to get Nicholas for surgery this morning around 7 and when we arrived downstairs, there was no GJ tube to be found. We were delayed waiting for that. The anesthesiologist came out to talk with us and it came out that he had NO CLUE Nicholas was a Mito patient and needed MH precautions for anesthesia b3ecause the resident who saw us last might did not get that info from his chart. He flipped. He did not have a "clean" machine (no gasses used in it) and he did not have any of the approved meds on hand, so he was scrambling to get what meds he needed and a clean machine from somewhere since they did not have one. They decided to bump us and take the patient behind us ahead of us to give them time to make the proper arrangements. So, we sat in his bed in the hallway and waited. He finally was taken back around 9:45. They had trouble placing the tube as the release string got caught and they had to remove the tube and start over again. His scope looked great though and we hope the biopsies they took will look good as well.

In the PACU he woke up well, but then got violently ill from the anesthetic they used...it is now on our NO NO list. He retched (like dry heaving) for ages, but since he had the fundo surgery as a baby he cannot throw up. So he just heaved for what seemed like forever. Finally we got him hooked up to a venting tube and 2 doses of Zofran later we thought we had it covered.

On the way back to the room, they called a code pink (missing baby) and all the elevators shut down, so we were stranded on the 6th floor (we are on the 9th). As soon as we finally got back up to the room, he started retching again. We hooked him up to a gravity drainage tube and he is now draining a lot of dark green bile. Blech.

Things finally settled down for a while and then their Mito dr came in. Dr K was there for all of 5 min when he went into another violent retching episode right in front of her. I think it really freaked her out. He was turning purple from the effort. She ran out and got the nurses scrambling for more Zofran and once he got it, he pretty much passed out from the exhaustion. After sitting and talking to her I think we have come to terms with the fact that he just cannot manage full day Kindergarten right now. It is just too much effort and energy for him. So, we will try cutting down his day some to see if we can get him used to it enough that maybe we can add in more time later in the year.

As of right now, the plan with GI is to wait until noon tomorrow to start feeds. We will start with 5 ml/hr and sit on that for 24 hrs to make sure he is going to tolerate J feeds. Then, we will advance the feeds by 5 ml/hr every 6 hrs as he tolerates it until we get to 55 ml/hr. He will need to be hooked up to the pump 24 hrs a day. If all goes as planned we are looking at Sat morning for discharge right now. It is going to be a long week.

We sat down with Nicholas once he started begging for food and told him he cannot eat right now. He is just devastated and cannot understand why he cannot eat when his tummy feels fine now. It feels fine because there is nothing in it. If he were to eat, he would go right back to feeling bad again. He is not understanding this. There were a lot of tears and "it's not fair"...and you know what? He is RIGHT. It isn't fair. It just plain STINKS. After years of fighting to get him TO eat, never did I think I would find myself fighting to get him to leave food alone. I dread to think about needing to send him to school when he cannot eat.

Please pray that the feeds go well and he will become more accepting of not being able to eat. Eating is such a normal part of our lives that it is going to be a major adjustment for him to lose that. Please also pray for us to have the right words to reassure him when he gets upset. He has a long road ahead of him right now.

Nathan, thankfully, is feeling much better today. The ped thinks it is something viral, but put him on Amoxil as a precaution just in case. He is going to be hanging out with my parents this week while Doug goes back to work tomorrow.

Thanks for all the prayers and thoughts. I will continue to keep everyone updated as I can. I am hoping for a quiet day tomorrow. Take care and God bless!

Melody

Sunday, September 21, 2008 4:35 PM CDT

UPDATE: It looks like we are booked to go to the OR at around 7ish Monday morning for the GJ placement and possible scope. He will be placed under general anesthesia.

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Well, Nicholas is in the hospital. I am sorry I have not kept a better update, but between Nicholas' health issues since starting school and the hurricane, I have hardly had a minute.

Just to give some background...Nicholas started school about 4 wks ago. We have been dealing with an overall decline in his GI tract for about 3.5 of those weeks. He has come home exhausted and wakes the next morning complaining about how tired he is even before getting out of bed. His behavior was awful and his health was suffering. Nathan caught a GI bug Nicholas brought home from school and was hospitalized. Nicholas was sent home from school with diarrhea, likely due to the same bug, but seemed to do ok with it. Since then, things have gotten bad. We have dealt with cramping, nausea, retching, belly pain, bloating, and a general intolerance of anything in his stomach. It all came to a head yesterday when he was unable to tolerate straight pedialyte without bloating. That is when we realized we were not able to do anything more for him at home and the drs wanted him admitted.

So, the general concensus right now is that Nicholas' stomach is not working...or if it is, it not working well at all. He is not able to tolerate anything in it without bloating and other issues cropping up. He needs to be vented every day. We are unsure if this is a result of how tired and energy depleted he has been from starting school, residual effects from the virus he had wreaking havoc on his GI tract, a combo of those 2 things, or something totally different. His GI is looking at placing a GJ (gastro-jejunal) tube tomorrow to see if bypassing his stomach altogether and feeding into the small intestine would be a better option for him right now as it would allow him the ability to have his stomach rest. The downfall to this is that he would need to be on the pump 20 hrs out of the day (which complicates school that much more) and either lose oral eating altogether or decrease what he can eat by mouth. This is expecially upsetting after how hard Doug and I have worked to get him to eat by mouth in the first place. It kills me to see all that progress lost. I will know more details tomorrow about what is going to happen, but I will keep you posted. I am guessing we will be in for longer than a few days as we were admitted to the IMU and not the general floor. This is the level of care in between the ICU and general care.

Nathan has been running fever on and off. He is acting ok though he has watery eyes, a runny nose, and a cough. We are hoping he will improve on his own without intervention as I am not sure I could handle having 2 kids inpatient at once. Thankfully, Daniel is ok. He is staying in the hospital with Nicholas and me as he is still nursing.

We are in the hospital with 2 mito friends of ours, so at least we are in good company. They have really broken in the staff here for us!

On a side note, everyone in both our families have power back now from the hurricane. We are so very thankful for that! Take care and God bless. We will keep everyone updated as we can. Thanks for all your thoughts, prayers, and support.

Melody

Saturday, September 13, 2008 12:48 AM CDT

Oh my goodness what a night! It was pretty scary...and we were far from the worst of it. We had wind gusts that we estimated to be at least 75 and probably higher and sustained winds we figured were around 55. We lost power overnight, but got it back around 6. Apparently we are one of the few who have power in the entire area. When the power went out, Doug went to get Nicholas up (he PANICS when the smoke detector beeps as the power goes out and he was totally FREAKING and screaming) and once he came into our room, Doug had to go back and get a screaming Nathan since I was feeding Daniel. While he was gone, Nicholas and I heard this giant ripping sound. Turns out the screen on our bedroom window just ripped off the window...and it was screwed in with EIGHT screws! Doug ran outside to get it before it blew away and caused damage.

We lost some shingles and our gate was damaged. We also have a rain gutter that is coming loose from the eaves. There is debris everywhere and limbs in the yard and street. Thankfully, we are all ok and the house did not sustain any major damage. My parents and sister and family, and Doug's mom and brother and family are all ok.

What a scary night. From the reports I am hearing on the news, this storm was worse than they expected. Entire blocks of Galveston burned to the ground as nobody could reach the houses to fight the fires. They are unsure if people were in the burning houses as 60% of the island stayed. The hotel where we stayed to escape the painting fumes had an 80-90 ft section of brick facade just torn off the wall. UTMB (hospital) in Galveston was under 11 ft of water sometime last night. The JP Morgan Chase high rise downtown lost most of it's windows. The hospital where Jen and Ali are had windows broken out on the 8th and 10th floors and the roof tiles were flying off and some broke the glass roof of the atrium. Somewhere near Jasper, there was a tornado spawned that was travelling 92 mph!! The roof of Reliant arena has giant chunks of the retractable roof missing...the damage is bad.

It has been a wild night for sure. Now we wait for the rain to stop and the winds to die down and see the damage that has been done.

Here are some pics of our yard and street.

Down the street to the left...everyone is coming out to survey the damage...

Down the street to the right...

The backyard...swingset and patio...

Our MN friends are struggling even more with both their Mito kiddos. Our friend who is inpatient at our hospital was intubated last night, but in stable condition. Please continue to pray for both of them and also pray for all of those affected by Hurricane Ike. Take care and God bless...

Melody

Friday, September 12, 2008 8:48 PM CDT

Well, here we sit waiting for Hurricane Ike to arrive as our zip code is a "shelter in place" area. The cars are in the garage, the things on the patio are up against the house, we have all our storm supplies, and we are as ready as we will be I guess.

We are expected to get winds up to 110 mph even way out here where we are. It has been really scary to watch the storm and what it is doing on the news all day. It is a huge storm...almost 600 miles across. The eye is at least 50 miles wide and expected to pass right over us. The storm surge is expected to be at least 20 feet high and they are projecting the storm surge will go as far as 20-25 miles inland. There has been one casualty already and numerous houses and buildings have already either washed away or burned to the ground. Flooding is bad and the outer rain bands on the storm just started moving inland. 250,000 people already without power already. 22 men stranded on a tanker ship and adrift in the gulf at the mercy of the storm. It is going to be ugly.

We have the boys godmother with us as she lives by herself in a 3rd floor apartment and we wanted her here with us to make sure she would be safe.

The boys are hanging in there. Nathan has recovered mostly from his tummy bug and is doing ok. He is drinking fine, but the appetite is still slowly returning. Nicholas is having a bit more trouble. His GI tract is going haywire somehow and his motility is slow and he is bloating badly and retching. He is not tolerating his feeds well at all and we are struggling with that. We could have taken him into the ER for evaluation, but then we would have to be separated during the storm and that is not what we want. They are only allowing 1 parent to go with each child who is a patient as the hospital is on lockdown. He is doing ok for the moment and is holding his own, so we will keep the status quo for the time being. I only hope we do not lose power for long as Nicholas needs his tube feeds via pump and both older boys need nebulizer treatments, each of which require electricity.

Please pray for us and everyone in the path of the storm. This storm is turning out to be worse than they thought it would be and that scares us. I will update as soon as we have power back and let you know how we fared through the storm. Please also pray for Landon and our MN friends as they are in need of prayers at this time. Take care and God bless!

Melody

Friday, September 5, 2008 10:51 PM CDT

Update Sat Sept 6 (Happy Birthday Colton!)

Nathan was discharged today. He is keeping down food and drinking well enough to be home now. Nicholas is still having some diarrhea though, so he is back on the pump...I hope it is gone for good soon. Thanks for all the good thoughts and prayers.

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Nathan was admitted to the hospital today for dehydration and fluids. He started vomiting yesterday afternoon on the way home rom PT after not acting like him self in the later morning. Well, we thought we had a good handle on the vomiting and got him to keep down a Gatorade juice box before he went to bed. I checked on him before I went to bed and he was awake...and lying in a huge puddle of "throwed" (as Nathan calls it). Apparently the entire juice box came right back up and he had not told us and laid in it so long his feet were pruny from the moisture. That plus the fact he had a diaper change at 2:30 and did not pee in it until 8:10 and had not peed again by 11:15 made us make the decision to take him in. Nathan and I arrived at the ER in the med center (where Jen and Ali are with their boys) just in time for him to pee again AND vomit in the waiting area.

The started an IV and took blood and were bolusing saline since he was pretty dehydrated. The IV lasted about 30 min before it blew and Nathan went into screaming crying hysterics over it. They decided to call in transport to start the next one and, after FIVE hours, they were still "too busy" so the new shift nurse started one and he was finally able to finish his saline bolus and perk up some. Dr K and L came by at 11 and took one look at him and said "He looks terrible...you're staying."...so, here we are finally up in a room. He is running D5 and finally looking ok again. Dr K said even his skin looked dry and Nathan would not even interact with her at all, which was so out of character for him as he *loves* her. He also started in with diarrhea while she was in there.

I am hoping to only be in here overnight as we are sooooo ready to be home already. Dr K told us to keep Nicholas away if we could to make sure he stays well. Too late...Doug had to leave the ER this morning after Dr K left as Nicholas was getting sent home from school sick with diarrhea. Seems a bug is going around in his class and quite a few are sick or went home sick. Pray for Braden as he is still ok. We think Nicholas brought the bug home and Nathan caught it and we think it also explains Nicholas' recent weird stooling issues.

So, in the bed earlier were Nathan with his IV running fluids and Nicholas on his pump on continuous running pedialyte. They were super quiet watching Sesame Street and looking pretty puny. At least Nicholas is doing well enough to not be admitted, but anyone who knows my kids know they are NOT quiet unless sleeping, so we are hoping this bug passes soon and does not take anyone else down.

Please pray for the bug to pass quickly so we can get home with the rest of the family soon. Take care and God bless!

Melody

Thursday, July 31, 2008 4:01 PM CDT

THANK YOU to everyone who visited and prayed for our friends. He is now doing MUCH better! He is out of the PICU, off of the ventilator, and officially "out of the woods"!! It turns out he had a really bad septic infection of yeast. He is a true fighter. Now begins his long recovery and the family could still use your prayers.

Nathan currently has pneumonia and has been pretty sick. We visited the Immunologist yesterday for our regular 3 mo follow-up. Nathan was the first order of business. We had gotten the xray report from the ped to take with us to make sure she got a copy. She said, from the sound of the report, that it looked like he had a viral pneumonia in both lungs. She was going to check out the xrays herself after we left and compare them with the ones he had back in March to see if it is the same thing or not. She listened to him and he was wheezing, and she saw how hard/fast he was breathing, so he is now officially dx with asthma. Poor kid. So, he will need to take prednisone for 5 days (she apologized for rx it as he gets so moody on it) and we will need to do breathing treatments 2x/day every day now instead of only when he is sick. She also gave us a rx for a peak flow meter and we have to test him every morning with that as well. We are to continue wth the abx as well. She said no hospital since she does not want to risk him catching something else right now, so I am relieved that we get to stay home. He is doing a bit better today anyways, so I don't think he would have been admitted anyways. Dr K (neuro) had already told Jen that if we were admitted we would have to be as far away from Landon as possible to avoid getting him sick...guess she knew we would have liked to be close for the company if we were both in...lol! Smart Dr!

Nicholas came next and she was really happy with how good he looks now that he has put on a bit more weight (as we are not constantly trying to tube wean anymore). He has been healthy (knock wood) and doing well despite his last Ig levels coming back not so great...but I will take low levels to have a kid who is otherwise doing well!

Daniel came last. She was happy that he was looking good this time, too (knock wood again). His one ear was a tiny bit red, but she felt it was due to healing and was not concerned. She nixed the idea of starting him on solids as soon as it came out of my mouth (even tho everyone else was ok with it), so we will wait until 6 mos for that like we did for the other boys. The good news is that, even tho his IgG is low, his IgA which was non-existant last time was now in the 20's! WOOHOO!! His is better than Nathan's! So, that is wonderful news to see his immune system kicking in. She said at our next visit, or at 6 mos, whichever comes first, she wants to start him on Singulair for allergies as it looks like he is going to be the allergic one of the bunch.

Nathan had to pee in a cup and got sent for bloodwork for the 2nd time this week and was anything but happy about that. She was going to get Daniel poked again for more testing, but when she heard he had just been poked already on Mon (which BTW came back FINE so they got it this time! YEAH!), she said she would wait until he got more bloodwork ordered as he is a hard stick. Before we dropped the urine off, she had her nurse (YES SHE HAS ONE NOW AND SHE IS NICE!!!!!!) do a quick test on it to see if it looked like he had a UTI, ketones, or was dehydrated and it came back looking ok for all those things. So, I am glad we avoided the hospital with him.

I have more updates, but will end here for now. I hope this update finds you all well. Take care and God bless.

Melody

Tuesday, July 22, 2008 10:29 PM CDT

I will come by and add a full update soon...I promise. Right now, we have friends who are in desperate need of prayers. They are friends of ours who see our Mito specialist (and most of our other specialists as well). They just moved down here from Missouri and live down the street from us now. Their 3 yr old son, Landon, is fighting for his life in the PICU right now due to sepsis and acute liver failure. He is very ill. We have been watching their older boys so both Jen and James could be at the hospital with Landon. PLEASE pray for them. Here is his site if you want to check it out...

www.caringbridge.org/visit/landonweber

Our MN friends are in need of prayer right now as well.

Thanks so much to everyone who comes here and prays for us and our friends. Take care and God bless.

Melody

Thursday, June 12, 2008 0:02 AM CDT

Ok...I have to say I just LOVED both Dr J and Dr B (the endocrinologists we saw today)...but man..Dr B is amazing! He is retired, but comes in to consult on cases that intrigue him. I guess our Mito group is a really interesting bunch! We are very lucky to have him consulting with our boys. He just commands attention and respect and REALLY knows his stuff! I guess after doing this for 30+ yrs, you get good at what you do.

Basically, the boys are bone growth delayed, but nobody knows why. Bone age delays tend to mean the body was stressed at crucial growth times and the growth hormone was somehow suppressed. Since Doug and I hit puberty at a "normal" time, we know we were not bone growth delayed, so it is not from that. They are hoping the delay means they will have the potential to continue growing beyond their same age peers for the amount of the delay they have, but because of the Mito, they are unsure if this will be the case or not. If the stress on the body causes growth hormone suppression, would that continue to happen during puberty and not allow the body to "catch up" as would be the case in someone without Mito? They don't know...

Because the boys have never had any growth hormone workup, they had that done today to make sure that was not a factor in the delay. Nicholas is 2.5 standard deviations below where his "average" height would be at this point and that alone would qualify him for growth hormone replacement therapy. Nathan is not to that point as of now, so he would not qualify unless his bone age delay continued to widen the gap.

A mito mom friend of mine and I were talking the other day about her daughter before the results of the bone scans done after her arm fracture were back (came back that her 9 yr old has Osteoporosis) and I realized that Nicholas has had some breaks (4) by the time he was 3 and thought I should mention that at the appt. They thought it was concerning and are checking his Osteocalcin (?) levels in his blood to see if the bone activity is normal or not and that, plus some other things, will help them decide if Nicholas needs a bone density scan or not to check him for Osteoporosis. Nathan is not a concern at this point as he has not had any breaks.

We talked about the appropriateness of growth hormone replacement therapy and there were several good points raised. First, since the IGF (growth factor) has to do with insulin, how would adding extra insulin to a body predisposed for diabetes (because of the Mito) affect the system? Second, how would adding in growth hormone to an already energy diminished body affect other systems? It would be like robbing the energy from Peter to pay Paul, so would this possible redirection of energy cause crisis elsewhere? They don't know, so these points would have to be researched and considered before starting on any replacement therapy. That is ok with me as I am not convinced of replacement therapy at this time anyways...

They also decided to recheck Nicholas' thyroid function because his previous testing came back showing a low TSH with normal T4, so they want to recheck it just to be sure. They think it was a lab error, but want to double check.

So, we left with a bunch of bloodwork to be done and now await the results to see where we go from here...

Nicholas' gtube site is still a yucky draining mess. We have NEVER had a leaky tract since the tube first went in and was healing...now it is draining tons of goop and drying into major crusties every day. Sometimes the drainage is mixed with blood. We had granulation ONCE as it was healing right after placement and Silver Nitrate sticks did the trick. We now have Silver Nitrate resistant granulation apparently as the GI tried to burn it off last week Mon (9 day ago) and it never went away! We have been using Silvadene (abx) cream and also Traimcinolone (steroid) cream with NO success either. It has been like this for almost a month with no signs of letting up. The GI is at a loss...UGH!

I am sick of drainage all over everything. I am sick of his site looking like a nightmare after over 4 yrs of perfection. I am sick of him wincing in pain and guarding it every day because it hurts for us to be constantly cleaning it. I am sick of Nicholas now being afraid to let anyone look at it because he KNOWS they are going to have to make it hurt him.

We ran into the GI nurse after our endo appt today and even he had no advice for us except to try changing out the button...well, I would, but I am afraid it is going to KILL him to do it as he is in so much pain from just us leaning it a tiny bit to look under it or to clean it. The best that we could come up with is to hope that the chlorinated pool water he will be swimming in for the next 2 weeks for his swim lessons will help clear this mess up. I think I am going to have to put him in a swim shirt every day tho as I am afraid anyone who looks at it is going to be completely grossed out by the sheer looks of it. It is NOT infected tho (much to everyone's shock) as we have had it cultured 2x already with nothing growing either time. Poor kid... :o(

Tomorrow is therapy for both boys and we are going to a local UMDF support group meeting in the evening. Our mito dr asked us to go so we will be going to check it out...and see her, of course! Before we leave, we will be going to look at a house for rent on our street since our MO friends are moving down here and think this place would be the perfect fit for their family! It would be amazing to have them living so close...our kids would attend the same school and ride the bus together, they can play with each other, and we would both have people close by in case of emergency with one of our kids. I am praying that God will allow the plan to unfold as it is meant to be and pray that this house is in that plan for them. One of the bedrooms is adjoining to the master bedroom, so their son would be able to sleep right next door with the option of leaving that door open at night to ensure his health and safety...so it would be a perfect set-up. Praying, praying, praying...

Thanks for checking in on us! We so appreciate each one who cares about us enough to take a peek at our site! Please sign the guestbook so we can see who has been here! Take care and God bless!

Melody

Saturday, June 7, 2008 1:17 AM CDT

Well, what a difference a week makes. Our MO friends who are inpatient are going home tomorrow...or should I say today?? YEAH!!! Thank you for all the prayers...they have worked!

Nicholas' tube site is looking better. But, better from what it was is still not great yet. I wish I knew what was going on. We saw both our mito dr and immuno when we went to visit our friends at the hospital earlier and they both wanted to follow up on the 2nd culture that was done. I am not sure if it is looking better enough to avoid having to go to a tube temporarily. I guess we will find out on Mon.

I am feeling a lot better. I still have a cough, but it is now a fraction of what it was. Our mito dr was concerned when I emailed her an update of that appt and asked for copies of all my PFT's. UGH. I hate this...I was not too worried about it until the mito dr was concerned. Now it makes me wonder what concerns her. We are waiting on her to review those records to find out what she thinks.

Daniel had another vaccination today. He has been crabby and fussy, but he was so brave and did not cry. What a way to celebrate your 3 month birthday. Here are a couple of pics so you can all see how big he is getting...

Nathan has been "off" for the past 2 days. I cannot put my finger on why tho. He has been running slight fever, but has NO other symptoms of anything, so I am assuming it is a temp issue. Maybe that is what is making him feel so puny, but I cannot find any other reason for it. So, we wait and see what happens with him.

The mother's group I am a member of took a "field trip" to one of the local fire stations yesterday. It was a lot of fun, tho HOT and the kids got to see what a fireman looked like dressed up in the full gear, including their head covering and air mask. Here are some pics...

Hearing the fireman speak...

Hearing about the pumper truck...

Daniel in the sling hanging out...

Checking out the goodie bags the fireman gave them...

Fireman Nicholas...

Fireman Nathan...

Nicholas also had his belt testing yesterday. He earned his black stripe belt in the cubs program. The next belt he earns will be the graduate belt and he will move up into the juniors program! He is so excited and so proud of himself as he has worked VERY hard to get this far...

Catching the ball. Those who know Nicholas know how big of a deal this is...it took him years working on this in therapy as his arms are the weakest part of him. He caught it on the FIRST TRY! I so appreciated the instructor too as he stood closer to Nicholas and threw it a bit easier to him than the others. It meant the world to him to have been succesful. This is one of the reasons I *love* this place so much...

Waiting for the belt awards ceremony to start...

Receiving his new belt...

So proud of himself. He is so red-faced here because the air was out in the practice mat area where the testing and awards ceremony was. Poor kid was sitting there in long sleeves and pants with an undershirt on as well...

Posing with his 3 favorite instructors (the 2 girls just graduated high school and will be gone in the fall :o( )...

Showing off his new belt...

So, that has been our week thus far. Other than that, we have laid pretty low and done a lot of resting and catching up on sleep. The boys are sleeping in until 10-11 some days and then still taking 3 hr naps in the afternoons. I hope they will be caught up soon...theyhave just been so exhausted.

Thanks to everyone who visits us. We so appreciate all the support. Please take a minute to sign our guestbook if you stop by as we like to see who has been here. We also really like making new friends, so please sign even if you haven't before. Take care and God bless!

Melody Pohla

Monday, June 2, 2008 9:41 PM CDT

Well, what a day it has been.

We started off with me taking the boys to my parents' house so they could have lots of fun while I went and had allergy testing done. Daniel went with me since I figured he needed to be with me in case he got hungry, but the older boys went and had a LOT more fun than me! I knew the appt was not off to a good start when the nurse wanted to check my peak flow (asthma test) and it was NOT good. I had to do the PFT n the computer and that was not good either. So, the dr was concerned and had the nurse recheck me even before I got any allergy testing done. The 3rd round was just as bad. Then, the nurse went and gave me 56 pricks in my back. I was not even reacting to the histimine, so I had to get another histimine and boy did that one react. Nothing showed up on the skin testing so they moved on to the intradermal testing. 32 needle injections later, the dr decided my PFT's were so bad I needed an albuterol treatment. I got an inhaler and had to redo the PFT's on the computer and thankfully, they were a bit better. When the allergy testing was done, I reacted only to ONE mold and not enough for shots!! YEAH!! This is a miracle for me as I have been taking allergy shots more than half my life. Guess they actually DID desensitize me! Then, the dr decided I needed a CT scan to check my sinuses. Big surprise they were 50% blocked and really inflammed and swollen. Her theory is, that with the pg, each time I got sick I never got back to baseline and the baseline got lower with each illness. Now, my baseline is so low that the sinus infections are affecting my pulmonary function. My PFT *WITH* albuterol is now worse than my sick PFT when I was first pg. So, she has loaded me up with meds in the hopes of getting me "over the hump". I am now on albuterol inhaler every 4 hrs as needed for my cough, pulmicort inhaler 2x/day, clindamycin for 20 days, prednisone for 3 days, and I have to come back in 3.5 wks for a rescan of my sinuses and to redo my computer PFT's. If they are good, I can go off the pulmicort and see how I do. So far, after 1 pulmicort and 1 prednisone I am breathing much easier and coughing much less. Now, to figure out how to keep from getting thrush and passing it back to Daniel in the midst of all these meds...

