Adeno...What?

Sorry for the delayed update. Things were going well with Evan and we were getting ready to go over all discharge directions and head to Ronald McDonald House...that is until Monday when we found out that Evan's cultures from Friday showed adenovirus in his blood. What exactly is adenovirus? Well, it's just a virus really. Much like any other virus it causes colds, flu like symptoms, fevers, sore throats...in normal people that is. Of course, as will any illness, it can be much worse and can cause many other issues in immune compromised people (like Evan). Because of Evan's positive test, the docs wanted to begin treatment right away so that his body could get rid of the virus. They started first with IVIG. (Intravenous immunoglobulin) This is to help increase the effectiveness of Evan's immune system. Unfortunately, after one treatment his levels of the virus increased instead of decreased. This baffled all the doctors and nurses since clinically, Evan was presenting great. He has been getting happier, more active, and more playful every day (since his fevers stopped last week). The Physician's Assistant said when she saw the results she kept thinking there had to be a mistake and even called to make sure it was correct. Either way, the fact that Evan is presenting well is a really great sign. Since the IVIG didn't seem to work for Evan, the team decided the best treatment would be cidafovir. If you look it up on the internet, cidofovir can have some really serious side effects on the kidneys. Because of this, there is additional treatment that goes along with administering cidofovir that helps to prevent this damage to the kidneys. Evan is responding well and his kidney functions are awesome with the preventative meds. His liver functions are also GREAT and the docs are really pleased with how his liver is doing.  The plan at this point is to continue with the cidofovir therapy to reduce and eliminate the virus. The docs are hoping for Evan to only do a couple of more inpatient therapies (every 48 hours) and then he will continue them outpatient until the virus is completely gone. So far so good! Hopefully not too much longer til we can get him out of the hospital and staying with us.

On that note...we had to move out of Ronald McDonald House :(  Because many of the children staying at RMH are also immune compromised, Evan cannot go there until he has ZERO virus in him. We are sad to be leaving the support system we have created there. We have met some wonderful people who completely understand how we feel and what we are going through. On the other hand, we are excited to get to some sort of "normal" instead of being in the hospital all day every day. We found a very nice furnished one bedroom corporate apartment not too far away and rented a car (15 minute drive). The apartment will be more comfortable for the three of us and it will be nice to have our own space. We will most likely be there for another several weeks if not longer since Evan will need to continue to go to Liver Clinic to have his numbers checked and his medications adjusted. He will also most likely need to continue with the Cidofovir treatments as well since it takes time to completely rid a body of a virus.

For those of you who have sent things to RMH, we will be stopping by there a few times to check on mail and they also know to call us. As soon as I have a good mailing address for the apartment I will send it out to everyone. Thank you to everyone for the continued support! Much love to you all! 

"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! 

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!

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! 

Is this really happening?

Each day the reality that Evan's transplant could actually happen gets closer and closer! Today I spoke with the insurance and got the name and phone number of the air transportation company that is going to transport us when we get "the call". Of course, as any mother would do I called them to get my questions answered (finally)!

Will both parents be able to be transported with Evan?
How do we get his carseat to Cali?
How much luggage can we bring?
How long will we have to get to the airport?
Where will be meet the air plane?
What kind of plane will it be?

SO MANY QUESTIONS!
Here are the answers I got...most likely, both parents will be able to go, but that will mean no luggage. (That's okay...I'd rather have my whole family together than my clothes anyway!)
Evan's carseat will be strapped to a stretcher and that's where he will sit for the flight.
We will most likely have about an hour and half to two hours to get to the airport.
We will meet the plane at the private fueling area of the airport. They will give us an exact address and phone number when we get the call.
It will most likely be a leer jet that picks us up.

Honestly..I am just relieved that we will most likely NOT have to worry about getting one parent a commercial ticket to fly out to California. That was my biggest fear..that we would not all go to California together. That one of us (most likely me) would have to sit alone in a waiting room while Evan was transplanted while the other was trying to make his/her way there.

Tomorrow Evan's status should be changed to 1B which is only below children who are nearest to death.  That means that this is really happening and it will be sooner rather than later. This means the wait is getting shorter. By how long...who knows.

We are still waiting and the waiting is anxiety ridden and frustrating.  The phone rings and all I do is wonder..is it? When will it be? How many more rings before it's the one?

Getting ready...

It's been a crazy week since family left. We have been getting things in order so that when we get "the call" we aren't scrambling to make sure things are taken care of. Bags are almost packed and directions have been given to those who will watch the house and the dog. Sadly...we still don't know which company has been contracted for our transportation. We know it's been approved, but we don't know who it's going to be which makes it difficult to figure out if we are all traveling together or if one parent will have to travel commercially (We are PRAYING that is not the case).

