Liverversary

I can't believe I haven't posted since the transplant! On May 13, 2014 at 8:30 PM, as I was making t-shirts for the annual CF Great Strides Walk and Seamus was taking a bath, we got the call.

"Julie, we think we have a liver for Seamus. Can you get to Philadelphia right now?"

Those words changed our life forever. We all jumped in the car and took off, leaving half of our pre-packed luggage behind. The 3 hour ride to Philadelphia was the longest trip of my life.

They had told us another child waiting for a transplant was also called and once they examined the liver, they would determine whether Seamus or the other child was the best match. I know many people waiting for organs go through many "dry-runs" before actually having their transplant so I was trying to not get too excited, but it was nearly impossible. At 4:30 AM, my cell rang and I heard "It's a go!"

Within an hour, Seamus was wheeled away into the OR with a smile on his face and our family went to the waiting room for a very long wait. Through the 8 hour period, we got many updates that things were going  well and finally the surgeon came out to tell us it was done. She said they were surprised at how awful his old liver looked because he always seemed in good spirits and didn't complain. I told her we've been hearing that from doctors his whole life. She said he was very lucky to get the new liver when he did.

His recovery went well, with a few bumps along the way, mainly with his GI system getting used to the new liver but he quickly adjusted and after two weeks in the hospital, we went to stay at the Gift of Life House for another week to be close for follow-up appointments. On June 3rd, 2014, we headed home.

This past year has been great. Seamus' liver has been healthy and he is back to being an active kid. He's gained more than 13 pounds and has gotten 3 inches taller. He hardly missed any school this year and he made the Honor Roll all semesters.

On his first year liverversary, I wrote the letter below to his donor's family thanking them for this wonderful gift. The Gift of Life House will send it to them and I'm hoping to hear back.

Dear Donor Family-

I am the mother of a wonderful nine year old boy who is alive today because of the precious gift that he received from your loved one. I will never be able to express my gratitude and thankfulness enough to your family for such a selfless act in your time of grief. I think about your loved one and your family every day and I am so sorry for your loss.

My son, Seamus, received his new liver after waiting ten months on the transplant list. His health had continued to decline until he was very sick and unable to attend school full-time. He wasn’t able to play with his little brother or walk very far without the aid of a wheel chair because he was so exhausted and in pain.

Originally, it was planned that I would donate part of my liver to him but because of an anatomical complication, I was unable to be the donor. This was a huge disappointment for our family. After that, he would ask me every day if that would be the day he would get “the call” and as time went on, we were all getting discouraged and were afraid he wouldn’t get it in time.

I am so happy to say that now he is an active little boy who runs around and loves to ride his bike and scooter.  He takes karate class and is back in school full-time in the 4^th grade. His liver is functioning extremely well and the doctors couldn’t be happier with his progress.

Seamus plans on being a pediatric anesthesiologist when he grows up and I know he will accomplish his dream. That wouldn’t be possible without the enormous gift he received last May. I do not have the words to fully convey how much it means to my husband and me to see him have that chance.

If you would like to contact our family and find out more about Seamus, we would love to talk with you. I know it may be difficult for you right now, so if you are not comfortable yet, I understand. Whenever you are able, feel free to contact us.

With sincere gratitude-

Julie

Time Flies

Where to begin? Well, we made it to Denver and saw Dr N. He was 100% certain that Seamus does need a transplant and also some very complicated motility testing to determine once and for all what the hell is going on with his GI tract. So we came home very excited and thought things were going to happen! 

Well, it's 8 months later, Seamus is still waiting for a new liver and will finally have the week-long motility testing done here in DC in three weeks. These things take a lot more time than we anticipated. We decided we wanted to increase Seamus' chances of getting a liver so we took a trip to Philadelphia and got him evaluated at Children's Hospital of Philadelphia (CHOP). We loved their program and felt very confident with the doctors. We also met with the transplant team at Hopkins and decided to list at both places. Originally, the plan was for me to be a living donor. I went through all the evaluations and tests and Hopkins thought I looked really good. I just needed to lose a few pounds. CHOP had other ideas. They looked at my imaging and thought I had some anatomical issues that would make the surgery too risky for both Seamus and me. Because CHOP has much more experience with pediatric transplants, we felt we couldn't ignore their advice so we discarded the living donor plan.