Then, it was off to the hospital for Nicholas' GI appt for his tube. UGH. I decided not to clean it up this morning as I think seeing the site so clean yesterday at the ER did not help the drs help us. Boy, was it NASTY by the time we went to the appt. We had just called the ID to find that the preliminary cultures came back negative so far and the GI was shocked...by the look of it he could not figure out how it could NOT be infected. So we rescured a piece of the crusted on drainage for them to reculture there and the GI used silver nitrate sticks on the granulation tissue to see if we cannot get rid of some of it. That made Nicholas scream, as the med burns the tissue to get rid of it. After several phone calls were made to talk to different drs and get several other specialists in to look at the stoma, he came up with a plan for us. He is keeping Nicholas on the abx just in case it is infected and also changed the topical from bactroban to silvadene, an abx cream used on burn patients. He wants us to change him to a regular foley tube until it can heal if it is not better in a week and he thinks Nicholas will need the next tube change to be done under some kind of sedation since it is so painful right now. That will also give him a chance to look around in the stoma tract and see what is going on in there. If it is bad, we may still need to consider surgical removal. We also asked for a script for a new pump so we can see if our current ins will get us one for a backup. Since they did not buy this one for us and have not paid for anything regarding a pump all this time, they hopefully will approve it. We also got a script for some foley kits to keep in the diaper bags so we have somethng in case of emergency...hopefully that will keep this from happening again.

No news from the mito dr about Nicholas' difficulty remembering things. It seems to come and go now. So, I guess we will see what happens with that.

Tomorrow is a day where we have NOTHING going on...so we will have a jammie day and relax and sleep. We all need it. Doug needs to actually work a full day at work as he has not done that since this past Wed due to all the hoopla over Nicholas' tube. I am so ready to get back to life as we know it soon. This craziness is really difficult on everyone. Thank you for al the prayers you have been lifting up for us. Hopefully, this infection will be gone soon and Nicholas will be feeling better. Take care and God bless...

Melody

Saturday, May 31, 2008 9:45 PM CDT

Well, looks like June is NOT off to a good start. UGH. Nicholas woke this morning with his gtube draining fromt he infection like usual, but instead of it being yellowish green as it has been, it was RED like it had blood in it. After some calling around to the drs, we finally located our mito dr who told us we HAD to get him into the ER. Of course, they had no explanation for it and decided it must be ok, so we were sent home. At least things went smoothly this time and we were taken right back...but we were also one of only 3 patients in the entire ER. I think it helped that our mito dr called them to tell them we were coming and "educate" the ER docs some. We follow up with GI hopefully sometime in the next couple of days.

Nicholas is also having difficulty remembering things right now and that is a concern as well. He got a bear on Friday night. He named it Charlie. He remembered it's name all Friday evening. Yesterday, he did not play with it. Today, he decided he wanted to bring it to the ER. All morning, he kept asking what the name of his bear was and we did not think anything of it as we were dealing with the ER staff and it was not something we had the time to pay close attention to. He continued to ask through the aftewrnoon. Sometimes we would catch him calling it the wrong name and ask him about it and he would start crying and ask what the name was again. We would then hear him repeating the name to himself under his breath. I think he was trying to make himself remember it. This continued on until after dinner and we were in the car getting gas and within a 5 min stretch of time he had forgotten 4 times and cried each time because he could not remember, even if I had just told him 30 sec ago. He was almost in hysterics by the last time and I just
told him before he could ask since I could hear him frantically whispering,
trying to remember and getting upset when he found he could not remember it. Nathan would say something to him while he was trying to repeat the name and he would answer and lose the name abecause he had stopped repeating it over and over, and freak out and get hysterican while he tried to remember. It was heartbreaking.

I called our friend who is inpatient to make sure that I was not overreacting and she could hear
Nicholas repeating the bear's name over and over and over again and he even made up a song to try and remember. He keeps wanting to call it Sophie, but refuses to rename it as it is a boy bear and not a girl...and yet he cannot remember the boy name he picked out and is so frustrated. It is hard to see him struggling to remember something. We call him an elephant because he can remember things from seeing or hearing them once. So, this is very uncharacteristic of him to not be able to remember something this simple.

I will update more once we hear something back from the drs about all of this tomorrow. Prayers would be gratefully appreciated. Thanks, everyone.

******************************************************************************
I, for one, am glad May is almost over. It has been a really rough month. We have had too many tests and major stress in our family, and 3 online friends who had or were being worked up for Mito passed away. The rest of our online friends have had a lot of stresses going on this month as well. I am hoping June will be a much better month for us and all our Mito friends.

Nicholas has an infection on his g-tube stoma. I think it started from the trauma of him getting the tube pulled out when he slid down an inflatable slide on his belly at a friend's birthday party 2 weeks ago. It was hard to get it back in for all the granulation tissue blocking the stoma, so the reinsertion was somewhat traumatic as well, but it saved him from going to the ER for a dilation to get it back in...that would have been worse. It is not looking or acting like the MRSA he had on it before behaved. The infection is not cellulitis and spreading across the skin, but looks like it is deep in the tissue of the abdomen and coming up from there. Because of the location and how bad his last MRSA stoma infection got (5 day hosp stay), we know better than to play around with it. Our ID could not see us as he was out of clinic, our GI was out of the office all this week, and our ped is out on indefinite medical leave at this point and we had no idea which ped to see. Our neuro was concerned, so we ended up at the ER at CMHH. This was Thurs night. Saying the ER visit was a disaster would be a gross understatement. It was not the most pleasant experience we have had in the ER. We basically sat around in the waiting room for about 3 hrs before we finally left. They pretty much totally disregarded the protocol letter, even tho it was shown to both the receptionist and the triage nurse. We had to wait about 45 min just to be triaged and the triage nurse actually said to us "Tell me how you know the site is infected...". We have been dealing with MRSA ever since April of 2006...and that first one was the worst and also on his gastrostomy stoma. I am pretty sure we both know what cellulitis and abscesses looks like by this point. It is red, draining yellowish green discharge and crusting over, and there is a red area spreading out to his midline from the side of the stoma and he is in pain when anyone touches the button to look at it. I thought it looked pretty obvious. I was pretty offended about this remark.

At the 3 hour mark, we again inquired about when we would be going back as we were growing increasingly wary of having the boys in the ER waiting area around people who were sick, coughing, etc and we just could not find a place to sit that was away from everyone. She basically told us there were way sicker kids than us there because "everyone there tonight was serious" and Doug asked her what was the point of a protocol letter if they were not going to follow it and she basically told us we had to wait our turn like everyone else and it was going to be "a good little while" before we were going to be taken back. She totally missed the whole point about the protocol letter. We are not to sit around with people who are sick for the risk of getting something else. We are also to be evaluated right away as Mito kids can quickly crash from something that would be pretty benign to anyone else. It is not about asking for special treatment or not wanting to wait our turn at all. If we did not need the letter, we would not have it. That is when we decided to leave. I could go on about other things about that visit, but I won't. I am too frustrated with the whole thing to go over it again. By this point, it had been 5 hrs since Daniel had last eaten and he was beside himself and they had no place for me to go to nurse him. Both boys had missed nap time as we had gone there straight from OT and PT and they were exhausted, hungry, and totally falling apart in tears about every 5 min (this is a mito kid's reaction to stresses and fatigue). There was no way we could stay under those circumstances when they had no intention of getting us checked anytime soon. Fortunately we were able to get an appt with the ID we use for 11:30 the next morning (otherwise we would not have left), so we loaded him up with Bactroban that night. We just felt that, with the other illnesses the patients there that night were sporting, that was a bigger risk to all the boys than waiting a few more hours would be for Nicholas.

It was disappointing tho, as our other experiences with the ER there have been great. Our Mito neuro was not thrilled with this either and this prompted action to be taken by her as we were not the first people to have the protocol letter ignored by the ER staff. This could be dangerous to a Mito patient...and even deadly, so it is not something she took lightly. she said she would have direct admitted him herself when we first contacted her about it had she known, but by the time we spoke to her on the phone, the hours for direct admitting were past and we had to go through the ER.

So, we had an appt with the ID for 11:30 the next morning, but we got bumped as they thought we had been taken care of in the ER. So, we were worked in at his afternoon clinic clear across town...a 1.5 hr drive from us. The ID confirmed that Nicholas' stoma is indeed infected, but since it is not looking or behaving like his last round of MRSA, he is not sure that is what it is. He cultured it and we should have preliminary culture results back sometime Mon or Tues. He is choosing not to admit him at this point as he is not running fever. What he thinks happened is that there was a lot of trauma to the stoma when he got the tube pulled out sliding on his belly 2 weekends ago and then more trauma occurred when I had to try to get his button back in,. There appears to be quite a bit of granulation tissue around the outside of the stoma and inside the stoma tract and he feels this tissue may be infected and there is likely also a pocket of infection somewhere inside the stomach and that is why the infection is draining out from the stoma and not spreading across the skin. He feels that, by us putting Bactroban on it every day, we avoided it turning into cellulitis, but we would have had no way to know there was infection inside the stoma or stomach area since we could not see it and the topical could not treat it on the inside. He is putting Nicholas on Bactrim for now since he said that will also cover gram negative bacteria and we will reevaluate treatment when we have some culture results back. We are to go back to see him if it is not looking better by mid-week.

He also wants us to go get the granulation tissue looked at as he feels there is so much it may need to be surgically removed from inside the stoma tract. We are hoping to at least be able to talk to the GI by Mon or Tues once he returns to see what his thoughts on the granulation tissue are. We have also been using the Triamcinolone steroid cream on it with no success, so silver nitrating the granulation tissue is our only other option before surgical removal anyways, but we have no idea if that can remove it from inside the stoma tract tho. The surgeon who has worked onb both of our boys is now only operating out of TCH, but there is a surgeon we know from the group he used to be with who has seen both of our boys and I am pretty sure he can operate at CMHH so we can use anesthesia there and not have to worry about mistakes being made with that. So, that is where we are with that right now...not a happy place, but what else can you do? He has now outgrown the liquid Bactrim doses, so he has to swallow pills and that has been hard for him as it is so gross and melts quickly, but he is being such a trooper and taking it like a champ anyways. He is much more tender there today, so I am really hoping the abx kicks in soon and we can avoid the hospital. There has been way too much drama over this the past few days and I am ready for him to start feeling better and get over this so we don't have the threat of an admit over our heads anymore.

Our Mito dr also emailed me in response to me asking about the boys' bone growth delay (see last entry for testing update and results) and she said that NO amount of bone growth delay is ok and she is concerned as is the endo she contacted about this when she set up our appt. This defintely does not make me feel good about this. I am just hoping the appt we have with them will provide some answers.

The boys are all having temp issues right now with the heat. Nicholas seems to be worse than Nathan and I am not sure why. He gets so red within minutes of going outside and stays that way for a while after coming inside...even with a cooling vest on. Nathan gets the same way, but takes longer to heat up and less time to cool down. Daniel got overheated when we were at the park Thurs morning and took an entire 40 min car ride and 30 min inside, both in the A/C, to get cooled back down.

This has been hard for me this week. There have been too many reminders and reality checks this month of this awful disease and it is piling up on me right now. I am not in a good place right now and I am hoping to get out of this funk really soon as I am not one to wallow and I hate how this is making me feel. I am hoping with the start of a new month, I can let this all go and start fresh.

Thanks for visiting. Please continue to keep all our Mito friends in prayer. Our MO friends are finally turning a corner and hopefully will be discharged. Our MN friends have won a small victory in court and are on their way back to some semblance of normalcy. I am sure much of this is because of wonderful people like all of you who read these prayer requests and take a moment to pray for those who are in need. THANK YOU. Take care and God bless!

Melody

Wednesday, May 28, 2008 11:35 PM CDT

Well, we sure got a lot of mostly WONDERFUL news today!! We heard back from the Mito dr and she had the results of all of our testing...

Daniel's sacral ultrasound...NORMAL
Daniel's Echocardiogram/EKG...NORMAL
My MRI/MRA/MRS...NORMAL
My Echocardiogram/EKG...NORMAL

YEAH!!! WHat a relief to know my scans turned out normal. The mito dr even put in a little comment behind that saying "whew...that's a relief for me"...I am sure it is not as much as the relief I feel!!!

As for the not so great news...

Nathan's bone age scan...bone age of less than 2 yrs (14 mo delay)
Nicholas' bone age scan...bone age of between 2-3 years (3 yr delay)

I am not thrilled with these results. We knew they were delayed as they are both so small for their ages, but Nicholas' delay just keeps getting more and more significant over time and the gap is widening as he falls behind on growth. My 5.5 yr old is the size of a 2-3 yr old child. UGH. We already have appts for both boys to see Endocrinology (yay...one more specialty to add to the list...UGH) and our mito dr wants us to discuss the results with the dr and get some more in depth growth hormone testing done...so it looks like a full workup of growth will be done and I am so not looking forward to those results. Bah. She also confirmed that both GI and Immunology concurred and have officially diagnosed Daniel with Laryngomalacia and Reflux...that was no big surprise since we knew it already, but it is official now. So, that is the big update on our overloaded day of testing last Friday.

Nicholas is the proud new owner of a Leapster and LOVES it. We bought it as a therapy tool actually. He needs to work on letters this summer...he can write all the upper case ones, but the Mito and hypotonic hands he has makes his letter making painstaking for him. So, we bought this learning to write game for the Leapster to make practicing handwriting fun for him and he ADORES it. Somehow this is FUN whereas practicing it on a manuscript paper is WORK. Go figure...I will take whatever works!

We have finally won a small victory with the school district. I am not going to go into all the ridiculous details of our past struggles with them in regards to Nathan, but Nicholas had a screening not long ago so we could get the ball rolling for him for Kindy in the fall. We figured we would start now to make sure we had all the proper things in place for him when the first day rolled around. The district felt otherwise however, and decided he qualified for NO services. We begged to differ and contacted the building principal. Some things I am willing to fudge on, but other things are totally non-negotiable. Nicholas absolutely HAS to ride a bus with air conditioning. Nicholas absolutely cannot be held accountable for all the PE class entails as he physically cannot do everything the other kids will do without seriously paying for it later. Recess is also going to be a bone of contention as well. With the heat down here in TX, I am not sure he will ever be able to play outside safely when the heat is over 90 deg for that length of time. He starts having trouble at 80 deg! So, we contacted the principal and she called back today saying that she had been talking to several people at the ad building trying to figure out what to do for Nicholas and he will be starting the year with a 504 plan in place for a bus with a/c, PE modifications to be specified, and something for recess, tho we are not sure what at this time. We are to come in sometime once school is out for the summer to fill out the paperwork for the 504 and make those decisions and she also wants us to get the Mito dr to fill out the OHI (other health impaired) form as well to have on hand if we need to pursue a special ed referral. So, that was a small victory for us to start off this public school journey with Nicholas. The principal has been very nice and accomodating so far, so I am looking forward to working with her and hope that things can stay this amicable in the future.

Tomorrow we are going to the park with our friends from my mother's group and the boys have therapy. I am hoping the cooling vests work as well for Nathan and better for Nicholas tomorrow than they did today so they can play with friends and not get overheated. If they do ok, we will plan to stay for a picnic lunch as well.

Friday Daniel has another vaccination...fun fun fun. We are in the process of finding a new and hopefully temporary pediatrician. Ours is currently out on indefinite medical leave at this time and nobody knows why. We are understandably upset as our boys love him and we need his continuity of care for them. Our Mito dr has worked closely with him, helping him to know how to best help them and I am worried about going to someone new who does not understand Mito. I hope he is ok and we have been praying for him and his health so he can return to work at some point.

I have allergy testing next Mon and am soooooo not looking forward to that either. I am hoping the allergies have gotten better. Guess we will see. If they haven't, that may explain all the lovely issues with sinus infections I have had lately.

PRAYER REQUESTS:

*Please pray for our MO friends who are inpatient here. He is teetering on the edge of needing to be intubated and we are so hoping he can avoid that as he may have serious difficulty coming off the vent if he goes on it.

*Our MN friends are going through some very difficult family issues right now and could use continued prayers as well. Please pray for strength and wisdom for them.

*Our Mito dr is very stressed and consumed right now with treating our friends and has so many other patients in need of her right now as well. Please pray for her to have the time and energy to do everything she needs to do right now without compromising her ability to be the fabulous and exceptionally caring dr she is to us all.

*Please pray for all the Mito friends we have made. One was in a car accident this weekend, one is in the middle of 2 tortuous months of never ending appts with no answers thus far, one is struggling with her son's inconsistant eating, one is struggling with her own health while caring for her 2 sons, one is dealing with various illnesses and issues with each of her 3 mito kids, and one is having to deal with GI issues with her mito child.

Thanks so much to each and every visitor here. We welcome and appreciate every single one of you. Take care and God bless!

Melody

Friday, May 23, 2008 11:16 PM CDT

So, the past week has been busy!!

Wednesday, Nathan had his first round of allergy testing. He was so brave and barely cried. They did a reduced panel for him since he is only 3. And...he reacted *barely* to dust, but it was so small that they did not even measure it. Other than that, nothing. Just like Nicholas. So, no allergies yet!

I am so proud of him, too. He has gotten really good at blowing whistles lately, so we decided to try him with a PFT to see how he did. He did 90 on the first try, then 80 and 70! He did better than the average PFT for a child his age! The office staff were surprised that he did so well as he is only 3...so it looks like he will be able to get his PFT done from now on when we have appts.

So, good news all around on that front!

Wednesday was also Nicholas' last day of school. While we are all sad to see that chapter in his life come to an end, he also needs the chance to sleep in and rest more to tank up from the year. he had a pizza and ice cream party the last day and had such a good time!

Both boys had therapy yesterday. Nathan had his first PT session with Nicholas' PT and did so well. She told me she is going to have to adjust her frame of mind when it comes to Nathan as he has less stamina than Nicholas did. By the end of 30 min with her, Nathan was laying down and complaining of being tired, so they had to end early. She is going to try to figure out a better way to get in what they need while conserving energy enough to allow him to make it to the end of his session every time. Nicholas was a bit jealous when he saw Nathan with "his" therapist and was a bit distracted watching her with Nathan. He was excited tho, as the OT sent him home with TheraPutty to use for hand strengthening.

So, we were woken up at 4 this morning with Daniel junky and having trouble breathing...he had been fine at 12:30 last night, so who knows what happened... He had a fever of 100.1 as well.

Got to the hospital at 8 and registered all 4 of us. Got to MRI at 9 and fed Daniel only to find out they were running VERY behind. Apparently, Mito scans are done ONLY on a T3 (I think?) MRI scanner and they only have one of those out of the 5 scanners they have. So, since it was occupied, I had to wait. Doug took the boys upstairs for their bone age scans and they had time to do Daniel's sacral ultrasound. So, since I was waiting anyways, I went up and did the u/s with Daniel while Doug handed off the boys and carseats to his mom when they were finished with their scans. She took them for a fun-filled day of train rides and museum visits! Daniel was done with his u/s after about 15 min, so we left and they were almost ready for me when I got back down. I went in the scanner at 11.

The MRI/MRA/MRS I had took 1.5 hrs. No music, no videos, no conversation, no company...NOTHING to do for 1.5 hrs. And NO MOVING either. UGH. It did not help to be claustrophobic either, especially when they put that cage thingy over my face! Somehow I made it through. My arm fell asleep and my legs were soooo restless. I did not realize I was having a dizzy spell inside the scanner since there was nothing to see moving (I just felt funny), but I sure knew it when I tried to get up when it was done and I fell over. The tech caught my arm and helped me wobble to a chair. They would not let me leave for a litle bit and made me drink juice and looked concerned. By the time I got upstairs to our friend's inpatient room where Doug was hanging out, apparently I was pale as both of them remarked about it when I walked in the door. I did not feel much like eating either and was cold, so I wrapped up in a blanket she had. Doug had Dr R (the GI) check Daniel's lungs when he came to check on our friend's little boy, and they were clear, so that was a relief.

At 1, and after a lot of confusiuon about where we were supposed to go, I had my echo and EKG. I was so tired, I was falling asleep on the u/s tech and she kept having to ask me if I was ok.

Got back up to our friend's room and we all ate a really late lunch. Then, they took our friend's son out for his fundogram while we waited in his room in case Dr K came (as she wanted us to take her hostage until she came back!). They came back and so did our boys and the boys visited for a bit while Dr K (neuro) and Dr P (immuno) came in to check up on her son. Both drs were kind enough to give Daniel a once over to be sure he was ok and then proceeded to take turns hijacking him from each other and our friend while we chatted. The boys all laid in bed watching WonderPets while the adults (drs included!) sat around and talked about a lot of things and it was a nice visit. After the drs left, we did too after a bit since our friend's son was getting worn out from the visit.

Dr K said she would have the results to us early next week. It was a long day and I am SURE glad it is done!!! And, can I add, that I NEVER want another MRI again!! Even Dr K told me she could not have done that long of a test unsedated. It was NOT fun.

We are looking forward to a long restful weekend, just hanging out and getting caught up on some much needed rest and family time with just us. Not sure what we are going to do, but it will be something fun! We all need that!

Hope you have all had a wonderful week! Take care and God bless! Please keep our mito friend and her son in your prayers as they are inpatient with her son's aspiration pneumonia. He needs to get better soon so they can go back home to the rest of their family in MO. Please also pray for our MN Mito friends as they are going through a family crisis right now and could definitely use the prayers, too.

Melody

Monday, May 19, 2008 1:34 AM CDT

We have had a really busy week! Seems like this week was all about Daniel!

On Monday, Daniel had appointments with Immunology and GI. The Immuno was not happy with his illness rate since he was born. She said, while it was not unheard of to have ear infections in a child as young as Daniel, it is certainly not common and warrents looking into. That plus the 3 bouts with Thrush he has had concerned her.

She also heard the stridor and dx him with Laryngomalacia. She checked his tonsils and said they were small...looks like it runs in the family. My mom said she gets told that all the time by drs. I am guessing mine must be small as well.

She looked in his nose and said his mucous membranes are "pale" and apparently that has something to do with allergies or congestion or something. He had some green mucous in there too, but not much. She is thinking she wants to start him on some allergy meds, but wants to wait until the GI tweaks his reflux meds first to see if he will need them after the tweaking or if he will be ok without. We also decided to only vaccinate one vaccine at a time as Dr K and the immuno felt it was too risky to overload his little body with too many vaccines all at once, so we are doing one at a time spaced out with 2 weeks in between shots.

She decided it was time to do testing, tho she was hoping he would do well enough to wait. He had the whole shebang drawn and HATED it. The tech tried so hard, but blew his little vein as soon as she stuck him, but she made it work so she would not have to stick him again. It bled, but barely. He sure got a big bruise. She took the bare min of blood for each test since he is so small and could not afford more blood than was absolutely necessary since there were so many vials to draw. I think she did 5. He also had a chest xray. Hopefully we will hear some results soon.

On a better note, the bigger boys had the rest of their T and B cell testing come back in and all was normal and she was happy about that. Back at the end of July....

Then, it was off to GI. Daniel has been having a lot of trouble with refluxing lately and that has caused problems with his Laryngomalacia. We talked about testing, but with the isk of anesthesia and Mito, we would prefer to wait until there is a real need for the testing. So, we have decided to try to treat with meds first. He left the Prevacid untouched at 1/2 solutab 1x/day, but decided to add in a motility med to see if that would help decrease the refluxing and helping with the stooling some.

Then began the big debate about which med to use. Erythromycin is out as it is toxic to Mitochondria. He had no idea and left to call Dr K (our Mito dr) in the middle of the appt to get more information about this. He made sure it was ok for Nathan to be on it and Dr K thought it better to "not rock the boat" and leave it alone. Nathan has been on it since he was under a yr old and has done well on it as far as we know. She did say NO WAY for Daniel tho. So, that left Bethanechol and Reglan. Since Nicholas did not respond at all to Bethanechol and did really well on low dose Reglan, and in light of possible respiratory stuff along with the reflux, he decided Reglan might be the better option. So Daniel left with a script for that and the threat of a GES and possible other testing hanging over his head if things were not better by the follow up visit in July. Since the appt, his stooling has been better, so we are holding off on filling the Reglan for the time being to see if things will work themselves out without meds.

Tuesday he had an appointment with Cardiology. He also had an Echocardiogram and EKG then as well. Everything came back looking GREAT! So, the cardiologist said he looked good enough to only get annual heart testing like his brothers. YAY! That evening, we met up with friends for ice cream and Nathan broke a tooth on the railing there while jumping in puddles. He hit it so hard, he scraped the paint off with his tooth! We got him in to see the dentist the next day (Wed) and thanlfully, things looked ok. Daniel also got another vaccination at the peds.

Then, we met up with friends for dinner and playtime on Thurs and again last night after a birthday party. The party was interesting as Nicholas decided not to listen to us and went down the slide on his belly and pulled his tube out. It was hysteria as we tried to calm Nicholas down and get home to put the tube back in. It was hard to get it in, but I finally got it. Nicholas was so relieved as he so did not want to miss the rest of the party to go to the hospital for a dilation.

Today, we had Daniel baptised at church. Here are some pictures from the day...

Doug and me saying the vows...

The minister getting ready to baptize him...

Doug and me standing with the boys, our parents, and the minister...

Daniel hanging out at lunch afterwards...

Smiley boy showing off his baptism outfit...

We had a good time going out to lunch with both our families afterwards and had a wonderful lunch of tasty Italian food. YUM!

Hope you all are well. Please keep some mito friends of ours in your prayers as they lost their 6 yr old daughter to mito last week. She was laid to rest a few days ago and now her parents and siblings begin to live their lives again without her. Hug your little ones a little tighter tonight... Take care and God bless!

Melody

Saturday, May 3, 2008 10:57 PM CDT

Boy have things changed with the addition of one more to the fold! I certainly do not have much time to sit and do anything anymore!

Nathan is out of the hospital and recovered finally after a few weeks of getting back to his baseline. He ended up with a bad virus that turned into pneumonia and he was given IV abx and sent home on more oral meds. That following Mon we went to our immuno appt and found out he also had a sinus infection on top of the pneumonia and so he had another rx for 2 more weeks of abx once he was finished with the pneumonia one. The ER protocol letter that our Mito neuro gave us was wonderful. We never had to sit in the waiting area more than 5 min and all the proper precautions were taken. It was so wonderful and we had such a good experience compared to past experiences with the ER at the other hospital. He is still going to preschool, but is not going to continue after this Thurs as he did not qualify to continue special ed services. He is also starting PT with Nicholas' therapist in a couple of weeks. His testing placed him at an 18-23 mo level for gross motor skills overall...he is 3 yrs old.

