"Wash Out"

Evan had his surgery today to drain the hematoma exiting near his liver. The hematoma contained mostly blood and fluid. The docs did a liver biopsy (pretty standard post transplant) and a washout to clear out any possible lingering infection. We still don't have any cultures back from the fluid, but the docs don't think it was concerning. Hopefully this procedure will have cleared out whatever is causing Evan's fevers and he has no more so we can get out of the hospital soon. The surgeons said that Evan's liver is working beautifully! We are so pleased to know that! The next couple days will be telling as to whether or not the hematoma or a possible bug in Evan's belly were the cause of his fevers. Evan is doing well and resting (thanks in part to some pain meds).

While Evan was in surgery, JR and I were able to walk the campus a bit. Stanford has a beautiful campus and the church in the center if campus is absolutely stunning! It was nice to get some fresh air and a bit of a refresher to get ready to tackle these next days if recovery. All is continuing to go well. This was just a hiccup in the road to full recovery.

UPDATE

I know many of you have probably been checking daily and waiting for the next update. Things have been busy (as to be expected).

Evan was officially transplanted on March 18th, just one day before his 1st birthday. The days that have followed have been filled with many emotions. Evan's recovery started out very well. He was out of the PICU in 3 days (Wednesday March 21st is when he was moved) and the nurses on the regular floor (3 South) were so excited that he was back up so quickly! Just two days later on March 23rd, his bile bags were removed. Evan was still doing very well. He became more and more active each day and was responding well to his medications. His liver functions were normalizing as the docs wanted. Everyone was very pleased with his progress.

Unfortunately, the luck seemed to stop there. On March 24, Evan spiked a fever of 101.5. The docs immediately ran blood and urine cultures and started him on antibiotics. His blood cultures grew quickly and they believed there to be a contamanent on his central line. They decided it would be best to remove his central line and did so the next day, March 25th. Evan spent all of Monday without a fever. Unfortunately, by Tuesday evening his  fever was back and full force at 102.5. Another set of cultures were taken and a CT and Chest Xray were ordered.

This afternoon of Wednesday, March 28th, Evan received a chest and abdominal CT as well as a PICC line insertion. Not long after the that was done,  the docs were certain they knew the cause of Evan's fevers....there is a hematoma next to the cut portion of his liver. They need to go in tomorrow to drain them. This means that Evan will once again be sedated, intubated, and have his abdomen cut open. It sounds so simple being written out, but it's really not. It's scary to know that once again Evan will be going into major surgery. All I can do at this point is pray and ask that you all do the same. Pray that Evan is strong and can handle this surgery. Pray that this hematoma is the cause of Evan's fevers and that once drained we will not experience any more hiccups. Pray that the Lord guide the hands of the surgeons and watch over everyone in the operating room.

I can't say that I didn't expect hiccups...I did. Having talked to many parents who have been through transplant for MSUD, I know that there are many different challenges that we can face...especially at this point of transplant. However, I also know that parents who have faced even  bigger challenges with the liver transplant still would tell me that I am making the right decision. I know families who almost lost their children during recovery and faced many hiccups on the road to recovery. The same families have told me that despite the difficult recovery, they would still choose transplant over MSUD.

Regardless of expecting hiccups, I can't say I am not scared. I am extremely scared, but I also know that the Lord is here with us and will guide us through this as he has before.

On that note, I am going to head off to get some sleep. I need to be back at the hospital early tomorrow. (Tonight is my night to rest at Ronald McDonald House). I'll update again when I can.

"The Call"

At around 2:00 yesterday afternoon we received the call we have been waiting for. I was told that the surgical team had accepted a liver for Evan and that they wanted us to come soon even though the surgery wouldn't be right away. They were worried that there was potential for bad weather that would make it harder to get into Palo Alto.