In the meantime, Seamus was working with his therapist trying to get used to the idea of transplant. Initially the whole thing freaked him out to the point of refusing to talk about it. The only thing that made it a little easier was knowing he was going to get part of Mommy's liver. When that fell through, we were really worried. Thank God, his therapist totally gets him and has worked with him so well that now he is completely on board. He understands his liver will come from someone who "didn't make it". At this point, he talks of nothing but his transplant and is getting very frustrated that the call won't come.

So over the 8 months, Seamus' condition has quickly deteriorated. He continues to go to school but is having a very difficult time because of fatigue, nausea and vomiting, and bleeding issues. He has also developed hepatic encephalopothy which causes confusion, poor memory and difficulties with motor skills like handwriting. It's been a definite struggle working with the school to understand his issues are medical and not behavioral. His teachers seem to think of him as a nuisance. We are working on pulling him from school part time and having a home/hospital teacher come to the house to work with him on the off days.

He also continues to have current hospitalizations due to his lowered immune system and frequent GI bleeds. His portal hypertension is getting worse and his liver and spleen have gotten so big, he looks like he's  8 months pregnant.

The plus side of all this is he has moved up the transplant list a lot. When he was first listed in August, his PELD (Pediatric End-stage Liver Disease) score was actually -6. That's because the score only takes into account a few items, all of which Seamus liver was doing in a borderline okay way. After writing an exception letter based on his other health and deteriorating quality of life issues, CHOP got him up to 28 points and Hopkins got him to 25. They are both in the process of writing new letters asking for 30 -35. That's a really high number and we expect to receive a call very soon if the letters are accepted by the national board.

So right now, we are in a holding pattern. We don't make any plans because we never know what is going to happen next. Seamus continues to fight and lead as normal a life as possible. Hopefully, it won't be another 8 months before I update this with news he got his new liver!

Progression

Let's start with my progression. BMI is now 31.9. It's coming down slowly but surely and with the last few months we've had, I'm not going to beat myself up about it. I've lost 17 pounds total.

In April and early May, James spent 11 days in the hospital with a terrible lung infection. It caused empyema which is a build of infected fluid in and around the lung which caused it to collapse. He thought he had bruised or broken a rib and didn't get it looked at right away. It had progressed very far by the time he got to the hospital and he needed surgery to clear out all the pus from his chest cavity. He is finally feeling better now.

Not to be outdone by his father, Seamus had to make a hospital appearance as well. Seamus had been having a hard time with his nightly feeds for weeks and on May 19th he vomited again but this time there was a fair amount of blood. I contacted his GI doc who said he should get an endoscopy soon to see if he had more varices that needed banding but it wasn't an emergency. The next day he went to school and vomited again with a lot more blood. We headed straight to the ER.

He was admitted and an endoscopy was done the next day. They kept telling us they were sure it was just a tear in his esphogus from all the vomiting and if he had more varices, he wouldn't be acting so happy. I told them "You don't know Seamus".

After the procedure, Dr K asked us to talk in another room which is never a good sign. Usually the docs talk to the parents in the waiting room right in front of everybody. He told us Seamus had two grade 3 varices which they banded and another grade 2. Grade 3 are very serious. It means the cirrhosis has progressed and the portal hypertension has gotten worse causing the pressure to build up. The blood vessels were so big they nearly filled up the whole esphophagus.

While he was under, they changed his feeding tube from a g-tube that goes into his stomach to a g-j tube so the feeds could bypass the stomach and go directly into the intestines. This is to help his nutrition level because currently he is severely malnourished, partly because of his cirrhosis and partly because with his chronic constipation, food is not being absorbed. Immediately after the surgery, he vomited and dislodged the new g-j tube so it was unusable.

Two days later, they put him under again to replace the g-j tube and things went fine until a few hours later when he started feeling ill and spiked a fever of 105. They started him immediately on antibiotics which was good because he ended up having sepsis. During the procedure with all the manipulation of his gut, bacteria crossed over into his blood causing an infection. Sepsis is very dangerous, especially in people who are weak and have low immune systems. They, and we, were very worried about him.

During that time, we were in discussion with his doctors about how to proceed with his care. At this point, he has them perplexed. He was only diagnosed with liver disease a year and a half ago and it's progressed faster than anyone could imagine. It's definitely time to start talking about transplant. We saw the medical director of the CFF in clinic a month ago and he told us we should probably talk with Dr Michael Narcowicz in Denver because he is the top GI/liver doctor in the CFworld. We brought this up to Dr K after the procedure and he agreed it would be a good idea. Besides just discussing tranplant options, he would like Dr N's advice on improving Seamus' nutrition (which is necessary for Seamus to recover from a transplant) and any other ideas on handing his chronic constipation. And on top of everything else, we can't forget about his lungs. Although his lung function is still very good, his x-rays do show CF lung progression.