Evan had more labs drawn on Monday and along with that his current weight (8.97 kilo) and height (72.7 cm) have been sent of to Stanford so that his transplant list status can be moved up to 1B this Friday. I can't believe it's been almost a month since Evan was placed on the list for a new liver. Man, the time sure does fly! We are hoping the call won't come too long after the status change. The wait is going to be killer!

We had a tiny glimpse into what it will feel like the moment we get "the call". On Monday morning at 2:45 am our home phone rang. Of course it woke us out of  deep sleep and I jumped when my brain thought of Evan and his transplant. I kept thinking we weren't ready because he's not supposed to be eligible yet because of his immunizations. Once I answered the phone and realized who it was I relaxed for a moment. It was JR's work calling because they had a real world mission and needed him to go into work. An autistic teen had gone missing while hiking with family in White Sands NM.  He had been missing for more than 12 hours at this point and ground and air searches had been unsuccessful to that point. By the time Gary's crew had arrived the teen had been missing for several hours longer. However, an hour and a half into their search, they spotted the boy, who was in remarkably good shape. He was carrying a red sled (for sand sledding on the dunes). That is how they saw him.  Because the boy was in such good shape, they were able to return him to his mother rather than take him to the local hospital.

Here is a picture of the rescue crew and Angelo (the teen they rescued) 

I am so proud of my husband and his crew for a job well done!

Evan's first Christmas!

Evan's first Christmas was a success! Uncle Jimmy (my brother) came to town on Wednesday and we could not have been more excited to have him come to visit us for the holiday! On Friday evening we had a few friends over for dinner to celebrate an early Christmas dinner. Lots of kids and friends so it was a busy holiday house! Absolutely loved it! Christmas eve was calm and uneventful. We went out to dinner and just enjoyed hanging out together. Christmas was nice and relaxing...not something I am used to since I am used to being around lots of people and going to at least 2 different houses to celebrate the holiday.  We opened gifts in the morning. Evan got lots of new toys: a riding/walking car, a ball popper, a ride on train, a wagon, books, a 3 in 1 trike, and lots more! He definitely enjoyed his new toys. As for opening gifts, that was a first for him. He wasn't too excited about it, but once he saw toys underneath the paper he was definitely interested. By the end he seemed to tear the paper a bit more and was more excited to play with each new toy.

My breakfast casserole was a flop :( I guess that's what I get for trying something new on Christmas morning.Oh well...we got a good laugh out of it!

Monday brought on an interesting day. Evan had been fussy and not eating as well for a couple of days, but he was eating. We think he's growing more teeth. By Monday, he was SCREAMING at food (which he loves) and wanted nothing to do with the bottle. We got some cereal and apple sauce into him and only 8 ounces of formula by 4:00 pm. We decided enough was enough and took him to the ER after talking to his geneticist. They admitted us overnight just to watch and make sure Evan wasn't getting sick. After about 5 hours on the IV of D10 (sugars) he was much happier and started to eat better. By the next morning he was back to taking 6 ounce bottles so we were released that afternoon. It's never any fun being in the hospital, but at least we were only there a day and Evan seems to be doing much better now!

Only a few more days and we will ring in the new year! It's crazy to look back on this year and think about the changes in our life and the many things we have learned. It's been an eventful and emotional year, but we are all doing well! We are looking ahead to 2012 and the many things we will encounter. Most likely Evan will have a liver transplant by the middle of the year (if not much sooner) and this, again, will bring on a new way of life for us all. We will no longer be fighting MSUD on a daily basis, but we will be managing on monitoring Evan's liver to make sure that he is not rejecting and is on the right meds. It's amazing to think of how far we have come, but how far we still have to go. As of today we have 21 days until Evan can accept a liver...it is also 21 days until he will be listed at Status 1B which will bring him closer to his new liver and his new lifestyle. We continue to pray each day that he stays healthy and eats well.  With the many feeding issues we have seen over the course of the last nine months, we are praying that we can get him transplanted before he says enough is enough and needs a G tube.  (Not uncommon in children with MSUD who refuse to eat).  We have spoken to families and doctors and know of children who almost immediately following transplant completely change their feeding habits. We are praying this is also the case with Evan as feedings have been one of the biggest challenges that we face.

Oh 2012...what do you hold for us? We pray it's all good! And we pray that all our friends and family have a blessed, happy, and healthy 2012 as well!

Why transplant?