Nicholas is starting back to OT with a new therapist as his moved at the end of last year and we have been on the waitlist since then to get in with someone else. He had his reevaluation after a 3 month hiatus with PT and did well...so well in fact, that he does not need to go back for another SIX months! He will remain on consult so he has someone to help with his orthotics and in case he has a regression, but we sure are excited! Amazingly, with the bad virus going around, he barely got more than sniffles. I got really sick and Doug got sniffles as well, but I am so glad Nicholas did not get more sick than he did. He had his Parent/Teacher conference at school and is doing well! The only areas of concern are all fine-motor related, so we are really thrilled! We are already working on the concerns, so overall it was a wonderful conference. He is now registered for Kindergarten in the fall (eek!) and was screened for special ed last week. We are hoping to have his issues addressed by the time school starts so he can ride the bus (with a/c) and not have to worry about PE and recess and all the writing they are going to be doing. He also tested and got his rd stripe belt in TKD and he is stil doing wonderfully in there as well.

Daniel had been sick with a cold (caught that nasty virus Nathan had before it turned to pneumonia) for abut 3 weeks and ended up with an ear infection that was attributed to his reflux. He also has stridor that is believed to be caused by reflux irritating the larynx and causing laryngomalacia. He is on Prevacid for the reflux now and doing better. Because of the stridor, laryngomalacia, ear infection, and reflux, he now has emergency appts with Immuno and GI in the next couple of weeks. He just started smiling and is such a wonderful baby!

We ordered cooling vests for both boys and they got them yesterday. Boy, were they excited! They have wanted to wear them since they arrived and they cannot wait to use them. I am hopeful these mean the end of being a recluse in the summer. The heat here is way too much for their bodies to handle, so I hope this will give them the freedom to be like other kids for a change.

We just went to the neurologist who treats their Mito yesterday. We were actually her entire afternoon. That made it nice actually since we did not have to wait for others to finish up and we did not feel like we had to rush so she could get to other people, either...well, until it was 5 and we were all trying to get out of there to go home!! By then, the office was empty and we walked out with all the drs as they were leaving...it was interesting! Ok...bear with me as this is going to be LOOOOOOOONG!

So, let's see...when the neuro first poked her head in, she asked Nicholas if he wanted to "show off" for her friends. Now, those of you who have met Nicholas know he is a total show off, so of course he is willing...and Nathan was jealous and had to get in on the action too. In the hall, Dr K had a group of about 6 other drs and residents and asked the boys to run up and down the hall. Nicholas made it 3 laps down and back before he was dragging and asking her to stop. Nathan made it down, stopped, came back, stopped, back and stopped for longer, and then walked back. They were sweaty, breathless, and so tired and they both were begging for their Gatorade. She used this as an example to teach the other drs how obvious it is that they have no energy reserves like other kids. It was pretty cool, actually...they were all asking her questions and it was interesting to hear her explain it to them.

Nicholas...

He has been having some weird temp issues. Even before our appt, at lunch, he turned BLUE from the cold at the restaurant where we ate and his skin was icy cold. The rest of us were fine. His lips looked like he had a blue sno-cone or something, but he pinked up as soon as we went outside. His temp issues are obviously getting worse, but there is not much we can do about that unfortunately. We talked quite a bit about school and modifications and she was really helpful. She got to watch him color and could see how much he was fatiguing from just a few minutes holding the marker. School is going to be hard for him with how weak his hands are. We also talked about upping his Carnitine as it is weight dependent and he has gained enough and has been acting "off" recently. I hope the med change helps him settle down some. He is also being referred to Endo to get a growth workup since he has not had one. She wants him AND Nathan to get a bone age scan (Nicholas had one about a year ago that showed a 2.5 yr old skeleton at 4.5) to see where they stand and Nicholas needs his growth hormones checked.

Nathan...

So, we talked about Nathan and school and about how tired he has been and we decided that honestly, as much as we woud like him to keep going to PPCD for preschool, maybe he just needs to work up to longer periods of time at school and to have days in between going so he can rest up for the next day. I think I agree. While he is definitely the most physically challenged in the class, he is not cognitively challenged and I think that is more the qualifier than physical issues. We can just modify reg preschool for that. We discussed his EMG/NCS results and she feels this is responsible for Nicholas' twitching and his own, as I discovered he does it too. He did it when I slept with him in the hospital. We talked about hydration as he does not have a tube and is not drinking as much as I would like and she said it could be because of his constipation that he is likely staying more hydrated than we think. As the stool sits there in the colon, the longer it sits, the more water comes out...she likened it to wringing a towel...his just wrings more than most...and he could be staying more hydrated because of that. Interesting...

Daniel...

Daniel had the bulk of this appt since she had not had a chance yet to give him a good once over. Doug had to take the boys out since she was trying to get him to track with his eyes and they kept distracting him. She said, overall, he looks the best of the 3. His muscle tone is good right now, tho she did say when I asked that that could change so we have to wait and see. He was doing everything he is expected to do right now, so that was a relief. She said it was very encouraging to see that. She saw his sacral dimple in his back and said it was spina bifida occulta and said she needed to do either a spinal xray or ultrasound (don't remember which) as she needed to confirm that it was not a big deal as it seems to be very superficial. No cocktail meds for him yet. I did nurse him there so she could see what happens when he nurses and he did a really spectacular honk/squeak (stridor noise) and choke for her and I could tell by the look on her face that it was worse than she was thinking it was. She asked again when we were seeing immuno and GI and I told her both on the 12th and she was so glad. I think she said she wants him to see a cardio, but I cannot remember why...hmmm...she did seem surprised that neither of the boys had ever been to one. And, it is offocial that Daniel has Mito as well. Once you read the rest of the update from the appt, you will understand why. Thankfully, he is doing the best of any of the boys so far, so we are hopeful he may be less affected than his brothers.

Me...

So then we came to me. My bp has not come all the way down since the pg. I have also had this weird thing happen to me that had me concerned. When I go from sitting to laying down in one movement, my head feels like it is going to explode and I can hear my heart pounding in my ears and then I have a splitting pounding headache for the next about 30-45 min. She said in light of that plus the higher bp, she wants me to have an EKG, echo, and aortic ultrasound done just to be sure. We also talked about getting me an MRI since I have never had one and she is on the fence. We will need to decide if I need to get one or not. She started me on Carnitine as well since I have been beyond exhausted since I was pg...but she wanted me to wait until after I had Daniel. We talked about the pg and delivery since it has been so long since we have seen her.

Other stuff...

Then, we talked about the genetic testing. She went into this 30 min long explanation of the boys' mutation. Basically, the gist is...3 genes make up an amino acid and several amino acids make up a protein. Some mutations do not change the function of the protein, so they are "mis-sense" mutations, meaning they are no big deal. Other mutations WILL change the function of the protein and that causes trouble since the protein cannot function as it should. Our boys actually have TWO mutations that both change the function of a protein, so this is what is causing their Mito. Also, all the mutations AND polymorphisms are on ND1 and that governs Complex I. Since Nathan has a deficit in Complex I form his muscle biopsy, she feels both the mutations AND the biopsy deficit add together to give us a concrete answer for their Mito. It is also present in ALL their mitochondria in their body, so we will not know what it will affect or when...or even if, but she felt like since I am an adult who functions relatively well, they are looking to be in pretty good shape. Because their mutations/polymorphisms are identical, she expects mine to be identical as they had to come from somewhere...and she expects Daniel's to be the same since he got them from me. So, she does not even feel it is worth sticking me or Daniel to check unless we just want to...ummm, yeah...that's ok! I think we will skip that! She did pay me a nice compliment tho, telling the resident that I probably knew more about Mito than most med students and later I heard her telling the resident in the hallway once we were done to remember that appt...that she probably learned more about Mito in that appt with the parents of mito kids than she has learned in med school and will learn in all her residency.

We also chatted some about the immune stuff she has been working on with Dr P, the immuno, and they *think* they have figured out the mito/immunodeficiency connection and now they just need to test it out. Should be interesting.

Guess that was about all from the appt (told you it was going to be LONG!). So, that was our about 3 hr afternoon yesterday! We go back in 3 mos as we all agreed 4 mos was just waaaay too long.

In other news, we are in the midst of a media frenzy! The boys are going to be plastered all over town. For the ad campaign for CMHH, they are putting up 6 billboards of our boys in the next 2 wks, they have their story up on the CMHH website, they have radio spots airing on pretty much ALL the major radio stations in Houston, they have banner ads linking to their story on the hospital website on the Houston Chronicle website, and they will be having magazine ad spreads coming out soon. It is so amazing! I am just so excited to be a part of getting the word out about Mito! Here is a link to the boys' story on the hospital website...

http://www.memorialhermann.org/services/PS_Pediatric_CH_NichNath.html'

Hope this update finds you all well. I am going to try to make a concerted effort to update much more often and go back to daily if I can. There is just too much to update if I let it go too long. Take care and God bless!

Melody

Thursday, March 27, 2008 10:37 AM CDT

Sorry for the lack of updates. Nathan started preschool on Mon and then became very ill after his 2nd day Tues. We have already had him into the ER for fluids Tues night and have been trying to keep him at home to avoid the hospital, but he is getting sicker by the day and the 103-104 fevers are relentless and not going away and are just taking too much out of him. His mito dr wants him admitted for fluids and observation, so I am waiting for Doug to come home and pick us up to take him in. At least from the ER testing the other night, we know he does not have the flu or RSV...it seems to be just a really nasty virus

I am so grateful for Doug's work being understanding as he really has *no* time off left and we are on borrowed time right now as it is. I am hoping I can convince him to go back in for a few hrs once we are settled into a room, but he is stubborn, so we will see. Thankfully, my parents are taking Nicholas so he can still go to TKD today...he has given up a lot since the baby was born and I am glad for him to have a relatively normal day today. I think the hospital will have Wi-Fi, so I will try to update later if I can.

Melody

Tuesday, March 4, 2008 11:28 PM CST

UPDATE 3/10/08 -

Baby Daniel Aaron arrived on March 6 at 12:53 weighing in at 6 lbs 13 oz and 19.5 in long. He and Melody went home on Sat.

Since then, his bilirubin levels have gone up and as of this morning, they were 17.5 and he is being admitted to the hospital. Any prayers would be appreciated...we will keep you posted.

*********************************************************************

I am so sorry for the lack of updates. The kids are keeping us on our toes and, by the time the evening rolls around, I am too wiped out to update. Here is the lowdown...

On the boys' health...

Nicholas is ending his 3 month hiatus from therapies and will be reevaluated this month to see how he has done.

Nathan will be starting PPCD on a 30 day temporary placement March 24th and he will be going every morning and riding the bus to and from school. He is so excited! He ages out of ECI on his 3rd birthday, which is coming in just about 3 wks!

Both boys have been pretty sick on and off with mostly sinus infections. They have spent weeks on antibiotics since Sept, averaging 15 days with each infection.

Both boys had testing done after Christmas to test their Mitochondrial DNA and they were both found to have the exact same gene mutation and several polymorphisms the same as well...nobody is quite sure what this means as far as finding the reason behind their mito as this is a rare gene mutation not seen before. We are still awaiting word on what this means and how this will affect them.

Nathan had his EMG/NCS done and was found to have muscle movements NOT govorned by nerve impulses. This is not normal and chanracteristic of Mito, according to their Mito dr. This may explain his leg pains why Nicholas has the twitching in his sleep that did not show up on his EEG...if the nerves are not involved with the movements, it would not correspond with EEG findings. The good news in all this tho, is that there is NO findings of nerve damage or muscle damage.

On the boys' appts...

The boys saw their current immunologist and she basically has had no answers for us as to why the boys cannot keep their vaccine protection. She wants to reveiew their records and see us again in 4 mos to review then.

Well, our Mito dr was NOT happy with this plan as she feels they have been WAY too sick this school year and are at much higher risk of metabolic crisis because of the illness rate they are having. She set us up with an immuno she knows and this immuno feels their immunological issues and Mito are related somehow. She decided to start all testing over fron scratch since Nicholas has not had certain tests done since 20 mos of age and Nathan has not had any of these tests. She also wanted to review their medical records and will see us back at the end of March to review everything. The boys may need to be placed on IVIG at this point to help improve their health and lessen their risk for metabolic crisis. Much as we are not thrilled with this, we know something has to give at this point. They have been too sick for too long and it is time...

On the baby...

Thurs, March 6 is THE BIG DAY! We are understandably very nervous about the whole thing as we are not sure how healthy the baby will be when he is born and we fear another NICU stay. That being said, we are more hopeful than before that things will go well. I have had NO INPATIENT STAYS with this pg and only ONE observation. I have been contracting for 19 wks basically, but have not had major issues with this and have NOT required bedrest. My blood pressure has been borderline, along with my blood sugars, and while I am on the verge of polyhydramnios, I am still one unit under the amt required for polyhydramnios to be dx. Not too bad! I will be 38 wks on delivery day and this will be the first time a baby of ours has been born in the month he is due AND the longest I have been pg. God is good!! Please continue to keep us and the baby in prayer. If he shows any signs of anything being wrong, the boys' mito dr wil have him transferred downtown to have him under her care. While this is obviously the best thing for him, it will mean he and I will be separated and that tears my heart out at the thought of it. We will try to update the site as soon as we can!

On the family...

We lost our one dog last summer to cancer and lost our other dog this past week to severe GI issues. She has been dealing with gastric ulcers for years now and has not been doiing well since the loss of our other dog. They were inseperable and she has never been the same since he passed. She has been having a bad uncer flare this past week that meds were not even helping and the day she passed, she was vomiting frank blood in large amounts. We knew it was time and Doug took her in. I am heartbroken. We have had these dogs for 12 yrs and the house just seems so empty now. I am not sure I will be able to have another dog for some time...it has just been way too hard to lose them both within months of the other.

Prayer requests...

Many of our newly found Mito friends are struggling right now and our friends on MN are going through a very hard time as well. Please pray for everyone along with our new baby as we need a smooth and easy delivery. Take care and God bless. Be sure to check back for pics!!!

Melody

Sunday, December 2, 2007 11:08 PM CST

Ok...here is the update I know you have all been looking for. Our lives have been such a whirlwind lately, I feel as though I hardly have a moment to stop and take a breath before needing to move on to the next thing! I guess we should start with some fun stuff...

The boys had a great Halloween! Nicholas was Peter Pan and Nathan was Blue from Blues Clues. Nicholas had a great party at school and even had a parade there wearing his costume and walking with the rest of his class. We visited the pumpkin patch a few times and had a great time. Here are some pics...

The boys with the pumpkins they painted (with a little help)

Nicholas during his class parade...

Our sweet little Blue trick or treating...

Nicholas had a fabulous birthday party! He had it at a place called Musical Chairs and he and his little friends had a BLAST! Can you believe our little guy is now FIVE??!?!?!?

Playing with friends...

Nathan posing for a quick pic with me...

Opening gifts...

And the fabulous dinosaur cake Uma made for him...

Getting sung to at his birthday dinner...

We took the kids to the mall to see Santa after church last Sunday...they were dressed nicely, we were already there for another errand, and the line was short. Well, as luck would have it, there was a TV crew there filming and both our boys were interviewed for the news. Nathan was crabby and hungry and did not have much to say, but Nicholas ate up the spotlight and was featured on the news! Here is a link...

http://video.khcw.com/global/video/popup/pop_player.asp?clipid1=1958625&at1=News&vt1=v&h1=Holiday+Shopper+Crowd+Houston+Malls+11%2F25%2F07&d1=116567&redirUrl=http://khcw.trb.com&activePane=info&LaunchPageAdTag=homepage&playerVersion=1&hostPageUrl=http%3A//video.khcw.com/global/video/popup/pop_playerLaunch.asp%3Fclipid1%3D1958625%26at1%3DNews%26vt1%3Dv%26h1%3DHoliday+Shopper+Crowd+Houston+Malls+11%252F25%252F07%26d1%3D116567%26redirUrl%3Dhttp%3A//khcw.trb.com%26activePane%3Dinfo%26LaunchPageAdTag%3Dhomepage&rnd=75913396

Ok...on to the medical updates...

Well, let's see... Both boys had heart Echos and EKGs and all came back NORMAL...wonderful news!!

Nathan got his new AFO's and has done super with them! No issues whatsoever!! The ones he got are this swirly blue pattern called Ice Age.

Both boys have been battling ongoing sinus issues for what seems like forever. Both have been on around 2 mos worth of abx and then Nathan got sick yet again and needed another week's worth of Zithromax. Once they were finally well enough, both got revaccinated with several shots to boost the low levels of titers they showed on their last draws. We go back in a month to recheck levels. Nicholas got sick with some unknown viral illness and ran fever for 6 days. We were really afraid we would need to cancel his birthday party, but the day before the party, he FINALLY was fever free all day, so we were ok for the party. He missed an entire week of school and was really sick. He had to be on his pump with pedialyte g feeds almost all day long to make sure we kept him good and hydrated. Had we not been able to do this, he would have almost certainly been admitted. The mito dr was on the fence about admitting him the entire week, but he was doing *just* well enough to stay home. They are both scheduled to see the ENT next week to see if more "agressive" treatment needs to be done to help them with these sinus issues.

Nicholas had some pretty intense GI testing and procedures done a few weeks ago. He had an endoscopy, Bravo pH probe, Botox injections to his pyloric sphincter muscle, and some bloodwork. The initial biopsy reports from the scope came back looking good, but they were also being analyzed more closely to look a the mitochondria in the gut. The probe came back looking ok, but the internal probe unit took 10 hrs to empty his stomach, so our fears of his emptying getting worse were confirmed and it is looking as though the Botox injections did not work as we had hoped. We go back to see him this Thurs, so we will likely be getting more info then. This will also likely be our last visit with him as he is relocating to Tennesee. We are devestated about this, but understand he needs to do what is best for himself and his family. We have been working with him and the boys' Mito dr to find someone new here in town and think we have found a good match in a dr who knows quite a bit about Mito and it's effects on the gut, so we are hoping a new set of eyes might help us.

Nathan has been having ongoing leg pains. He wakes in the night screaming out in pain and it seems to be getting worse as time passes. His Mito dr has scheduled him for a nerve conduction study and EMG. We are not happy about this as, from what we have heard about this test, it is painful and traumatic. We would only agree to it if it was scheduled to be done sedated. We are hoping it will give us some useful information and direct us towards something we can do to help him.

As for me, I underwent some testing myself and it looks as though I might have Mito as well. I am dealing with this thought just fine as it would explain quite a bit. My mom needs to get testing done as well as she has more symptoms of Mito than any of the rest of us. The baby will be tested at birth to see if anything shows up so we can start treatment as soon as possible if need be.

And...now for our exciting family news...

The boys' Mito dr called us last week to check in on the boys and ask us a question. She had been asked to participate in the hospital's ad campaign for the upcoming year. She was also asked if she had any families who would be interesed in helping her with the ad campaign and she said she immediately thought of us as we were a "memorable" family and illustrated what Mito can look like if it is mild and you are not cognitively involved. Of course we said YES...she has done SO much to help us and we thought this would be a great way to say Thanks and spread awareness about Mito at the same time. We are really excited. I have already spoken to a reporter to give her the boys' medical story and we are scheduled for a photo shoot with their dr later this month. The ads will run in print...newspapers, magazines, and billboards. Whatever the kids get pictured in, we will get copies of to keep. We are so excited to be able to help out. We will let you know what we find out as this all unfolds.

And, for the most exciting news of all...we are having a baby BOY!! The boys are so excited! They are so ready for the baby to be born already. We have started buying things for the baby's room and are now just waiting for the big day to arrive! I am feeling great (knock wood) and so far, things are going well. I am praying the 3rd time is really the charm like they say it is! Here are some u/s pics of our newest little bundle...

Baby looking down with arm and leg bones visible underneath...

Baby looking down...

Baby looking up at us!

On a more somber note, many mito families have lost their little ones recently, or are facing the upcoming anniversary of losing their little one. This disease is so vicious and does not care who it takes. I often wonder why we got so lucky and are so mildly affected, when I look at others who have such severe involvement. I am counting my many blessings right now and praying for all those who are facing one of the worst things that can happen to a parent. Many prayers are being said for them and sending strength, peace, comfort, and healing their way...may God be with you all...

Thanks so much for your patience in waiting for this update. I am really going to try hard to keep you all better updated. In the mean time, take care and God bless each and every one of you!!!

Melody

Sunday, November 11, 2007 0:19 AM CST - Happy 5th Birthday, Nicholas!!!!!

Sorry it has been so long since I have updated. We have had a lot going on between illness, a preterm labor scare that required a hospital trip, family issues, and dr appts and testing. It has been particularly stressful for us. I promise once things settle down, I will give you all the update you have been waiting for.

I just wanted to post and wish my sweet Nicholas a very HAPPY BIRTHDAY today as he turns 5 years old!! You are so very special to us and we love you very much!!

Love,

Mama and Daddy

Friday, October 5, 2007 9:00 PM CDT

Soooooo much to update today....

Tues, the MRI went ok...not as smoothly as we would have hoped, but we got most of what we needed, so that is what counts. The day started off well...Nicholas needed an EKG before anesthesia to make sure he would be ok to go under...and he was. He did not cough in front of anyone who mattered, so he was allowed to go through with it. They began sedating him and he did well, for the most part. He was under for just under 3 hrs and they used a med called Presidex for the general. He began waking during the MRS portion and they felt it safer to let him come out of the anesthesia instead of letting him go back under again, so the MRS was not completed. Nicholas woke from the anesthesia very agitated and upset and was screaming, feverish, and very red from a reaction to the meds. He also had an upset tummy. After some Zofran to quiet his tummy, he slept for a bit and after some Tylenol, did well enough for him to go home. We arrived at 5:30 and were leaving at noon.

Wed, the boys had their A/A appt and boy, was that one a shocker! We went in with the hopes it would be an easy appt and we would be able to go to Pulmicort as needed. Bye bye dreams of that!! The boys have SEVERE sinus infections. Nicholas' sinuses are 95locked and Nathan's left side of his sinuses are completely blocked and the right side are 1/3-1/2 blocked. No wonder he has been complaining about the left ear hurting...it has to be from the pressure! Both had a CT scan to find this out. Nicholas also has reduced PFT's from the last visit, so when we go back in 3 wks, she wants a computerized PFT, CT recheck, and environmental allergy skin testing. Nathan goes back in 4 wks for a CT recheck and follow up. In the mean time, both boys are on a load of meds (Nicholas 5-3 new, and Nathan 4-2 new) and are to be on breathing treatments 2x/day for the entire winter! UGH! No wonder they have had coughs forever!

Yesterday, Nathan had his first transition meeting to go from ECI to the 3-5 program in our local school district. It was very interesting... Last time Nathan had PT I was discussing this meeting with her and I wanted to ask her to come, but thought it would be awfully forward and did not want to take up her time, but before I could ask her, she offered to come. She said she wanted to be sure things went well for us. I was sooooo relieved. Upon arriving, we met the woman who did both boys' initial evals and she is now the director and remembered both boys from when they were babies. Then, we also saw the woman who used to be Nathan's service coordinator...remember the pg one who refused to come anywhere near our house because of the MRSA and she was never seeing Nathan and we finally asked for a new one? She was so fake to me and I was glad to have a distraction so I could end the conversation. Anyways, we went and I had my mom with me as she used to work as a SLP for the district and knows her way around this kind of stuff. They explained a lot and I sure got a whole lot of "But he LOOKS so GOOD!", to which the PT would immediately pipe in with a reply like "Yah...TODAY he does". They were surprised he needed a stroller to get around. They were surprised at looking at what a good day he was having, that he could have bad days, too. I was SO glad the PT was there...she set the woman straight every time and it was nice to have it coming from her who they know and respect, and not from me as the possibly overreactive mother. I think that was the exact reason she was coming as I think she knew this would come up. I swear this woman is a godsend to us. It worked out so well, especially since our service coordinator was out sick today and could not be there. TG the PT was...I don't know what we would do without her some days! So, we wait until Jan to have the next meeting where they will set up the testing and ARD dates for sometime in Feb. I asked for early Feb as I am worried about when this baby is coming and I want to have all this DONE before the baby comes if we can so that will be one less worry after we have 3 to care for.

This morning, we went to get Nicholas' AFO's adjusted. He has been intoeing severely since he got these new ones. Turned out to be a totally crazy thing! When the orthotist was adjusting the braces the other day, he did not shave enough off the soles to allow it to spring up under his toes, so it was not bending at all and was like he was walking with his feet strapped to a board. He was turning his feet sideways to walk so he would not need the brace to bend at the toe. Now that he fixed it, Nicholas' rt foot is back to being straight and the left is back to the level of intoeing he was doing before the new braces. If it does not completely resolve by the time we go in 2 wks for Nathan's casting, he is going to try adding in a wedge to force his foot back out. I am just glad it was an easy fix!

Today the boys had their follow-up with their new neuro, who is also a local mito dr. I have to say this burden it feels like we have been carrying around forever has been lifted quite a bit today. We had a great appt with Dr K to discuss the boys.

First off, since I know you have all been wondering...the EEG is NORMAL (NO seizures, praise God!) and the MRI was NORMAL!!! She said there was really no need to worry about trying to get the MRS since we know he has had history of elevated lactic acid in the blood, there is no need to go looking for it in the brain since it will likely tell us what we already know if it shows anything. She said the risk of anesthesia is too great if there is no immediate need for the test...whew.

As for all the urine and blood testing...both boys had elevated lactic acid...Nicholas was actually the 2.8 and Nathan the 4.3. She said treatment for elevated levels is actually controversial since the body is used to functioning with higher levels and will compensate by producing more base elements to balance out the acidity. If we were to treat, it could actually make the levels of the base elements too high and cause other issues, so better to leave it alone unless it is extremely elevated. Both boys had elevated Pyruvate levels as well...she said 3x the levels they should be. I am not sure what normal levels are, but theirs were around 0.33 for each boy. For the others, they each had a handful of elevated or low amino and organic acids, so it just carries on with the trend from the other testing they have had.

She said based on Nathan's biopsy and the fact that their labs are almost clinically identical, she is dx them both with Mitochondrial Cytopathy, since she is unsure of the specific type at this point. She said it should not be necessary to do a muscle biopsy on Nicholas since they are so similar and there is no reason to put him through anything unnecessary at this point. I am just relieved to have a dx since it changes nothing really at this point except knowing what we are dealing with, what to do for them, and how to treat it so they can have better quality of life. I know it seems weird to be relieved at a dx that is not such a great one, but she said there is no indication they will not be able to grow up and lead relatively normal lives, aside from the challenges they will be facing. That leaves me with a great feeling. It is also relieveing to know with certainty what is going on...up until now, we KNEW something was wrong, but just never had a name or a concrete plan for what to do to help them. We were just treating things as they arose and doing nothing to try and prevent things from happening. It is a good feeling to know we can be more proactive now and it is just a good feeling in general to have a name for the beast we have been facing for so long now.

