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Sunday, May 20, 2007 8:37 PM CDT

Hello to all,
I just wanted to let you know that we cancelled all the Evan's appts. in MN and our attendance at the MN regional meeting for this past weekend. Leah had two seizures Tuesday morning. I brought her into the ER after the first one and they ran lots of labs and did a head CT. She then had another siezure while we were there. They then admitted us and ran an EEG as well. All the tests came back fine. The peds. neurologist is hoping it was an isolated incident and called it a possible neurotropic viral infection. So, we thought it might be a good idea to stick around home for the next couple of days. We will reschedule Evan's appt. soon enough. Kim

Monday, May 7, 2007 2:48 PM CDT

I know I just added an entry 5 min. ago but I forgot to mention that we went to a huge water park this past weekend. Evan loved it all and ran from the time his head lifted off his pillow to the time it laid back down. He loved the big tube rides, the wave pool and being with all his friends. This is an event we have done for the past 6 years with a bunch of families from the lamaze class we had when expecting Claire. 8 adults and 11 kids (oldest being 7 y/o). I guess our heads hit the pillow as hard as Evan's did at night!

Feel free to see our new pics! Kim

Monday, May 7, 2007 2:28 PM CDT

Hello to all,

Just wanted to drop everyone a line to let you know how Evan is doing. He had his follow up with the GI doc he saw two months ago at Milw. Childrens. He thought Evan may have grown and gained some but not to much. He thought it would be a good idea to see what Dr. Wagner (BMT doc we are seeing in two weeks in MN) and Dr. Petryk (MN's peds. endocrinologist) thought about his weight. He is not opposed to putting Evan on an appetite enhancer but didn't want to jump into it either. I think Evan's weight is about 31 lbs and is 32 in. roughly.

Evan's growth study through Cinn. Children's is going smoothly. They are upping either his thyroid medicine or his placebo after getting labs drawn two weeks ago to check his thyroid levels. He takes his medicine in the AM so good. We break the capsule open over a big spoonful of his Auntie Shana's strawberry jam... he loves it. We will recheck these levels mid - July. He will be on this medicine for 5 more months and then switch to the medicine he has not taken yet - either the placebo or the tryroid med.

Otherwise, Evan had his karate competition tree weeks ago. He took home two 2nds and one 3rd. He was so proud and his Dad was a little proud too! We have been spending alot of time outside since the weather has been getting warmer. His T-ball started and he has hit the ball several times without the tee. Now I just need to get him to stop wearing his baseball hat backwards. This summer, he will be in swim lessons at the local pool, bible camp and hopefully a karate camp.

We will be leaving on the 18 of May for MN. Evan is seeing the ped. endocrinologist for his annual and for a bone age test. He will also see Dr. Wagner and have his annual bone marrow biopsy done. After giving Claire the choice, she wants to come with. We will be seeing the docs Friday and Monday and then at MN's FA regional meeting on Sat. Claire and Evan will be with their Uncle Pauly so we can go to the meeting. Rae and Leah will be home with our right hand, Miss Aryany. Sunday we are hoping to catch up with a good friend of ours and take in MN.

One last thing to mention... We held our second annual Croppin For A Cure three weekends ago. Thank you to our wonderful friends who help this event be such a big hit for all who came to scrapbook and for all the money that it raised for FARF. It is a wonderful example of how you can take something that you are passionate about and make it a FUN fundraising event. John and I are very grateful to all those who attended and those who helped make CFAC possible. We have so many great friends and we love you all. Please know that all of your efforts have already bought Evan more time to laugh, live and love! Kim

Thursday, April 19, 2007 9:01 PM CDT

Hello to all,
Just wanted to update you on Evan's labs today... and to tell you we had a wonderful Easter and hope you did as well.

ANC - 2530 **was 1550 (consistanly below 1000 indicative of transplant) , platelets - 168 ** was 162 (normal 138-400), Whites - 5.5 ** was 5.0 (6.0-18), Reds - 3.85 **was 4.32 (3.9-5.3). All in all, still do well... We also had a Free T4 and a TSH drawn for the study he is involved with. We also weighed him and got his height with no changes in the last month in either :0( After this appt., he had a dentist appt. for a cavity that needed to get filled. What a day. I gave him a frog Webkinz I have been saving for the right moment when we got home today. He was a champ through it all.

Other than that, not much going on. We are set for a bone marrow biopsy and appts. with his endocrinologist and BMT doc the third week in May. There is an FA meeting that weekend at the hospital so we just planned all his appts. around that. We are also working on scheduling an appt. with the public school system here in town now that Evan will be entering kindergarten next year... we will get him on their radar. Also, he has an appt. with Dr. Noel, the GI doc he saw at Children's Milw. throught the Nutrition Center, to reveiw the last couple of weeks since we saw him. That is the first week in May. Always busy!

Easter was great. Lots of Easter egg coloring, finding and eating! We traveled in IN to see John's family and then made it back here for Easter supper with my family. The kids loved it. Then we had a week of Spring Break were we hit appts., the Children's Museum and spent two complete days in our PJs... in a row! Now, we are back to reality which is a good place to be too.

Take care to all, Love, Kim

Monday, March 26, 2007 2:18 PM CDT

Hello to all,
I just wanted to update you on our trip to Milwaukee to the Children's Hospital Nurtition Center this past Friday. We were so impressed. Now we know why it took so long to get the appt. scheduled and to get all their requested paperwork to them... these people knew their stuff! We met with a GI doc, a nutrionist, a child's psychiatrist and several nurses all at once in one room. They knew all about Evan's diagnosis and history. They asked us more questions to get them current to Evan's nutritional situation. They also watched him eat and watched us interact with him while he was eating through one of those windows that looked to us like a mirrow... I felt like I was on CSI!

When the all came together again, here were their recommendations:
1. We are not look at Evan's weight for age but his weight for height. He is right on the boderline of being average to below average in his weight for his height. So they agreed that it would be nice to get him to gain some weight but it wasn't life threatening by any means. Also, their hopes were that if Evan gained weight, maybe he would grow some as well...
2. He should go to the bathroom prior to sitting down at a meal then not be allowed to go until he has finished his meal.
3. Meals should only last 20 to 30 minutes. After that time is up, what he has not eaten should be taken away and he is not to be allowed to eat until the next scheduled time.
4. One of us should sit with him, just until he gets used to this idea, and coach him to eat faster. One bite after another...
5. No snacking or grazing between meals. Breakfast, a schduled AM snack, lunch, scheduled afternoon snack and dinner... and to conintue his 1/2 c. Ensure Plus before bed. It is Evan's nature to graze but he usually does not to this alot to begin with.
6. To keep meals to 2-3 food only. The more foods he sees on a plate will only look more overwhelming to him.
7. We got some great high caloric recipes to try for desserts as well.
8. We will revisit Evan's weight with a recheck to the GI doc. the first week of May. If there is no improvement, Dr. Noel thought we might want to try a medication to boost his appetite. Also, by then, we might want to do an endoscopy to look at Evan's stomach. He has been getting canker sores lately and with his predisposition to frequent stooling and complaints of stomach pain, it might explain why we aren't seeing any improvement in weight by then.

It was a great appt. and we will keep you updated on how things are going. So far, so good. He has finished every meal since then in under 30 minutes eating the same amount of food as usual. AND... without leaving the table to go to the restroom. After all the specialist left us alone to come up with a care plan for Evan, I asked him how he thought it was going and if we left anything out. Evan first corrected me saying I told them that he ate most of his peas at supper the night before when in actuality he at none. Secondly, he says "Mom, I just wanted to tell them that I want to be done eating when everyone else is." Sweet boy...

On a different note, Evan also started the growth study at Cinn. last week. He is now taking a small dose of thyroid medication or a placebo for the next 7 months and then switching to the other. The study is questioning that in FA kids who have poor growth and a normal to low thyroid level... if giving them additional thyroid medicine will help their growth. We will keep you posted on this as well.

I just wanted to say thank you to Amy, a very good friend of mine, who watched Claire and Rae while we took Evan (and Leah) to his appts. on Friday. They had a blast with her and hopefully didn't wear her out to much!

After the appts. at Children's were done, we picked up Cliare and Rae and headed to a water park in IL for the night. Everyone had a blast until Claire and Evan had to share a bed at night. "Mom, Evan is touching me." and "Mom, Claire is taking all my blankets!" Uggghhh! And then on Sat., we were right where we needed to be to attend our youngest nephew's first birthday party. What a great time we had. After the party, we drove home without any issues. What a great weekend.

As always, thanks for checking on our Evan. We hope you are enjoying the nice weather we have been getting up here - LOVE IT! Kim

Sunday, March 11, 2007 10:22 AM CDT

Hello to everyone!

Yesterday was Evan's Enchanted Evening...

What an amazing night. We had a hall filled with 180 people who were just their to show their support for what John and I, and so many family and friends, are trying to accomplish... finding a cure for FA. The night was very successful. Larry, our chairperson, did an amazing job. We had plenty of people who helped set up, work the evening and take down. The evening had great food, drinks, dancing and shopping. Our guest speaker, Rachel Grossman, did an incredible job telling all of us about their son Jacob who also has FA. Everything was just perfect! And... we raised some serious dollars for FARF. YEH! I attatched some pics of the evening. Again, thank you to everyone who donated, attended, helped in anyway - we will be forever grateful! Kim

Friday, February 23, 2007 1:29 PM CST

I know I just added a journal entry yesterday but I am also trying to get some birthday pics on the site. Hopefully, they will be there now. Thanks, Kim

Thursday, February 22, 2007 3:12 PM CST

Evan is five years old and he is telling everyone who will listen!

The marathon birthday started this past Sat. when Evan had 10 of his best friends to The Little Gym for fun and cake. Sunday we celebrated with our in-town family at Grammy and Grampy's house. Tuesday we had Evan's school birthday celebration (frosted, bone, 'Scooby snack,' chocolate chip cookies included!). Wednesday was his actual birthday with phone calls from a ton of people wishing him a great day (and a chance for him to tell everyone that he is five now!), Today, Evan had his five year doc. visit (we brought treats!)and he got a gift in the mail from his cousins from IN. Tomorrow, he is expecting one from his Uncle Pauly! We might actually be celebrating Evan's fifth birthday when he is six!

His five year check today was uneventful. I think we talked more about Rae and Leah! He got his kindergarten shots which he was not at all excited about. He is pretty sore this afternoon at one of the sites and I actually gave him some Motrin for it. He is 39 3/4 in. (up to 5%!!!!! - 2 in. since Oct.) and 30.4 lbs (less than 5%). All was status quo.

It looks like Evan is going to qualify for the Cinn. Childrens' growth study through Dr. Rose. We are just waiting on a start date...

Evan has an eval. with the Nurtition Center at Milw. Chilrens' on March 23...

We scheduled Evan's next bone marrow biopsy in MN for the end of May and he will also see the peds. endocrinologist at that time. I think she is planning on doing a bone age test as well.

Until then, things are great. We are in full swing for EEE '07! If you are able, come and join us! It is always an amazing night. This year, we have another wonderful FA mom speaking (Rachel Grossman). We hope to raise lots of money for FA research!

With all of our love, Kim

Thursday, February 15, 2007 9:12 PM CST

Hello to all,
I hope you are doing well. We have good news! Leah's DEB breakage test came back normal... she does not have FA!!!!!! Getting that call is the worst as you wait those few awkward moments before the person on the other end of the phone gives you the results. I am so glad that is over.

Evan had an appt. with the peds. cardio. today. Everything looks great. His PDA is still clipped and there is no signs of leakage. There is no cardiomyapothy. His aortic valve is still a little goofy with one of the valves slightly adhered to the wall of the aorta but it is working just fine. As the doc said today "There is more than one way to get the job done!"