Many people have asked us why we are choosing to transplant or how a transplant will help Evan. It has been a very difficult and very personal decision for JR and I. We have done much research and talked with many families and feel that this is going to give Evan the BEST quality of life!
I am going to share with you an article I read that explains in fairly simple terms the risks of MSUD and the benefits of a liver transplant to cure the metabolic symptoms of MSUD. This article was written in 2006, but the information is still valid. There are more articles out there, but this one really explains it well. If you prefer to read it on its own website, here is the link: http://www.sciencedaily.com/releases/2006/04/060410161437.htm

Liver Transplants Provide Metabolic Cure For Rare Maple Syrup Urine Disease

ScienceDaily (Apr. 10, 2006) — Liver transplants cured the metabolic symptoms of 11 patients with a rare but devastating genetic condition known as Maple Syrup Urine Disease (MSUD), according to a study by researchers from Children's Hospital of Pittsburgh and the Clinic for Special Children.

All patients from the study (ranging in age from 1-20) are alive and well with normal liver function, according to the researchers. Amino acid levels in the study patients stabilized within 6-12 hours of transplant and remained stable since transplant despite unrestricted intake of protein.

MSUD is a metabolic disease which causes amino acids from proteins to accumulate in the body. The disease gets its names from the sweet smell of the urine. The accumulation of amino acids in the blood can cause metabolic crisis at any age, which can lead to brain swelling, stroke and even sudden death. Over a patient's lifetime, chronic instability of blood amino acids can result in serious learning disabilities and mental illness.

Before transplant, the only treatment was strict adherence to a diet almost devoid of protein. Despite adherence to this diet, patients were still at risk of metabolic crisis from something as simple as a common cold, which can disrupt the body's metabolism and cause rapid neurological deterioration.

In 1997, an MSUD patient at another hospital received a liver transplant due to an unrelated medical condition and physicians noticed the symptoms of her MSUD were alleviated.

Based on this serendipitous result, physicians from Children's and the Clinic for Special Children, located in Strasburg, Pa., began working collaboratively to develop a liver transplant protocol for MSUD which optimized patient safety. With a comprehensive, multidisciplinary protocol established, Children's transplant surgeons began performing liver transplants on MSUD patients in May 2004. Children's has performed 18 MSUD liver transplants since then.

The study by Children's and the Clinic for Special Children involved 11 of these MSUD patients, including the original patient. Results of the study are published in the March issue of the American Journal of Transplantation.

"The development of liver transplantation as a treatment for MSUD has dramatically improved our patients' quality of life," said George V. Mazariegos, director of Pediatric Transplantation at Children's and one of the study authors. "Our MSUD patients and their families had lived in fear of everything from a chicken nugget to a common cold. Liver transplantation is not without risks, but for some patients, it is the best option and it has allowed these recipients and their families to live without fear of simple things most people take for granted."

Kevin A. Strauss, MD, a pediatrician at the Clinic for Special Children and a co-author of the study, said that over the past 15-20 years, early diagnosis of MSUD followed by careful nutritional therapy have improved the health and developmental outcome of affected individuals.

"Nevertheless, the risk for metabolic crisis and acute neurological injury is always present, and many older individuals with MSUD suffer from depression, anxiety, and impaired concentration and learning," Dr. Strauss said. "Liver transplantation protects patients from these acute and chronic neurological complications. It is a reasonable alternative to nutritional therapy, particularly for patients with poor access to specialized medical care. However, liver transplantation is not without serious risks, and decisions about the best course of therapy will vary on an individual basis."

For more information on Children's transplant program and its MSUD transplant protocol, please visit www.chp.edu.

It's Official

Evan is ON the transplant list! We got the call a couple of hours ago! I am still so amazed at how quickly this process is going! As I believe I have posted previously, Evan will be listed with a score of 30 out of 40 for the first 30 days. Once he has been on the list for 30 days, he will be bumped up to a Status 1B which is the 2nd highest status for a pediatric transplant patient.  Once he is on status 1B we will hopefully get "The Call" fairly quickly. The transplant team seems quite confident that it will not be a long wait. The longest they have waited for another child was 6 months, but usually they transplant within 4 months of listing.

So far so good! We are so blessed to have had such a smooth road so far.  As much as I feel blessed, I am waiting for the other shoe to fall. I worry that we will hit some snag or something will change.

But for today, for right now at least, I feel so positive! Thank you again to all of you for your continued support! The REAL wait has officially begun!

College Acceptance

Most people don't pray for college acceptance for their kids until they are about 17 years old or so. However...we are pleased to announce that Evan has been accepted to Stanford University! Okay...so, he's not a student, but he's still been accepted!

