Cousin Thomas (and Aunt Terrie and Uncle Tom) Comes to visit

From October 6th-10th we had visitors! We were so happy to have the Wnukowski family come visit and to get the boys together. Thomas and Evan seemed to have a lot of fun together (makes me wish we were closer).  We got to do a lot of fun things while our visitors were here. We went to the zoo, the pumpkin patch, the balloon fiesta (twice), and the KAFB Air Show.  Here are a few pictures from our visit.

Face off!

First Halloween costumes!

  Evan and Aunt Terrie

The Pumpkin Patch

Balloon Fiesta 2011

Air Show...Sitting Under a B52 Bomber

Waiting....

We had an appoitment last Monday with Evan's geneticist, Dr. H.  He said Evan looks fantastic, but his numbers are still high. We are decreasing his protein intake even more.  He is doing well, though.  We also asked Dr. H to contact Stanford for us and to get the referral going for a liver transplant pre-evaulation. He emailed yesterday and said he contacted Stanford and we may be getting a call from their transplant coordinator.  We are nervous and excited all at the same time! We are sure this is the way we want to go, but don't really know what is going to happen. And we know the waiting is going to be difficult once Evan is placed on the list. Either way...we feel this is right for us and for Evan!

Nana and Papou come to visit

As promised, here is a post about Dad and Kristy coming to visit.

This was taken at the zoo before we headed home.

We didn't do too much while Dad and Kristy were here as Evan was teething (and of course later we found out his levels were off) and was super crabby.  Dad and Kristy were completely fine with that because all they cared was that they got to spend time with Evan.  We did get to take an outing to the zoo which was a lot of fun! Evan really seemed to enjoy watching the monkeys and was definitely more aware of the animals than he has been on past trips to the zoo.


Although Dad and Kristy's visit was short, it was very nice that we had them here and got to spend some time together!

Here are a few pictures from their visit

Nana wanted to sit in the big chair and take a picture with Evan

This was when Papou and Nana first arrived.
 Can you tell Evan is happy to see them?

Papou wanted to be the only one to push the stroller



Papou and Evan couldn't get enough of eachother!

Frustrated!

As usual...life has taken over, so I am sorry I haven't posted for a while. 

My dad and stepmom came to visit last week, but I will save that for another post.

Right now I need to take a moment to let out my frustrations.

It has been 6 months since Evan was born, which also means 6 months of weighing his formula every day, weekly blood tests, weekly weight checks, constant diet changes, worries, and difficult feedings.  Of course there are also good days that are all happy and smiles, but right now I am frustrated.  Is it selfish of me to wish my child were "normal" and didn't have to have these tests every week? Is it selfish of me to wish that I didn't have to worry every time my child didn't want to eat a whole bottle or was extra fussy? I know it could be worse...especially since a former colleague of mine just lost his 16 month old son to leukemia.  That is definitely worse.

What brings on my frustrations right now, you ask? Well...again...Evan's levels are elevated. His numbers are 800. What is most frustrating is that last Monday he was tested...we didn't get results until SATURDAY...5 days later! His numbers were elevated to 500 and the doctor didn't recommend a diet change. I should have gone with my gut and cut Evan off of protein for a day.  Of course I am not the professional so I did as I was told and continued to feed him his protein and MSUD formulas. Then he gets tested this Monday, we get the reults today and he is now at 800...or was on Monday I should say.  The Dr. recommended we lower the amount of leucine we give him by cutting some of his formula.  I feel like it's not enough. I feel like his numbers have obviously been going up for 2 weeks now. Maybe 24 hours without protein would be better. We've done it before.  I just don't understand how the decisions are made! I will definitely be asking for further clarification come Monday when we have our genetics appointment.

It's also frustrating because as a mother you want to protect your child from everything and I am doing my absolute best yet my son still ends up with elevated numbers.  What more can I do? I barely leave the house unless I have to. I fight him to make sure he finishes his bottles so that he stays healthy. I love him and play with him and try to teach him new things every day. But still...it feels like nothing is enough.  It feels like no matter how hard I try he just needs more.  Today was hard. Evan cried...a lot! I thought it was his teeth, seeing as he has two of them coming in.  Then I get the email telling me we need to do a diet change because his numbers are elevated.  Now I don't know...was he screaming because of his teeth or because his leucine levels are too high? Was he fighting the bottle because it hurt his teeth or because his numbers are high?

I HATE MSUD! Yes..HATE! It sucks...it's completely unpredictable and so scary! We are talking to the geneticist on Monday about getting a referral to meet with the transplant team in California. Hopefully we can meet with them soon and get the ball rolling on a "cure" for Evan's MSUD. (At least that's what people in the MSUD transplant community call it). 