Evan is getting labs tomorrow which should finalize him being in a growth study through Cinn. Children's. They are trying to determine that in patients who have FA and low normal thyroid counts, if they still gave them thyroid supplements... will it increase their growth? He should be starting the study soon. I will keep you posted.

Therapy has been slow moving lately as we switched form one therapy facility to another because of insurance. We are just getting to know the new therapist and they are getting to know Evan.

Everyone has been sick in our neck of the woods. We have all had colds but other than that, not to bad. Evan has remained fairly healthy through it all! YEH and then knock on wood!

It is Evan's birthday on the 21st. My handsome little man will be 5 years old. What a miracle. He is having a birthday party with a few of his good friends this Sat. and has been planning on who to invite and un-invite for months (depending on how they have treated him that day!). Needless to say, he is very excited for it all.

Lastly, Evan's Enchanted Evening is March 10th. It is going to be a great night of dinner, dancing and an auction. Please contact us for tickets if you are interested or if you can help in anyway. We have always contemplated if he should go to the event or not. Now that he is getting older it is easier to see how he feels about it all. And accourding to Evan's comment a couple of nights ago he must be doing OK as he said to me "Mom, is my enchanted evening coming up soon?" What a card!

Have a great day. Kim

Sunday, January 14, 2007 2:47 PM CST

Hello to everyone,
Just thought we would update you on Evan's labs from this past Friday. He first had an ultrasound of his liver because his liver function tests have been elevated since August. All looked good on the u/s and there were no abnormal signs. Labs: His liver function tests were very close to normal - finally! ANC - 1550 (consistanly below 1000 indicative of transplant) , platelets - 162 (normal 138-400), Whites - 5.0 (6.0-18), Reds - 4.32 (3.9-5.3). So all looks pretty good.

We do have all of our paper work in for our eval. at Milw. Childrens' Nutrition Clinic and for the growth study being done at Children's in Cinn. We will keep you posted. I do have to mention that when we were at the hem./onc. clinic on Friday, I asked for a weight and height on Evan. He was up 2 lbs and somewhere between 2-3 in. since his last check in Oct. That is amazing!!

Evan is starting at a new therapy clinic in town. Because of insurance issues, we are switching. Evan is starting at Aurora's therapy clinic. We met with the PT and OT this past week and they were very professional and sweet to Evan. We will go twice a month until further notice.

We also found out that Leah is a 3 out of 6 match for Evan from her cord blood. That is not a match. We are so fortunate to have Claire be a 6 out of 6 match for Evan, but still it would have been nice to have cord blood and save Claire from donating. That will be quite a decision on her part when the time arises.

I updated the photo album with some fun pics of Evan shaving with Dad. He got a Scooby Do shaving kit and loves to 'shave' with his idal - Dad.

I hope everyone is doing well. Thank you for checking in on Evan. We are taking every day as it comes and holding our breath as things seem to be very stable right now! :0)
Remember, our forth year of Evan's Enchanted Evening is come up on March 10th. It is going to be a great night of dinner, auctions and dancing. Tickets are $75 a person. All proceeds go to research for FA. Email or call us (920-339-0503) for tickets.

With all of our love, Kim and John

Monday, January 1, 2007 2:13 PM CST

Hello Everyone! January 27th, at 8:39 at night, Evan became a big brother again to little Leah Michele. She was 7 lbs. 15 oz. and 20 in. long. She is so adorable with a full head of thick, black hair. Evan adores her and just continuously tells her he loves her and announces to anyone who will listen that this is baby Leah... his new baby sister! She is quickly adopting the name Baby LeLe from Rachael and Claire just holds her on the couch with her face against Leah's. She is truly a blessing. Love, Kim and John

Thursday, December 21, 2006 1:28 PM CST

Hello to all,
I just thought I would sneak in one last entry before the holidays arrive. Evan has been doing wonderful these days.

No new counts to update you on - YEH! There are plans in the works for Evan to get a CMVP (cytomegalo virus - sp?) blood draw. This is one last attempt to try and figure out why his liver function tests are elevated. They also suggested getting an MRI of his liver to look for any abnormal growths which would explain the elevation as well. We will keep you posted.

We finally, after about 6 hours of paper work, got Evan signed up for the Milwaukee Childrens' Nutrition clinic. We are just waiting to hear from them and set up our first appt. with them. Through this clinic we will also be seeing their GI doctor. We are hoping he has some better advice for us on Evan's constant complaints for stomach pain and frequent GI issues.

Evan's therapy is going great. He is down to just once a month for both PT and OT. We do hamstring stretches at night and Evan continues to wear his braces just at night as well.

Other than that, Evan has just been consumed with Christmas. He keeps calling the Natvity sets he sees in peoples' yards 'Erector sets.' He is very much into imagining things lately, like knights and killer bees and raging pitbulls. He talks my ear off all day long with "Mom, what would happen if all the bees in the world would sting me at the same time?," or "What if all the houses started on fire and there wasn't enough firemen to help, what would we do?" or "What is stronger, a viking or a mermaid?" I don't know if I should laugh or pull my hair out! (But, or course, I laugh!)

We are all so excited for the baby to arrive. 38 weeks and counting. We had an induction scheduled for tomorrow but the hospital got so busy and we had to cancel. It is sort of ironic that this happens right before Christmas. We know know how Mary and Joseph felt when there was no room at the inn for her to have her baby. I would consider a stable at this point as long as it had an anesthesiologist on call! Evan keeps saying "I just love the new baby so much!" Again, we will keep everyone posted. :0)

Please, keep one of our FA families in your prayers. A little angel, Nina, just lost her fight with FA yesterday. Her family and friends are deeply in our thoughts and prayers.

We hope this holiday, you and your family are able to spend quality time together making wonderful memories. As always, we are so blessed to have so many wonderful friends and family checking in on Evan. Your thoughts and prayers are making the difference! Love, Kim

Monday, December 4, 2006 4:03 PM CST

Hello to all,
We hope everyone is doing well. Evan had labs on this past Friday to monitor his liver function. They grabbed a CBC as well because Evan has been looking pale these days with some reddness under his eyes. He is fighting of a fairly mild cold so he has been dragging a little too. Here are the results: Liver function tests: AST 96 and ALT 104 (Both are more elevated than in Oct. but I guess we will continue to just watch them), RBC 3.95, WBC 6.5, Platelets 150 and an ANC of 3705. Just about everything dropped since Oct. but again, he is fighting off a cold and it is to be expected. Overall, they still look pretty good.

We are also gathering release of record froms and doing the paper work to get Evan seen at a feeding and nutrition clinic at Children's in Milwaukee. We are hoping this will help him gain some weight or better his eating habits.

Evan has been doing really well in OT and PT. So well that they are moving him to just be seen once a month. Yeh! He still wears the braces on his feet at night. We have been taking a break a few nights here and there with the braces due to a red spot he has been getting from them on top of his foot. We have decided to watch his hamstring tightness and to not do Botox treatments at this time. We are doing exercises to try and improve his range of motion in that area.

And the count is on... only 4 weeks until our due date. All is going well. We do plan on saving the cord blood and then testing this little one for FA at birth. Ultrasounds so far have showed no birth defects and the growth has been really good. All of this is a good sign that the baby is healthy.

So we are just busy getting ready for baby and Christmas. Both events are busy enough without trying to do them at the same time. Evan is so excited for the baby and loves to feel the baby kick. And of course, he has a ton of cut out pictures from flyers in an envelope he plans on sending to Santa. I think Santa better get a second job!

Again, thanks for checking on Evan. We will keep you posted. Enjoy the Christmas season! Kim

Monday, November 6, 2006 8:12 AM CST

Hello to all,
Just a quick update on Evan's lab results from this past Friday. We expected things to be on the low side but all was within normal limits! RBC - 4.0, WBC - 6.8, Platelets - 171, ANC - 2992, Hemoglobin - 12.9. His liver function tests are still slightly elevated but better than they were. So, YEH! And might I just add that Evan said in the parking lot of the hospital before going in that he was going to be the bravest yet... and he held out his arm with not even a flinch as the needle went in. Isn't it amazing that my four year old is teaching me a thing or two about bravery? Take care and good blessings to all of you, Kim

Monday, October 30, 2006 3:22 PM CST

Hello again,
Hope everyone is doing well and enjoying the Halloween spirits! Just a quick update on Evan. After he had the stomach flu last week, he still remained very hyperactive and a tad more sassy (which is usually how he acts if he is tired or fighting something off). We finally had enough of it by this past Thursday and called his ped. and hem./onc. doctor. After we all thought about it, we discovered that for the past 2-3 weeks, Evan has been suffering from side effects from his nasal spray (Nasonex) which he was taking for a retracted ear drum! Once we took him off, a day or two later it was like we had our sweet, sweet Evan back! Thanks goodness because we were about to set him on the front yard with a for sale sign soon!

He also saw his ENT doc last week and has fluid behind both ears. His left tube fell out in August and his right tube is still in but blocked. We have a recheck on Nov. 13th and will see if the fluid is still there. If it is, we will consider a third set of tubes.

Evan also saw the neurologist a week or so ago. He feels Evan does not need Botox in his calves this time around and felt that him wearing his AFOs (leg braces) at night is doing the job with keeping his leg range of motion nice and loose. BUT... he wants us to consider giving him Botox now in his hamstrings. He explained that Evan's neurons fire quicker than normal in his brain causing his gait to be quite short and causing him to strike his foot down on his mid-foot instead of his heal. We talked this over with Evan's PT and decided to hold off before we do this. If at his next nuero. appt. this looks worse or has any other worsening side effects, we will consider it agian. It just sounds really painful. And if the Botox only works for a couple of months and then wears off, leaving Evan to come right back to here - why?

I think that is about it. I will list a pic of the kids in their halloween costume after tomorrow. Evan is pretty cute... Thank you for checking on him and keeping him in your thoughts. Love, Kim

Tuesday, October 24, 2006 12:41 AM CDT

Hello everyone,
Just a quick update on Evan. He had the flu Sat. night into Sun. He could not keep anything down and at one point said "Mom, I really don't like days when all I do is PUKE!" He seemed better by Sun. evening but still ran a fever in the middle of the night. We kept him home from school on Monday even though he begged to go. He seemed good all day and today is back to school! A pretty uneventful stomach flu. Now we will just try and make up a day of no calories... ice cream, here we come! Kim

Tuesday, October 10, 2006 2:04 PM CDT

Hello to all,
We are so sorry it has taken so long to update you all on our trip to MN. It just seems that once we got back, things got pretty busy. Here are the details.

Evan's labs in MN looked awesome, probably the best they have been in over a year. I don't have the specifics but just about everything was within normal limits! Yeh.

We saw the endocrine doctor and she thought Evan's height was still increasing at slow but steady pace according to her data. Even though it looked like he hadn't grown since January, her numbers still looked good from a year ago. Her recommendation was to do a bone-age X-ray when we comes back in June and to try to get Evan to gain some weight.

The GI doctor ran some malabsorption studies on Evan which all seemed fine and ordered Evan to have a gastric empting test here at home. We did have that done and all looked well, even though I will probably never get Evan to eat oatmeal after having to eat it with radioactive material in if for the test! Our local peds. doc is referring us to see a feeding clinic in Milw. at Children's to try and address the many eating issues Evan is still having. We will relay this to the GI doc from MN if he should call and follow up (which we do not expect).

We saw Dr. Wagner as well, Evan's bone marrow transplant doctor. He thought things were going well but that we needed to get Evan's weight up. Some of his labs did come back showing possible malnutrition or at least failure to thrive. His liver function tests were elevated again and we are to have them rechecked with our local hem./onc. doc in the next week or two. If they continue to be elevated, Dr. Wagner will want to do a biopsy when we are out agin in June. Evan will also have a bone marrow biopsy and aspirtation at the time so it will probably be easy enough to get done.

