Tuesday, February 25, 2014

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!


Rick said...

Thanks Jules, those "preggers" were a bit surprising, but I'm encouraged by the willingness of both hospitals to pursue a higher priority rating. I wonder if the two letters might help justify board approval. Hang in there, y'all:)

Anonymous said...

We will be praying for Seamus, and hoping everything goes well for him. Very glad to hear an update, we follow you here from the UK. Especially as my boy Bee who is 10 wcf has now been diagnosed with (mild) liver disease.

Meggy mum to 2 boys