If you made it through this post...thank you! Please say an extra prayer tonight for us that Evan's levels drop quickly! I want my happy baby back! :(  And please, say a prayer for angel Micah who was taken from us too soon! I think of him and remind myself that no matter what struggles I am facing with Evan, we are still blessed to have him here. Things can always be worse...even when it doesn't feel like it.

Home Sweet Home!

Just wanted to let you all know we are home. No one really knows why Evan wasn't eating. It could have been a small virus or he could just not like his MSUD formula. We have started mixing it a bit differently and he is doing better, so I am thinking it's the latter of the two listed possibilities. He could also just be a stubborn kid! Either way, we avoided metabolic crisis by going to the hospital and having Evan put on a feeding tube. It allowed him to get enough nutrition to make sure his body didn't have a negative reaction!

Right now, we are just glad to be home and back to "normal"...whatever that means! LOL

Why won't you just eat?

As some of you know, Evan is back in the hospital. For some odd reason the kid just doesn't want to eat! For most kids, not eating as much isn't such a big deal, but for a kid with MSUD it can be an indication of illness, metabolic crisis, or something even more serious.  Or...it could just be a child acting stubborn!

Here's what is going on with us, around Wednesday August 24, Evan became very fussy with eating. He didn't want to finish bottles and went from 5 ounces to fighting after 3 ounces. We were still able to get him to finish his bottles, but he wasn't eating normally at all. By Thursday night, he refused to take more than two ounces at any given time. Very strange for him.  He was still behaving normal at all other times of the day, but when it came to feedings, he was NOT having it! Fast forward to Friday morning...Evan awoke as usual for his 4 am feeding. He took 3 ounces from his bottle without problem. I stopped, burped him, changed his diaper and went to give him the bottle again. At that point, he vomitted what looked like the entire 3 ounces he had just eaten. For those of you who do not know, vomitting is the nemesis of MSUD. I couldn't take it anymore so I woke up JR and told him that I felt we needed to go to the hospital to have labs drawn and to have the doctors observe him.

We got to the hospital around 5 am, Friday morning.  In the ER they drew labs and Evan's geneticists came in to observe him. Based on his behavior they were pretty sure it wasn't his MSUD acting up, but there really isn't a way to tell until his levels come back...which took until SUNDAY! So frustrating! Evan did not want to eat more than an ounce every few hours if that while in the ER, so they started an IV of D10 which is basically fluids with a high level of sugar to give him calories.  They admitted him and several hours later we were finally up on the general peds unit...and here we still sit!

Evan's levels have all come back normal, so now it's just getting him to eat.  A feeding tube was put in Saturday night and was run on continuous feeds until the next day. On Sunday we went to bottle then bolus feeds to mimic the normal eating pattern during the day and a continuos feed at night. Evan is starting to eat more by bottle than by tube which is a step in the right direction. Hopefully he continues to move in the right direction.

We still have a lot of questions...many of which no one has an answer for. Of course the biggest question is why won't he eat? We may never know and that is soooo frustrating!




Here are a few pictures of Evan during his current stay at the hospital.  

Evan LOVED playing with the activity gym we were able to borrow from the Child Life program.  He would bat at the toys and kick and laugh. (Basically the happiest "sick" kid ever! LOL)



Our friend's Jen and Travis came to bring us lunch and visit with Evan.  Travis had not met Evan as he just returned from a 6 month deployment to Afghanistan. Evan took to him right away and LOVED when Travis put his hat on his head! We are truly blessed to have great friends here! :)



Feeding tube in and still laughing and smiling! :) We are lucky to have such a happy boy!





Cereal!

Evan tried cereal for the first time today!!! He did great! Opened his mouth for the spoon, but of course spit quite a bit of it out. I don't think it's that he didn't like it. I just don't think he knew how to keep it in his mouth and swallow it.  He definitely seemed to enjoy the experience as did mommy and daddy. We will be doing this each night for the next couple weeks until we get the hang of it and are ready to move on to veggies!

So you all understand with Evan's MSUD, adding new foods can be scary! I kept putting off giving him food because I am so concerned about monitoring his protein intake. With close monitoring and discussions with his dietician, we will do great! He is eating "normal" rice Gerber rice cereal. It has 1.1 grams of protein in 1/4 of a cup. Since I am only making 1 Tbsp. right now mixed with his MSUD formula, there really isn't much protein being added to his diet from the cereal.  I have to write down what he eats (just like I have been) and we will continue to have weekly blood tests, but right now, it's more of a learning stage for all of us. Once he is eating enough for the food to be .5 g or more of protein and is eating more during the day we will start to decrease his protein formula and instead he will get his protein from his baby foods. 

We take each new experience and challenge as it comes and just continue to monitor and pray that Evan's protein levels stay where they should!