So that's where things stand. He is now doing 18 hour feeds everyday as well as 1.5 hour of medicines through his tube so he is basically constantly attached to his pump. He is in good spirits although he has "the worries" often and is seeing a psychologist to help him feel better. We are all looking forward to going to Denver next week but I am very nervous because I think our life may be changed completely after meeting with Dr Narcowicz. I'm trying not to think about or make any plans for the future because things may all change.

Our wonderful friends set up a fundraising site to help us with the travel and medical costs and it raised $3000 in just a few days. I can't thank them enough, that money will help us out tremendously.

Here's a cute video of the kids in the sprinkler this weeked. After I stopped taping, Mackey really got into it and we couldn't pull him away.Sprinkler fun!

I'll post again after we go to Denver.

Update

So let's get it out of the way... After realizing on-line Weight Watchers just wasn't going to cut it for me, I joined Jenny Craig. My life is so crazy right now, I really need something very structured so I don't have to make any more decisions than I already do. After one week, I lost 3.6 pounds. I had lost a few before so total loss is 7.3. Current BMI - 33.7.

Mackey had his second birthday a few weeks ago. The poor guy really gets the short end of the stick. It was a low key event at our house but he had a big celebration at daycare. He was thrilled to get presents so I don't think he minded not having a party. I will write a post strictly about Mackey soon. He is growing up so fast and I want to make sure I record some of the wonderful things he does before I forget.

Some of Seamus' symptoms seem to be changing so we spent 7 hours at CF and GI clinic on Friday and got x-rays and an ultrasound done. The concern was he might be developing ascites which is fluid build-up in the abdomen because the pressure has increased so much in the liver. The doc called us Sunday night, which is usually not a good thing, to tell us there was no sign of fluid but he has lots of trapped gas and they can't figure out why. He had some stool but isn't obstructed. He constantly looks like an 8th month pregnant woman. We also got definitive proof of the cirrhosis which up to this point they had assumed but hadn't proven. This doctor has only been following Seamus for about a year and I think he's finally starting to understand the uniqueness of the situation. He admitted that Seamus is "a mystery" and his liver (and GI) disease is not following the path of most CF patients. We had another long discussion about transplant on Friday and he still believes Seamus isn't ready but we are concerned about waiting too long because he could become too sick to be eligible.

The best part of the weekend was meeting with the Make-A-Wish volunteers. They had lots of questions for Seamus about his likes and interests and really listened to his request. He has a very specific request so we are hoping they are able to do it. He makes to go to the Activision offices where they make the Skylanders video game and create his own character called Man-atee. It's half man, half manatee. The face will be his face. He's come up with all the powers he has and whole story line. So hopefully, he could actually work with the designers to create it and incorporate it into the game, at least a version just for hem. My biggest concern is they wouldn't fully understand what he wanted and would just send us out to meet with the designers who would give us a tour and just show him how they work. I think he would be very upset if we went all the way there but he couldn't make Man-atee but it may be too much work for them.

He has a second wish, just in case, which is going to the Disney resort in Hawaii. Obviously, James and I wouldn't mind if that wish was granted but he really has his heart set on the first one so I hope they can do it or at least some comparable version of it.

So we're still waiting for culture results and more liver blood work to come back but currently Seamus is feeling good which is the most we can ask for. I'm hoping we get that huge snowstorm everyone is talking about so we can all stay home together and snuggle.

Weekly update 2/8/13

Okay, this week I did well with eating healthy and went to the gym or did something at home every day. I lost 1.2 pounds which isn't a lot but I had lost 4.2 pounds before my last post so overall I'm down 5.5 pounds so I feel good.

BMI 34.1

Weighty issues

I have always been overweight and like most overweight people, I've tried scores of different diets, some more successful than others. I've lost a few pounds and inevitably, gain it back over time, especially after having two kids. I know the statistics, how I'll be healthier and live longer if I lose weight, but of course, I think nothing will really happen to me. For the first time, I no longer have an option of taking my weight lightly (pun intended).