Dr. Wagner did mention talking more about a feeding tube in June if Evan's eating issues don't improve, which is why John and I are doing everything in our power now to get some weight on our little Spiderman. We also saw a nutritionist last week. She really didn't have much to say that we didn't already know and it seems there are other issues than just his caloric intake, such as him having to go to the bathroom three times every meal, that she could not address. It will be nice to be seen at the feeding clinic in Milw. The appt. is not yet been made but we will keep you updated. We are also going to start up on Ensure Plus at his lunch time as well as him taking it at night, which he had done for almost 6 months now.

Evan has been a little on the hyper and sassy side the past week or so. This usually clues us in that something is brewing. He does seem better the last day or two so hopefully whatever it was, has passed leaving little effect on his counts. He has had a cold and today woke complaining of his left ear hurting. I was bringing Rachael in for a cold and had Evan come along to have the ear checked out. It is not infected but is retracted. He got a nasal spray and we are to give him an antihistimine to try and dry it up before it gets infected. The tube in this ear fell out as of late August.

Evan is doing good in PT and OT. PT is only once a month now and OT is still weekly but I could see it decreasing soon as well. Evan was really walking on his toes quite a bit and the PT suggested having Evan wear is AFOs at night. We started that about a month ago and it has seemed to make a huge difference. Evan doesn't mind wearing them at night but usually around 2am we hear a bang, then some heavy footsteps to the bathroom and then everything in reverse as he goes back to bed! Evan has an appt. with the neurologist this Thursday and we are really going to push that he not have the Botox done this time or, hopefully, anymore. In MN, Dr. Wagner suggested that if Evan's tip toe waling doesn't improve, we should see an peds. orthopedic doc to talk about having his tendons cut to prevent any tighting or further complications without having him continuously getting these Botox injections.

Well, I think that covers the last month or so... it seems to have been a busy time with school starting and all of these doc appts. But are so blessed to report that our ultrasound of the baby looks to be completely normal and healthy. We are saving the cord blood in hopes it will be a mactch to Evan's bone marrow but if not, Claire is still a match. We are 28 weeks and I feel, and look, 38 weeks. WE are excited and as Evan says "Mom, we are really busy with one baby... how are we going to handle two?!" It is always a joy to see Claire, Evan and Rachael play and live together. We cherish each like they are the most precious gift we could be lent from God.

We also have to thank everyone who planned, participated, shopped or attended Shop For A Cure in Indiana. It was a huge success raising almost $6,000 for research of Fanconi Anemia. We are so very blessed to have such wonderful friends and family that have always surrounded Evan with love and hope... we are forever grateful. Kim
PS - I will add some new pictures! Kim

Saturday, September 9, 2006 8:32 PM CDT

Hello to all,
Just wanted to update you on Evan's trip to see the ortho. peds. specialist at Children's in Milw. They did several X-rays that showed Evan had a few extra bones in his cervical spine area and that he had some extra cartilage in between his scapula and spine on the right side. Dr. Tassone met with us after veiwing the x-rays and said remarkably, Evan's extra bones in his spine balance out almost perfectly and his spine remains straight. Also, for whatever reason Evan has extra cartilage or tone in his right shoulder, it doesn't seem to be affecting his range of motion to a large extent. His advice was to get an MRI to obtain a baseline and then just watch him early to make sure all continues to look good. WOW! We are thrilled to hear this news and it is just one more reason Evan is such a miracle.

We go to MN Sept. 20 and 21 to see a GI doc, endocrine doc and his bone marrow transplant doc. It will be nice to get some follow up in these areas. We will keep you posted.

Other than that, Evan started school two weeks ago. He is going 3 full days and seems to be loving it. It is a change to not go to school in the same classroom with Claire but they both seem to be adjusting well - it is Mom we is having a hard time adjusting :0) Take care and God bless, Kim

Monday, August 21, 2006 7:46 PM CDT

Hello to all,
I hope everyone is surviving the end of the summer/school starting transition...

I just wanted to update you on Evan's recent fever. He had the fever from Thursday to Saturday afternoon. He has been without a fever since Sunday and seems to have no other symptoms/side effects from it. I have noticed he seems more drained at the end of the day and a bit more "punchy" throughout the last two days. There is really nothing we can do but hopefully watch him improve as whatever effects the fever had on his body will improve as he stays healthy. I will keep you posted :0)

As always, with lots of love, the Connellys

Thursday, August 17, 2006 8:19 PM CDT

Hello to all,
Just wanted to let you know that Evan woke up tired and whiney today. By 1pm, he was begging to take a nap. By early evening, he was complaining that his stomach and legs ached. His temp at bedtime was 102.5. We just gave him some Motrin and will check in throughout the night. We are disappointed to see a fever because his counts are just starting to come back up from the Hand, Foot and Mouth Disease episode in June. Hopefully, whatever he has, will pass quickly. Say a little prayer for him if you have a second to spare... Kim

Friday, August 4, 2006 10:20 AM CDT

New Photos!!! How is that Kristen?!

Today we are off to South Haven, Michigan. We are taking a ferry and the kids are so excited. Hope everyone has a great weekend. Kim

Wednesday, August 2, 2006 8:22 PM CDT

Hello everyone,
Evan had his referral to the orthopedic today for asymetrical issues in his shoulders and weakness on his left side of his trunk. The ortho. doc looked him over and said he would like to refer him to a ped. ortho. spine specialist at Children's Hosp. in MW. He has suspect that it is something called Klippel-Feil Syndrome. He was kind enough to run some info. off for us from the web. When reading it, it sounds like it is a genetic disorder that presents with fusion of two or more cervical vertebrae, a scapula that is radiated ourward, along with possible other abnormalities. Along with this, patients with KFS also are at risk for renal issues, hearing loss, facial asymmetry and cardiovasular issues, along with the issues of the fused vertebrae. While I agree with the possible issues of fused vertebrae and scapular abnormalites (along with other possible deformities unknown in that same area), I don't think Evan has this syndrome. It seems like alot to be diagnosed with two genetic disorders?? I think Evan already has his share of abnormalities that can be explained by FA and one of FA's characteristics is skeletal abnormalities. His possible fused vertebrae (etc.) can be explained by the FA and doesn't necessarily mean he has KFS. But, as you know, I am not a doctor. We are NOW scheduled to see this ped. spinal orthopedic on Sept. 6th. I will keep you updated...

Evan remains, as always, to be such a wonderful part of our lives. His strength and attitude guides us and teaches things we never would have learned without having been in his life. He is truly a miracle in our eyes. Kim

Sunday, July 16, 2006 1:36 PM CDT

Hello to everyone,
We just thought we would update on Evan's labs from this past Tuesday. His WBC are up from 4.9 to 5.8 (normal 6-11), RBC are up from 3.67 to 3.70 (normal 4.5 to 5.5) and platelets are up from 121 to 157 (normal 145-440). His hemeglobin is down from 11.9 to 11.7 (normal 12.5-14.5). Evan's liver function tests are also improved but still not normal. Our game plan is to do labs in another three weeks to hopefully see more of an improvement in his overall labs and normal liver function tests.

We are also coordinating a trip to MN to see his bone marrow transplant doctor, a GI doctor (Evan goes to the bathroom 1-3 times during every meal), and the endocrine ped. doc since his height has not changed in the last six months. We are hoping we will get to MN before school starts but who knows.

On the 24th, we will be seeing our ped. regarding the worsening scoliosis and muscle imbalance in Evan trunk and upper body. I will keep you posted. Hopefully, it is nothing and Ms. Mary, Evan's amazing therapist, can really start to push him and begin to see improvements in this area.

Evan had Safety Town this past week and so I have been hearing about water, electricity, fire, stanger, animal, car and bike safety all week. He says to John and I in the car "Click it or Ticket!" His spirits are great and other than napping during the day, his energy level is good too.

Also, we are proud to announce that we are expecting again around the beginning of January. We are very excited to be expecting our fourth child and our little "caboose." Accourding to ultrasounds, everything looks healthy but we will do an indepth ultrasound at 20 weeks to look for birth defects. Evan and Claire are constantly kissing my belly and even though the subject of Christmas far outweights the subject of expecting our "new" baby around that time, the kids are thrilled :0) Take care and I will post again when something comes up. Hopefully, it will not be for awhile! Kim

Tuesday, June 27, 2006 10:23 AM CDT

Hello to all...
I just added a journal entry two days ago so if what I am talking about makes no sense to you, check the journal history for details.

We had our appt. with Dr. Brandt, Evan's hem./onc. doctor yesterday for a re-check on his liver function tests, a CBC and a complete physical. The liver function tests are almost completely improved, which is incredible since they were so high. Dr. Brandt said that the liver has the most potential for healing itself so he has no concerns for long-term effects of these high numbers on Evan's liver. He does want to recheck them again in two weeks. We think it was the virus he had that got the liver so excited and it is really uncertain what will happen next time Evan gets sick.

For Evan's CBCs, mostly everything dropped further. His WBC are now 6 (4-12 normal), RBC are 3.84 (4-5.3), and platelets are 137 (150-450). We will recheck these in two weeks as well in hopes that the virus he had (we think it was Hand, Foot and Mouth Disease - very common in young kids)knocked them out and in time they will come back up.

Upon Evan's pysical with Dr. Brandt, we notice Evan has plateaued in his growth and height over the past 6 months or so. It is his recommendation that we see Evan's endocrinologist. I will be making the appt. to see Dr. Petrik who is in MN today.

So all in all, this virus really took a toll on Mr. Evan. We are hoping that with some time and no more getting sick, he will replenish his counts and be back to normal in no time. As alwalys, I will keep you posted. Thank you for checking in on Evan and always, keep the little guy in your prayers. Kim

Sunday, June 25, 2006 10:02 AM CDT

Hello to all. Yes, it is with new concerns that I am writing today. It seems that I am adding new entries more than ever lately and that makes me nervous.

Evan started with a high fever (104.0s) on Wednesday. We had to give Tylenol and Motrin to keep it down. Evan was scheduled for routine labs at his hem./onc. dr.'s office, Dr. Brandt, the next day on Thursday. We debated even going in but Evan seemed better with the fever reducers on board so we brought him in. The wanted to still grab labs so we did. We are watching his liver function tests these last couple of weeks waiting for them to go back to normal after, we guess, some viral infection elevated them. Evan only wimpered a little but was VERY excited to get a Rescue Heros bandaid! Without event, we went home. About a half an hour later, Dr. Brandt called to say his labs were off and he wanted Evan to be seen that day by his pediatrician. Evan's liver function test(SGOT)was 572 and normal is 1 to 45. His SGPT was as high as well. His red count was 3.84 and normal is 4 to 5.30 and his platelets are now down to 137 and normal is 150 to 450.

We were worried and Evan was starting to get his fever again. We brought him in to the pediatriician's, Dr. Hoyer, for an exam. It was not of concern what he had but how to bring down the fever. Tylenol is hard on your liver and Motrin is hard on you platelet count. But we had no choice, at 104.8, we gave both to get it under control. Evan was hot, lethargic, shaking and crying.

The fevers lasted for only another 24 hours and whatever it was he had seems to be gone. We are going back in to Dr. Brandt's office Monday morning for a complete physical and to try and find out what is going on with Evan's liver. I will keep you posted. We will also continue to monitor his CBCs hoping for them to fall back into normal. A good thing is his ANC level, how he can fight an infection, is good.

It is funny how as Evan ages, John and I need to be more aware of what he is hearing and what he wants to know. As he was so sick and I told him we needed to take him back into Dr. Hoyers office, after just getting home from Dr. Brandt's, he says "Mom, why do I need to go there now." I found myself saying to him "When they take blood from your arm, it shows them how your blood, bones and body are working. This morning, your blood showed that it and your body might need to be looked at by another doctor today to make sure everything is good." And Evan says "OK Mom, lets go." So matter of fact and part of his life... I wish I could do it for him, all of it...