She had a list of things she wants the baby tested for when it is born to check a few things from the get go. She is getting with an endo she knows to check and see what we need to do about the bone age scan results for Nicholas for his bone growth delay, and will get back to us with that info. She also gave us the names of 2 GI's she works with who are familiar with mito and how it affects the GI system in case we need a new one when ours leaves...she wants us to check with our GI to see what he thinks of them, but this way she would be able to keep tabs on them if they ever needed to be admitted for GI as she has no priviledges at TCH. We spoke about the tube feeding and she thinks it is likely Nicholas has such a high metabolism form the mito that due to that and his volume restriction from the fundo, he is not likely to go off the tube feeds anytime soon since he obviously needs them (evident by him not tolerating the weaning). I am just glad to know something for sure so we can stop worrying about the weaning for the time being. She also has some specific requests for the GI to check on Nicholas' upcoming endoscopy to look at the mitochondria in his gut. That should be interesting...

She ordered more testing for the boys (which they drew today) and wrote Nathan's rx for new orthotics. She also gave us our protocol letters. She also wants ME tested for several things as she said, from the sounds of my medical history, it would be worth looking into, especially since many forms are maternally inherited. Since I am pg, it is especially important to know if I have it or not. Based on the boys labwork, she is upping their Carnitine (uh-oh insomnia!), and adding in CoQ10, Riboflavin (B2), and Folate based on weight. So, they are OFFICIALLY on a Mito cocktail now.

Guess that is all the high points...sooooo much to remember! The boys LOVED her again and warmed up quickly to her this time. Nicholas knows her name now and calls her "the nice lady dr who is in the tall big building". She wants us back to follow up in 3 mos.

What a week. The last few weeks have been like living in constant stress and fear and now that we have a dx, we are SOOOO ready to move forward out of Limboland and get on with the rest of our lives!! Thanks to all of you who have supported us on our journey thus far and we hope you will continue to check up on us as we start this new chapter of our journey with Mito as a part of our lives. Take care and God bless!!

Melody

Friday, September 28, 2007 10:44 PM CDT

Well, this week has been full of surprises...and not all of them good ones.

We finally all recovered from all of us having the coughing crud this last weekend. Nicholas and Nathan still have a cough, but not too bad.

Tues, Nicholas was just finishing up his TKD lesson and Nathan all of a sudden just puked EVERYWHERE! The first heave went down my back and the 2nd landed in my lap. My friend, who has a dd in Nicholas' belt class, was sitting next to me and jumped up to grab some paper towels and help me clean up. She is awesome! Then, while I cleaned up the couch and myself, she went to get one of the instructors to get some cleaner so I could sanitize the couch. Nicholas' instructor came over and told me to let him help...that he had 7 brothers and sisters and he did this kind of stuff all the time...and then told me to go home so I could shower and he would take care of it. Ok...ANY single childless man who offers to clean up someone else's kid's puke has got to earn major kudos from me!! I kept trying to get him to let me do it, but he insisted. With being pg, I was all too relieved and grateful and I let him. I am just so glad my parents left when they did. My dad just had spinal surgery and is in a brace and he would not have been able to handle the smell. UGH. The puking continued several more times during the night. We still do not have our protocol letters, so in desperation and fear, I emailed our mito dr and she responded 13 min later with all the info we needed. Thankfully, he recovered well and did not end up dehydrated enough for the ER, but it was scary nonetheless.

Wed, I had my 15 wk OB appt. I got all the results back from the ultrascreen and the prenatal panel they did 2 wks ago. Everything looked awesome! My risk for any of the trisomies actually were better for this pg than they were for Nathan's, which surprised me. BP was 120/68 which is amazing, even for me normally and especially during pg. I lost another lb tho so I am only +1 lb total for the entire pg instead of the +3 I was at my appt 4 wks ago. A first in my ob relationship happened today...she actually had to fuss at me for not gaining ENOUGH! Usually I am gaining too much, so that was a weird turn of events. I swore to her I really was eating and we think it may have been from that bad sinus inf I am still trying to get over. I promised to try to do better. The best news of the appt was my cervix measured in at 3.6 and anything over 3 is great, so I am super relieved about that. The baby sounded good and HR was 152 and even tho I don't look very big on the outside, she said inside the uterus was measuring right where it needed to be. She said that when they give me my RhoGAM shot at 24 (or was it 26?) wks, they will also give me the lung maturing steroids for the baby, just in case. she said why take risks when it is such an easy thing to do. I am really glad to hear she is taking precautions. Hopefully I will not need them, but better safe than sorry. I go back Oct 24 for the u/s to find out the sex and see her for my next reg appt. Only 4 more weeks until we see the bean again!

Thurs, Nicholas had his fitting for his new braces. He had to get new ones because his current SMO's were making his knee hyperextention so much worse. Dr K wrote the script for a basic brace and gave the PT and orthotist leeway to work out the specifics. I was expecting what he had before with the SMO's with a piece up the back and a strap at the knee. That is what we had discussed. I asked the PT about the brace on Tues at therapy and she tried to explain it, but I could not get a picture of what she was explaining so I figured I would just wait and see for myself when we pick them up. We went Thurs around noon and he has BOTH an SMO insert inside the brace and an exterior articulated AFO with straps. This AFO is more supportive than the old ones Nathan has and he was having so much trouble walking in them as he was desperately trying to put his knees back and fight the brace...so much so that the orthotist went back and adjusted them even more to make the dorsiflection more so he would have a harder time fighting the brace. He is going to have to relearn how to run and jump in them too. He was sad and I was even sadder to see how much regression he has had in needing to go from a basic SMO to this. What makes me even sadder is that if Nicholas does well with these, Nathan is getting the same ones. He has a casting appt set for 3 wks and the orthotist had to increase his dorsiflection in his old AFO's he is back to wearing now, as he is able to overcome the brace and put his knees back at times. Thankfully tho, he still fits them well enough that he can squeeze about another month out of them until he can get new ones casted and made. I know they are going to be better for the both of them...after walking in them for a bit, Nicholas told me they were comfortable and felt good, even tho they still felt funny. I am glad he is taking to them well. I just hope the kids at school are nice about them when he comes in with them on Mon. I guess I just need to get used to seeing these regressions, but man it hurts when you realize how much it has gotten worse and know there is nothing you can do to stop it. Hopefully, this will do the trick and we can get him back to doing his usual stuff without all the problems he has had going along with it.

Today we had Nicholas' sleep-deprived EEG and MRI/MRS with general anesthesia. We had to keep Nicholas up all night so he would sleep for the EEG. It actually went better than I thought and it was really nice to spend time one on one with Nicholas when we usually do not get a chance to. I usually only get the one on one with Nathan these days while Ncholas is in school. We went out to eat, just the 3 of us, after dropping Nathan off at my parents' house, closed down Target shopping, browsed at WalMart, came home for a before midnight snack, watched DVD's, played games, went out to run an errand and got gas in the car and drove the car through the gas station car wash, and then Nicholas played his Vtech baby video game before we left. We had a good time...the hard part was trying to drive downtown in rush hour traffic in the dark and keep little man awake. I think he hated me by the time we got there. They had to send us 2 places to check in and we finally got downstairs and set up for the EEG. Nicholas never complained at all. The goo smelled like lemons, the pencil almost put him to sleep he was so tired (even tho he said it did hurt some), and he really thought the idea of being a spaghetti-head was funny...he was too sleep deprived by then and everything was funny. He barely made it through the one min she needed to record him awake and then he was OUT COLD. He started right in with the twitching and I was able to look for the twitch and then look over the tech's shoulder to see if anything looked different and it did when he twitched. It would go from small bumps to big jagged looking peaks, and then back each time. Not sure what is normal or not and I did not ask her then, so I have no idea. I am just glad he WAS twitching for the test seeing as how the kids NEVER choke during swallow studies and that kind of stuff and then we miss getting to see what is really going on when that happens, kwim? He was SO tired he went into level 3 (or was it stage 3?) sleep pretty quickly and she had to have me stimulate him and she also flashed a strobe light into his eyes to keep him from sleeping so deeply. Then, one min recording awake again (which he was super upset at) and it was done. She said they would read it today and our mito dr would have it by Mon at the latest. I will be interested in seeing what the report says. Then, we were taken upstairs to do the MRI/MRS. Poor baby was a wailing sobbing mess at this point and finally fell back asleep. Meanwhile, the anesthesiologist came in to speak with us and when she heard he had a cough (that was now 3 wks old and almost gone) she was saying that, according to their MH precautions, they are unable to intubate anyone with an active cough unless it is chronic and as good as it gets for that patient, esp anyone who has a history of airway issues and being treated for them. We were devastated. I burst into tears from the stress of this week, having to orchestrate all this today and thinking of having to do it all over again, dh having to take MORE time from work, the exhaustion of being up all night, and from being hormomal and emotional in general. I then started to feel crampy, almost like I was contracting, and that freaked me out. I was stressing, likely dehydrated from not drinking in front of my clingy little man, and stressed to the max. I was shaking as well. It was not fun. She looked like she felt just awful and was trying to see whatever she could do to help. Since he was out cold at that moment, she suggested trying it unsedated. He has been ok with CT's unsedated before, so I was game. We took him down and he woke up and we knew he was not going to sleep again, so she tried Versed and all that did was make him loopy, out of it, and not able to follow directions well. We still tried it hoping he would relax, but after 2-2 min scans were a bust, we gave up. He tried SO hard, but just could not hold still enough and his brain looked like a blurry blob on the screen. We are now rescheduled for Tues at 7:30, arriving at 5:30. UGH. PLEASE pray for the cough to be gone by then!!! As for me, once I was able to come home and relax some, I realized a lot of what I was calling "cramping" was actually coming from my lower back and when I touched either side of my lower back, it was tender. I did not realize this was the actual issue in all my rushing around and strsssing, and I think my kidneys were hurting. When we went back and looked over the past 24 hrs, I remembered having 2 glasses of iced tea for dinner last night and I think it must have been caffinated. Add into that not drinking in front of little man when he was NPO from midnight to after 10 am this morning, not eating, and stressing, I think I must have gotten WAY more dehydrated than I actually knew. I have been downing water and cranberry juice this afternoon like there is no tomorrow (almost to the point of being sick to my stomach from forcing it) and I am finally feeling better. I was so concerned about the pain at one point, that I got out our doppler to check the baby's heartbeat, and after a frantic long few min, was able to locate the baby and the heart was perfect at 152. That was reassuring enough to let me sleep for quite a while this afternoon with everyone else in the house.

Next week is going to be obnoxious...a rescheduled MRI/MRS, both boys see A/A, Nathan has his first ECI/TYKE transition meeting, and then Fri is our BIG appt with the mito specialist to review ALL the labwork and testing. Then, the next day is Nicholas' Fall Festival for his preschool. We are going to meet ourselves coming and going! A prayer request...Doug and his family have had some things come up this week that have caused some great concern within the family. Please pray for them all right now as they all could really use any you could spare. Our MN friends are also in need of prayers...their daughter and son are both ill and their little girl is not healing as they had hoped she would. They certainly could use prayers as well. Take care, God bless, and know we all think of you and appreciate you checking up on us.

Melody

Sunday, September 23, 2007 11:21 PM CDT

***UPDATE*** Tues evening...***

Please pray for Nathan. He started vomiting this afternoon and has been throwing up everything he tries to take in. We are not to the point of the ER yet, but it is getting closer. He has no tube to keep him hydrated like Nicholas does, so this is worrying. we have already been in contact with their new mito dr and she knows what is going on in case we need to bring him in. Please pray for him to stay hydrated and feel better...

_________________________________________________________________

I am definitely getting better at the updates...or at least trying! Hopefully once a week will work!

Monday we worked like crazy to get Nathan's urine sample in the tin foil covered cup to keep it protected in the light and I literally put the lid on the cup, packed it up, put Nathan in the car, and drove straight to the lab. I handed it to the lab tech exactly 15 min from him urinating. I had to prep Nathan before walking into the lab tho...after the traumatic last visit there, I wanted to make sure he knew he was NOT getting stuck when we were there. He was visibly relieved when we left and asked "no booboo?"...poor kid.

Tuesday we got an email from the mito dr. She wanted to let us know she had not forgotten about us and to also let us know both boys came back with elevated lactate. This is NOT good for mito kids and it is pretty indicative of mito issues. Normal is <2.2...Nathan's was 2.8 and Nicholas' was 4.3 . Nathan's makes sense as he was struggling against the draw (as it was drawn on the 3rd stick)...he was also really worked up and crying hard, so that may have affected the level slightly. Nicholas, however, was sitting perfectly still and not fighting, struggling,or crying at all...just sitting. I know there is no way anything he did during the draw affected his lactate. These results are now delaying the protocol letters she is writing for the boys as she wants to see what else the labs show before finishing the letters. We hope to hear from her soon about the rest of the labs, but will definitely be going over them at our next appt on Oct 5th.

Tues was also Nicholas' belt test. He was testing for his blue belt. He did AWESOME and I could nto have been prouder!! Here are some pics from the day!

Nicholas got chosen to demonstrate how to give a proper greeting when they were practicing before the testing began...

All lined up and ready to go...they are saying their tenets in this pic...

During his portion of the test, he had to stand on one foot for 10 sec...he chose his stronger foot and still had a lot of trouble...but BOY did he try! I was proud!

Here he is demonstrating proficiency at palm heel strikes...the woman helping him is one of his fave instructors...we just learned she is only 17, a Sr in HS, and she is a 4th deg black belt!

Receiving his new belt...the guy tying it on him is the head instructor of the academy and a 5th deg black belt...

Standing with the instructors...the main 4 are the tall adults in the back...the ones with red stripes are in the instructor trainee program and the small kid on the left with the glasses is only 9 and a 2nd deg black belt already!!

Showing off his new belt and certificate...

We went to San Antonio Friday for the boys' GI appts and he sure hit us with a shocker...he is moving to Knoxville, TN. So, if any of you around that area are looking for a GREAT GI who really cares, you are getting super lucky. He said he was going to try to work it out with the hospital here in San Antonio that he can come back a couple of weeks avery 2-3 mos to follow certain patients (us included in this group), and if that is the case, we will still hopefully get to see him. If the hospital will not allow this, we will be out of luck and needing a new GI. I am SO upset...he has been with us since the beginning with Nicholas and for Nathan's entire life. I so don't want to start over with a new dr. He told us the name of one at TCH he said was the one who he would recommend over all others, so we will try to get an appt with him set up Mon as I am sure the wait till be around 6 mos and that will put us close to the time when our GI is leaving.

Nicholas is doing ok...he wants to check and see how well Nicholas' reflux is being managed on the meds, so he wants to scope him and likely do a Bravo pH probe, which is the pill camera one. Before he sets this up, he wants us to talk to the new mito neuro we are seeing and figure out if/what she would like done while he is doing the scope. He said if she wants biopsies, he will take them for her, but wants to know what kind...full thickness or whatever...and he will take them for her during the scope. Also, if she wants anything specific looked at, he wants to know ahead of time as well so he can plan or that also. I thought that was so cool of him to want to help her if he can. He wants Nicholas to see Endocrinology due to the 2 yr delay in bne growth they found on his bone age scan. He said that delay is significant enough to warrent looking into it further. Ugh...adding in another dr...soooo not thrilled with this development. We are also to talk to the new neuro about Nicholas' tube feeds and see if she has any insight as to why he is having such trouble weaning off them. If he needs the calories due to mito, that changes things and he wants to have more info before figuring out what to do from here. He may need to change formulas to make it better absorbed or something, too.

Nathan was pretty straightforward. Pretty much all we did was move his daily dose of Prevacid from pm to am so we can better control his reflux symptoms during the day.

Guess that is about all regarding GI...boy am I bummed about him leaving. We started driving the 3 hrs one way to San Antonio when he left Houston to move there. It was not ideal for us, but best for the boys to have continuity of care, so we have driven the 6 hr round trip for the past 3.5 yrs every 3 mos. I sure hope we can find someone who cares about the boys as much as he does. Nicholas names his GI by name as one of his 2 fave drs and this man has offered to have us over to his house to see his new pony and told us he would treat us all to lunch when Nicholas gets off tube feeds. Guess that won't happen now. Tennessee is sure going to luck out when he moves there early next year. Nicholas is going to be devestated when he finds out.

Nicholas will be getting his new braces this Thurs. We are FINALLY getting along with this situation pretty well now that our Phys Med dr is out of the picture. Nicholas' PT called the new mito neuro and LOVES her. She said they had a wonderful conversation, she felt the dr really listened to her, and took her concerns to heart. They came to the conclusion that Nicholas (both the boys really, but only Nicholas is with this one PT) is going to be a kid that is likely going to need 2 separate sets of braces. A set of ankle high SMO's for good days and mornings, and a set of knee high SMO's for bad days and later in the day when he needs more support. The new neuro agreed and wrote the script for the knee high SMO's and approved the current SMO's to get cut down to a brace height that he can use without it encouraging his knees to go back like they were before. Dh got the braces in to be cut down today and Nicholas now has an appt for next Thurs to get his new knee high braces once they finish making them.

As for Nathan, his PT came over Thursday to work with the mobility company to help with the tie downs and stroller mods (can you believe the tie downs FINALLY came??) and while she was here we discussed things. Nathan still somewhat still fits into his old AFO's. She was really upset by how bad his knees had gotten as now the left knee is doing some weird inward rolling sort of thing during his stride while he walks and she wants him immediately back into the AFO's for a few weeks until we can get his braces situation sorted. She wants us to have the orthotics guy check the AFO's for fit next week when we pick up Nicholas' new braces and if they are ok for a little bit longer, she wants to see how Nicholas does with the knee high SMO's and then she may order them for Nathan. We see the new neuro a week after Nicholas get his and if they work out well, she wants the neuro to write the scrpit for Nathan to get a pair. If the fit of the old AFO's is not going to be ok for him, she wants to call the new neuro and he will probably get another pair of articulated AFO's until we see how Nicholas' braces work out for him. Whew...what a relief to be almost done with this major mess!!! His PT is so awesome too...she blockd out Nathan's 1st transition meeting to KISD on her calendar so she can go and make sure they understand that, while Nathan looks good on paper, he has issues that need to be addressed and she is going to explain what she is seing from a therapist's standpoint. I think it is going to help us a lot and she is going out of her way to help us. We just love her and are going to miss her so much once he ages out of ECI.

In addition to all the above, we have all been sick with some sinusy/allergy stuff this week. I started getting sick Mon and Nathan did then as well. Nicholas was coming down with it Tues and by Thurs, Doug had it as well. We have sounded like a bad chorus...all coughing and sniffing and sneezing. I think we are finally getting over it...thank goodness!

I have an OB appt on Wed and Nicholas has his sedated MRI/MRS this Fri. It looks as though he is going to have to be put under general anesthesia as they think it too risky to allow him to be sedated without constant supervision. The only way he has supervision like this is to have general and an anesthesiologist with him. I am not thrilled, but better safe than sorry.

Guess that is about all...the next week is going to be packed full as is the week after that. I am tired just thinking about it. We will keep you posted on what happens. Take care, thanks for checking in on us, and God bless each of you!

Melody

Sunday, September 16, 2007 10:00 PM CDT

So, another week and a whole lot to update...

Nicholas started school last week! FINALLY!!! He was soooo excited. He is in the ladybug class and has so many neat things planned for the year. His best friend from last year is not in his class, but he DOES have recess with her class 2 different times during the day, so he is really excited to be able to see her every school day. He has another little girl from last year's class in his class and he has been playing with her some in class since they know each other. Here are some pics of him his first day!

Sitting by his school bag, packed and ready...

...and standing by the door ready to go!

Nathan has had a hard time getting used to "N-key" being gone at school. When we go to pick Nicholas up, he starts calling out "N-key, coming!!" over and over again once we get into the parking lot. He is super excited to see his brother when we get into the classroom to get him.

Both boys have something brewing...likely another URI, so we are upping breathing treatments and starting decongestants again. I am hoping this is the end of it for a while, but this is starting to look like it may be a long year, illness-wise. Thankfully, both boys got their flu shots already and are set for the year in that respect. We are still trying to decide how to approach the revaxing situation since we need to make a decision soon now that sick season is almost upon us and neither has a decent amount of vaccine protection right now.

I have been sick since last weekend as well and finally just started eating normally again this weekend. I was so nauseated and ill I could not eat and lost 1 of the 3 lbs I had gained thus far and the OB was not happy. She prescribed Zofran for me to take, but luckily, the symptoms began to subside and I ended up not needing them after all. I am finally starting to feel human again, thank goodness...keeping up with the kids was really hard last week.

Both boys had a lot of testing done for the new neuro this week...saying it did not go well would be putting it mildly. I sat for almost 3 hrs Tues morning to try to get urine samples for the dr. With Nathan not being potty trained and trying to avoid him being cathed, I did it (tho it was awful). I packed up the urine in a cooler and ice packs and took it with us to therapy and then we MISSED TKD to go get the blood draws to make sure it got done. This made one little 4 yr old NOT happy, which did not help matters any. The lab tech was...well, there really is no word for her honestly. She flat out refused to draw some of the labs since the boys had not been fasting. I told her the Dr knew they would not be and was ok with that. I went on to explain that they have a suspected metablic disorder and should NOT be fasting...even for fasting testing. She still refused and said "she is JUST A DR...she does not know about this stuff. They order the testing but have no idea how the testing is really done". I told her this dr was a METABOLIC dr and she KNEW this stuff, but to call the dr and ask for herself. By some stroke of luck, when she called, our neuro was RIGHT THERE and talked to her. She set the lady straight about the boys not needing to fast before this testing. Well, after this was all said and done, the urine samples I spent ALL morning collecting were worthless since they needed to be "light protected" and ours were obviously not. I was able to get it from Nicholas, but Nathan WILL have to be cathed since it has to go straight into a protected cannister and everything. I am not sure I can do that trying to catch it. This tech went on to make me feel stupid for asking if we needed to haver the light off when he peed...well, she made it sound like ANY light would damage the sample. Then she also said they cannot do the Pyruvate there as it has to be mixed with some acid and they cannot do that there. So since I had no idea when we would make it clear to the other side of town to get that one done, I asked her could she just do the rest so we would not hold them ALL up for the one. She then made me feel like a terrible mom for "wanting" them stuck 2x...like I WANT any of this, lady??? I finally burst into tears and said "fine then...I am leaving". She then had the NERVE to ask me if I was OK...ummmm...what do you think genius??? I am bawling my eyes out.... stressed to the max... pg and hormonal... tried for HOURS this morning to get worthless urine... come here to be judged and belittled by a lab tech... told I had to drag 2 misbehaving kids to another lab clear across town to get this draw... told my child was going to HAVE to be cathed... nobody told us about all the specific precautions so how was I supposed to know... I am still sick and feel horrible already BEFORE this whole mess... and you have the NERVE to ask me if I am OK??? NO I AM NOT OK!!! I was so upset I just wanted out of there...I accidentally left the cooler bag and ice pack there. Who cares...they can have it. I had to schedule a nurse appt for Fri morning to get Nathan cathed. I am NEVER setting foot back in that lab again. BTW - the reason we HAVE to use that certain lab is because it is the ONLY one we are allowed on our ins...THANKS A LOT UHC...GRRR! Doug was so ticked he called to complain about her.

Fast forward to Friday morning...so, we get up and go to the peds Friday morning to get Nathan cathed for his urine sample. The nurse threads the catheter in and would you believe the kid's bladder was TOTALLY DRY??? She put that tubing so far up there she just barely had the end in her fingers and NOTHING...not even ONE DROP. I was shocked into tears as the poor kid was writhing and screaming and it was all for NOTHING. So, we leave and go grab breakfast at McDonald's to try and regroup before getting the blood draws. We go to the lab and I had to tell the techs Lactate has to be drawn with no tourniquet...the look they gave each other should have been a bad omen, but I ignored it and now I wish I hadn't and just walked out then and there. Nicholas went first and they tried to stick him with NO tourniquet at all!! I tried to explain USE it for the first tubes and then release it when you are about to draw the lactate, but they did not listen until after they blew the first vein going in without the tourniquet. Nicholas is usually the easy stick ever and never requires extra sticks...these women took THREE...yes you read that right...THREE sticks to get 4 vials of blood from him. Poor kid sat there face all scrunched up, but he never cried and all he said was "ow...that hurts" and they were digging that needle in there until they got it right. I could hardly look it was so bad and I KNEW Nathan was going to be a nightmare if they had that hard a time with Nicholas. They did worse with Nathan...3 sticks and a vein blown so bad he has a HUGE knot on his elbow that still has not gone down and has turned a nasty shade of blue. Before the draw we had tried to get a urine sample on him there, but were not able to get one, so they sent us home with a sample cup and directions to use tin foil to cover the cup and bring it in immediately after getting the urine. Poor kid was SO traumatized when we left the draw room he threw himself on the floor of the lobby and had the biggest world'class tantrum I have ever seen and I did nothing...he deserved to get it all out...it was sooooo awful. Ok...so by this time I am a mess, the kids are even worse and Doug is stoic. We leave and take them to ToysRUs for a toy as that had to be bar none the WORST stick ever for both of them. I am afraid to try for the sample at home for fear of messing it up, but am more worried about taking him in for a 2nd cath and having to torment him again and possibly have it be for nothing a 2nd time. I told Doug between all the sticks for the mito and also for the immune deficiency, something has got to change if the number of draws they are going through does not decrease. We cannot keep putting them through this crap all the time. I am just heartbroken...pray for us tomororow as we try for the urine sample. We are sure going to need it.

I had the ultrascreen for real this past week. The baby has grown quite a bit since 2 wks ago. The heartbeat was strong and the baby was moving all over. She had a really hard time getting to measure the baby's neck as it was literally bouncing off the walls. I told her I had thought I had felt the baby moving a couple of times, but my sister was sure it was too early. She said with how much the baby was bouncing around, she thought it was likely I had felt the baby...it was a wild little thing!! According to her theory...looking at this little bump thingy...she guesses a boy, which is what I had been thinking, but said even tho with this theory, her guesses are usually accurate, sometimes she has been wrong, so don't count on anything just yet. Otherwise, she said everything looked really good and the baby looked perfect! I am so excited! Here are a couple more pics...