The flight over was uneventful. Evan had a hard time on the flight. It didn't seem he understood what was going on and was upset until the flight nurse switched seats with me so I could sit next to him. Then he was calm for the remainder of the flight. We flew in to Moffett Air Field and were met buy an ambulance which took us to the hospital. Evan didn't seem to mind the ambulance ride too much.

Once we arrived at the hospital, the craziness began! We were quickly brought up to the floor where the nurses checked all Evan's vitals, started his IVs, and just basically poked and prodded for a bit. We were told that Evan was scheduled to go into surgery at 7am, although anything could change. He was not allowed to eat after midnight, but they put him on his D10 IV and gave him lipids (fat) to keep his calories and fluid up. The night was difficult, Evan couldn't sleep and of course that meant we didn't sleep much either. Come 6:00 am we were wondering why we hadn't heard from the nurse or the docs about when we would be taking Evan down for surgery. By 6:30, we found out why. There was a delay in harvesting the organs and the surgery had been pushed back. No one could tell us what time, as the donor organs were still not harvested.

At this point, we are still waiting. The surgical team feels confident that this liver will be good for Evan. All they could tell us is that the donor is in the 5-10 year old age range. This most likely means the liver will need to be cut a bit to fit into Evan. Of course with this, there comes added risk for infection and bile leaks (all which are treatable).

This is not how I pictured this would go. I pictured we would get here and within a few hours Evan would be in surgery while we anxiously paced the halls.  Instead, we anxiously wait in a tiny and noisy hospital room with no clue as to when his surgery will be. It could still be tonight or it could be in the morning. All depends on the donor and what is taking place on that end. The team here is ready...so are we!

Of course, it is difficult not to think of the sacrifice the family of the donor is making. They have lost a child, yet they choose to make an unselfish decision and share his/her organs so that children like Evan can grow and hopefully have a wonderful healthy life. I cannot imagine the grief and pain this family must be feeling. I pray that God can give them peace in knowing that their child did not die for nothing. That in their child's death there is healing for other children.  I am sure they have feelings on anger, sadness, and just plain frustration wondering why they are losing their child.  I wish that I could give them an answer. I wish I could hug them and tell them that out of this death comes new life. Would that even console them? I don't know. We were told that after the transplant at some point we could send a letter to the donor family through the organ procurement program. They could then decide if they wanted to meet us. What would that be like? Would they even want to meet us? Would we even write the letter? It's something we will think about and most likely will do down the road when Evan is recovered and doing well. I have seen videos of donor families meeting recipient families and they are quite emotional. I don't think we will be ready for that kind of emotion for a while as we are still going through our own emotional roller coaster...and I am sure the donor family is too. 

Please pray that this truly is Evan's time for a new liver. Although everything seems to be in order, there is still always a chance that the liver arrives here and the transplant team decides it's not the right liver for Evan. We have faith that this is his time and that God has a plan for us, Evan, and the donor family. 



I will leave you with this, "The measure of a life, after all, is not its duration, but its donation"


The donation this family is making is immeasurable to us! It is the greatest gift that we will ever know! 

The Right Words - Rare Disease Day

Today a friend posted on his Facebook about MSUD and I loved the way that he had explained all the details of living with MSUD on a daily basis. I got permission to use most of his words (but adjust as needed for Evan's specifics) to share with you what we do each day to make sure Evan stays healthy.

Since today is 'Rare Disease Day', I thought I'd explain Evan's metabolic condition in case there were people who didn't fully get it. Evan has MSUD.

MSUD is Maple Syrup Urine Disease. It's a biochemical disease and it means that Evan is missing a family of enzymes in his liver that break down 3 specific amino acids.

When you eat protein it breaks down into 20 amino acids and your body uses them to grow, build muscle/tissue etc. Each day your body only needs a certain amount of each amino acid and the rest is overage and this overage is very toxic to you so enzymes blast the overage into smaller safe bits. For Evan, 3 of the 20 amino acids cannot be blasted so they collect in his body very quickly and 1 of the 3 is highly toxic to the brain and will cause brain damage, coma or worse. So Grayson cannot eat meat, fish, eggs, diary, soy, tofu, grains, nuts or anything normally thought of as rich in protein.