Evan has started napping again too. His tiredness is probably representative of his low platelet and red count. He has been really tired for about 3-4 weeks and doesn't even fight me to nap. By bedtime, he is asking to go to bed again. The extra rest is good for him and gives him the energy to get through the day. I hope as his counts rise, so will his energy level. We has started really pushing his Ensure intake to stop any wieght loss that might be happing from his poor eating lately too. His therapy is going well. Without his leg braces on the past two months, he has started tip toe walking again. We have him in firm shoes right now and notice it does help. But we are trying to avoid the clunky AFOs in the summer if at all possible.

So I guess things are going all right. We have faith that these things we can not control will be handled by God in Evan's best interest. It is our job to try and make informed decisions on Evan's behalf and love him to pieces. Please keep him in your prayers for a full and speedy recovery. Thank you so very much, Kim

Wednesday, May 17, 2006 10:21 AM CDT

Hello to all,
I think I have been posting journals more in the last couple of weeks that in the last four years! I just wanted to let you know what we made out with Evan's liver functioning test and his complete bone marrow biopsy results. Last Thursday, they did an ultrasound of Evan's liver since his SGOT and SGPT were elevated. The u/s looked fine and they reran the labs again. The SGOT and SGPT were still elevated but down from the initial labs. We will have them redrawn again next Thursday to make sure they go down to normal. Dr. Brandt thinks they are elevated due to some viral bug. I have also heard from other FA families that their FA children tend to have a very sensitive liver to viral infections and often have these labs elevated for no particular reason. When I find out the results from next week, I will pass them along.
We also got the cellularity level of Evan's bone marrow back from the biospy just done. He gets this checked each year and so far his numbers have been excellent. The cellularity number is given in a percentage and is the number of bone marrow producing cells there are mixed with fatty cells in his marrow. Usually, the number is 100% minus your age. Evan has ran in the 80%s for the last three years. This time, he was 25-35%. Knowing we were concerned, Dr. Brandt said that the number is only a representation of the the area where the marrow biopsy was taken and doesn't necessarily mean that Evan is doing so much worse. We will continue to look at his red, white and platelet cells through CBCs. If this is truly representative of Evan's bone marrow, we can anticipate his CBCs to be dropping more steadly than they have been. We are hoping the boipsy was just take from a poor area but we do not know for sure. Always something...
Other than that, all is well. His left tube in his ear has fallen out with out incident. His therapy is going well. We are trying Evan's walking again without his braces and watching for him to tip toe walk agian. I see him doing it more in bare feet but not yet in his shoes. He has this wierd rash under his eye right now that I am debating taking him in for. And of course, he fell from the top of the couch this morning as he was launching a paper airplane and then jumping off the couch (without the cushions in it - it is their favorite sport these days!)to try and catch the airplane mid-air. Did he not think he would eventaully get hurt? What can you do - he is all boy!
Again, we hope all is well with you and your family. Thank you for thinking of Evan and if you have time, keep the little munchkin in your prayers... Love, Kim

Monday, May 1, 2006 8:28 PM CDT

Hello to all, just wanted to update you since there seems to be enough going on with our little Evanator lately... we hope you and your families are doing well and thank you for taking the time to think of Evan.

He had his annual bone marrow biopsy and aspiration done last Thursday here in Green Bay. All went well but now that Evan is getting older, the lab draws are getting harder. He asks for them not to be done and whimpers through them. The biopsy and aspiration went so well do to the complete professionalism and caring nature of the entire staff at Dr. Brandt's office. They are amazing people to work in pediatric oncology and hematolgy and are truely gifts to our family. We got the results back from the the morphology test and no signs of cancer or luekemia were found in his marrow. His CBC had dropped but still is alright. The concern is his liver functioning tests (which is about the only organ in his body that has not been evaluated before). His SGOT was 97 (norm 15-41) and SGPT at 124 (norm 17-63). We are uncertain why this is and Evan is going back in for further lab work on Thursday and an ultrasound of his liver. We will keep you posted.

Evan has been working hard in therapy and got to ride a horse last Wednesday as part of his therapy. Riding on the horse works his upper body and overall muscle tone and coordination. He is making huge strides there and his therapist is very sweet, encouraging and patient.

I think that is about it. We are just concerned about his liver function and I guess we will find out what that is all about on Thursday. Evan, these days, believes he is either Spiderman or Rocky Canyon of the Fisher Price Rescue Heros. His energy is never wavering and his love for life is encouraging to all around him. He is such a blessing to this family and to all we know him. Have a good day... Kim

Monday, March 6, 2006 9:07 AM CST

Hello to all,
It has been a VERY busy last couple of weeks. This past Saturday we held our third annual Evan's Enchanted Evening. All the numbers are not added up yet but we are pretty certain we did better this year than last year (and last year was great!) Thank you to everyone who donated items, services, time and/or attended. The evening would be nothing without you. All proceeds go to research for Fanconi Anemia through the Fanconi Anemia Research Fund. Evan and Claire even showed up for about a half hour. When I get pictures, I will post one.
As for Evan, he is doing well. The only thing new with him, since he isn't do for labs for another month, is his evaluation for therapy. When Evan had his IEP (screening for high risk kids through our local school system), they recommend he could use work on his "rib cage expansion and breath control." Evan on his birthday two weeks ago (four years old!), needed us to stand behind him to blow out his candles so he wouldn't know we were doing it. When we went for the evaluation, I was prepared for the therapist to say "Sure, he could use some therapy but it isn't such a big deal." When we walked in, she immediately noticed one shoulder is higher than the other. After checking it out and watching Evan do certain things, she said he is missing most of his muscle tone on his left side of his upper body. His shoulder blade is also higher. Maybe because of his heart surgery and the incision on his back, Evan is also not able to raise his right arm up completely. She noticed some coordination issues in his trunk and he was not able to do certain things like stand on one leg. Her recommendation is therapy weekly for sure for 6 months.
It was so hard to see Evan failing at these things and to see his body in a way I have never looked at it before. To me... he is perfect in every way and unique only because he was chosen to be special. To watch him be given these tasks and fail at them, I watched for him to sense that he wasn't do good at them but I don't think that he did. Well, he is not a stranger to working hard to accomplish everyday activities. His braces on his feet are going well and with a few exceptions, he wears them all day. I think we are even starting to see an improvement in his walking when he is not in them.
As always, we have faith that everything will be alright and that Evan will have the strength to do what is needed. In the light of things that could be going wrong, this is nothing. John and I will do our best to help Evan with his therapies, make his days full of laughter and normalcy and to stay strong for him, Claire and Rachael. Thank you for your support of Evan. Kim

Monday, February 6, 2006 7:48 PM CST

Hello everyone,
I just wanted to tell you that Evan got his new AFOs this past Thursday. They are braces that, with the help of the injections in his calves every 3 months, will allow his muscles to become less tight and help him walk flat footed. He should also then stop falling as much and complaining about the pain in his feet and calves. I was worried that his first impressions of them would be negative and that we would have a hard time getting him to keep them on. But so far he has worn them everyday almost all day long. I do notice he goes to bed extremely tired these last couple of nights but I am so thrilled he wears them. They make him a little more clumsy right now but if you ask him, he will tell you that the motorcycles on the straps (which he got to pick out) will make him "very fast like a motorcycle!" He knows what they are for and every afternoon he asks to have them off but then will say he will keep them on because he knows they will make his walking better. Once again, I worry for nothing! Thank you to everyone who hyped them up and told him they look cool - he takes what you say to heart. Speaking of hearts, have a great Valentine's Day! Love, Kim

Friday, January 27, 2006 8:41 AM CST

Hello everyone. The sun is actually shining here in De Pere, WI! That is a rare occurance lately. We will take it!

I thought I would update you on Evan's labs. They were drawn on 1/23 after Evan continued to be tired and pale. Whites - from 5.4 to 7.0 (normal 6.0 - 18.0), Reds - from 3.99 to 4.23 (normal 3.90 - 5.30), Platelets - from 169 to 151 (normal 138 - 400). His platelets are down, explaining the paleness and tiredness but everything else is up. All in all, the labs look good and no precuations to take at this time. We will just redraw in 3 months. Yeh!

Evan in at school today. It is not his normal day to go to school but it is 100 Day! The school is making a big batch of chex mix with alot of different ingredients. Claire has to bring 100 cheerios and Evan had to bring 100 M&Ms. M&Ms are his favorite so counting them without eating them was very hard to do! I can only imagine the germs in in this bowl of chex mix! Take care. Kim

Wednesday, January 11, 2006 8:10 PM CST

I can not believe it has been so long since my last post on Nov. 4. I guess it explains what kind of holiday we had - a blast but very busy! I hope the holidays have been good to all of you and that Santa was brought you want you wanted.

We still have not had labs drawn since the last post when they were low. Evan remains the same. He still looks pale and most of the time has this reddness around his eyes. He has been falling asleep on the couch some after noons lately. I am not sure if that means anything but, of course, everything makes us worry. Still, his activity level is just fine! We have labs drawn again on Feb. 1st and we will post them as soon as we get them.

Evan saw Dr. Edgar last week who is his neurologist. He has diagnosed Evan with idiopathic (no explanation) tip toe walking. He got Botox shots in both his calves which he did not like at all. He also got fitted for AFOs or braces that will make him walk heel to toe. We were very disappointed to hear Evan would need these braces again but Dr. Edgar's felt that if we didn't get him walking heel to toe, Evan would continue walking on his tip toes which would lead him to become tight in his muscles and eventually need surgery. If you see Evan in them, make sure you tell him you think they look cool...

Other than that, all seems to be well. Evan got a set of drums for Christmas that he LOVES! His Christmas was a blast and it was so much fun seeing him and his sisters enjoy each part of the holiday. We are truely a blessed family. Lots of love, Kim

Friday, November 4, 2005 3:12 PM CST

Hello to all,
Just wanted to touch base with everyone. Evan is doing well. He was Spiderman for Halloween and loved it. I will try to get it in the photo album.

He had his initial review with our public school system. Cognitively and developmentally, he did great averaging about 4 to 4.4 years old in alot of areas (He is a little of 3.5 years old now). Physically, he had just a few issues when his size came into play. They only wanted to re-evaluate him for speech in 6 months and that is all. They recommended he see a physical therapist to help him gain lung capacity to talk louder and stronger without taking so many breaths in between words.

Also, the last two weeks or so, we have noticed he is falling quite a bit, looking pale and bruising more. So we got labs two days ago. His whites went from 6.7 to 5.4, his reds went from 4.19 to 3.99 and his platelets went from 206 to 169. His ANC dropped from 3752 to 1944. I am not sure what the follow up is but he goes in for a flu shot on the 17th and I will ask then. I really don't remember him being sick to have had depleted his counts other than the cold he had beginning of October, which he faught off better than Claire did. As always, we will hope that his counts come right back up. I will keep you posted. We hope you and your family are doing well. Love, Kim

Tuesday, October 4, 2005 10:30 AM CDT

We got results back from his labs done on 9-16. They were redrawn because labs on 8-26 did not look good and we wanted to see what was happening. Everything from the latest draw has went back up (Whites - 5.8 to 6.7, reds - 3.94 to 4.19 and platelets - 138 to 206) and he now has an ANC from 2668 to 3752. We are actually in normal limits for a change! It was concerning that his body was so depleted by the viral bug he had at camp. It was recommended to us by Dr. MacMillian, one of Evan's BMT docs, that he should have an immunophenotyping done to look further at his immune system.

FA D2 patients have shown a trend in the past to have weaker immune systems. Evan does not always catch every bug flying around but he does seem sick more often than Claire, which we always expected since he had FA. We should know the results of the immunophenotyping in a week or so. And crazy enough, Evan has a head cold and a cough for the last 5 days. Acutally, I think it is going through the whole family with the exception of John (who never gets sick). Now I am concerned what this cold will do to his counts. Still, I guess you can't be checking his counts after he gets sick every time. He will have another draw in the middle of December. That is a long time to worry...