Here is one of the baby in it's back and you can see the little arm reaching up and the foot above the belly...the dark spot is the stomach with fluid in it...

Here is the baby again with the little hand resting on it's belly and you can see the stomach again in this one too...be sure to check out the tiny footprint by the belly...

Hope that will tide you all over for a bit! This week holds for us GI appts in San Antonio, trying to get a urine sample, and several therapy appts. Thanks to all who continue to check in on us and keep us in thought and prayer. We really appreciate it. Take care, all, and God bless!

Melody

Friday, September 7, 2007 11:09 PM CDT

Ok everyone!! I officially had the best birthday present ever today! This new neuro dr the boys saw today was the BEST! She was nice, pleasant, GREAT with the kids, conscientious, and VERY thorough.

She thanked us for bringing in their records and complimented us on how organized they were. She said she would read every page before deciding on what specific testing to order as she did not want to put the boys through anything unnecessary. She did flip through Nathan's neuro section to see what results she could quickly find and reviewed those with the resident before coming in.

Her first comment to us was that in kids under 5, even those who have OBVIOUS mito (genetic proof, etc), only 50% of biopsies will actually show abnormalities...even if they have proven mito. I thought that was interesting. Then she said because Nathan's DID show findings consistant with mito, this was VERY significant and very indicative of mito. Then she went on to say that she did concur with Dr Parikh in that she could not be certain if the mito was primary or secondary to something else as he STILL has not had enough testing of other things to rule out the possibility of a currently undx primary disorder.

She then went on to examine the boys and was SO good with them. They both liked her and laughed at her when she was messing around with them. She made the exams she needed to do into a game and the kids had so much fun. She asked us if we minded her talking to the resident and explaining stuff to him in front of us and we said not at all. I appreciated her asking permission and it was really interesting to hear her observations to him about the boys. After that, she sat down to talk to us and explain some things and then allowed us to ask all our questions. She even came back after another patient to answer some things we had forgotten to ask!

She ordered a TON of bloodwork saying she was looking for mostly non-mito related stuff and wanted to check and see if she needed to add anything to their cocktail. She wanted to check CK levels which have never been checked before, and also wanted us to put a sample of urine from both boys into the sun for 1 hr and see what it does and then email her to tell her what happens. That should be interesting! Apparently if it does something, it is significant. Not sure what we are looking for, so I guess we have a science experiment on our hands. She is also sending us a letter saying ER protocol and we are NOT to sit out in the waiting room and are to go straight back to a room with a list of things to do. She said at the ER at this hospital where her clinic is, they know her by name and take her letters very seriously. I am relieved to know we would not have to do the marathon waits we have had to in the past at TCH. She is also going to take over our orthotics and said she defers to the therapist's judgement as to what the kids need, so I am thrilled we are going to be able to get out of WW3 with our phys med dr and PT. I am to give her email and ph# to the PT so she can get in touch with her. Our PT is going to be in HEAVEN!! She also ordered Nicholas' 1st MRI...praise GOD! It is about time we are going to get a baseline on him in case we ever need to have something to look back on for comparison.

Ummm...I think that is it. She said to email her anytime we have a problem and wants us back in 1 month after she has adequate time to review their records and decide what needs to be done.

Can you tell how impressed and thrilled we are to have had such a wonderful appt with this dr? She is such a Godsend and has been SO promt to reply each time we have emailed her...even before seeing us when we were not "officially" her patients yet. Nathan had an incident earlier this week where we found brown urine in his diaper and we were too afraid our current neuro would blow us off and our ped would not know what to do, so we emailed her and she replied back within 30 min to say she would rather be safe than sorry and take him in to do these labs as soon as you can. We feel so very blessed to have found a good dr so close to home who is willing to help us.

In other news, we sure had a wonderful holiday weekend last weekend! Doug had that Fri off as his off Fri and also Mon for the holiday, so we had a 4 day weekend to spend together doing fun stuff. Fri we took the boys to the science museum for the first time to see the butterfly center and to look at the museum exhibits. Nicholas was lucky enough to have a butterfly land on him and he has been talking about it ever since! They LOVED it! They were also pretty impressed with the dinosaur skeletons, but I think Nicholas expected LIVE ones at first! He asked a lot of questions about them. They also enjoyed the energy exhibit as well. They left with a dinosaur and a butterfly book and each chose a stuffed dino. Sat we hit the malls for some shopping and Nicholas had TKD. Sun we went to church and had a swim party with the boys' godmother. Mon we took the boys to Galveston to visit Schlitterbahn, a water park. It rained while we were there, but 1/2 the park is under a roof, so we were not out in the rain then and we only had 2 episodes of lightning where we had to exit the pool. Both boys had SO much fun! This was their first time to a water park and they enjoyed it! They had special double seater tubes which had one part with a mesh bottom for small kids to sit in so they did not fall through and also stayed attached to their parents. It was nice to keep them close by and not have to worry. We had dinner at our favorite seafood place there and came home very happy and tired.

Nicholas starts school Mon and hopefully things will be settling back into our usual schoolyear routine really soon. Thanks for all your good thoughts and prayers, especially for today. We appreciate you ALL!! Take care and God bless!

Melody

Wednesday, August 29, 2007 10:07 PM CDT

Well, I know this update is LONG overdue, but I promise...for those who do not know yet, it will be well worth the wait by the end of this update!

Let's see...so much has happened, I think I should just do the condensed version...

* Nicholas has his Phys Med appt and that went well. He was casted for his new braces and the PT and orthotist HATE them on him. Both boys are having worse issues with knee hyperextension in the new ones (same braces). This has led to the BIG battle going on right now...PT/Orthotist VS. Phys Med dr. It has not been pretty. The drama is ongoing. I will update when someone shows the white flag of surrender and they come to an agreement as to what needs to be done.

* Nathan failed 2 of 3 parts of his recent swallow study. Thin liquids are a problem...he takes too big of sips and when he swallows, there is not room and it forces some of the liquid into his trachea and with every swallow, liquid was actually touching his vocal chords. This is not normal. He did not even choke or cough, so the SLP was sure he was aspirating when he does show distress. He swallowed a cheeto whole as well, so he failed solids as well. Purees went well tho, so that was the saving grace of the day. We are awaiting news from the GI about this as well as waiting on the report so we can start him on Oral Motor therapy through ECI.

* Nicholas had a bone age scan and that was interesting...a simple x-ray of the hand. Turns out he has the skeleton of a 2 yr old and not a 4.5 yr old. That surely explains why he is SO small and is roughly the same size as a lot of 2 yr olds we know (39 in). Waiting to hear back from GI on this as well to see if an endocrinologist consult is needed or not.

* Nathan had his annual ECI eval and did REALLY well! He did better than we would have ever thought! So well, in fact, that we have dropped PT to 1x/mo! That sure is a far cry from 1x/wk like he was at first! We are likely increasing either OT or ST to weekly due to the recent swallow study and addition of oral motor therapy tho.

* Both boys saw immunology...our immuno is out on maternity leave and we saw a dr who does not know our boys, so it was a very anticlimactic appt. They drew lots of labs to test Ig levels and vaccine titers...results are pending. We are hoping to hear in the next week or so. We will update when we hear something.

* Both boys have been sick with a cough and URI. Nathan has been worse off and has needed much increased breathing treatments. He is finally on the downside and it is about time since we have been battling this for over 2 wks now.

* Nicholas is doing well in therapy as well. He starts school on Sept 10. He is super excited and so are the rest of us. This LONG summer has taken it's toll and he is READY to go back! He is doing awesome in TaeKwonDo and has his green belt. He is testing for his blue belt in a couple of weeks.

* Both boys have dental appts tomorrow. They also both have appts with the new neuro next Fri (my bday). The greatest bday gift I could get is a local dr who is knowledgeable and willing to help us. Let's just say our local neuro has been less than cooperative or helpful and we will never darken his office doorstep again for other reasons I will not go into here.

* Lastly, but not least, is our family's biggest news of all. We will soon be adding a new little one to our family. I am expecting our 3rd child and am 11 wks along. I have been soooo tired and that has made updating difficult. We saw the little one for the first time on u/s today and what a beautiful sight it was to see! A wonderful little 2 inch moving bean on the screen! Nicholas thought the baby was waving at him...it's little arm sure was waving around, so I bet it was! The OB was so pleased with how well things are going this time around...she says it is the best pg I have ever had thus far. Getting my PCOS under control was the best thing I ever did for myself. This pg has been sooo easy compared to the past ones and I am sure hoping things stay that way. Here are some pics of the little one!

The first one is of the baby on it's back with a fist by it's face (at the left), a foot over it's belly, and a little leg sticking straight up (at the right)...it was stretching while we were watching!

The second one is just of the baby on it's back with the fist next to it's face just chillin'.

We are just in love already...

And on that fabulous note, I will close this update. Prayers would be gratefully appreciated next Fri as we meet this new dr and pin our hopes on the help she can offer us...and also for this new little life, that the pg will be easy and the baby will be healthy. Also, please keep our MN friends in prayer as they are going through a very rough time again with the health of family members. Take care, God bless, and know we think of you all!

Melody

Monday, July 23, 2007 12:53 AM CDT

**Update Sat, July 28, 2007**

Please pay for our sweet dog Rudy that he is now running pain-free in heaven with God. He passed away this afteroon at around 1 pm. :o(

__________________________________________

Sorry for the delay in updating...so many things going on and I have not really been in a place to update.

Nicholas has been having big sleep issues since returning from Cleveland. He basically averaged anywhere from 3-6 hrs a day (no naps) for about 6 wks. As you can imagine, that made for one super overtired, crabby, grouchy kid who was having meltdowns all the time. It was seriously awful. We have gone through every tactic we (and anyone else) could think of and were getting nowhere. We had appts with the neuro and ped and got help with some techniques to try from his therapists...all to no avail. We tried Carnitine to see if that would help. Then we thought it was the Carnitine making things worse, so we took him off...no difference. So, we finally have chalked it up to Nicholas getting used to sleeping with us in the same room in Cleveland and somehow he had convinced himself he could ONLY sleep with us with him. After a week of letting him cry it out, he is finally beginning to sleep better with fewer wakings and fewer meltdowns in the night. The rest of the family (as well as Nicholas) are understandably exhausted and every free minute has been spent trying to catch sleep where we can. I hope someday we will be caught up. Weeks of barely sleeping is no fun for anyone.

Nathan got his wheelchair stroller a couple weeks back. They still did not fit it with the tie-down system, so they have a retrofitting kit on order for us and will fix that when it comes in. Nathan's PT was on hand when it arrived to help fit him for it and she was amazing! I will post pics when I get a chance.

Nathan also got his new SMO's and we already are having problems. We went from too much support to not enough and he is having issues with knee hyperextension. The orthotics place went through their bag of tricks and nothing helped, so they are remaking them from the mold with higher backs to see if that will help. He should get them on Thurs.

Nicholas sees the Phys Med dr Wed and we will figure out what kind of new orthotics he will be getting. He has basically trashed this pair and they are hanging on by a thread right now...we are hoping they make it until his new pair is finished. It is looking like he will be getting knee-high SMO's according to the PT's assessment since his knee hyperextension has been so bad lately. The good thing about these are that they can be cut down to ones like Nathan will be getting when he no longer needs the full support of the tall ones. He will be getting casted for those on Thurs when we pick up Nathan's new ones.

We had a nightmarish appt with the neuro last week. It was so disappointing, I am not even going to go into detail. Let's just suffice itto say we are looking into getting a new dr immediately as this one is no longer working out for us. He has decided the kids need no more medical help from him despite info from the specialist in Cleveland who says otherwise and he basically refuses to carry out treatment requested by the Cleveland dr here locally. We have an appt with a local mito researcher who sees patients in Sept. She came very highly recommended by an online friend who met her at this year's Mito Symposium and she was very impressed with her. I hope she can be of some help to us. If not, we have an appt with another geneticist who has an interest in Mito that might be a good fit otherwise. I guess we will see how things work out.

Last week we had VBS for the first time and the kids LOVED it. Nathan was not accustomed to being left every day for a whole week and had a hard time with drop-off in the morning, but did wonderfully during the rest of the time. Every day when I would go to pick him up, he would say to me "have fun!". Nicholas had fun as well and especially enjoyed the singing. I taught a 3rd grade class there as well and Doug even was a sub on his Fri off since they needed teachers, so it ended up being a family affair. We all had a good time and are planning on doing it again next year.

Other than that, both boys have been doing well. Rudy is hanging in there as well. He is having trouble with his eyes now and is getting so thin. I look at him and wonder how much longer he will be able to hang on. He does not look good at all, but since he is still acting as perky as he can, I am not sure he is ready to go yet. Since he is breathing ok and still eating fine, we will continue to watch and wait and pray we will know when his time is coming so we can let him go peacefully.

Guess that is about all for now. sorry for the delay in updating. We have been living in a sleep deprived fog for a while now and it is hard to think straight most days, so I have been putting off an update until I can have some coherent thoughts. Thanks for your patience and understanding. I am hoping that his sleeping will be back on track really soon...fingers crossed. Please pray for our MN friends as they really need some more nursing or medical care help for their kids. They are having difficulty finding someone willing to drive out to where they live and willing to deal with all the medical needs their kiddos have. I know any prayers you can spare would be gratefully appreciated. Take care and God bless all of you.

Melody

Saturday, June 23, 2007 10:37 AM CDT - HAPPY BIRTHDAY JON!!!

** Please see previous journal entries for updates from our Cleveland trip to see the Mito specialist and also to see pics!! **

Yesterday, we had our regular GI appt in San Antonio. It was an eventful appt. We talked about our trip (which had come up since our last appt with him) and he was happy to hear we were on our way to getting some answers for them. He decided to redraw liver enzymes since Nicholas' liver was a bit enlarged (by about 1cm he said) and also because he wanted a baseline for them both in case things changed over time, so he would know. Both boys got a stick, but Nathan fared much better this time and I was so relieved. I asked for hot packs to help dilate his veins to make the stick easier and they definitely helped. Nathan was none too happy to have to sit still with me holding them on both arms while there was a playroom just feet from us, but he did so well during the stick, his complaining was worth it. Nicholas had found a Blues Clues toy that also helped distract him and the stick went very smoothly and we got plenty of blood. Nicholas did well also. Depending on the results of the bloodwork, he will order a liver ultrasound for Nicholas (or I guess Nathan too) if it looks warrented at that point.

We also spoke about the choking Nathan has been doing recently and asked about a swallow study since it has been about a year since he has had one and the GI thought that would be a good idea since he has evidence of aspiration in the past (on his bronch) and the choking thing has only just recently began to be a problem again. That is an easy test, so hopefully it will go smoothly and we will figure out what is going on.

In going through the boys' records to familarize ourselves with what was in them before travelling to Cleveland, I happened to notice quite a number of xray reports over the past few YEARS that talked about getting Nicholas evaluated for skeletal dysplasia because of his sz and his bone growth. Amazingly this has not ever even once been brought up to us and needless to say, it was unnerving to see after we have been asking about his height and slow growth for so long. We mentioned this to the GI and he decided we needed to get a bone age scan done on Nicholas to see if he is delayed in bone growth or not. He is 4.5 and just hit 38 in for the 1st time at the drs yesterday.

We also continued the discussion of scoping/probing them from the last appt and he wants to wait until the next appt to make a final decision. He wants them to have a Bravo pH probe instead of the one with a wire and the youngest they will do these on kids is 2.5 and Nathan is not quite old enough yet. For this probe, they swallow a pill (he would place them during the scope instead since they do not swallow pills yet) and wear a trasmitter box and the pill just travels through the body and records info much better than the kind with the wire. Then, after about 2 days, they will get rid of the pill probe in their stool and we turn in the recording device and want for the results. Sounds MUCH easier to me than the one with the wire. I can SO see Nathan pulling the wire out!

We are trying to wean Nathan off his Erythromicin and see how he does. If he does well and the testing results come back looking good, we will discuss weaning him off reflux meds entirely...YEAH!!! Good news for him! As far as Nicholas goes, we discussed tube weaning again and will be trying a VERY slow wean and see how he does. Very slow meaning around 20-25 cc's at a time, which is just under an oz. Hopefully this will make a difference because it is a tiny amt at a time and we can be successful this time.

We got together with our SA friends and the kids got to play at their neighborhood water park and had a blast! It was a good day overall.

Last week, Nicholas saw his OT at the new place for an eval and he is actually doing very well with his therapies! He might even be up to par with his development in as little as 3 mos and no longer require intensive OT like he has been getting! YEAH!! Barring any setbacks, we are hoping for good progress!

This coming week, Nathan has an appt to get his new SMO's, so be on the lookout for pics! We are so excited to get rid of the tall braces this summer so he can have a chance to have at least part of his legs out and open to the air. He should be able to stay a lot cooler this summer without knee socks and tall braces, so that will be good for him. He also has his adaptive stroller being delivered sometims this week (hopefully!) and we will be done with that as well. Nathan's head is almost better as well...just a small amt of bruising left and a small bump, so hopefully it is almost better.

On a side note...God works in mysterious ways. We received a letter yesterday from Boston stating they are no longer accepting new patients for the time being. So, had we not contacted Cleveland when we did, we would have been starting over from square one today. God is GOOD!

So....that is about all for us right now. We are now in the process of trying to get appts with all the necessary drs to disseminate the news we got in Cleveland, so I can see this being an ongoing thing for a while until we can see them all. Thanks again for all the prayers...they were surely felt and appreciated. Take care and God bless!

Melody

Thursday, June 14, 2007 10:20 PM CDT

*** Please see the previous 2 journal entries for updates from our initial appt and follow-up appt with the Mito specialist in Cleveland ***

Well, we are home and have been trying to settle back into a routine, but it has not been easy. The kids are pretty exhausted still and have been napping quite a bit. Nicholas started back to TaeKwonDo after missing 3 lessons while we were gone and BOY was he happy to be back! He sure missed it!

Yesterday, Nicholas had his PT eval with his old PT at the new therapy place where she is now working and it went well for the most part. He is doing better in areas involving his legs (YEAH!!), but has actually lost strength in areas involving his trunk since his last eval. He used to be able to do sit-ups, but since his setback a few weeks ago, he has lost the ability to do those at this time. He is also still struggling with areas involving his arm strength as well, but that has not changed one way or the other since the beginning. He has his OT eval with his old OT at the same new place this coming Monday, so we will see how he is doing then with his OT skills.

We are in the process of trying to update all the therapists one at a time since we have returned. Nathan saw OT yesterday and PT today and both are happy with us returning with more answers than we had when we left. It is helpful for them as well to have this new info so they can do their best in working with Nathan.

Now, onto some pics from our trip!!

Here are some pics from our visit to see Doug's uncle. He is bedridden and has not met the boys before. We had a great visit and the boys were so good.

Sleeping sweeties...

Good morning!!

Fooling around with daddy...

Doug, the boys, and his aunt and uncle...

We also took a side trip to Cedar Point, an amusement park about an hour away from Cleveland. The boys had a blast! Nicholas rode his first roller coaster ride and both ended up being way more daring than I would have ever guessed riding the rides! The weather was iffy tho...when we were driving there a weather alert came over the radio for Cedar Point and we got there in time to unload the kids and run to the picnic shelter when the rain started coming down. We were arriving when everyone else was fleeing the weather! It was about 30 min of nasty weather, but cleared up and since a lot of people had left, we barely had any lines. We had to buy sweatshirts tho as the temp dropped about 20 deg and we did not bring our coats as it was HOT when we left!

The boys on a helicopter...

...and on a tank...

...and on a rocket...

I know you are sick of pics at this point, so this is the last of them!!

The boys and me in Little Italy after having the BEST pizza for lunch!

The boys playing in the water table at the Children's museum...

Playing in the bus at the museum...

The boys and me watching a movie before bed in our room at the Ronald McDonald House...

Finally, the last set of pics...it would not be a trip of ours without something totally off the wall happening to us. Nathan had an unfortunate accident about an hour before our first appt with the Mito specialist. Here are some pics of my poor baby. He was playing on the playground...Doug was pushing Nicholas and I was standing with a friend we met for lunch, chatting with her. Suddenly Nathan ran towards Nicholas and got whacked by the swing before anyone could stop him. Doug even yelled out to him, but he never stopped. He got hit so hard, he went airborn and flew through the air a few feet before landing on his back and by the time we got to him a few seconds later, it was bruising immediately.

Here is a pic of him about 15 min after it happened (Thursday)...

And here he was Sunday sporting a bump/bruise still and also 2 black eyes...

He now has a big yellow bruise on his head and still a small bump and 2 black eyes. I told Doug enough with waiting for the facial bruising to go away to get the 2 yr old pics (have been trying since Feb!) and we should just get some boxing gloves and take them right now. It is definitely Nathan right now at this point of his life!

So, that is about all for us right now. Hope you enjoyed the pics of the lighter side of our trip...we tried to make it as fun as we could in between the appts.

On a side note...PLEASE pray for our MN friends. Their 6 yr old son is fighting for his life right now and is unstable and in very critical condition in the PICU. They are unsure if he is expected to make it at this point. Their 17 mo old daughter is scheduled for testing inpatient next week to investigate her breathing issues. They are struggling right now between the severe medical issues of both their kids. Please pray for comfort, peace, strength, and healing right now. I hate Mito...:(

Take care and God bless...

Melody

Monday, June 11, 2007 8:05 PM CDT

***See previous journal entry for the update from our 1st appt!!!***

Well, we had the boys' follow-up appts today and went, armed with our page long list of questions.

We spoke at length about Nicholas and the tube weaning and he gave a suggestion that had not been mentioned before, but also said that because we had hit such a wall with the level where he currently is, maybe he just has that level of caloric needs at this time and he was totally ok with leaving things where they are for the time being. He mentioned that he thought that we would know when it would be time to drop more time since Nicholas would probably show us with weight gain, so I am relieved to hear we can leave it for now. I think the GI will be receptive to that.

They will not need to be on any special diet as neither has any fat malabsorption issues, so that is not an issue for them at all.

As for testing when we return, he will let us know the results of the testing run while we were here (and NO CATH by the way, as Nathan peed in a cup the next morning after us holding one down there for 30+ min...WOOHOO!), he gave us labs to run the next time Nicholas is sick so we can start building a case for him (less testing and data for him than Nathan has) as least invasively as possible for now. Nicholas will also need a liver ultrasound to look into his enlarged liver, tho he is not too concerned since the liver enzymes have always come back ok. Both boys will also need to be followed closely by the opthalmologist as well and have a more comprehensive visit than most to monitor their eyes. He said he would leave it to the dr's discretion here as to what to do beyond that. He will be sending a comprehensive care plan for both boys to TCH so the drs there can know what his recommendations are for them.

As far as returning for follow-up, we spoke at length about that as well and we were told that he deals with all kinds of follow-up...from every 6 mos to every 2 yrs, to as needed. He also said he was available to any of our drs who needed him via phone or email to answer any questions they have and to let them know of any new testing out that would help them get to the bottom of exactly what may be going on with them. When we spoke at the end about a specific dx for them, he is certain they both have a Mitochondrial Dysfunction. His gut feel on Nathan is that he does have Mito and he just needs a little bit more to get the "proof" he said he was looking for to tell us for certain. As for Nicholas, he is relatively sure that what they both have going on IS genetic and once we find out Nathan's issue, we will have Nicholas' answer as well. He suspects Nicholas has it as well. Thankfully, both are mild and pretty much, what we heard is "what you see is what you get" in that they are likely to not get much worse unless something drastically changes for them. We are to just treat them as if they have it until we have a concrete answer one way or the other and we will continue testing them as new things come available and see what we find.

So, for the most part, pretty decent news! I am SO glad we came and I am really excited that we will be able to manage things and feel more comfortable when they get sick or have hydration issues. My mind is much more at rest and peace after talking to this dr and I am confident that they will be getting good care from their home drs with the help of this dr. Whew...what a relief... Thanks for all the good thoughts and prayers coming our way while we were here. Tomorrow we return home. We are just so blessed with the support we received form all of you and we are grateful. Take care and God bless!

Melody

Thursday, June 7, 2007 8:17 PM CDT

We arrived in Cleveland yesterday safe and sound AND after receiving a phone call 10 min before boarding our flight that RMD house had a room for us...YEAH!!!! Unfortunately, Nicholas' tube feeding formula had been overnighted to the hotel instead and when we arrived at the hotel to pick it up, they could not find it. UGH! We had to use the emergency 1 day stash we take with us on the plane for last night's feed. After calling the HHC this morning and finding out when it was delivered and who signed for it, Doug called back and let them know that info PLUS how much it was going to cost them to replace it ($200) if they did not find it. Amazingly, they called us right back, having located it, and we were able to go pick it up.

Now onto THE appt update I know you have all been waiting for...

Well, it was LOOOOONG, but went well. The dr had this computer program where they have some type of scale where they assign points for certain things...he asks you questions, inputs the answers, and it gives a score. The score is compared to a range...a certain # and below is Not Mito, a certain # and higher is Definitely Mito, and in between is Possibly Mito. Both boys were right smack in the middle of Possibly Mito. Still no definitives yet, but this dr was surprised that certain tests had not been done and some new tests have come out since he had testing done and he wants those done as well. We are still focusing on Nathan for testing right now since he has the muscle tissue still out there and they have more data to compare the testing to, so he was the (un)lucky one to earn a urine and blood test today. He wants us to have a dr who is current enough on Mito that will know when new/revised/better testing comes out and will recommend us getting these when they do come along...if we cannot find one there, we can stay with him and continue to travel. Not the best option, but we will if necessary. He gave us a protocol letter (YEAH!!!!!) and wants both boys started on CoQ10 and Carnitine and he said with kids in the mild/moderate range like ours, they see the best results with these meds, so he was confident they would help the boys. He told us that he was not sure with the mild/moderate symptoms they were having if they had a primary Mito or if the Mito was secondary to some other genetic something going on, but did tell us whatever they have IS genetic. Unfortunately, the boys have just enough atypical things ("twists" as he called them) that he was not quite sure what to tell us for certain (just like my kids to NEVER fit), so right now he can tell us that there is definite Mitochondrial Dysfunction, but he is unsure why they are dysfunctional...but they boys did fit most closely into either a Complex I or Complex IV deficiency, so he is starting the focus there and also with some genetic disorders NOT related to Mito to see if the Mito might be secondary.