So what does he eat? Well he has a formula, just like all kids, but his has quite different in composition. This medical formula gives him all the fats, vitamins, minerals etc but instead of also having protein, it has the 17 amino acids in powder form that he can process and is without the 3 he can't break down. He still needs to get those missing three aminos, as the body needs them every day, but we do math and use a scale to determine how much real food to give him so that just enough leucine is in his body, with no overage and no underage (if that's a word?). He mainly eats fruits and vegetables. He does also eat some pastas, rice cereal, and risotto. Gerber baby food company is wonderful and has been able to share with us the amount of leucine found in most of their foods so we are able to give Evan more than we otherwise would.  It's a very delicate balance as we must expose Evan just an exact amount of protein each day...to the decimal. Right now he gets 63mg of leucine (leucine is the problem amino acid) per kilo of his body weight. This week he is 9.45kg so that means he gets 63 x 9.45 = 595.35mg of leucine per 24 hours. On average a gram of protein has 68mg of leucine so that means he can have 418.4/68=8.755 grams of protein per 24 hours. To give context, a measuring cup of skim milk has 9g of protein and Evan can only have 8.755g/24 hours. (I learned through my friends that this is more than some children. Grayson, the original poster's son, only gets 40 g/kilo. which is less than 7 grams of protein per day for him) Evan gets his blood checked weekly at a local lab and his blood is sent out of state because to be analyzed.  There is no machine here in NM that can analyze his blood, so we often wait almost an entire week to get the results. (Many of the other families we talk with get their results same day or within 48 hours). He has a dedicated dietician at UNM who reviews he protein intake every week and makes adjustments and he has a dedicated geneticist. He cannot eat a gram more of less of his required intake or he can go toxic.

Food isn't only one way the body gets protein. The other way protein is introduced into the body is if the body metabolizes its own resources (which is how you lose weight). This is called çatabolism. Your body goes catabolic when you don't have enough of a specific resource it needs (fat/sugar/protein etc) and it eats its own reserves. Since muscle is protein (just like a chicken breast is a great protein source or a steak), if Evan goes catabolic it can release uncontrolled amounts of protein in to his body and therefore increase his leucine levels. The body also goes catabolic when there is a stressor like a cold, or teething or an injury etc. Also, blood contains whole proteins in it. Two nights ago my friend's little boy, Grayson, was standing and then fell and hit his lip on the drawer and cut his gums. Since he would be swallowing his own blood and that whole protein would break down into amino acids in the stomach, the Dr's had them decrease his protein intake by 40% for 12 hours just to be safe. That was something I had never considered in regards to Evan.

So it's the tightest of tightropes. He can't have anymore protein in his diet that what his body needs for that day, but if he eats any less, the body will simply go catabolic to get at it. This means that each day we measure and calculate and hope that we got it right.

This is a little bit of insight into the inner workings of our day to day with Evan. In addition to his MSUD, he also suffers severe reflux issues and will vomit sometimes once a day. It's a struggle, but we have been lucky so far that his levels have stayed normal for most of his 11 months of life.  We are also lucky that liver transplant is an option for us. We have learned that many of our friends both in the US and outside the US have difficulties in either getting their doctors on board, getting insurance approval, or even in their country's belief in using liver transplant for MSUD treatment.  At this point we are still waiting for Evan's liver transplant so that we can begin our "new normal".  We are hopeful that we will get the call soon. 


Today is Rare Disease Day~ The disease may be rare, but hope should not be! Hope, it's in our genes! 