Also, Evan is being evaluated by the special needs program at Westwood School, our public school system. He had an evaluation last week and four this week including speech, occupational and physical. We will meet with his case worker and the entire therapy and medical team on Oct. 24th to determine what we should be helping Evan with. Always something going on...

We hope this entry finds you and your family happy and healthy. Oh, if I haven't already mentioned it... Evan's Enchanted Evening '05 is March 4th, 2006! And a new event -Cropping for a Cure is April 1st, 2006. Attendance, prayers, support, donations - anything and everything is welcome and appreciated. In addition, we are selling Entertainment Booklets again this year for your area, please contact me if you are interested in purchasing them with proceeds going to FARF. I think that is it! Kim

Friday, September 9, 2005 2:15 PM CDT

Just a quick update.

We spoke with Dr. Brandt today, Evan's local hem./onc. doctor. We faxed him the latest labs yesterday. He would like Evan to have his labs redrawn no later than next week. We will keep everyone posted.

I spoke with the pediatrician who is filling in for 'our' Dr. Hoyer while she is out. She gave us some suggestions for changing Evan's diet to try and lessen the times of diarrhea. And actually, it seems to be doing better lately all on it own. He still complains of his stomach hurting him throughout the day. His Fecal Quat. Fat number came back high in the tests they ran which probably just shows malabsortion. We will try the changes in his diet and see the docs in two weeks to evaluate how everything is going (Again, no pun intended).

Thanks for thinking of the Mr. Evan. Kim

Wednesday, September 7, 2005 9:44 AM CDT

Once again, it is time to update update our journal. Things have seemed fairly busy lately dealing with the FA issues in our life. We went to Camp Sunshine, a camp for families with FA, in the beginning of August. Evan was battling a temp of 103 to 104 and Claire came down with a double ear infection. Despite them not feeling 100%, both seemed to have a great time at camp. John and I found the meetings to be educational and informative as always.

Evan's labs were drawn on August 26th. Here are the results: Reds 5.8 (normal 6 - 18), Whites 3.94 (normal 3.8 - 5.8) and platelets 132 (nomral 140 - 400). The red cell morphology, or shape and size of the cell, came back abnormal and we will have to check in this further to see what that means. Obviously, things do not look all that great but we do not have to take any extra precautions at this time. The nurse did say to try and keep him low key and then we both laughed at the obserdness of the suggestion knowing Evan.

Evan just recieved botox injections in his calves again. His degree of flexion in his feet are down to 10 degrees. Dr. Edgars, pediatric neurologist, said we have to do the injections before Evan has a difficult time walking flat footed at all. We also had to find him a pair or high tops to keep his foot flexed at all times. So if you see him, comment on his "cool magic running shoes." If the shoes don't work, we will have to go back to AFOs which are braces that go up to his knees. We are trying to stay away from these since he started school and is just trying to be "one of the kids."

Evan also has been struggling with stomach pain and constant diarrhea. We did a bunch of test on him and all seemed to come back fairly normal. This is a common complaint of FA children and, usually, nothing is ever found to explain it.

Evan is also going to be evaluated by our local school system for a possible speech delay and any other help they feel is necessary. We should be setting the date once they recieve records on his previous therapies.

Also, we will be doing some immune studies on Evan through Dr. Wagner's office. It was recommended by Dr. MacMillian in Minnesota to have this done as it seems FA D2 children seem to have more issues with their immune systems.

On a good note, Evan ear's have infection free and his hearing is fine - just selective! He started back at school and is doing well. Currently, they are doing chores around the house to raise money for Hurricane Katrina relief. My house is, for the first time ever, clean! Apparently, I am pretty boring as he misses Claire now that she is in all day Kindergarten. He continues to adore Baby Rachael and her newest nickname by him is "Ray Ray the Day Day!"

Of most importance, I would like to tell you of a HUGE discovery just made in the last couple of weeks. The National Institue for Aging, part of the National Institute of Health, has discovered an additional FA gene - FA M. It appears to be responsible for DNA repair. As you know, the unability of our FA children's DNA to repair is why there are birth defects, bone marrow failure and cancers/luekemias/tumors. This discovery narrows our search for a cure dramatically, and now we know where to look and what questions to be asking! This research project was supported by the Fanconi Anemia Research Fund (FARF). Our friends and family have been monumental in raising incredible amounts of money for research through FARF. You all should be VERY proud to have helped make this happen and move us a huge step closer to finding a cure. If you would like to read more on this, here is a news article explaining it better:
http://www.registerguard.com/news/2005/08/23/a1.faconianemia.0823.p1.php?section=cityregion


I hope you and your family are happy, healthy and safe in the current climate of Hurricane Katrina - where life seems so very fragile. Love, Kim

Tuesday, July 12, 2005 7:29 AM CDT

I just didn't want to leave anyone hanging with the results of our trip to MN a week and a half ago. While we are still waiting for actual copies of all the results to be sent to us, I can tell you this: all the endocrine lab work looked fine along with this CBC labs (which we sort of knew because they were just drawn 2 weeks prior and all was OK then too) BUT the bone marrow biopsy and aspiration proved to be interesting. We are concerned with the bone marrow cellularity percentage which is the number of bone marrow producing cells compared to fatty cells in the marrow. The number should be 100 minus your age roughly. At one year of age, Evan had a cellularity level of 50%. At two y/o, his cellularity was 60%. So this year we joked with Dr. MacMillan that she had the magic touch last year and to please increase Ev's cellularity level by another 10% this year (but in all reality it is typical to jumb around a little in celularity levels just depending on the place the aspirate was taken from). Well, his cellularity level was 70-80% this time! Can you believe it! There were also no signs of luekemia or early cancer cells in the marrow. We are so excited that everything looked good from the doctors' prespective. We might have somewhat of a normal year!

Evan is getting seen by the De Pere public school system just to introduce their staff to him for future needs and to have him evaluated for special needs and possibly speech therapy. We are also going back to Evan's neurologist to see, once again, if there is anything else we can do for his tip toe walking and spasticity in his legs. We will keep you posted on that. Otherwise, he starts his first soccer class tonight where they get to kick around a ball and do activities like bouncing balloons off their heads. He is very much looking forward to it.

Life is so great for us right now. We are having a busy summer but are so appreciative of Evan's overall health and all those people that help us in regards to his health care and we keep Evan in their thoughts and prayers. Thank you. Kim

Thursday, June 30, 2005 10:11 PM CDT

Well, we just got home tonight from our annual trip to Minnesota. We saw Dr. MacMillan who, with Dr. Wagner, will be someday assisting us with Evan's bone marrow tranplant and who help us navigate Evan's daily FA issues. Dr. MacMillan was so good to us and we talked with her for about an hour discussing Evan's growth issues, ear troubles, lab results over the past year, outlook for the next year and his plan of care. We also saw Evan's ped. endocrinologist who was pleased to see some growth and weight gain over the past year. She determined that his height and weight percentiles compared to others his age is only minus 2-3%off the growth chart. She felt this was minimal enough to continue doing what we have been to maximize his caloric intake and also wrote for labs on his thyroid function and growth hormones.

Next, Evan went for his bone marrow biopsy and transplant. He was such a trooper and the nurses could not believe how outgoing he was with all they needed him to do. He would lift his shirt for them to listen to his heart and lungs,assisted in putting on and taking off the blood pressure cuffs, helped to get height and weight measurements and held the probe under his arm for a temp. There was a child life specialist that came to talk with Evan in the playroom about his possible anxiety about going back to surgery. We had to keep telling him to get back here and listen to the nice lady and stop playing! He was hoisted on the bed, kissed us good bye and was gone. When Dr. MacMillan came out to let us know the biopsy went fine, she added that he had all the nurses and staff in the OR in stiches before they put him under. He was so excited to breath in the "mask" like a firman does! When he woke up, he was very calm and his recovery nurse fell in love with him for his brave and mature attitude to what was going on. Plus, she gave him spiderman stickers so he loved her right back! He recovered quickly and then began to say "I am all done with this," so we dressed him and headed out. The 5 hour drive home was uneventful and he is now sleeping in his bed.
We won't know results to the biopsy or labs for a week or so but will post them when we have them. Again, thank you for thinking of Evan. I don't know where he would be without all of your kind thoughts. Kim - "Mamma"

Thursday, June 16, 2005 6:34 PM CDT

Labs from last Friday (6/11):
WBC - from 6.9 down to 5.7 (Normal 6 - 18)
RBC - from 3.94 up to 4.42 (Normal 3.9 to 5.3)
Platelets - from 214 down to 180 (Normal 138 to 400)
ANC - from 3588 down to 1938 (Normal is anything above 1000)

If anything, we learn to not take one tab result to mean anything serious, but rather, to look at them collectively over a period of time. Since Evan was born, overall, his counts have all dropped significantly but they do tend to flucuate up and down over the course of several draws. We will look to see what the bone marrow biopsy and aspiration will show us on June 30th. Since the bone marrow is the "factory" the makes all these cells we have checked in lab, if the "factory" is doing fine we will continue doing what we are doing now. Hope all is well with you. Kim

Wednesday, June 8, 2005 10:00 PM CDT

Wow. It doesn't seem like April was the last time we made a journal entry but I guess this website is telling the truth. Evan is doing great. He had his last day of school today and everyone went to a park for a picnic. He had a ball. Evan is loving summer and his knees have more bruises from climbing, falling, tripping, etc... He doesn't miss one minute out of any day - that is for sure!

His ears... we went in a couple of weeks ago for a post op appt. and the doc pulled out a huge piece of dried blood from his right ear. Apparently, this was blocking the way for the post op ear drops and so the ear drum was inflamed and the tube was not working. So the ENT put him on steroid ear drops for the next month and a half which we give before bed. I just brought Claire and Rachael in this week for ear infections and they took a look at Ev's. Already, the tube is working and the swelling seems to be going down. Yeh! Finally. When you ask Evan, he says "My ears are feelling MUCH better." That says it all...

Evan went to see the eye doctor two weeks ago and all is well there. His right eye is not quite 20/20 but not so bad that they are thinking glasses at this point. We don't have to go back to the peds eye doc for another two years!

Our little man is due for labs this Friday so I will post what they are when we get them. Also, he is going for his annual peds endocrinologist appt., visit with Dr. Wagner and bone marrow biopsy/aspiration on June 30th. We are hoping all will go well.

Other than that, we have just been loving being a family of five and Claire and Evan adore Rachael. He calls her "My Rachael...!" And then him and Claire argue about whose sister she is and how they don't want to share her with each other. They are both looking forward to a great summer of lot of cool activities, and of course, a trip to Maine for Camp Sunshine!

Hope all is well with each and every one of you. Lots of love from all of us, Kim

Friday, April 22, 2005 9:50 AM CDT

I know we just updated everyone on Evan's most recent labs but I could not believe the number of people we signed the guest book regarding Evan's ear tube and adenoid (Which Evan calls them 'astronoids')surgery. You can look back in our journal history to see labs if you wish. Tubes and Adenoids went great. I went back with the nurses by himself this time without any sedative either. I think I needed a sedative not to be there when they put that mask over his face to put him to sleep. But... he did great and had the surgery room cracking up from what I understand with his interpretation of how he blows up a balloon (I am sure the ballon topic is their ploy to get him to breath into the mask.) Anyway, he woke up and was fine. Recovery went smooth and we were home in no time.