Nathan had to have had the all-time WORST blood draw ever when we went down to the lab after the appt. I cannot even describe just how bad it really was. After 30 min of screaming, struggling, fighting, and hysteria, the tech finally got about 2 in of blood...1 in in each tube. He was not sure if there was enough. UGH. They tried 3x to get urine in a bag and it did not work, so we are likely to have to go back in tomorrow and have Nathan clean-cathed to get the sample. I already told Doug it was HIS turn to help torture the poor kid tomorrow. We felt so bad for Nathan afterwards that we bought him the gift shop and Nicholas got lucky and rode Nathan's coattails and got stuff too. Each got a balloon, a stuffed Thomas train engine and a sucker. We are such suckers....but it really was THAT BAD.

On a side note, we got to meet up with a friend from an online site and her adorable son at the feeding clinic here. She was a lovely person and bought us lunch and we had a great visit. After lunch, we took the boys out to play on the playground and Nathan ran under Nicholas while he was swinging in this huge swing and the corner of the swing hit him so hard, he went airborn and was thrown back a few feet onto the ground. Poor kid had a bruise as soon as we got there to pick him up and has the hugest goose egg on his forehead. It was just awful. Thankfully, this dr we saw was a metabolic neuro, so he checked him over and said it was superficial (thank goodness) and looked worse than it was...we got soooo lucky. We have pics, but will have to wait until we get back to post them...sorry. That is it for today. We have our follow up appt Mon afternoon, so I will post more then.

Thanks for all the support...it really means a lot. Take care and God bless!

Melody

Wednesday, June 6, 2007 6:59 AM CDT

Well, today is the day. We are just about to pack up the car to leave for the airport and will be in Cleveland by dinnertime. Please pray for us as we are hanging quite a bit on this appt in the hopes of getting some answers once and for all. It is also an uneasy time as we are having to board the dogs and had found out just last Friday that one of our dogs, Rudy, is dying of a vary aggressive and fast moving cancer. There is literally nothing to be done and treatment would only prolong his life for weeks at best, so we (along with our vet's guidance) have decided on pallative care only for him. He thinks he has several weeks to maybe a couple of mos left. We are just hoping he does ok while we are gone and is still hanging in there when we return. We will not be staying at the RMD house when we arrive as the waiting list was too long, so we will be at a hotel. We will be calling over to the RMD house to hopefully move over there once space becomes available, but until that point, we may or may not be able to update the site with a report from the boys' appt tomorrow. We will try our best. Please pray for safe travels for us and for answers to be had at our first appt tomorrow. Thanks once again to all those who have supported us and take care and God bless!

Melody

Tuesday, May 29, 2007 0:29 AM CDT

Just a quick update to ask for prayers...

Nicholas had some sort of episode earlier this week where he had an increase in weakness and a decrease in muscle tone...he had his therapists really worried. He seems to be bouncing back ok, but is still not back to baseline and we have NO idea what happened. All of a sudden, things he could do with ease 2 wks ago, he was struggling with in therapy. He has had a rough week behavior wise and has had an increase in sleeping as well, taking 3+ hr naps several of the days this week. We are just hoping that whatever happened will resolve itself in time.

Nathan is fighting something right now and has a very high fever. He woke with a 104.2 rectal this morning several hrs after a dose of Motrin and we can only get the fever down around 103 piggybacking Tylenol and Motrin. To get it lower, we have to give him a tepid bath, which makes him scream and is terribly uncomfortable for him. The ped's office did not transfer their phones to the answering service this weekend apparently, so we have been unable to contact anyone regarding his fever. He did ok in the middle of the day today, but after we left Uma and Papa's house where we were visiting, we started having problems getting the fever under control again and have not been able to get it down much since then. He currently has NO symptoms, so we are hoping it is a simple ear infection, but if not, we have no idea what is causing such a high temp. He has a Physical Med appt first thing tomorrow morning and we are hoping to have her get a peek into his ear to see if it looks infected or not and then we can decide if he needs to be seen at the peds or not.

Please pray for them both right now, along with our MN friends, as we all could use some right about now. This appt in Cleveland cannot come soon enough for us. Take care and God bless...

Melody

Tuesday, May 22, 2007 10:54 PM CDT

Well, what a past few days it has been. Our trip had been planned perfectly down to the flight arrangements and appts and such and then we got the mail on Saturday. We received a new patient packet for Nathan from the Cleveland Clinic and so I put it aside to fill out once all our records were ready to be sent. Then, yesterday morning I saw it sitting there and something made me go look at it. The appt date said 8/1/07!! I had a heart attack and called Doug immediately to tell him there was a problem! Come to find out there was a scheduling mistake and the new patient appt was scheduled for Aug instead of June! After some wrangling, we were able to secure Thurs appts the same week we were to be seen on Fri and we had to change our flight arrangements up a day to Wed, which cost us almost $100 more, but it was nice of the airline to waive the flight change fee since this obviously was not our fault. Thankfully, it worked out, but boy we sure did NOT need the extra stress right now while preparing for this trip!

Nicholas' fasting levels came back in and they looked better for the most part. He still had some high levels, which the neuro said could be indicative of Mito, so he said he was looking forward to us going to Cleveland and seeing what they had to say. I am ready too...

Doug and I celebrated our 12th wedding anniversary this past Sun. We took the boys to the Melting Pot, a fancy and pricey fondue restaurant, in lieu of gifts to each other. They had a great time and I really enjoyed a nice dinner relaxing with our sweet little boys. I think we may start this as a new tradition...we decided we do not need any material things anyways and this is a geeat gift the whole family can enjoy.

Now...for some totally exciting news!!! I am SO proud of my baby!!! He earned his yellow belt today! He is so proud of himself!! The white belts were last to go up, but he waited patiently and quietly for his turn. He had to recite the Tiger Cub promise from memory, recite the 3 rules of concentration, demonstrate high blocks and punches, and demonstrate proficiency in all 3 listening positions. Here are some pics of the testing and awards ceremony...

Nicholas waiting with the other white belts for his turn to come up in testing...

Nicholas on his mark waiting for the testing to begin...

Demonstrating a high block in the skill portion of the test...

Nicholas receiving his belt during the awards...

Getting his new belt put on by one of his instructors...

Sitting with his fellow yellow belts (Tiger cubs are all white belts, but earn colored stripes in the middle of their white belts in the same progression as the juniors and adults program)...

The tiger cub testing class (Nicholas is near the left side end of the front row)...

Nicholas with his instructors (his main one is the guy on the left)...

Nicholas showing off his belt...

And his certificate and uniform patch...

Sorry for getting carried away with the pics...I am so proud of him right now I could just burst!!! He has come SO far from the delays he had as a baby! He is still not quite caught up, but man has he done well!

Anyways, that is about it right now. I will keep everyone posted on our trip. So far, everything looks ready, minus the records we STILL need from San Antonio, but that is another angry post and I am in too good of a mood after tonight to post about downers. Hope all are well. Take care and God bless!

Melody

Wednesday, May 9, 2007 11:25 PM CDT

Hi everyone! It's time again for another update.

Might as well start the update with a bang...today we got the best news! As many of you probably remember, we have been busily preparing a referral to see a Mito specialist in Boston. It is taking quite a bit longer than expected due to the amount of time it is taking to receive copies of out of town records. We had considered Cleveland Clinic in Ohio as a second option so if should something fail to work out with the Boston referral, we would have a backup plan. Well, Doug had called them around the time we contacted Boston, and we had heard nothing back, so we proceeded with the Boston referral and did not worry about Cleveland. Today we actually got a call from the Cleveland Clinic! We ended up being late to PT because of it, but wow...what a great reason to be late! The woman asked me to tell her about the boys, and so I told her a summary of the boys' symptoms and the things we have been dealing with recently and after all that, she said that it sounded like they might be able to help the boys and that the dr would love to see them! I was elated...especially because I am getting frustrated with the endless flaming hoops we seem to be having to jump through to be able to get the boys seen in Boston. She then asked me when we would like to be seen. I immediately assumed it would be several mos until we could be seen as the wait for Boston is 6 mos and for TCH specialties can be anywhere from 4-7 mos. When she told us the earliest available for both boys to be seen the same day was JUNE 8 I about had a heart attack!!! I was almost going to ask if she meant this year...lol! So, now we are in a mad rush to get those records in and sent off to Cleveland to see Dr Parikh (Dr Cohen's partner) now instead of Boston and make travel arrangements to Ohio to get answers once and for all!! I cried tears of joy and relief today because someone finally is taking responsibility for our boys and wanting to help us get an answer once and for all. The woman I spoke with said she felt like we had valid concerns about our boys' health and that their symptoms sounded a lot like what they see on a daily basis there in their clinic, so I felt better at least, knowing that there is a problem we need to get to the bottom of. Please pray for the boys and this dr...that we can leave with answers once and for all...whatever they may be.

In other news, Nicholas is doing awesome at his TaeKwonDo!! He has belt testing coming up May 22 and will be trying for his yellow striped belt. He is very excited and focused on getting this belt, so I am looking forward to seeing him accomplish this! He has 3 more school days left and has done so well. Doug and I went to his parent teacher conference and he did fabulously well. He got checks for every single thing and his teacher had nothing but good things to say. He truthfully is ready for kindergarten, but has one year yet as he is not old enough. We are just so thrilled he has adapted so well to school, enjoys it, and is excelling at it socially and academically!

Nathan is progessing well in his therapy. Since we had his ear tubes replaced, his speech has really taken off. He is repeating so many more things and sometimes surprises us with things he says out of the blue all on his own. He is even starting to point to letters and name them or their sounds!

We met up with a special needs playgroup through our local ECI the other day. Nicholas enjoyed meeting another child with a tube, both enjoyed playing with the other kids there, and I enjoyed chatting with the other moms there about drs and therapies and other things I don't really get to talk about like that with most people. We had a great time. Unfortunately, the heat really sapped the kids, even in the shade and low 80's. We paid for it yesterday as Nathan had trouble walking and keeping his balance and was falling constantly and both boys were grouchy. Nathan barely made it through OT and then crashed on his couch for over 2 hrs after that, just laying there and watching TV and resting. Thankfully, both are better today.

Nicholas had some basic testing done (finally!) in preparation for our trip and his labs came back looking not so great. Of the 31 amino acids they tested for, 16 came back high...some mildly and some very high. We are repeating the testing fasting Fri morning to see how the results compare. I am not thrilled with the fasting since it may not be the best for Mito kids, but I guess we will do what we have to in order to get to the bottom of things. I guess we will see how those come back this time.

That is about it for us right now. Thanks for checking in on us. We appreciate each and every one of those who come here to support us. Take care and God bless...

Melody

Tuesday, April 24, 2007 6:43 PM CDT

Well, what a few days it has been since we updated last!

The biggest news we have is that we are seeking a referral to see Dr Korson in Boston as we are just not ok with waiting for a year to see what happens in the next year, like we were told to do by the metabolic specialist here. The wait for an appt is 6 mos and once we get our referral packets in (they came Sat), they will be able to call us and hopefully schedule us an appt. This dr is one of the top 3 Mito specialists in the country and we are hoping he can put to rest the question of Is it Mito or not? If it isn't Mito, maybe he will be able to point us in the right direction to find out what it is. We are hoping to have all the paperwork sent in by the end of next week. Please pray for this to be a quick and easy process...the longer it takes, the longer we will have to wait for an appt.

Nathan has been doing wonderfully since his new tubes were put in!!! I am SO excited with all the new vocalizations we are hearing and all the new things we are hearing him try to say. He learned how to give real kisses too the other day! They are big wet sloppy kisses and I LOVE them!!!

Nicholas has big news! He started Tae Kwon Do today!! We have been looking to put him in some type of program for some time now, but had no idea what he would like and what things would be easiest for him to do where he would not be compared to others on a team or have something be frustrating for him as it is a bit too hard for him. After talking to a number of therapists (his and Nathan's) and several parents, we decided to look into Tae Kwon Do. We stopped by several places and took Nicholas to watch a class of kids his age and he was SO interested! We bought a starter pack of 3 lessons to see how he liked it and after his first lesson today, he LOVED it! Can I just say thank goodness it was FINALLY today??? If I had to hear "is it Tuesday yet" one more time, I was about to scream!! And, he did such a good job for his first class! I was so proud. He listened better then some of the kids who had the yellow striped belts in his class! His uniform is huge tho...they gave him the smallest size they had...a 000...and he still had to roll the waist 3x and the cuffs of the pants and sleeves once! Here are some pics...

Nicholas in his uniform before class...

Nicholas sitting in the mat room waiting for the class to start...

Nicholas trying a kick on the paddle target thing...

Listening to the teacher (note how much bigger the other kids are in his class...he is the smallest one out of the 4-5 yr olds)...

Nicholas blocking...

And here is Nicholas tonight during his breathing treatment...I think between school and TKD today coupled with no nap, we have one exhausted little man on our hands...

We also got to take the boys to Space Center Houston on Doug's last Fri off. We had such a good time. We took a tour of the building where they keep the Mock-ups (replicas of the shuttle, space station, etc) that the astronauts practice on, we saw an IMAX and a show on how people live in space, we touched moon rocks, and walked inside models of different rockets, and ate dippin dots...space ice cream! We all had a blast! Here are some pics...

The boys and me in front of a huge mural of astronauts...

The boys standing in between parts of the Saturn V rocket...my dad helped design/build parts for this exact rocket back when he worked in the aerospace industry!

Nicholas trying out an astronaut's helmet...

Daddy helping Nathan touch a moon rock...

The boys inside a rocket...

Finally, here are some pics on how an astronaut gets into a space suit on Earth. We went on a tour to this palce where they keep life sized replicas of all the shuttles, space station, Soyuz, etc. To go on this tour, you have to get pics taken for security reasons, get issues a temporary visitor's pass, and they only stop at the one stop instead of several stops like they used to. We went a week before the hostage was taken, btw. Since we went on a Fri during the work week, while we were in this building we saw a REAL astronaut climb into a REAL space suit!! Dh and I were totally impressed! A little group of us on the tour almost got left since we were watching this and not paying attention to the tour group! The guy had to climb a ladder and slip down into it. The part on the back that looks like a backpack opens like a book to make an opening for the astronaut to get inside it. Here are some pics...

The astronaut waiting in his cooling suit (he is in the middle of the bottom of the pic in the white)... You can see the space suit getting adjusted by the guys in the yellow hard hats...

Climbing up the ladder and getting ready to get in...

Sliding into the suit...

In the suit and getting locked into it...

Anyways, that should hold you all for a while! I will be sure to keep you all posted on the referral to Boston. I hope you are all well. We are light on the appts this month, but next month will be making up for that for sure! Hope you are all well. Take care, know we think of you all, and God bless.

Melody

Sunday, April 8, 2007 8:53 PM CDT HAPPY EASTER!!!

We had a wonderful Easter today, despite the cold, wet, and yucky weather! We ended up having to purchase new Easter outfits for the boys as they were supposed to be wearing shorts and short sleeves. No way was that happening when it was a high in the 50's and a low in the 30's here today! I had to dig out a white sweater twinset to wear in place of the short sleeved sweater I was originally supposed to wear. We all had a good day and things went smoothly in spite of the strange weather. The boys woke and enjoyed checking out their baskets to see what they bunny had left for them and then got ready for church. Nicholas spent his first time in "big church" today. We attended the 8:15 (early!! we usually do the 11:00 service) service and he was amazingly well behaved. Thus far, since being a baby, he has been to the Children's Christmas service several years and otherwise goes to Children's church with his Sunday School class, so this was a big day for him. He LOVED the music and quietly colored his Children's program during the service. I was so proud. After that, we went to my parents' house for our Easter get-together. We had a yummy ham dinner for lunch and my parents had made a delicious bunny cake the kids LOVED. We sure do have a lot of talented cake-makers in this family!! We also had an egg hunt for them before the kids were so tired, it was time to head out for naps. Once naps were over, we gave them each an Easter present just from us and have lounged around in jammies the rest of the day.

Yesterday, we celebrated my sister-in-law's birthday and went to a fun egg hunt at their church. We celebrated with Doug's side of the family for the birthday and an early Easter. We had a great visit and the kids thoroughly enjoyed the egg hunt. There was a clown who made balloon animals, a pretty princess who painted faces, dancing, games, and more eggs than I could begin to count! They boys had to quit the hunt when it was obvious their baskets could hold no more eggs! Each of them won a golden egg in a drawing as well...it contained a dollar!! They were excited to be able to put it in their "penny" (piggy) banks.

Here are some pis of today (we forgot our camera yesterday :o( ).

Nicholas showing off his Wiggles towel from his Easter basket...

Nathan smiling about the goodies he found in his basket...

Family pic taken at my parents' house...the kids were SO done with taking pics by this time! And I am wearing makeup in this pic...mark it down since it does not happen too often!! ;)

The adorable (and yummy) bunny cake my parents made...

Nicholas checking things out with the new binoculars my parents gave him...

Nathan vacumming the play mat...

Nathan opening his Easter present from us (it is a remote controlled cement truck)...

And Nicholas showing off his present (a train set he has been begging for forever)...

We had a wonderful weekend spent with family. It was really nice.

Before I end this update on such a good note, I would like to ask for all you who read this to PLEASE pray for our MN friends tomorrow. Their daughter is having surgery and has had a rough time recently. It is risky and they are worried about how she will come through it and how her recovery will be. They need all the prayers we can send up for them...I really appreciate any prayers you all can spare for them. They are a wonderful family who have been such a good support for us with our boys. Take care and God bless. Hope you all have a blessed Easter!

Melody

Tuesday, April 3, 2007 10:52 PM CDT

Ok, ok...I know this update is LONG overdue. Things have been super crazy around here the past week and a half!

First off, Nathan had his T-tube surgery on Friday and did wonderfuly well and it all went relatively smoothly. We had a small disagreement of sorts with the anesthesiologist about which med she was going to use. Doug had called the anesthesiologist Thursday to talk about precautions for Nathan's surgery. She was going to use gas and he told her that Nathan cannot have gas. She says ok then...we will just use Propofol. He tried to explain to her that he is not supposed to have that one either. Well, she tried to explain to him 7 different ways that it should be ok for short periods of time. Finally, she told him that we would discuss it tomorrow. Well, the day of his surgery she comes in and says that they were going to use the Propofol and I said NO...he CANNOT have it. She then tries to explain again how it would be ok. I then asked her, so are you saying that there are NO other alternatives to the Propofol then? And she backpedals and says there are other options. She gives us the choice of Ketamine (sp?) and then proceeded to tell us all the reasons why it would be better to use Propofol then the Ketamine...the risk of increased secretions, the need to give him Robinul to control the secretions and Versed for some reason, the need to stay in recovery longer for him to come out of it (since it would take longer), etc, etc, etc. I finally told her that was fine with me...give him whatever extra meds he needs and since we have nothing planned the rest of the day, we can stay as long as he needs to, but NO Propofol. She finally realized I was not going to back down, so she relented and said if that is what you want...YES...it is. My stomach was literally tied in knots by the time we were done talking...I was so afraid she was not going to change her mind and I was fully prepared to walk away if that were the case...way too much stress for that hour of the morning!! She did request LMX for him since he is such a hard stick and the Ketamine has a pain relieving property, so he woke in a much better mood than he would have otherwise. The only issue we had really, was the fact that he developed Nystagmus from the Ketamine and it has since gone away. It is a side effect from the drug that goes away when it leaves the system. Unfortunately Doug did NOT tell the anesthesiologist that Nathan cannot have the lactated ringers and he came out of the OR hooked to a bag of it. Nobody mentioned it to us and we had no idea they did not know proper Mito protocol. Ugh. Thankfully it was already off when I saw it on him, but he had already had some. She was not too happy we wanted something different than what she had planned, but it worked out actually better in the long run.

The ENT said his left ear was clear and looked good (this was the good tube that fell out, so the abx he was on for 2 wks must have helped that ear) and said the right was VERY congested and packed with junk. It drained a lot when he placed the tube, so he cleaned it out and it still drained a TON on the sheet after they sent him to recovery. This was after he had been on 14 days of abx, too. Yikes! He woke slowly and had some trouble maintaining his O2 sats after coming off blow-by, so they put him back on it and he did well the next time they tried to turn it off. His mind recovered faster than his body (he coild not stand/walk until late in the day Fri) and he was his usual attitudy self pretty quickly, but boy was he MAD that he could not move around like he wanted to! Thankfully, he was not able to move well, so he was not able to pull his lines out...and only 4 sticks this time instead of the usual 8-10 so hooray for LMX!!! We were out of there by 11ish and got to do the cancer walk with my mom that night, so he recovered well!

Since the surgery, Nathan's almost constant coughing has all but stopped. He is breathing better and sleeping MUCH better with no wakings! YEAH!!!

Both boys also received their Medic alert brwcelets we ordered them! They call them their "pretties" and they rally like them. I am glad we have not had a fight on our hands to get them to wear them. Nathan also had his Theratogs fitting last week and he is going to be getting the full suit. He will be using the vest and chaps bottoms with a small X cut strap to pull his shoulders back and 2 thick stretchy straps to hold his ab muscles in. He looked SO good...straight and tall!! he had a straight line from his ear to his knee for the first time ever!! The therapy place where Nicholas goes will be ordering them soon. I will post pics when they come in!!

To add to the confusion of preparing for Nathan's surgery, the boys' furniture was supposed to be delivered Fri, but Nicholas' bed was damaged so then they told us Tues, so Doug took off the day to be home to take down the crib. Turns out the truck broke down between here and Dallas and the shipment did not make it. We were ticked...we have too many appts and stuff to just waste time off like that. Then they said it would be in the next day...and it was, so another day off of work. The problem then was that Nicholas' drawers did not fit (too big) and they had to reorder them. Today, the drawers were finally installed, but they very nearly didn't. Somehow they had brought over another whole new bed instead of just the drawers and the men were telling me they only had a bed. I asked them to please open the box and check to see if drawers were in there and thankfully, they were. Whew...finally all here and all taken care of. We have been culling toys and other things here and have made some big donations lately and will have a delivery truck coming to pick up some big things tomorrow. We do not have an inch of space left in this house! We got all new knobs for everything in Nathan's room so it all looks like it matches and Nicholas got new knobs for his bed that go with the handles on his furniture as well.

Nathan did great his first night in his new bed! The only real issue we have had recently is that he whacked his head at 4 am the morning of his surgery. We had no clue what could have happened and I nearly had a heart attack because on the monitor it sounded like he had fallen out of the bed! Turns out he hit his head on the windowsill...somehow we missed that it stuck out, so $1.49 and some sticky corners later, all is safe now with that area! He will wake and play with his toys and then call for someone to come get him, but he has not yet tried to climb out. We are SO proud!

Here are some pics for you!!

My old childhood dresser, scrubbed with a magic eraser and with brand new knobs and handles (we decided Nathan did not need the 70's girly pink/blue flowered handles it used to have!)

Nathan's new bed...it has 3 drawers on the bottom and a pull out trundle...

Nathan's new toy storage unit...

Nathan's mural on the wall...I freehanded some of the pictures on the comforter and painted them...

Nicholas' bed...this was taken before the drawers arrived today. Iput a drawer front we had up against it so you can get an idea of how it is supposed to look...

Nicholas' toy storage unit...

Big boy in his new big boy bed!!!

Nathan actually asleep the first night...

Margo...this one is for you!! The boys having their breathing treatments on their new chairs!!

Another interesting thing that happened ot us this weekend was that we went into the car dealership on Saturday to pick up the car from being serviced and left with...a NEW car instead!! The car Doug was driving was having problems and we took it in. We thought we had the 5 yr 75k mile warrenty, but that was the van we were thnking of. The warrenty ran out 5k miles ago. UGH. The part that was malfunctioning was the BCM and that was the computer "brain" of the car and to replace that was pretty pricey, especially with no warrenty to cover it. Well, turns out Saturday at the dealership they were having a sale and our salesperson we used for our van came and said let's look and see how much it would be for you to leave with a new one today. We kind of humored her and stayed since it was a free lunch and it turned out when she came back with the figures, to put down what we would have been paying to replace the BCM, the monthly payments for a brand new model of what we currently have would be $30 LESS per month than what we are currently paying! We were totally shocked!! Since we knew we would be paying that money anyways, why pay to fix a car with no warrenty if you can drive out of there with a new car for less per month? We got pretty much the same thing he was already driving...a 2007 Saturn VUE in storm gray. I think God was looking out for us since we were getting worried about what we would do when this warrenty ran out as it was coming up soon.

Anyways, guess that is about it for this installation of our lives! Hope you are all wel. Take care and God bless!

Melody

Monday, March 26, 2007 0:10 AM CDT

Well, what a weekend it has been! My little Nathan is now a 2 yr old and he sure welcomed this stage of life in with a BANG at his party!

We went for his birthday dinner Weds night at Kids Night at Rainforest Cafe with the boys' godmother. We had a blast...balloon animals, clowns and costumed characters, yummy food, singing, a sparkler, and a volcano dessert...what more could a boy ask for? Then, Friday, we had a little get together at Chuck E. Cheese with our playgroup friends for Nathan's birthday...it was his actual birthday then. The kids had a wonderful time. We ate yummy pizza, heard Chuck E. Cheese sing, played games, and got to pick prizes with our tickets! Everyone enjoyed cupcakes as well before opening presents. That night, the boys were supposed to get their new beds (hence the birthday dinner earlier in the week), but Nicholas' somehow got damaged during shipping to the store, so we opted to wait until the new bed they reordered for him came in before they delivered it. Hopefully it will be in by the middle of the week. Nathan opened his presents, which included his new bed set for his big boy bed. He also received a special package from a dear friend as we were leaving to go eat and when we opened it, we were greeted by a Bues Clues fantasy! Nathan wanted everything opened before we left and the inflatable chair he got is now his new breathing treatment seat in front of the TV! The Blue from the playset even travelled with us to dinner that night!

Yesterday, we had the family party...all 17 of us in the family over for a cookout. We grilled out hot dogs and sausages, and had chips and queso, baked beans, salad, and all the trimmings. The kids played outside on the swingset and in the sandbox and the adults had a good time visiting. After we ate, it was time for presents. Nathan enjoyed the first one, then freaked out about the rest. He screamed, arched, and struggled to get down and by the time we were done with unwrapping the gifts,I was drenched in sweat. We finally calmed him by putting him in the crib and leaving the room...before, he was just hitting and kicking at me and screaming and struggling, so I didn't know what else to do. It broke my heart...especially to see the rest of the family as they watched him open gifts. Instead of a happy time full of smiles, it was a rush to get everything opened before he escalated into something really bad. Finally, he calmed enough for us to sing to him and so he could eat his cake. He did ok during this and then went straight down for his nap when everyone left. Doug's mom really outdid herself on the cake for Nathan's party...it was AWESOME!! Everyone was super impressed with how wonderful it looked.