Support

Every time I start to feel like things are getting too hard something or someone does something to pull me back and remind me that we are going to get through this. Sometimes it's another parent of a child with MSUD who is looking for help or who is there to listen. Sometimes it's my husband or a friend telling me I do a great job with Evan and how impressed they are with my ability to stay strong through all of our struggles. Sometimes it's just someone asking me how we are doing and how Evan is doing. Not just asking, but genuinely caring and wanting to know. Somehow God knows just when to bring those people or those moments so that I can regain my strength and keep doing what I do for our little guy!

Evan is doing great! He makes my job as mom so much easier! He is generally a happy boy. He smiles a lot and babbles all day long! He is starting to take his first steps and it's amazing to watch him learn new things each day. He is a very active boy as most boys are. He has been eating well lately, which is a pleasant change from the way things have been before.  He sleeps well too, which is great for us as it helps to make each day a little easier.  Everything is easier with a good night's sleep!

For those of you wondering about the transplant, we are still waiting. Evan is staying healthy, which is wonderful and makes it easier. Regardless, I still spend each day worrying and wondering about when we will get the call. I just remind myself that it will come when the time is right. In the meantime, thank you all for the continued support. Our faith, family, and friends will carry us through this!

Rare Metabolic Disorder Awareness ~ Glutaric Aciduria -1

In honor of Rare Disease Day, February 29, 2012, I am going to be posting some of the things I have learned about different metabolic and rare diseases. Most of these things I have learned by talking to other parents of children with rare diseases or reading about them on some of the sites I have been to.

The first rare disease I will showcase is Glutaric Aciduria

GA1 effects about 1 in 40,000 children. It is a rare metabolic disorder, much like MSUD, in which the body cannot breakdown amino acids found in protein. In GA1, these amino acids are lycine and tryptophan(that stuff in turkey they say makes you sleepy).  If you don't remember, in MSUD those amino acids are leucine, isoleucine, and valine. If these amino acids build up in a person's bloodstream they can cause brain damage or even death. Symptoms can include seizure, no appetite, vomiting, etc... What's difficult, is that even with perfect dietary management, a person can deal with the effects of GA1. Stress, childhood illnesses, not enough calories, teething, etc... can all cause levels to elevate. GA1 is often misdiagnosed as Cerebral Palsy or "shaken baby syndrome".

I am going to share with you a video that a parent posted to help teach about GA1. Dustyn was born with GA1 and this is his story:

You can also read the family's blog at http://rmdawareness.blogspot.com/

Just don't forget the baby

" sometimes all you can do is not think, not wonder, not imagine, not obsess… just breathe, and have faith that everything will work out for the best . . ."

A friend posted this quote on her Facebook the other day. It keeps running through my mind. It's one of those quotes that reminds you that things happen when you stop stressing over them (like getting pregnant, taking a big test, or waiting for something in the mail).  

I know that it's in Gods hands as to when Evan will get his new liver and I truly believe he has a plan for us. I just wish I knew that plan. It is so hard not to spend every waking (and when I'm supposed to be sleeping) moment wondering when we will get "the call." Then of course I wonder where Gary will be when it happens. Will he be flying? Will I have to get the First Sergeant to have to call his aircraft back from a flight? Will it be in the middle of the night like they say it usually happens? Are our bags really packed enough? Will I forget something? 

I spoke to a new friend today who is 25 with MSUD and awaiting her call for a new liver.  As I was telling her about all my fears and wondering how I would handle things when we do get the call I realized none of that really mattered. All of those things would work out. So what if I forget a toothbrush..there are stores in California.  I finally told myself that nothing matters...JUST DON'T FORGET THE BABY! 

So back to that quote...don't think, wonder, imagine, or obsess....harder said than done! Just wish there were a switch that I could turn off and allow myself to breathe and let my faith carry us through this.  I pray each day that God gives us the strength to get through the next day, week, month, however long it may be. I pray that he keeps Evan healthy so that we don't have the added stress of him being sick or having his levels off. 

I am hoping that by writing this all out I will be able to stop thinking, wondering, imagining, and obsessing and just breath and have faith!