The doc said Evan's left ear had a layer of crust over the eardrum and that the tube was not working anymore. He cleaned things up, took out the old tube, put in the new one and found fluid behind the eardrum as well. The right ear was really bad. The old tube had fallen out and gotten lodged in his ear crooked. Then from all the ear infections since there was a build up of blood, wax, and infection around that tube. The doctor pulled that out to find fluid behind it. Then when he made the incision for the new tube, there was puss and infection behind the eardrum as well that just had no place to go since the old tube was blocking the entire canal with debris. It sounded pretty bad and it explained the pain Evan has been complaining about in his right ear for months.

Then last night, Evan was all tooked in bed and he started crying and complaining about his ear hurting. I went in there to tuck him back in since he couldn't have anything else for pain meds and I heard this squishy sound in the dark. I turned on the light to see blood over his ear, face, blankets and hands. We called the doctor he said Evan must of re-aggrivated a spot that was trying to scab over from that morning. We are now filling his ear with gauze and keeping a small pressure dressing on it for the next couple of days. Ugggg! When I finally cleaned Evan up last night and tucked him back into bed, he said to me "Why does my ears always have to hurt me?" It tugged at my heart strings a bit. Today (the next day) we are suppose to be leaving for a water park with friends for the weekend that was planned for months now. We are going to have him wear an ear band over his head to keep his ears dry and safe. Crazy.

Anyway, it is so sweet that so many of you were thinking of Evan yesterday. We appreciate every thought and kind word so much. Take care, Kim

Tuesday, April 19, 2005 11:23 AM CDT

Hello to everyone! We got labs drawn last week and here are the results: White cells 6.9 (normal 6-18), Red cells 3.94 (normal 3.9-5.3), platlets 214 (normal 138-400), and ANC 3588 (under 1000 is not good). Overall, these are excellent. Evan's labs two months ago were 5.6, 4.22, 145, and 1624 respecfully. His reds are down but other than that, everything is up! Yeh!

We also got news that Rachael FA test came back negative so she is perfectly healthy. And her cord blood is a 4 out of 6 match for Evan. That means that if we didn't have anything else for transplant we would use this but because Claire is a 6 out of 6 match, we will still use her bone marrow.

Evan goes in on Thurday to have his tubes put back in both ears and his adenoids taken out. We are glad this is being done because he has hand one ear infection after another since about January.

That is it for now. Again, thank you for all the thoughts and prayers. We so appreciate them all. Kim and John

Tuesday, April 5, 2005 11:36 AM CDT

Hello to Everyone!

We are thrilled to announce that Evan has a new little baby sister as of March 21st! And... we just got result back yesterday telling us to is perfectly healthy and does not have Fanconi Anemia! Evan and Claire are in love with her and she is such a sweet, beautiful baby. It is like she was meant to be with us all along. Evan says to everyone we meet "Want to see my new baby sister?" and tells her "Baby Rachael, I love you SOOOO much" all day long. We are still waiting to hear if her cord blood will be a match to Evan to be used for his bone marrow transplant in the future. We will keep you updated.

Evan is doing well. He is still battling ear infections continuously and they have just decided to take out the old tube that is wedged in his right ear and clean out all the debrie that is wedged in there, put tubes back in both ears and remove his adenoids. This will happen April 14th and we are guessing it will have minor effects on Evan. It will be good not to continuously be giving him antibiotics and Motrin/Tylenol when he complains that his ears are hurting him.

Evan is due for labs next week so we will post those results as well. He has also been tripping and falling alot, and back to his bad habit of tip toe walking more than ever. We are concentrating on doing his stretches in his calves and foot muscles to see if that will help. If not, we will pay another visit to the neurologist to see if he recommends doing the botox injections in his calves again or doing the leg braces again.

Other than that, life is great at the Connelly household. We are having a blast watching Claire and Evan interact with Rachael and adjusting to the addition of a new born baby. Again, thank you for your thoughts and prayers for Evan. We hope you and your family are also doing well. Take care and I will write next week. Kim

Friday, February 18, 2005 9:06 AM CST

Hello and thank you for checking in and seeing how Evan is doing. He will be three on Monday and wants a Blue's Clues cake. He isn't much into Blue's Clues anymore but it was the cake he had last year. I think that he just thinks that is the way it should be. He seems like such a little man lately. He is potty trained, going to school three days a week, and sleeping in a bunk bed! He is a very sweet little guy and often tells the ones he loves "I love you so much in the whole world all night!" Still, he is very much boy and into name calling. Lately, everything is labeled as a "poopy stinky diaper." We are trying to break him of this but as you know, he is very strong willed.

Evan had labs drawn last week. He is getting older now and knows when we wait in this certain waiting room that he is going to get a "poke." He cried this time but recovered quickly with the help of the three stickers he got to pick out. Labs: Whites - 5.6 (normal 6-18), Reds - 4.22 (normal 3.90 - 5.30), Platelets - 145 (normal 138 - 400) and ANC 1624 (any thing under 1000 would mean going to tranplant). As you can see, he is continually falling. He has been sick for the last month with double ear infections that just don't seem to go away with several different antibiotics, several colds, and this past week, Influenza A. With a low white counts, he will be doomed to get whatever is going around until (hopefully) it begins to go back up. We try not to worry about his counts and hopefully we can contribute them to him being relatively sick the last couple of weeks. But realistically, it shows he is heading toward bone marrow failure as his bone marrow can not replace the blood cells so need when you are ill. We will continue to monitor his labs and the next draw will be in two months. We will keep you posted.

Evan, now almost three, is discharged from the Birth to Three program and will no longer get any therapy by them. We had him screen at our county's Developemental Days and he did wonderfully. It will now be John and my job to detect any kind of delay and get help through our local school system. Evan is also in school with Claire and we are confident that they will also be able to detect any delays as well. He loves school and being with other kids. It is also nice that he is in the same classroom as Claire and will be next year as well. She is quite the mother to him and I hear they play seperately most of the time which is good too.

Evan also saw the pediatric cardiologisth for a routine exam. His heart size remains large for his body but is not concerning to Dr. Cousineau at this time. We will continue to monitor that. As for the clip in one of his heart valvles (a PDA repair), it looks great and there is no leakage around the clip. His small hole in his bottom chambers is very minimal at this time and can only be detected by hearing his heart murmur.

Baby Connelly is 35+ weeks now and doing well. He/she tried to escape a couple days before our Evan's Enchanted Evening but after a long talk with him/her (and some Magnesium Sulfate), he/she settled down just in time to let me get discharged from the hospital and make it to the fundraiser. The night was a huge success and from what I hear, was enjoyed by all we went. I think we are already looking at a EEE #3! We are prepared to save the cord blood from this little one in case it is a match to Evan and would then prevent Claire from having to give of her marrow. Plus, cord blood is much more potent than bone marrow aspirate. That would only be icing on the cake. We also have to test this one for FA but so far there are no physical signs that he/she should have FA.

Other than that, things are busy but going well. Evan has been tolerating being sick like a champ and that is very helpful. We will continue to plug along and take one day at a time! Again, thank you for your thoughts and concern for Evan. We are truely blessed to have him and to have such supportive friends and family. Kim Connelly

Monday, December 20, 2004 4:07 PM CST

Alright, it has been a while since we updated and I am getting greif from some of you out there. You have to stay on my case. Before you know it, life takes over and some of these really important parts gets kicked to the curbside!

Evan is doing well. We got counts drawn last week and his platelets (which were low two months ago) are now back within normal limits and his white count is low. Again, we have been told and so I am telling all of you, one lab draw does not make for any concern. It is when you see repeat lab draws indicating the same problem. With the bone marrow aspect of Fanconi Anemia, if one part of his blood cells are in need, it will take his body longer to make enough of them to fall back into normal limits. The fact that his body is still doing this is a good sign. He has labs drawn again in beginning of Feb. and we will let you know the results.

Evan has been sick with some intestinal bug, which makes since with a low white count, and so his weight had dropped over the last two months or so. He tops the scales at 22 lbs. and his growth has not gained anything in months. We continue to fill his diet with high calorie foods and hope for the best... and buy him a belt! His physical therapy has been pretty status quo as well. He had an MRI a month ago to rule out a tethered spinal cord which would cause him to tip toe walk like he does. But... his spinal cord is fine. His neurologist says he might be 'wired' to tip toe walk and so now we are determining what is the best route of action. We are keeping his range of motion in his feet flexible and trying to verbally tell him to "walk down on your heels!" It works when we think of telling him but if this isn't helping, we will go back to the botox injections and braces. Evan starts school January 4th and I am so hoping he will not have to wear those braces to school. Already, kids his age call him a 'baby' and try to carrry him around. School will be good for him and since he is almost three and will be leaving the Birth to Three Program which regulates his need for therapy, his school will take their place in doing so. Oh, but I can't believe my baby is starting school. I will let you know how he does, or more importantly, how I do!

His post-op appt. for his thumb surgery went well and we do not have to make the drive to Chicago again until March. At first Dr. Light wasn't sure if Evan would need therapy in that thumb and so he took a look at it again last month after some more time of healing. He asked if Evan ever used his thumb to touch the tip of his pinkie on that hand. I said I never saw him do it. He said that the range of motion to do that would indicate a huge success for the surgery. So we instructed Evan to try it and he did it immediately and said "Look, see, my thumb is all fixed!" And so the need for therapy was gone!

We are still waiting on results from Claire's DNA FA test. I will update when we hear anything. We were told it would take a couple of months so we are trying not to worry. She is growing like a weed and eats us out of our home lately!

Baby Connelly is now 27 weeks along. We had an ultrasound done a month ago and a repeat a week or so ago. The growth of the baby is good and there were NO signs of birth defects to be seen. This is a very good indication that this little one does not have FA, but at birth we will do a blood test to check for sure. Baby Connelly was busy sucking on his/her feet during the ultrasound. When you ask Evan who has a baby in her belly, he will lift up his shirt and show you exactly where his baby is! And if you ask Claire if she wants a baby sister or brother, she will say it can be a girl this time and a boy for Evan next time! We are pretty sure that won't happen!

FYI: Evan's Enchanted Evening second annual is Feb. 5th here in Green Bay. If anyone is interested in supporting this wonderful night with your attendence or a donation, please don't hesitate to contact us so we can help you with that. All proceeds go to finding a cure for FA.

Before updating the website, I ran through all the comments left on this site for Evan and his family. I see that the site has been visited over three thousand times. With it being the holiday season, I find myself so thankful for many things. First of all for my wonderful children. Claire is a beautiful gift to this family who decorates our life everyday with the sweet things she says and does. Evan is an angel from heaven who constantly teaches me how to be strong and brave, and how to live life for the reasons it was given to us. For my wonderful husband who I could never imagine this journey without. And... for all of our friends and family who we are so blessed to share your lives with. I hope you have the merriest of holidays and a new year that is better than all the rest! Kim

Tuesday, October 19, 2004 10:09 AM CDT

I know I just added an entry updating you on the last month or two but I had a moment to tell you how Evan's right thumb surgery went last week. The doctor was able to remove the scar tissue left from the removal of the extra digit, cut the tendon that was keeping Evan from bending his thumb, and than he went into Evan's wrist and used ligaments and tendons to rebuild the structures of his thumb. If all continues to go well, Evan should have complete use of his thumb. He was in a cast that was slung to his chest. Yesterday, we had his post-op appt. where they removed the cast and just put on a splint. The splint has to be worn until the end of this week and then only at night.

Evan did wonderfully with all of this. He never once needed anything for pain and the day after surgery he was doing summersaults in our living room with one hand! We thought he would be agitated with not being able to use his right hand or arm at all being right handed, but it didn't even phase him. It is actually good therapy for his fairly new left thumb.

As far getting that skin sample, it seems for all the work it created for everyone, it may not be usuable. We will see how it grows out in the lab.

We got labs drawn last Monday as well. Everything dropped quite a bit again but we are trying not to worry about it until we see the next set of labs in two months.