Here are some pictures of the festivities...

Nathan showing off his smile and his monkey in a tree balloon...

I just LOVE this awesome pic of the boys and their godmother!!

Nathan eyeing his Volcano dessert and trying to blow out the sparkler...

Nicholas enjoying his dessert...

Nathan tasting his cupcake at Chuck E Cheese...

Nicholas helping Nathan put his game token into the slot...so sweet...

Nathan sitting with his birthday gifts...the space bedding and planet pillows are for his new big boy bed...

Peeking into a gift bag...wonder what's in here???

He loved getting sung to and he blew out his candles all by himself!!

The birthday boy eating breakfast and all ready for his big family Coos Coos party...

Nicholas got the party started outside...he drew a rainbow on the ground...

Nathan was our little sandman...he had sand pretty much every single place sand could go!

Me helping Nathan open his presents...this was pretty much the only one where he was NOT screaming...he was breaking in his new 2 yr old attitude already...

The cake...isn't it wonderful? It is 2 cake mixes, just to give you an idea of the size of it...

Nathan calmed down enough to have his birthday song and some cake...boy he liked the cake (along with everyone else)!

Hope you enjoyed the pics!! I will post an update later on the rest of things, but I thought our special little man deserved his own update all about his birthday! Take care, God bless, and know we appreciate every one of you!

Melody

Tuesday, March 20, 2007 1:09 AM CDT

Poor Nicholas...He woke from a dead sleep Thurs night just screaming out MAMA MAMA and sounding panicked. I ran in there to see what was wrong and he was totally in a fog at first...had no idea what was going on and he said that he didn't know why he was awake. After we had snuggled for a bit, I asked him if he felt ok. He said no, so I asked him to point to where he felt bad. As usual he pointed to his regular reflux pain areas. I asked him to tell me how it felt and he said it felt like a lion. I asked him why it felt like that, since I had NO clue what he was talking about. He said it was because it felt like a lion scratching him and he pointed to his sternum, which is where I have heartburn pain as well. UGH. I was SO glad we were seeing GI in the morning. I made him promise to tell the GI that so he knows how to help him. My poor baby...he was up later that night again with the same and I ended up sleeping with him because at that point, he was hurting too much to easily go back to sleep and we were running out of sleep time left before we had to get up and go. The GI was very concerned with what Nicholas told him and the fact that he has been having rough nights. He rx him Axid for right before bedtime to help with the overnight pain and wants to scope him again (since it has been 3 years since his last scope), but he decided to wait until the next appt (June) since he wanted to see what the metabolic appt Mon turns up first to see if anything they find might be an explanation for his ongoing issues. Nicholas DID have a growth spurt and was almost 38 in!!! He has gained back some of what he lost during this last wean. The GI was totally ok with us putting him back up on feeds and we have decided on a slow wean since he just cannot seem to handle the big cuts. We will try to cut 1 hr/mo depending on his weight and stop Periactin 1 wk prior to give the appetite a kick start when we drop the hour and start the med back up. He hopes this will help.

Nathan is still VERY constipated. He was pretty backed up, but not impacted yet. We are to go up a a full cap daily and then 1/2 cap with each hard stool we see. His weight was fine....almost 28 lbs, and he was 33 in tall! He is already in Nicholas' clothes from last winter. Yikes!

Nicholas has a mind like a steel trap, I swear. He looked at our GI out of the side of his eyes and asked him if he was going to be giving us a shot paper...the last several visits we have needed blood draws and he remembered. The GI had decided since we were seeing metablics on Mon, we will likely be getting a boatload of bloodwork then, so he decided not to put them through anything else right now. Nicholas was thrilled with no shot and yelled our YAY and clapped his hands.

We saw a dietician for the first time ever today and she basically said it sounded like we were doing everything we could and she had no suggestions for us. That was reassuring. We left with a fistfull of rxs and another appt in 3 mos.

When we arrived back Fri evening from our GI appt in San Antonio and visiting with an old college friend and her 3.5 yr old son and 1 mo old daughter, Nathan was burning up. We took his temp and it was 103.3 . Well, as dh was about to put him to bed, he puked everywhere. That was such a deja vu from his last bout with nausea and of course, this happens AFTER we get back from seeing the GI. So the next morning we woke bright an early after a long night to get an appt with the on call ped Sat morning. Nathan is now on his 3rd ear infection in about 2 mos. This is getting ridiculous. I had asked the ENT last Wed if we should keep him on abx until the surgery since it is still 2 wks out and we already had to be sent home froma surgery once and I am so NOT going to do that ever again if I can avoid it. He said No...he should be fine and if something comes up to call and they will just treat him for it. Oh I am SO ticked...had he just LISTENED he would not have to be going through ANOTHER infection!! Thankfully the on-call ped we saw Saturday offered to extend the rx for the full 2 wks instead of ending it at 10 days if we wanted to. I was so relieved as I would have asked had he not offered. I am sure we would have been seeing another infection pop up in those 4 days...groan. DARN TUBES!!! Thankfully he is feeling better now and we did make it to some of the rodeo Saturday. He was fever free by mid morning and acting his usual self by lunchtime after a dose of abx, so we made it by 2. He took a 2 hr nap while we were there and woke up just sunny and sweet and had a good time, so I am glad we went.

I am not even sure what to post about the Metabolic Genetics appt today. To say it was the hugest disappointment would be the world's biggest understatement.

We got there and spent a LOT of time talking about the boys and their issues. We went over a lot of info and talked a lot about their med history. The problem we have is that the testing we have on Nathan is conflicting. Some points to Mito and some looks normal. He is mild...borderline. There is way too much going on to say there is nothing wrong, but not enough specific to pinpoint something. So, we are in limbo-land. We cannot rule it in and cannot rule it out...so for the time being, we just assume he has it and go with that until we hear otherwise. Well, the neuro was wishy-washy at this last appt since he wanted to defer to the metabolic geneticist. The GI is holding some testing to see what the MG says. We were hanging a LOT on this appt assuming that since all these other drs were waiting on this appt for info, that we might actually get some. I guess I put too much into this appt and have left feeling unbelieveably disappointed...I cannot even express how let down I feel right now.

The MG agreed with the neuro that a nuclear DNA testing for the known complex 1 mutations needed to be done. We talked about the mito cocktail and testing to determine what he was deficient in to see what componants would benefit him most, if any. He came back later and told us there were NO labs in the US currently doing this nuclear DNA testing for mutations, but they were developing it and we might be able to have the testing done within a year...a YEAR. He will not do testing on Nicholas now as he wants to determine everything with Nathan first to see where the testing with Nicholas needs to go. So, we will know NOTHING for sure on Nathan for at least another year. And, we will not even begin looking at Nicholas for longer than that as we will not have that one piece of testing he wants done for at least a year. He did order the bloodwork on Nathan for the cocktail, but that was it. Told us to come back in a YEAR and he will be doing nothing to help us in the mean time unless he does want to start Nathan on some part of the cocktail.

I just want to scream...beat my fists on the wall and MAKE someone listen to me. I just do not understand why we cannot seem to get help for our boys. He even went so far as to tell us he hoped that we would be "one of the lucky ones" who would have their kids just magically somehow "grow out of all this by age 3-4". Well, Nicholas is 4.5 and STILL having problems...they are NOT going away or getting any better. I know if we closed our eyes and pretended they were gone, they would still be there later when we opened them again. When I asked him to explain all their similarities and the irregularities in the labs that will not allow us to rule this out...he said he had NO explanation, but agreed that SOMETHING was going on with them. Ok then...well if you and all these other docs can agree that "something" is going on, why can't someone step up and FIGURE IT OUT instead of passing us off to yet another doctor? I just wanted to have something to tell the school when Nicholas started K to see if there was any need for an IEP once we knew more of what was going on, but this is going to take so long, it will not be done by then.

I just feel very defeated and don't know what to do now...

Thanks for all the prayers today. They were definitely appreciated. Take care and God bless you all.

Melody

Thursday, March 15, 2007 5:12 PM CDT

Well, here are the LONG awaited pics from our vacation. But first, a small update from our follow up ENT appt this past Mon...

So Nathan had an ENT appt Monday to follw up on that tube that has caused 2 ear inf in under 2 mos. Our appt was at 4 and we did not see the ENT until 5:45, just to give you a picture of the frame of mind we were all in by the time we actually saw him...2 tired, cranky, hungry kids...need I say more? He looked and Nathan's left tube was out in the ear canal. It was still in when the Vail dr looked at him a week before the appt, so it fell out sometime in the week in between her seeing him and the ENT seeing him Mon. Already there was fluid building up in there. UGH. Then, he looked at the right tube and agreed it looked blocked. This, BTW, was the SAME tube that we had to use peroxide on for weeks last summer to try and unblock, so who knows if it was ever working like it should. So, he sent us for a Tympanogram and the left was almost abnormal, but not quite, and the right was a total flatline, so we knew for sure it was blocked. DOUGLE UGH. When we got back to the room, he came back in after talking to the audiologist and said the dreaded words...another surgery for another set of tubes. This will be surgery #5 for my not even 2 yr old and general anesthesia #7. TRIPLE UGH. He did say he was going to place T-tubes this time and not the PE tubes. Not sure why, but I am not going to argue if it means less procedures for my little guy if this is going to keep happening with reg tubes. These did not make it for a year even. I am bummed and so sick of all this. The T-tubes are a more permanent tube, so hopefully this will be the end of this mess.

On a lighter note, Tuesday we went to the zoo to see friends of ours from out of town while they were downtown for an appt. We had such a GREAT time and got lots of pics. I will post those soon so as to give you time to enjoy these vacation ones...

Tomorrow we are heading out to San Antonio for both boys to see the GI and then we are getting together with our friends as they just as a new baby girl Feb 12 and we are SO excited to meet her! It should be a fun day. We will see what the GI says about Nicholas' 2 lost lbs and us stopping the tube weaning...oh well, we have to do what is best for him no matter how much someone else disagrees. One day it will be the right time...

Saturday we have tickets to the Rodeo and we are so looking forward to going. We will get to ride the train, shop, eat cool yummy food, see the animals, watch the rodeo, and listen to Brooks N Dunn perform...what more could you ask for?

Monday will be our LONG awaited appt with the metabolic geneticist. I am SO ready for this appt. We will definitely update after this appt and let you all know how it goes.

AND NOW, WITHOUT FURTHER ADO...THE VACATION PICTURES...be sure to read up on the trip summary in the journal history for more info...

When we first arrived at our "home in the snow" as Nicholas called it...the boys learned a hard lesson about NOT sticking your bare hands in the snow when it is 6 deg outside...they were both crying here...

Some pics from the Wiggles...the boys watching...

And Nathan showing off his new Dorthoy the dinosaur hat and Wags the dog toy...I LOVE this one...

All of us eating at Bubba Gumps after visiting the dr...(sorry for the no makeup...it was a long morning)

All of us standing outside in Breckenridge on the way to the car...

And the boys...

The boys just mesmerized by the views on the gondola ride we took at Breckenridge...

Nicholas all bundled up for the snow (Nathan was too busy crying to take a pic)...

Nicholas making a snow angel...the ONLY thing he REALLY wanted to do in the snow...

Nathan crying in the snow...not sure if it was the bundling or the snow he did not like...

Me on the slopes at Vail...

Doug on the slopes...

Both of us posing on a scenic overlook..we got our pic taken here a few min later by a professional photographer, but Doug needs to scan it in before I can post it...

And a couple of the MANY scenery pics we took while there...

Hope you enjoyed them! I know some of you have been looking forward to seeing those! Please continue to keep our MN friends in prayer as they adjust to their new "normal" at home with their daughter. Please also keep us in prayer as we have several important things coming up in the next couple of weeks...including Nathan's 2nd BIRTHDAY...can you believe it??? Our baby is about to turn 2!! Where does the time go? He is looking SO forward to his "Coo Coos" (Blues Clues) birthday party. We will be sure to post pics of that as well as our recent trip to the zoo soon! Take care and God bless!!

Melody

Saturday, March 10, 2007 5:44 PM CST

We got back in from Denver earlier this week. We had a good time...all except the boys BOTH getting really sick. I swear that seems to be the story of our lives these days! Fri we flew into Denver early in the day and the boys were excellent on the flight...both slept for about 1/2 the flight. When we arrived, we got our rent car and drove around Denver some. We tried to stop at the Denver Mint, but it was closed, so we stopped and ate lunch instead. Then we drove up into the mountains through some pretty bad weather to Doug's parents' place, and went out for a yummy dinner...awesome burgers and fries for $5! Sat we had to exchange the rent car since it was making weird noises. Then, we drove back into Denver and went to see the Wiggles. After that was over we met my friend (who I taught 6 yrs with before she moved) for dinner. Sun morning BOTH boys woke up with 102 fevers. UGH. Apparently there are NO urgent care facilities in the mountains anywhere around where we were, so we had to take them into the ER at Vail. Thankfully, they referred us to a family practice clinic upstairs that would take walk-ins and be open soon, so we went there. That tip saved us $75 in copays per kid! The dr was really nice and helpful. Nathan had an ear inf (same darn tube that was not working in Jan and he got an inf) and Nicholas had a bad sinus inf. After some abx they were both feeling better so we stopped for lunch at Bubba Gump's (YUM!) in Breckenridge. On the way back, we drove under a gondola so we stopped to see if they did rides for people not skiing, figuring it would be a quiet, laid-back thing for the boys to do when they were under the weather. They gave us complimentary passes when doug asked about us riding, so we stopped and rode the gondola up Breckenridge's Peak 8 for a 30 min scenic trip. Both the boys loved it and it was just their speed since they were still feeling bad. Monday, Doug's mom watched the boys while Doug and I went skiing at Vail. We had a BLAST and so did the boys. The weather was awesome and we ate at a nice restaurant on the slopes...it was the first REAL date we have had in ages! We split a $12 cheeseburger and it was the BEST burger we have ever tasted...well worth every penny!! When we got back, Doug's mom took us to a fabulous tex mex restaurant where we visited Doug's old Aggie license plate on the wall there and ate the most wonderful seasoned tortilla chips. Tues, we left to come home and the boys were wonderful again on the flight back.

Both boys were better than I could have hoped for on the plane and we could not have been prouder. Nicholas LOVED the snow and made snow angels in the yard and had a great time. Nathan however, must have thin Texas blood and did not like it near as much. He cried most of the time we were out. Not sure if it was the bundling up or the actual snow, though. Maybe next time he will enjoy it more. Doug took them both on short rides around the yard in a sled and they both enjoyed that.

Don't worry everyone...we took TONS of pics and as soon as I can download them and upload some to photobucket, I will post them for you!!! I am recovering from a sinus inf myself that has turned into laryngitis. UGH. It was an awesome trip, but we have all been glad to be back home, sleeping in our own beds and recovering.

Nathan saw the neuro yesterday. We were a work-in appt because the dr we were to be seeing through the MDA clinic suddenly (Tues afternoon) was unable to be there yesterday. Well, this was unacceptable since we have not seen neuro since Oct and we have this metabolic genetics appt coming up the 19th and I am not about to go into that appt cold. Doug made a fuss and they worked us in with the neuro we saw last time and he was supposedly already double booked for the day and we were told to expect a long wait. Well, the wait was hardly abnormal for any TCH clinic, so I am unsure why they made it seem like it was going to be ages...hmmm. We wait for our Immuno appts regularly 2x as long as that and we have an actual appt. Ah well...

The appt turned out to be just like I expected...he is being wishy-washy about everything since we see the metabolic genetics dr on the 19th, so he was basically saying to wait and see what this other doc had to say on everything we asked. He wants us to wait on the Mito cocktail since he wants to know specifically what he needs and does not want to skew the testing the MG might want to do, tho he agrees (finally) that Nathan would probably benefit from it and is surely would not hurt him to be on anything. I agree with that line of thought, but it is still hard to see your child struggling when there are options out there that may help them. Thankfully, it is only about a week now until the 19th...

He would not comment on Nicholas...wants the MG to decide what testing and such needs to take place for him.

He did say that if what Nathan has does not turn out to be Mito, there are other reasons behind his symptoms...that they could be a secondary Mito to something else. Those can be tested for by the MG, so at least we will hopefully be able to get to the bottom of this either way.

He did write us a rx for Theratogs fitting and trial to try and see if we can do something about Nathan's poor trunk tone allowing his big belly to pull his spine swaybacked. He looks pregnant and sometimes will stand with his hands on the small of his back just like a pregnant woman will do. Poor baby must have a sore back at times. The PT is going to be thrilled about the Theratogs trial. Also asked about possible Ataxia (poor walking gait) from fatigue and he gave Nathan a good once over and said that he looked ok today (it was a good day), but we could discuss a gait analysis further down the line if need be. The good news he said was that there was nothing on the MRI in the motor areas that showed cause for concern tho, so that is reassuring.

I asked him about all the high/low flags we saw on the CSF testing from the spinal tap that he said was "normal"...one being the Glycine level which was more than double what the high end of normal should be (150 is high end normal and his was well over 300). He had to go back and look and then admitted that this was indicative of Mito or possibly some other things and that they would only cause concern if he had other things going on...like elevated lactic acid levels in the blood. Umm...he DID. So then, he deferred to the MG again, saying he would do further testing.

Also, I asked about sick levels to be drawn while sick and he said to call if this happened before ther 19th and he would see what needed to be done...otherwise...guess...wait until the appt with the MG to see what he says.

UGH. I hope the MG has all the answers. Until then...we wait...AGAIN. At least the kids each got a free book from the MDA lady...she loves to see them! So that was it in a nutshell. I will let you know how the appt on the 19th goes...8 days and counting...

Guess that is about all for our little corner of the world right now. I will be posting vacation pics really soon. Hope you like the new look of the boys' website...they are into Blues Clues right now...especially Nathan, so I thought a change might be in order. I am pretty pleased with how it turned out, if I do say so myself. :o) I have a prayer request if you have any prayers to spare...our MN friends are dealing with a lot right now. Their 1 yr old daughter was airlifted out Fri afternoon and is hospitalized right now. Please pray for her to turn the corner and heal from this latest scare. Take care and God bless! We appreciate you all!!

Melody

Sunday, February 18, 2007 12:04 AM CST

Monday, February 19, 2007 10:24 pm cst

Well, he took a turn earlier and he was retracting, breathing funny, and his heart was pounding so hard you could see his belly quivering because of it. We checked and his resp were 28, so good, and his heart rate was 105...that was ok too. His capillary refill was 1 sec (2 is dehydrated) and he had had a slightly wet diaper since the appt this morning. His cheeks were briight red and he felt very feverish and hot even though his rectal temp was only 99.2 . The peds office finally called back and told me to force feed him pedialyte every 10 min with a syringe to rehydrate him and not to worry about taking him in...that he would be fine. I am looking at this limp baby laying there almost panting and knew he was not ok. After him screaming through 30 min of pedialyte force feeds and having flashbacks of having to do this to Nicholas right before he went inpatient with the virus that untimately destroyed his gut and brought on emergency surgery for him and I was just in tears along with Nathan. I finally asked him if he wanted some water and his eyes perked up and he looked at me like Itis about time! and nodded his head, yes. So I gave him some water and he was gulping it down like he was dying of thirst. Stupid me had listened to the ped when he had said only pedialyte and not offered him something else when he obviously does not like the pedialyte. He had taken almost an entire sippy of water and eaten a couple of mini nilla wafers from the box I had left out after lunch and looks 100x's better. He was cool to the touch, cheeks are back to normal color, and was laughing and playing. I just need to remember about listening to my gut and not always going by the drs rules. Obviously Nathan is not playing the same game as his ped is right now. he ate a bit of scrambled egg and plain pancake for dinner and seems to be handling it well. So far, so good... Just thought I would let you know how he was doing and to thank those of you who have been praying for our little guy...we truely appreciate it.

Melody

Monday, February 19, 2007 1:42 pm cst

Poor Nathan...he was up again this morning vomiting. Because he had nothing in his stomach, it was pure bile. Thankfully, we already had an appt for Nicholas to see the ped first thing this morning, so he gave Nathan a good once over. Nathan was feeling much perkier then than he is now unfortunately. He was given Zofran to try to settle his stomach and he had a dose already. Thus far today he has refused food and drink and had a 30 min crying fit because he wants to eat and just does not feel as though he can. He is worrying me. With Nicholas and the tube, we put him on the pump on a slow drip of pedialyte and he is fine. With Nathan, there is no way for us to make him drink and he has already had a rough weekend, so this certainly is not helping. He is super fussy and clingy and lethargic, which is SO unlike him. We are calling the neuro to see if there is anything we should be doing. If we have not heard from him later today or Nathan continues to struggle, I will be calling the peds office and seeing what more can be done. I will keep you posted...please keep him in your prayers.

Melody

Sunday, February 18, 2007 12:04 AM CST

I am SO sorry for not updating sooner. I feel as though our life has been in fast forward recently and have not been able to slow things down to have enough time for much these days. Let's see even where to begin??

Nathan gave us quite a scare last night. He vomited all over the car on the way home from Doug's birthday dinner with his parents and the smell of Outback Steakhouse coming back up is NOT a good one, let me tell ya. We got home and it continued. After 5 vomits in less than 2 hrs, we called the ped since we know Mito kids can dehydrate quickly. Well, he then puked for the nurse on the phone and then again when the dr called, so they knew how bad it was...7 vomits in 2.5 hrs and counting and refusing all fluids. So, the ped told us to give it another 1-1.5 hrs and if the vomiting kept up at that rate, we would need to take him into the local ER for fluids and labs. If he was admitted, they would keep us overnight and arrange for a hospital transfer to TCH in the morning. If it slowed, we would be ok to keep him at home and keep an eye on him. Thankfully he fell asleep and vomited once more at the hour mark and again after another hour when we moved him into the portacrib in our room for the night, so we decided to keep watching him and see how he did since it appeared to be slowing. Well, he did well overnight and only vomited once more around 5 am when he tried to roll over and the motion made him sick. Since then, he has been sipping water/pedialyte mix since 10 and has been keeping it down. It appears to be the 12 hr variety of stomach virus the dr mentioned to us last night. Thankfully, he is perky and doing better, so I think we are over the hump and going to be ok. Here are some pics of him passed out on his Dora couch in between rounds of vomiting last night...poor baby was sooo sick.

This past week has been a flurry of activity when we heard from my inlaws that they would be willing to pay our way to visit them in CO AND we found plane tickets for $150/seat and will have enough $$ left over to pay for all the rest of the expenses without spending much out of our pocket. This we can actually afford, so we have been running all over town trying to scare up winter gear for the boys in Texas in Feb when winter is basically over. That was a feat, but it seems we have managed to outfit both boys AND buy new boots for me (as my old ones are too small due to pg foot growth) for under $175...not bad at all, esp for a place where it almost never snows. We will be gone for 5 days in the beginning of March, so we will be sure to take lots of pics while we are there.

We are curently trying to figure out preschool placement for Nicholas for next year. The scholarship he has this year is only good for a M/W class next year. Well, M and W are when he has therapy and T/Th when he is currently in school is when Nathan has therapy so we can cut down the disctraction for Nathan during his therapy. We would have to reschedule WAY too much and Nicholas' OT ONLY works M/W, so we were looking at having to lose her after 6 mos, so we are looking into other programs instead to keep from having to change too much. One looks promising AND it is where both of our Wed lunch playgroup friends go, so I hope this one works out. In the mean time, we are holding our spot at his current school just in case.

We heard back FINALLY from the HR at corporate and they DID decide to change the company insurance policy regarding tube feeding and formula, so now his formula is a covered item AND the coverage is retroactive, so all the back months are covered too! Praise God!! We have not see the specifics yet, but we are so thrilled to hear we will not have to struggle with this anymore. Both the boys also somehow got their orthotics covered as well. Someone with true genious at the orthotics place figured out a code the ins would accept, so that is another load off our minds right now as well.

Nicholas had been doing well with weight gain recently, so the decision was made to try once more a trial of tube weaning. In the first 2 wks he has already lost 1.5 lbs, so not a great start. We are giving it another week and at the weigh in on Wed we will decide if we need to increase it again or not. As disappointing as it is, I guess you need to keep trying to see if it is time to try again or not. One day it will be time...until then we plod on. It still amazes me, though, how it takes forever to put that weight on him, but can take only days for it to come off. Ah well...thus is the roller coaster of tube feeding I guess.

Our team participated in the MDA Stride N Ride Walkathon and we had SUCH a good time. Out of the mile it was to walk, Nathan made it 1/4 mi around in 3 parts and Nicholas made it 1/2 mi and then dropped. Both boys took marathon naps later to recover...Nicholas for 3.5 hrs and Nathan for 4.5 hrs and Doug woke him from a dead sleep for dinner. They slept all night and laid around a lot the next day to recover. It took a lot out of them, but they had a wonderful time! Nicholas was the star of the day, dancing around to Radio Disney with all the costumed people and getting the cheers from everyone. Nathan spent time hiding from the costumed people, so he was not in the mood for pics. Doug and both boys made the local TV news in the story that night!!! Here are some pics...

Nicholas with a tiger...

Nicholas gettin' down with a frog, dog, and tomato...

Nicholas on the stage dancing and soaking up the attention...

Nathan crossing the finish line...what a victory for him!!

The team...

My sister is also doing well and recovering from her surgery. She appreciated all the thoughts and prayers coming her way from this site. Thanks to you all for your awesome dedication to our family.

So, there you have it. Our hectic last few weeks in a nutshell. I tell ya, when I have time to update, I am dead tired and when I think about it and am not beat, I can't do it then. I apologize for the delay and am trying so hard to be better about it. One last prayer request please...our MN friends are still struggling with too much on their plate right now. Too much to go into detail, but please pray for them to have peace and strength in the midst of the medical chaos they are currently dealing with. Thanks again to all of you who support us with prayers and thoughts and also to those who sign and leave us encouragement and thoughts. We appreciate it more than you know. Take care, know we think of you, and God bless you all!

Melody

Tuesday, January 23, 2007 11:41 PM CST

Update - Tues evening...

My sister is out of surgery and doing ok. She has 3 drains in...one in her hip and 2 in her neck. They expect her to stay overnight in the ICU and, barring anything unforseen, she should be moved to a reg room tomorrow morning. They expect her total stay to be around 48 hrs (??? this seems short to me after such major surgery) and the drains will need to be removed before she leaves, so I am guessing her discharge will be dependent on the drain output. She had severe problems with nausea/vomiting after this last surgery even after giving her 4 meds for it, so this time they only gave her IV meds and no gas, and several anti-nausea meds, so hopefully she will not be sick this time. Vomiting after having major jaw surgery is so not a good thing. Everything went as planned...so whew...one hurdle is done. Thanks so much for the prayers and support...I appreciate it and I know she does too.