Our trip to Lucile Packard Children's hospital at Stanford University was a huge success. Evan did great traveling and we met with the genetics and liver transplant teams. Dr Enns, the geneticist, fully believes that transplant is the way to go when it comes to classic MSUD. He really believes the benefits of transplant outweigh the risks of MSUD.  Especially since MSUD is completely unpredictable.

Our talks with the members of the transplant team were great as well. They were very thorough in helping us to understand the risks and benefits of transplant.  They are confident and knowledgeable all while being friendly and kind. Evan just absolutely fell in love with one of the surgeons. He just snuggled her for a good 20 minutes as we talked with one of the lead surgeons. Her tenderness was exactly what I needed to see to know that this team would take good care of Evan...not as just another patient, but as our baby! 

The transplant coordinator, Marcia, was fantastic. She answered questions and gave us more information than we anticipated. She is going to be our guide through the ins and outs of this process. She will make sure we get them everything they need and she will be the voice we hear on the other end of the phone when we get "The Call!" 

She called yesterday to let us know that the team had presented Evan and made a decision to approve him for transplant! There are still some things that need to be done before he is officially listed. He will need to have an ultrasound done as well as a few more blood tests. He will also be getting his MMR and Chicken pox immunizations on his 9 month birthday (December 19).  Once all of that is done and the insurance approves everything *FINGERS CROSSED* then Evan will be listed. 

Evan will be listed with a PELD score of 30 (40 being highest need). After 30 days with that score, he will be bumped up to Status 1B...which is the second highest category for need. The transplant team has said that once he reaches Status 1B they should get calls for available livers and once they decide a liver is the perfect match we will get "The Call". They don't anticipate the wait being longer than a few months as the longest wait they ever had for a similar situation was about 6 months.  So..for all practical purposes...Evan should have a new liver by the summer! :)

While we were in California, we stopped by the Ronald McDonald house and put our name on the list for housing need. We are really hoping to stay there as it is very close to the hospital, has all the accomodations we need, and because there will be other parents there who (although maybe not going through MSUD related stuff) will understand how we feel and what we are going through.

We have jumped a huge hurdle here with Evan's "college acceptance" and hope that the rest of the process continues to go as smoothly as things have so far.

We love you all and thank you again for your continued support!

Headed to Stanford

Thanksgiving week was...eventful! Evan had a terrible cough and cold as did I. It, of course, messed with his levels and he was crabby and not wanting to eat. We worked with the geneticist to keep him out of the hospital and successfully got through this cold without a hospital visit! Thank goodness. It took adjustments in his formula as well as him eating smaller bottles every two hours while he was up since he didn't want to eat full bottles every 4 hours.  Luckily he is now back to his happy-go-lucky self and smiling :)  

We had a few friends over or Thanksgiving. It was very nice. Evan slept most of the time they were all here, but it allowed mommy and daddy to enjoy their company and dinner.  Quite a nice holiday! :)

So...now to the title of this post...we are HEADED TO STANFORD!

We leave tomorrow morning for our trip to have Evan evaluated for transplant. It took some work, but we were able to get the AF to pay for all 3 of us to travel instead of just Evan and one parent.  They even bought Evan his own seat so we will have some space for sitting (once we get the airline to recognize that an 8 month old cannot sit alone without his parents). 

Our schedule for the week is as follows, leave for Stanford Tuesday morning, meet with genetics Wednesday morning, and then meet with different parties of the transplant team on Thursday (all day).  We are really looking forward to meeting with all parties to fully understand the ins and outs of transplant as well as the benefits and risks that Evan could face.  This is just the beginning of a long journey, but we are happy for it to be starting.

I will update again when we return from California.

Evaluation Approved!!!

Yes...you read that title right! Evan's transplant evaluation at Stanford has been approved by the insurance! The next step will be on Monday when we meet with outpatient records and EFMP (Exceptional Family Member Program) to arrange for transportation and lodging to be paid for by the military.  We are so blessed to have this as an option.  Once we have that paperwork started, we will schedule our appointment with the team at Stanford. I spoke to the transplant coordinator today (she called because they too got the referral) and she said they have open appointments for the rest of this month besides Thanksgiving and they are open into December. We are shooting for December 1st as we know that the paperwork process will be a little while.  That means that in just a few short weeks we will be on our way to California to have Evan evaluated for a liver transplant. Woot Woot!!! This is the first step on a long journey, but we are looking forward to this and feel it is the best option in treating Evan's MSUD. Please keep us all in your prayers and pray that Evan's evaluation goes well and that afterward TriCare (insurance) will approve the transplant!