Also, last week we got a call from Dr. Auerbach in New York who has been working on mapping out Evan's mutation. There is a doctor in Europe who was collaborating with her and he just called with news that he found it. With nowing exactly where Evan's mutation lies, Evan now is a candidate for stem cell research and gene therapy WHEN it becomes available. Also, we will use this infromation to test against Claire's already saved DNA in their lab to determine if she has FA. That way no skin fibroblast sample is needed for her. This way will take longer to get results but all is stable currently anyway. Busy week, huh?

Take care and we hope all is well with you! Kim

Monday, October 11, 2004 7:34 AM CDT

Today we leave for Evan's last fore-seeable surgery to correct his birth defects. He is having his right thumb fixed by Dr. Light at Loyola in Chicago. They are not quite sure what will be able to be done. Dr. Light wants to try to get the thumb to function normally but if there isn't enough of the right material there, he is at least going to cut the tendons and ligaments to straighten the thumb. That way, when Evan pincher grasp he can do it at the tip fo the thumb instead of at his knuckle like he presently does.

Surgery is tomorrow morning but we are leaving tonight. It is outpatient so we will be home tomorrow evening. Hopefully, he will have the majority of the dressing or cast off by Halloween! We are also grabbing a set of labs this AM at the pediatrician's office to ensure all is fine before surgery. And... there is a research project being done at Dana-Farber/Harvard Cancer Center. They have asked for a skin sample of Evan for their FA study and since he is going to be under anyway, we elected to do so. Evan is such a rare form of FA that we feel obligated to contribute to research. It is just difficult to do that yet not at the expense of Evan. They was an easy decision.

Regarding Claire being considered to have FA, that is still not resolved. Dr. Auerbach, who had been researching our family's blood work in respects to FA, called a couple of weeks ago. There is a doctor in Europe that is pretty sure he has mapped out Evan's mutation. He just wants to clarify his results before giving them back to Dr. Auerbach. If this is the case, we can then molecularly check for Claire having FA against her already stored DNA. This saves her from having the skin fibroblast sample taken, which would be devostating for her and leave a scar on her thigh. Results, unfortunately, will not be gotten any time soon. We are probably talking at least a year. But that is alright since Evan is stable and Claire obviously doesn't present with any signs of FA today.

As far as Evan's tip toe walking is concerned, we are not going to continue the botox injections even though he continues to tip toe walk. We are scheduled to see Dr. Edgars on the 21st of October. We will discuss with him the possibility of doing the MRI on his spine sooner. If you remember, there is a possibility that his spine could be tethered to his sacrum causing two symptoms - tip toe walking and unability to potty train. It seems pointless to continue our regimen as is if there is actually a medical, fixable reason for him to be still tip toe walk. Plus, we have a problem persueing potty training if he can't physically do it because of this problem. We will keep you posted.

Evan is almost three and therefore will not qualify for his Birth to Three therapies soon. He is hardly delayed in any of the areas and the only problem really existing is the tip toe walking. That being said, he will probably not qualify for school assisted therapy. So we are almost certain the plan is to start him at Claire's school in January to at least keep him in a structured environment with lots of learning opportunities. We will have Evan evaluated sometime in spring to see if this plan is working or if he will do some private therapy.

Other than that, all is going well. If you already don't know Evan and Claire will be getting a new brother or sister in March. We opted not to have FA testing done on the baby thus far because of the risk of miscarriage. We will have a level II ultrasound in November where we can look for birth defects and growth issues. If all looks well, we can be half way certain this little one will not have FA. Still, we will have labs drawn off the cord blood at birth to test for FA and if this little one is a HLA match for Evan's bone marrow transplant. Cord blood has a one in two chance of being a match to a sibling since it is so concentrated with stem cells. Claire is a match already, but cord blood is four times a potents and will save Claire from having to donate the marrow when it comes time. The cord blood will be saved free at a cord blood registry since it stands a chance of saving Evan's life and being used during transplant. We are all so excited about the third Connelly baby and almost still in shock that we are expecting after so many miscarriages and infertility challenges. \

Our family is blessed in so many ways... Kim Connelly

Monday, August 23, 2004 8:29 AM CDT

I can not believe it took this long to update everyone on Evan's doings this summer. This might be a long one... Summer has been so busy with fun activities and also alot to keep updated with Evan's care.

As mentioned in the last journal, the labs during the bone marrow biopsy showed Evan had a growth hormone deficiency. We had plans to have a growth hormone stimulation test to be done in Minnesota the beginning of Aug. We also hadn't been to see our local hem./onc. doctor, Dr. Brandt, in two years so we saw him in July. He was pleased with the care Evan was getting and offered to do the hormone stimulating test at his clinic under the Minnesota endocrinologist's order so we wouldn't have to travel. That was done Aug. 10th. The idea is to give Evan two medicines that should stimulate his growth hormone at certain intervals and then to draw about 10 labs depending on when these meds. are given. Evan was so brave while they put in the IV plus there was a child life play specialist there who did a great job keeping him distracted with toys.

All seemed to go smoothly until we found out that the first two labs drawn off the IV were clotting and not usable. From here on out, they had to poke him for the remaining 8 labs. Now, since Evan is a small guy he has always been a poor stick for lab draws. I felt so bad for these nurses who had to repeatly draw Evan's labs. They tried in his feet, hands, and arms with often several attempts before getting blood. They felt awful and I am sure several of them went home to drink after their shift. Still, I felt worse for Evan. It was the worst procedure I have seen him go through since this all began. They had his hands and feet wrapped in warm towels and then in bags. He laid there and just cried poke after poke until he actual slept through a few from exhaustion.

There were several times John and I looked at each other with tearing eyes suggesting to stop it all and go home. Still, we were over half way done and we just couldn't imagine him having to get this far all over again. When it was all done, of course Evan could have asked for any toy or toys in the world and he would have gotten them but instead he wanted to go to Chuckee Cheese! So for dinner that night, we went to Chuckee Cheese and it was like nothing ever happened to him, except for the bruises all over his hands and feet. He is the toughest, bravest person I know - all ages included! We are still waiting for the results of that test and will let you know. Also, they checked a CBC and it is coming up slowly but surely from June.

As far as Evan's tip toe walking has been going, it is going slowly. His range of motion is great but once the shots to reduce his tip toe walking wore off, he was right back to doing it. We repeaded the shots July 26th with a new Neurologist. Again, Evan laid right down on the table for four shots in his calves. He cried until he got to pick out a prise from the treasure chest. On the way home, I said to Evan that Dr. Edgars was a nice doctor to give him a toy. He responded by saying "No, Doctor not nice. Gave Evan shot!" He is too smart. I guess the plan is to see what the results are after these set of shots and to take it from there. Dr. Edgars did say that if Evan is wired to tip toe walk, he may continue to do it when the shots are not in effect. Also, he mentioned the possibility that some children who do not potty train and tip toe walk may have a birth defect were there tip of the spinal cord is tethered to their sacrum. If Evan is not potty trained (which he had not even went potty in his chair once) by 3 1/2, they will do a MRI to check.

Evan also had an occupational review from our birth to three program to evaluate his level of skill in activities of daily living. His hands appeared to be weak in several actvities that made it difficult for him to complete but overall, he actually came out one month ahead! We were so proud. In mid August, we are getting our physical therapist and case superviser together to discuss his plan of care during the next few months. Evan only has until Feb. until he is discharged from birth to three and will then be our responsibility to get him care from the school system or privately.

Then last week we all traveled to Maine to Camp Sunshine. It is a camp run on donations and volunteers that devotes each week of summer to a life threatening childhood disease. Last week, families coping with FA all over the world attended this camp. It was our first year but FA has been doing this for years. During the day, John and I attended talks given by doctors and specialist who are world known for their work with FA. While we were in these talks, Claire and Evan were kept busy with programs run by the sweetest most amazing volunteers I have ever seen. Seeing so many people give of their time and talents for no purpose but to help us has completely renewed my faith in the goodness of strangers once again. At night, there was a talent show, a costume party, and even a night for John and I to enjoy a nice dinner with other parents and adult FA individuals. We left completely exhausted with information overload and a long list of follow ups and new appts. I need to make.

So, this week I am talking with Dr. Auerbach who is working on mapping out Evan's mutation of D2 (which we found out at camp is VERY rare and of extreme interest to every researcher known to FA) and also to see if she has determined if Evan is mosaic at all. We are determining if Evan should be in a study at the National Institute of Caner working with children with bone marrow failure diseases. I have made appts. for Evan to follow up with our local Ears, Nose, and Throat doc to check on tube placement and hearing, with the local cardiologist to have his annual EKG, with the dentist for his 6 month check and cleaning, and an appt. to see our pediatrician for weight checks. We are also seeing Dr. Light Sept. 13th to set a date for Evan's last hand surgery - hopefully. At the time of surgery, we will grab his every two month CBC and also a blood sample and skin sample to be sent to another genetist from Harvard working on D2. We are also writing an email to Dr. Wagner, our bone marrow transplant doctor, to discuss with him information we recieved at camp that suggest a change in our annual care for Evan.

Of extreme worry to us, one of the doctors at camp suggested to have Claire rechecked with a skin fibroblast sample for FA. She said that blood samples testing for FA may not show truthfully if the child has FA if that child is highly mosaic (or the stem cells of the blood have found a way to fix themselves but the tissue and organs still remain affected with FA). She pointed out a few features on Claire that are typical of FA children and said that since she is Evan match for bone marrow transplant, it would be tragic to transplant Evan with a FA child herself. John and I can't seem to get this out of our heads. If Claire would be affected with FA, that would be awful in itself but then Evan would loose his match which would be awful too. So I am setting up an appt. for a skin fibroblast sample to be taken from Claire with our local genetic clinic.

Also, this September our Katie Beckett for Evan comes up for renewal. This is a government funded program that helps support the medical expenses we incur. It is ton of paper work and a home visit from a social worker but I don't know what we would ever do without it. I would probably have to go back to work.

Yes, we have been busy. But during this all, we have visited just about every park in a 20 mile radius, spent two weeks on vacation with our families, went camping, and many day trips to great places. It has been a fun summer but it is always apparent to us that we have a huge responsibility to our family medically. And a responsibility to fundraise for research. We are having a meeting in September with our wonderful volunteers of friends and family to discuss what we will do in the next year.

Again, I am sorry this is so long but I really need to up date it more often. I promise. We hope your families are all happy and healthy and getting ready for the upcoming school year. Take care, Kim

Sunday, June 27, 2004 3:20 PM CDT

Hello to everyone. It is Sunday and we just spent the last 5 days on vacation in Green Lake, WI with John's family. We had a wonderful time and the kids played until their little bodies just collapsed in their beds/cribs. We were celebrating John's mom's 70th birthday, but called to get Evan's results while we were up there and had one more reason to celebrate. In his bone marrow, there were no signs of early luekemia cells or cancers cells. His cellularity last year was 50% (it should be 100 minus your age - roughly) and this year it was 60%! I guess the number can change slightly depending on where the sample is taken. So we can safely say his bone marrow has at least stayed the same.

His growth hormone was reported to us that it was normal the day we left Minn. but when we got home today, the endocrinologist left a message saying his growth hormone is low and she wants us to come back to do some more tests. Always something... I will call her tomorrow. Also, we will redraw Evan's labs tomorrow so I will update this site later in the week.

Overall, we are glad the bone marrow results are good. As always, thank you for keeping Evan's in your thoughts and prayers. I don't know what we would do without our family and friends. Kim

Thursday, June 17, 2004 9:19 PM CDT

Well, it is 9:30pm on June 17th and we just got home from Evan's bone marrow biopsy and aspiration at Fairview Hospital in Minnesota. He saw the endocrinologist at 8am, Dr. Wagner (his hem./onc. doctor, the one who will do Evan's bone marrow transplant and a specialist in FA)at 9am and then Evan had his bone marrow biopsy at noon. He did well considering he is not very fond of hopsitals, doctors, and especially nurses (excluding present company!). While he was under, they drew all labs needed by the endocrinologist and Dr. Wagner so he wouldn't have to be awake for it - which was super nice. Since he gets labs done every two months, we try make the experience as nice as possible since we know he just has to come back and do it all over in another two months.