Yesterday, Seamus had a follow-up visit with his liver doctor. We always dance around the topic of transplant at every visit. His opinion is some day Seamus might need one but as long as the liver is still functioning and his lung function does not seem to be compromised by the ever-increasing size of his liver and spleen, then he is not ready. His lung function has actually increased lately due to three months of inhaled antiobiotics but his liver lab work is always borderline okay, some times just under the acceptable numbers and sometimes just over. Every time he gets sick, like the flu-like virus that landed him in the hospital last week, his liver has a very difficult time handling it and bouncing back. So basically we do not know if, or when, the transplant talk will change.

That's where my weight issue comes in. I am the same blood type as Seamus but James is not. That means that I am a candidate to be a living donor for Seamus and I always assumed that would be no problem. Yesterday I found out that one of the biggest donor requirements is weight and I am too fat. It never really occurred to me that the fat is not just hanging off the outside of your body but your organs actually get a layer of fat as well and in order to have a healthy liver, your BMI should be around 23-25. That means I would have to lose more than 45 pounds to be an acceptable donor.

Since his liver could take a turn for the worse very quickly, I really need to be ready at any time. I could not lose 45 pounds immediately if he needs a new liver quickly. He could be listed for a cadaver liver but there is a shortage of organs and you never know the outcome of that.

So I have to take this weight loss thing seriously, extremely seriously, for the first time in my life. It's not a matter of just looking good in my clothes but it could literally save my son's life. I am going to post my progress here for everyone to see. Maybe that will shame me into keeping on track. I could use your support and advice.

2/1/13 - BMI 34.3

Catching Up

So the last two years in a nutshell:

 Mackey is almost two and is totally awesome; crazy, and defiant, and full of piss and vinegar. On days when he's throwing temper tantrums and saying nothing but "No, No, No", I may not think he's exactly awesome but to hang with this family you have to be tough and not take anyone's shit. I think he's ready for the challenge.

I am so glad we made the choice we did. Seamus is such a good big brother and the love they share is so special. Mackey absolutely adores his big brother, Bubba, and tries to emulate him in every way. He loves to sneak into the medicine drawer and grab empty syringes and "take his medicine" and we've caught him on a few occasions puffing on dirty nebulizer cups. He is completely obsessed with Dora the Explorer and does a mean Dora dance. Drawing is his other passion, much like his brother.

Mackey spent the first year at home being taken care of by Ga, my mom, who lives with us. After we caught him standing inside the dishwasher a few times and doing laps around the house, we realized he was too much for her to handle. He now goes to daycare and is doing great. His caregivers speak Spanish to him most of the time and he's picked up a lot. When he first started becoming vocal, we thought he was speaking lots of gibberish but then we realized he was speaking Spanish!

Seamus is doing well at his new school. He attends a performing arts school and has drama, art, music, or dance every day. Last year, he skipped a grade so he's now in the second grade. The only downsize is the large class size but that's the same in any public school. The nurse there is great and treats him like her own.

His health has been a concern. He has been hospitalized quite a few times in the last two years (now up to 33 admissions.) Some were for lung exacerbations but most were for GI or liver issues. He was diagnosed with CF liver disease in December 2011 and it has progressed much faster than anyone expected. His liver and spleen have become very enlarged and are in danger of rupturing if he accidentally falls or gets hit. He is not supposed to do any contact sports but he is still allowed to take karate for now. He loves it and is up to a yellow belt but he won't be able to continue much further because he can't spar.

He has cirrhosis and portal hypertension which means his liver has a lot of scar tissue that doesn't allow the blood to flow through. Instead the blood flows back into the stomach and esophagus and the pressure builds up so the blood vessels can burst at any time. He has had some bleeding so they went in and did a banding procedure to cut off the affected blood vessels. Normally, after a few weeks the band sloughs off and heals but in his case it didn't heal and instead formed an ulcer. One night, he woke up vomiting about two cups of blood from the ulcer and had to be rushed to the hospital. It eventually healed but the concern is the next time he needs to be banded, which is definitely a "when" not an "if", it could happen again.

He's starting to question things and some stuff does seem to bother him, but overall he still has a positive outlook. He is starting to have some trouble concentrating in school. We don't know if that's because the work is getting harder, if it's related to the liver disease, or if he's having sleep issues which are common to CF. He is going in for a sleep study soon.

Tonight, bedtime was a circus and James said something about how we aren't a "normal" family. Seamus asked "Is that because of my CF?" That's the first time he ever said anything like that. We told him of course that had nothing to do with it. There's really no such thing as "normal" and if there was, what fun would that be?