Melody

Well, thankfully, the night went ok even tho the infection kept spreading and still no fever. He choked down 10 ml of unflavored Clindamycin last night and again this morning along with the 5 ml of Bactrim. This morning, it had not spread any further and it was hard to tell where the red areas were since Doug put him in knee socks with ribbing last night. We went to the appt and I wanted to hear from his own mouth just what he had been told by the ER doc before he looked at Nicholas' leg. Basically he had been told that, since he had not felt an abcess under the skin, he felt it was "just a superficial cellulitis infection" and he was ok to go home. So, our ID agreed. He had NOT been made aware of the size of his infection. I knew this because he did not mention it and I watched his face carefully when I showed him Nicholas' leg. His eyes got HUGE and he whispered "whoa" under his breath when he saw it. He then says to me...that is a really big infection. I said, yes...I know. This is why I did not feel it could wait. He then told me that I did exactly the right thing by taking him into the ER and not waiting. He told me had I waited even another 6 hrs we were likely to have had a severe infection on our hands by that point. He thinks that by starting him so early on the Bactrim, we saved the cellulitis from turning into an abcess. From what I understand of him explaining it is that the cellulitis and abcess are the same infection, just the cellulitis is on the skin and the abcess is what happens when the cellulitis is left untreated for too long and it gets under the skin and forms a pocket of infection. So, by starting him early on the Bactrim and getting him into the ER to get the Clindamycin rx, we "dodged a major bullet". He thinks that doing 2 doses of both the meds helped us be able to get control of it finally. By the way he was reacting and talking, it sounded to me like he would have admitted him had he been the one to see him yesterday, but I can't worry about that now. He said we are ok to be off the Bactrim now and wrote us a standing rx for the Clindamycin in case this happens again. He also wrote us a rx for some flavoring for the bottle of Clindamycin we have as he was really surprised the ER dr did not mention it could be flavored. Ugh. We got it flavored with grape and Nicholas says it tastes MUCH better now than it did before. It is well worth the $3 for flavoring it if it means he will take it without complaint or disgust. The theory on how this cropped back up after all this time is that the SMO's probably caused the socks he was wearing that first day (not the ones we use under Nathan's but had not gotten any for Nicholas since we needed to see how tall the SMO's were to know what sz to get) to rub on his skin and irritate it. That probably caused the skin to be open just enough to let in bacteria and thus the infection grew from that point. The worst part of the infection, if you look at the pics in the old update from yesterday in Journal History, is just under where the SMO's end on his Achilles tendon, so it seems to make sense.

As for filing the complaint, we are calling that nurse that we posted the pic of her and the boys the other day. She used to work in the RTA where we were yesterday and she also worked in the ER before they had the RTA and we are going to ask her exactly what is the hospital policy for lancing abcesses and such so we can see what should have been done and what was not done. Then we can decide how to move forward with the complaint. That plus as soon as she hears what happened she is going to hit the roof and I am sure we will be hearing what we need to do from her. I am still so disgusted by it all yesterday, but just glad he is going to be ok and we avoided the hospital. This is why we usually both go with the boys if we can. One to be the comforter and focus on Nicholas or Nathan and one to watch the dr. I just could not do both yesterday with Nicholas being so hysterical. And Doug felt terrible since he had one day off left and wanted to save it in case Nicholas did end up inpatient since there would be no one to watch Nathan today. Ah, well...thankfully all is on the right track now and he is getting better. Thanks for all the prayers...we really appreciated each and every one!

Now...I have 2 more prayer requests, if that is ok...

First, my sister is having some major surgery tomorrow. She has been dealing with a reoccuring jawbone cyst for almost 2 yrs now as a result of a rare complication of a root canal. She has already had 3 surgeries to remove the cyst...the first 2 to just remove it and each time it came back so the last surgery they removed the entire section of jawbone and replaced it with a titanium plate. The plate broke a couple of weeks ago, so now they are going to have to repair it with a bone graft from her hip. She will be in surgery 6-8 hrs and they will put her in the ICU the first night. She is scared, but wants it over with. If you have any prayers to spare, I know she would appreciate it greatly.

Secondly, our MN friends have their baby girl in for more testing and med adjustments this week. she is not doing well again and the drs are not sure what more can be done. her family only wishes for her to be comfortable for as long as God chooses to let her be with them. They ar ein need of prayer right now to decide what to do for her.

Thanks...I know how much this means to them both and I am grateful for any prayers that can be said for each of them. Take care and God bless!

Melody

Sunday, January 21, 2007 0:04 AM CST

Update - Monday Jann 22, 9:30 CST

I am so upset I can hardly see straight. We were sent to the ER because the ID could not fit us in and knew he needed treatment. So, I pack a bag, make arrangements for Nathan and drop him off, and Nicholas and I go downtown. When we get there, we wait for 2 hrs to be seen. When we finally get taken back, we are not taken to the ER but the RTA, which is the 23 hr observation unit and also where we go to wait to be admitted. The dr comes right in and sees us within 5 min of us arriving in the room. So far so good...but that is where the good ended.

He spoke to me about Nicholas and asked questions about his med history, but every time I tried to reply, he cut me off. He was a very full of himself cocky dr. I explained about his immune deficiency and that the last time he had one this big, we were admitted for 5 days and on IV vanco, genta, and clinda for 4 days before he was well enough to go home. He said with his history, he thought we would be admitted to the 23 hr unit for a 24 hr round of abx and we would see how he was doing tomorrow and see if he needed an admit or could go home. He leaves and then comes back a couple of min later and announces that he is just going to lance it and send us home on orals. Not sure what caused the 180, but ok. By this time the infection had spread more than what it was when we left at lunchtime. He leaves and comes back with a bunch of supplies, wraps Nicholas in a sheet, and hands him to me. Meanwhile I was sure Nicholas was having flashbacks of the WWF nurse who held him down like that for his head stitches and was screaming bloody murder. The dr starts readying his supplies and by the time I look up from my freaking out son to see what he is doing, he uncaps the hugest needle I have ever seen and jams it into Nicholas' abcess...NO NUMBING AGENT!!! I was horrified and spechless! Even as an adult, I was given a numbing agent each time I had an abcess lanced and the numbing agent injection itself was excruciating! And this is a CHILDREN'S HOSPITAL!! I try to calm Nicholas down and see him jab again from the corner of my eye. He is squeezing and nothing is coming out. I knew it wouldn't because the surgeon's told us last time when they admitted Nicholas, that it was way to deep to lance and we needed to wait. By this time Nicholas is hysterical and begging the dr to stop, yelling NOOOO and I am nauseous and then the dr jabs him one last time and digs the needle in the abcess until it starts bleeding and then removes it. all this in the span of just a couple of min, but it seemed like an eternity. All he got was blood...nothing more and especially no drainage. When he left, I wanted to yell and scream at him, but I was so nauseated, I was afraid to open my mouth for fear of getting sick and I had to settle Nicholas before anything else.

When I got him settled, and wanted to speak to the dr, he was not available. We had to wait for a blood test to be done and we later found out (once we had left...he never told us we were getting one..the nurse did and she had no idea what it was for) that this was to check his Ig levels. The nurse came in to discharge us and handed us a script and told us "Here is your script and you need to follow up with the dr tomorrow. Um....what is that script for? Who do I follow up with...he talked to both the ped and the ID? She asked me if the dr had talked to me about all this and I told her no and she said in a frustrated voice...Well, he NEEDS to! and then she leaves. She comes back all flustered and says, well...it seems he has left for the day and signed your care off to another dr....UMM...WHAT????? So she says let's just go by what he has written here. I ask her so does he take the clinda with the bactrim he is on or just take it alone? She has no idea so she goes to ask the new dr. When she comes back she says we can do either and it is up to us...ummm...WHAT??? I am NOT the dr...that is why I came here!!! She says I can follow up with whichever dr I want. I look at his leg one last time before she leaves and notice that the black line the dr drew was now inside the red area and it had grown since he had seen us and asked the nurse of she was SURE that Nicholas was ok to go home with is still spreading and she told me that is normal (????) and he will be fine. By now the cellulitis is softball sz and the abcess is close to golfball sz. Nicholas is crying to leave, and I just want to get us out of there because I could feel the tears rising up in my eyes by this point. I leave and take Nicholas to the bathroom and bawl my eyes out to Doug on the phone. We had not eaten in 11 hrs and were just treated like they wanted to only do the bare min and get us the heck out.

Doug called the ID while I was on my way home and stuck in traffic and could barely see for the tears running down my face and he was shocked at how we were treated and told us to come in tomorrow at 11:45 to be seen. He is giving up his lunch hour to fit us in since they cancelled the appt they had us down for when we took him in., He kept apologizing to dh and saying how sorry he was for all Nicholas and I went through today and he was specially stunned that no numbing agent was used on Nicholas. All I know is that I plan to file a complaint about that dr and if we end up admitted tomorrow, I am so NOT paying for this ER visit today. If he needs to be admitted it should have come today and not put us through all this to send us home. Heck, I could have done everything that dr did but write the script and the ID could have and probably would have called that in for us. OMG I just want to SCREAM and then my poor baby tells me "It does not hurt that bad...I am sorry we had to take me to the ER today, mama". Talk about a broken heart to hear HIM apologize for this awful day. So, here we sit, waiting for our appt tomorrow and watching the cellulitis and abcess grow ever bigger. It is days like today that make me feel like the world's worst mother for putting him through that and doing what we are told, only to have it end up being a totture session and nightmare..and I feel so responsible for being the one to bring him there for that. I hope he will avoid an admit, but the cellulitis has spread since the dr marked it earlier. I guess we will see what happens tomorrow. Prayers would be gratefully appreciated.

Here are some pics in case you are interested...

Here is the line the dr drew for the cellulitis. The pen mark is the outline of the abcess, and the tiny red dot is the poke hole the dr made.

Here is the new line we drew tonight showing how far it has spread since we left the ER...

Thanks for all the thoughts and prayers alrady given. You all are such a great bunch.

Melody

Update - Monday Jan 22, 11:20 CST

We are not going to be able to be seen by ID today. He cannot wait to be seen until tomorrow when they can fit him in so he is sending us to the ER at TCH. We are hoping to avoid an admit, but it is still spreading. Any prayers would be appreciated. We will update later...

Update - Monday Jan 22, 7:00 CST

Nicholas has another abcess that has been spreading at a quick rate. It is spreading about as fast as the one he had last April when he was admitted even though he has been taking abx for it. The cellulitis area is tennis ball sized and the abcess is almost quarter sized as of about 6:30 this morning and takes up about half of the back of his calf starting at the top of the heel. We will be calling the ID's office as soon as it opens and seeing what to do from here. It is causing him a lot of pain when touched or moved. Please pray that we can get this under control before it spreads much more. It is scary that it is spreading despite being on the topical and oral abx as soon as it was noticed yesterday. We will update when we know more.

Ok...lots to tell! We have had a bunch of appts and I kept thinking I will just wait until this appt to post and then there is one the next day and I put it off again. Here is the lowdown...

Tues, Doug got a call in the afternoon about Nathan's MRI and Spinal Tap scheduled for Thurs. Turns out they had scheduled him for an UNSEDATED MRI and needed to reschedule him so he could receive the anesthesia. The only problem was they wanted to reschedule for Feb 28!! So not acceptable!! Nathan had had a horrible week prior to this and kept falling every few steps. It got bad enough we had to call the neuro and he was relieved to hear we had the MRI coming up so soon. It was not an option to wait that long. Doug got nowhere with the woman he was talking to and got so frustrated with the situation and lack of help that he called the Chief of staff of the entire hospital's office! That sure got some attention! We were rescheduled for Thurs at 2:30 instead of 7, with sedation. I was proud of him for taking on that task and doing so well!

We actually made it downtown on Wed, ice and all, to Nathan's appt with the wheelchair clinic for his seating eval. We met with an OT and a rep from a wheelchair co. At first they were all for a KidKart and I was unsure about that one since I thought Nathan was too mobile for something like that and did not really need all that support and the tilt and everything. Then they asked where we were from and I told them and the wheelchair rep asked us who our PT was. I told him and he was so excited since he has worked with her a LOT for years. I think he is her "equipment guy" she keeps referring to, but he said he did not think she knew he worked downtown at the clinic, too. He said "Well, if she is your PT, I know what she wants for you...she wants a stroller type with tie downs". He was right, so that was the last of the KidKart discussion. We looked at the Convaid Cruiser, Stealth Lightning, and the Quickie Mighty Lite. We ended up going with the Stealth Lightning for several reasons. First, we LOVED the stroller type basket under it. The other ones did not have one and that is something we use all the time. The other is that the Stealth has adjustable angle footplates. The others have fixed angle footplates and his legs are so short that his feet were not sitting comfortably on the fixed angle ones. They were so excited to be fitting someone for this one as they just got the demo in from the rep and have only been able to sell it to people who have specifically asked for it. We liked the design and adjustibility on it. The demo they had was an 11in seat and Nathan pretty much filled it already, so we opted to go with the 14 in (next sz up) to allow for growth room. He added lateral pads since Nathan slouches to one side in the stroller and got the double sz ones since he is going to be small in the 14 in and wanted something to stabilize his hips too. We also got the smallest sz headrest in case he falls asleep on the bus and we can take it off the rest of the time since he won't be needing it much, and also the transport option. We also got a canopy since he has trouble with the heat in the summer and this will help keep the sun off him and the strap pads. And, we got heel cuffs and ankle straps since he still has the froggy hips and this will help his feet sit ptoperly to avoid that. It also has a footbrake that is easier to use than our current baby stroller and it weighs about 22 lbs before the adders. Here is a link to it, but his will be in blue...

http://www.southwestmedical.com/Pedi...roller/14571p0

They will need to shorten the seat depth when it arrives, but we will be able to add depth to it later when he needs it back. It also has a headrest extension you can get and the footplates have a TON of settings, and the weight limit is up to 100 lbs, so he should be able to use this for a long time. Now, if we can just avoid the hassle I am SURE the ins will give us...groan...

Then, Thurs Nathan had his MRI/Spinal Tap. To say it was a long day would be an understatement. It was a very long day. We had pre-op at 1:45ish and they prepped him for the anesthesia. This is where we learned that he was NOT scheduled for the spinal tap as we had originally thought. Turns out when they cancelled us on Tues and rescheduled us, they cancelled the spinal tap as well. When it was rescheduled we were told the spinal tap had been rescheduled, but it actually hadn't. And, even worse it needed to be done via video flouroscope in Interventional Radiology because when they had tried to give him a caudal block back when he had his circumcision revision, the spaces between his vertebrae were so small that they had no room for access. This had been noted in his chart and apparently the neuro did not want to take chances with them going in blind. Because of this, we ran into yet another problem...who was to go to IR with Nathan while he was under general? Radiology has one anesthesiologist for the whole dept on at one time and he was going to be with Nathan while he had his MRI, but then had more patients after him and could not go with him to IR to monitor him for the general while he was in there. We were flipping out thinking what a huge mess this all had become. We met with the anesthesiologist and he was adament that he would find a way to stay with Nathan, no matter what so we could avoid having to subject him to general again just for that. He was SO nice and we were SO relieved! They had to stick him like a pincushion before they got IV access and he had been screaming for so long by that point that he was refluxing like crazy and he was getting dehydrated so they had to give him lactated ringers to perk him back up. He finally settled right before they put him to sleep and then we had to leave him (since he was under general). This MRI was to take about an hour, but ended up taking about 20 min longer. Then he was transferred to IR and had the spinal tap, which took longer than expected since he nearly aspirated! When they turned him on his side to look for access to place the needle, the anesthesiologist suddenly noticed red fluid dripping from the side of Nathan's mouth. He immediately stopped the procedure and grabbed the suction and suctioned out his throat and when stuff just kept coming out and coming out, he then went to his stomach. Turns out that at around 4:30 pm, he still had almost 200!! cc's of fluid in his belly, throat, and mouth from early this morning!! He was to stop clear liquids at 11 and I fed him a strawberry jello cup right before 11. This was STILL in his stomach plus a LOT more almost 6 hrs later! We did not get into to see him until after 5 and got to talk to the anesthesiologist at that time. I will never forget the look on his face...it was one of extreme concern over what had happened and how close he came to having some serious problems. I just thank God that this dr chose to make sure he went with him. Had he not been the one there or had he not noticed what he did when he did, this could have turned out very differently! Poor kid also had to have a blood draw for some other tests run and they had to stick him 3 more times to get access good enough for 10 cc's of blood. My baby looked like he had been poked to death. He woke up groggy and cranky, but ready to get out of there. He was trying to pull all his lines and get off my lap top get the heck out. The anesthesiologist stayed a bit past the time he could have left to go home just to be sure he was ok. You could tell that whole situation had really shaken him. He did just fine the rest of the evening and we hopefully will be hearing initial results sometime Mon. Thanks so much for all the good thoughts. I know those prayers helped make that day have the happy ending it almost didn't.

We called the GI Fri, but he was not able to get back with us yet about what happened during the procedures. They keep all the patient files in the basement of the hospital next door to his office and he had to request Nathan's file and wait for it so he could review Nathan's emptying scan again and see what he thinks needs to be done. It will likely be Mon before he has a chance to see it, so we will let you know what we hear then. It is pretty obvious that some type of medical intervention needs to be done after what happened Thurs since it could happen again while he is sleping.

Nicholas got his SMO's yesterday. We already went back once for adjustments and it looks as if we will be needing to go back again on Mon for more. Since getting them, he has started intoeing when he walks and, after talking with both the orthotist and PT, it seems like this is normal and happens because his legs have no idea how to walk now that he has to do it the right way. The PT said if he is still intoeing by Mon, we need to call her back and she will let us know what we can do about it. After getting them, we went to the Stride Rite outlet and bought some new shoes. Here are some pics...

Nicholas' new shoes and the SMO's...

Nicholas' SMO's...

Then last night, something else came up. Nathan almost aspirated (or so the anesthesiologist thought) Thurs during his MRI and Spinal Tap. He told us to be very vigilent and to watch for fevers. Thurs night Nathan was coughing overnight and woke yesterday morning with a very yucky stuffy runny nose...kind of yellowish drainage just running out all day. Around dinnertime, he started feeling warm, so we unbundled him and he seemed to get cooler, Then a bit later, he felt warm again and still had the same amt of clothing on so I knew he was running fever and I took it rectally and it was 102. I immediately worried about aspiration pneumonia because of what happened yesterday. I called the peds office and got the nurse line and according to her triage chart, he needed to be seen in the ER. When I looked at him tho, he was running and playing and he was NOT retracting or having his nostrils flaring or any pulling at his neck or anything. I hated to take him downtown to the ER at this time of year (esp on a Fri night) if I don't have to so he won't have to be exposed to all those germs there. I asked the nurse to please have the on call dr call us if at all possible. The dr did call me and after talking with her, she was ok with us keeping him at home for the time being, but watching him closely for any wheezing, retracting, breathing changes, or any increase in fever. She seemed pretty certain that he had an aspiration pneumonia and wanted him seen first thing this morning at the drs office...where our ped is the one seing patients, thank goodness. Well, we went in this morning and I sure was not expecting him to have an EAR infection! Thankfully, it was that and not pneumonia. And strangely, once I knew that was what it was, I should have known. He had the same symptoms yesterday as with every other ear inf he has ever had. UGH. The ped said it did not look like the tube was functioning properly, whatever that means. He only has it in the right ear, so we are guessing he must have been lying on his right side when they were doing the Spinal Tap and when the fluid came up, some of it drained into his right ear. It is very unusual for Nathan to have an inf in one ear and not both. I am glad we ended up keeping him home. We got up and checked on him all night long and he did fine, but I had such guilt for feeling like I was going against what the dr had told me on the phone. Our ped told me this morning that we know him better than a dr over the phone and we need to go with our gut since we will know when he needs to go or not and that he knows we will do the right thing for him. That made me feel better to know he trusts us about that kind of stuff. He is on Augmentin and boy does he HATE it. He has gone dusky on us twice now crying so hard from having to take it. Ihope he is ok with it soon seeing as how he needs to be on it for the next 9 days...UGH.

That was our last week in a nutshell. Lots going on and lots of running around. Nicholas is still loving school and doing so well with his therapies. He is drawing pics of people now, complete with 95f body parts and starting to identify letters wherever we go and trying to write them. He can almost write his name! I am going to leave you with some pics of my sweeties...

Nicholas...

and Nathan...

We will update when we start getting in results and calls back. Hope all are well and enjoying 2007 this far. Take care and God bless!

Melody

Saturday, January 6, 2007 8:54 PM CST

Well, the new year has certainly started off with a bang!

We went to dinner with friends on New Year's Eve and went back to their house for some pre-midnight fireworks. The kids had a great time and the adults did too. Doug and I rang in the new year with a toast to a happy and healthy 2007 for our family. I sure hope this proves to be true.

We did some deep cleaning to get the house ready to start the year off right. This was also to get ready for the inspector to come and take some pics for the home equity loan we are applying for. Finances have been very tight around here with all the medical and bills and the savings is gone, so we are hoping by consolidating with this loan, things will be better soon.

Some big news...Nicholas got casted for his SMO's yesterday. He picked a really cool pattern called Ice Age and it is this cool looking light blue, white, medium blue, this lavendery color, and slight grayish color and looks kind of streaky vertically and it really looks like ice. He picked light blue straps to go with it and I think they are going to look awesome! They had a sample SMO there to show him and it just so happened that it was just his size and almost a perfect fit (minus the girly pattern!) and he was able to try it on and see what the SMO's were all about. He thought they were not too bad and thinks he is going to be ok to wear them. I am glad as I was worried. The guy who casted him commented on his "significant ankle pronation" and it appears it is getting so bad that the alignment the ankle is taking on is forcing his heel to go outward creating a diagonal from the ankle to the heel. It seems like we are getting this treated just in time. We are hoping the ins will pick these up, but I am not counting on it and so we will most likely be paying out of pocket for these since he has not been dx with anything yet that MDA will cover. I am hoping they will not be too pricey...Nathan's are $2,104 and change, so I am hoping these are at least less than that. I will make sure to post some pics of him with them when we pick them up in 2 wks.

Some even bigger news is that NICHOLAS IS POTTY TRAINED!! I think that waiting ended up being the best thing we did for him, even with it being as frustrating as it was. After that awful VCUG, we did learn that he could hold the urine for an 8 hr stretch, so we knew he had the ability to do it. After visiting preschool before they closed for the holiday break, he knew he needed to use the potty and then had the motivation to do it. The combo of the 2 was just what he needed and best of all...it was HIS choice and not ours. I think for him, with as many things that happen to him that are out of his control, he tries to control as many things as he can and this used to be one of those instances. He went to Pull Ups, which gave him a terrible rash. They obviously changed the diaper material when they added in the velcro sides, so those were out. We then went to training undies (with the cotton padding in the middle) and plastic pants. Those worked well and he only had one acident he could avoid...learning to stop doing something you enjoy when you need to go got the better of him that time. He learned his lesson tho! Then, he was ready for regular undies with the plastic pants and did even better with those...especially with the addition of some new Cars and Diego ones! One day, he was sweaty and we took off the plastic pants and he did great, so those are gone now, too. He has not had an accident since! He tells us when he needs to go and is even learning how to hold it when a bathroom is not immediately available. He is doing AWESOME!!! We couldn't be prouder of him!!!!! :)

Probably the biggest news of all HAS to be that Nicholas started preschool last Thursday! Our baby is growing up so fast! He LOVED it and had such a great day. He did not cry at all. He did wonderfully, according to his teachers. They said he was a joy to have and they really enjoyed having him there that day. :) They had motor lab, no recess due to the rain, and goldfish crackers for a snack. They read the gingerbread man story and baked one, but he ran away before they could eat him. So, they had to look for notes he left so they could find him. He was hiding in a box! Everyone got to have a taste of him when they found him. He colored with markers and crayons and played puzzles and playdoh. I think that about does it. Oh...he used the potty the whole time and had NO accidents! He said he LOVED it and it was the best thing ever. Here are some pics of his very first day...

Nicholas shouting "Hooray...school!!!"

Nicholas so busy with Playdoh that he could not even be bothered to look up and tell his mama a proper goodbye!

And, not to leave sweet Nathan out, here is a cute Nathan story for you... Sometimes Nicholas will come up to me and tell me something he is excited about and I will mess with him a little and say NO WAY! and then he will laugh and say YES WAY! back to me. Well, Nathan wants to be just like his big brother and he will come up and babble something in that same excited tone of voice to me and so I said to him NO WAY! the other day just like I do to Nicholas and he smiled this HUGE smile and he said back to me Weh Weh!! I think he was trying to say YES WAY! like Nicholas always does. So, I have been saying it more and more to him and every time he grins this hugs grin, giggles, and says Weh Weh! to me back! It makes me smile every single time!

He is also trying SO hard to talk and is getting better at making the babbling sound like words. His cutest ones right now are Antaaaaaaaa (Santa), Seeeeeeee (see), Teeeeee (tree), and Baaaaaaa (bath). He LOVES to draw out the ends of words and his voice will get louder and higher the closer he gets to the end of the word. It is just too sweet!!

Well, I am enjoying this happy, good news entry so much, I am going to leave you with some pics from our Christmas. Hope you enjoy!

Doug and me at his work Christmas party...

Christmas Eve...

Nicholas enjoying his presents at Nana and Grampy's house...

Nathan snuggling his new Diego at Nana and Grampy's house...

Christmas morning...

Nathan flashing a smile while opening his stocking...

Nicholas digging into his stocking...

The boys enjoying their new therapy trampoline from mama and daddy...

Doug showing off a present...

Christmas Afternoon...

The boys posing for a pic in front of Uma and Papa's tree...

Me opening a gift at my inlaws' house...

And our family pic taken at Uma and Papa's house...

Hope you all have had a wonderful start to 2007! Nathan has an upcoming MRI and Spinal Tap and we would appreciate any prayers lifted up for safe procedures for him. These tests have us very nervous and it is hard for us to think about what is going to be done to him. We are also still at odds with the insurance over formula, therapy visits, and orthotics, so any prayers there would be gratefully appreciated as well. Please also continue to remember our MN friends as they are still struggling with their daughter and fighting for adequate care for her. Take care, know we think of you all, and God bless each one of you.

Melody

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