Evan cried from the time he woke up in recovery until they sent us home. He went from crying to once his little cheeks hit his carseat to asking for a cookie and Blues Clues on the VCR! Little stinker!

We will get results back from the biopsy next week. We did get some of his blood work back before we left. His TSH, free T4, and growth hormone are all normal. Here is the not so good news. His CBC, which has been within normal limits since he was born and even stable the last 4 months, was drastically down. His WBC went from 8.4 to 3.8, RBC went from 4.13 to 3.78, ANC was 500ish and platelets from 256 to 116 - all under normal limits, especially his WBC and platelet counts. Evan had a fever of 102 a couple of days ago that lasted for two days. We are hoping that this was a viral infection that drained his counts while fighting the viral infection. We are instructed to repeat the CBC in one to two weeks and wait for the results of the biopsy because they will tell us even more about what is going on.

I guess John and I always assumed Evan had some time before worrying about going to transplant. Tonight, we have no certainty of that at all. At Evan's age, transplant would be so difficult because he understands so little of what is going on. He is so brave and strong. Whatever happens, we will be there for him and Claire no matter what and when. I will keep you posted. Thank you for keeping Evan in your thoughts and prayers. Kim

Saturday, May 22, 2004 3:15 PM CDT

Evan got his braces on Thursday and the plan is to wear them whenever he is up running around for the first two weeks. He wore them all day Thursdsay, Friday and so far today too. He is not bothered by the braces much and it really prevents him coming up on his toes. We will evaluate in two weeks and see where to go from there. It would be nice to have this whole issue resolved once and for all. Other than that, all is still going great. We are schedule for the bone marrow biopsy and aspiration June 17th so please keep Evan in your thoughts that day. Take care, Kim

Tuesday, May 18, 2004 2:46 PM CDT

I thought I would update everyone on Evan's treatment for the tip toe walking. About a week and a half ago, we went in to get the injections in Evan's calves. This treatment is very common in CP children where there tone is adhanced and their muscle are tight. We discovered that Evan will only need the set of injections once and they should last 4-6 months. After that, we will see how well they are working and decide if they need to be repeated at that time. Already, Evan is walking flat footed 95% of the time. We had him fitted for braces about three weeks ago and will get them this Thursday. The braces will only reinforce the correct posture in walking heal to toe and continue to keep his calf muscle from getting litte use. The only obstacle might be Evan's tolerance to the braces. We got to pick out a design for them with is puppies playing baseball. Hopefully, he views them as fun and not therapy!

Evan has been battling this cold/sinus infection for weeks. He just finished a round of antibiotics about two weeks ago and still, he seemed like he wasn't getting over it. So the day we were to leave for the Wilderness Waterparks, we brought him back in to find from a chest X-ray that he had some pneumonia in his right lung. They put him on a hefty antibiotic that seems to have kicked this bug once a for all.

We also had a meeting last week with his speech therapist, physical therapist and their supervisor to determine how to continue with Evan's therapy for the next three months. I knew this was coming but we discontinued speech altogether since Evan seems to be surely at his age level if not ahead at this point. As for physical therapy, we are going to increase it to every week for awhile to maximize whatever benefits we can from those shots he had in his calves. Overall, everyone was pleased with Evan's progress and Mom couldn't have been any prouder.

Again, life seems to be on cruise control lately and we are just taking it for every day we can have it. Take care and thanks for keeping Evan in your thoughts. Kim

Friday, April 23, 2004 8:05 AM CDT

We got results back yesterday from Evan's every two month blood draw. Would you believe his platelets and white count are up again? His red count was down though this time. Overall, it was all good and still within normal range so no special precautions. He will have his next blood draw in June when he has his annual bone marrow aspiration and biopsy at Fairview Hospital in Minnesota. He will also have a complete endocrine workup and an EKG at that time.

Other than that, Evan has been a very healthy and happy two year old. He is on antibiotics at the moment for a sinus infection, but to be fair, it went through our entire house. His speech therapy is going wonderfully and he is now saying 5 word sentences. It is always so amazing to here our children begin to talk because it gives us a window into their minds. The other day in the car, Evan says to me "Momma, birthday party tonight. Blow out candles!"

Physical therapy has been a bit more stressful. His range of motion in his legs and feet are wonderful, still Evan continues to tip toe walk. We saw a neurologist a couple of weeks ago. Between her, our PT, and ourselves, we have decided to treat Evan with small injections into his calves that numb his over-toned and neuro-sensitive calf muscles. He will then be put in either removable casts or braces that will help to retrain his brain how to walk toe to heel. Numbing the muscles removes the hugest obstacle to him learning how to walk this way now. We hope it will only take a few weeks to do this.

Other than that, his weight just broke 20 lbs and his height is still 31 in. He is just beginning to wear 18 month clothes. But like I tell everyone, if you spend 5 minutes with him, there is nothing small about him! He begs to go to school with Claire and repeats everything she says. They are beginning to play little games together where Claire pretty much makes up the rules and he doesn't follow them. But still, it is cute. One day, we put up his big boy bed next to his crib and that night he just went to sleep in it probably thinking "...so what is the big deal."

Life is great and we are just enjoying the normalcy of everyday life. It feels good. Kim

Wednesday, March 3, 2004 2:02 PM CST

Hello to everyone and thank you for checking in on Evan. He is now TWO years old this past Feb. 21st. He is doing very well. At his check up he was 19lbs. 8oz (still gaining, slowly but surely) and 31 in. tall. He is a peanut but his personality seems to make up for it.

Evan had his three month eval. for speech therapy and he went from being 6 months behind to one month behind in only three months. His therapist said he might need a little help in the introduction of things but he is a very smart boy. Physical therapy is going the same. Evan still tiptoe walks and the therapist really doesn't know why he still does it so he is getting refered to a pediatric neurologist. We will keep you posted.

Evan also had labs drawn and everything looked fine. He usually drops a couple points every 2 months but this one looked much like that of two months ago. Yeh! Evan will have labs drawn again in April and is scheduled to have his bone marrow biopsy and aspiration in June at FairView in Minnesota.

It is so good to write so positive. We are so thankful to be blessed with wonderful family and friends. Without your prayers and thoughts, I truely believe Evan wouldn't be doing as good as he is. Thank you from JOhn and myself. I hope you and your family are happy and healthy. Kim

Monday, February 9, 2004 10:05 AM CST

Evan had his annual check up with his cardiologist last Tuesday and got a big thumbs up. We don't have to see him until next year! They did an echocardiogram of his heart to check that the clip was still in place and that his PFO was reducing. This was done first when Evan was 6 months old and for each time we had to sedate him since. This time... a Blue's Clues video and Evan didn't move! Amazing. Evan also had a checkup with his ENT (Ears, Nose, and Throat) doctor. All seemed fine. His tubes are starting to slide out but Dr. Block thought he would at least have them for the remainder of the winter. We will do another hearing check in 6 months as well.

Therapies have been going well. PT is now down to every other week. His coordiation issues are gone but he still tiptoe walks. He is all stretched out so that isn't the problem. The therapist, Karen, thinks he might do it because it is a neurological problem rather than tight muscles. We see Evan's pediatrician in two weeks and she wants to assess him and make a referral for either a ped. neurologist or an orthopedic. ST is going wonderful. Evan can mimic just about any word. Yesterday, he said upside-down! Now we are just working on getting him to use those words instead of whining and grunting! Both therapist are very pleased with attention span for doing things like puzzles, games, and reading books.

He has labs drawn in two weeks and he will get his vitals done then as well. We weigh him in ST and last he was 19lbs, 5oz! Still gaining. Height was 31 1/2in.

The lab in New York has been trying to find Evan's 'markers' for his FA type - D2. Knowing this, Evan could be helped with gene therapy in the future and other experimental research. We got a call last week that they have exhausted every avenue and just can't find it. They are still working on it but don't have any new ideas as to what to do next.

I will update when we get labs done. We hope all is well with your family. We thank God everyday for Evan's good health and enjoy him and Claire so very much. Kim

Tuesday, December 16, 2003 8:03 PM CST

Good news! Evan had his re-evaluation for PT today. He was 6-7 months behind when he started therapy again in September. Now... he is only 1-2 months behind and gaining fast. ST has been going well too. Evan seems to pick up on therapy so fast. They are good people.

Last Thursday Evan had a CBC check, weight check, and a flu shot. His CBC looked good but the white count (controls your immune and ability to fight infections/virals) took a huge nose dive. But you have to look at the labs over a long period of time and not compare one to its previous counts. Evan has been steadily dropping but that was to be expected. So far, no precautions have to be taken. His weight was #19lbs!!!!! He finally broke 19. We have been playing around with his supplemental drinks and finally found one he likes - Ensure Plus. It must be working. And, he gained an inch in one month. A couple of weeks ago he was very sleepy and falling alot, now I know why. So, things are good.

Thank you for your support for Evan. It means alot. Kim

Tuesday, December 2, 2003 10:07 PM CST

Evan started speech therapy last week. He is approx. 3 months behind but has a history of catching up quickly with the help of therapy. For those of you from my home town, Maria Aerts is our therapist. She is amazing already!

It has been a tough week for Evan. He seems to be falling alot lately and over the weekend split his lip open twice. He used to do this prior to starting physical therapy but then stopped after they began to work with him. He does have a cold but such a small one I can't believe it would affect his equalibrium. Plus, he has been sleeping a lot lately. He will go down for bed from 7pm to 7am and then nap 3 - 3 1/2hours in the afternoon. I called his ped. Dr. Hoyer and we decided to keep an eye on him for a day or two since he has CBC next Thursday. We will always worry. We will keep you posted. Kim

Thursday, November 13, 2003 1:55 PM CST

John and I are just starting a web page for Evan to update friends, family, and other concerned individuals about Evan's fight with Fanconi Anemia. As of today, Evan has just sailed through h is 6th surgery. It was to remove his left thumb which didn't have any bone in it and move his adjacent finger into that thumb position. Surgery went fine but Evan hates hospitals so the stay was rough. We were told not to expect movement for 3 - 4 months but after 1 month, he was refusing to wear the splint and having complete range of motion in that thumb. Dr. Light, the surgeon, could not believe his eyes when he saw Evan at that one month appointment. He said it was the best results he had ever seen! Other than that, our trip to Cinncinati in July was uneventful. Every specialist said Evan looked great for a Fanconi Anemia child and to keep doing what we have been doing. Evan is still small, 30in. and 18lbs, 11oz as of this past week. We try to maximize the caloric intake of each bite he takes but overall, he really has a great appetite just a fast metabolism to go with it. He gets physical therapy for a coordination issue in his trunk that causes him to fall often and for tip-toe walking. He is improving on both issues every week. Evan also is starting speech therapy next week. He says several words but there is concern he is behind so since he applies for therapy, he will get it until he catches up. The plan is to do blood counts every 2 months and a bone marrow biopsy yearly to catch the bone marrow failure early. With Claire as a perfect match, we are hoping it will go smoothly when the time comes. His bone marrow presently is working at 50% compacity but compensating well since his counts are only slightly anemic. Other than that, Evan has a zest for life that I have never seen in another human being before. He is smart and very compationate with others. He is a complete flirt and loves to be busy all the time. Claire and him get along wonderfully most of the time! We are busy planning a fundraiser March 6th with all proceeds to go to research of Fanconi anemia. But amoungst our busy lives, we will try to keep this website current with Evan's progress and medical status. We appreciate your concern and suport without we would never make it through. Kim and John Connelly

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