Boston, Day 2

Author: Chris

On day two of our Boston visit, the first order of business was blood. They needed some blood from Porter for lab tests. Eleven tests in all, I counted. Of course, we tacked on the weekly ammonia test as well. The lab staff made us all feel very comfortable and the nurse drawing Porter's blood did a flawless port access!

The infusion nurses always have awesome toys!

Day two was education day. First, we met with a representative from Infectious Disease. We learned all about some of the recommendations and restrictions for Porter following the transplant. The main idea is that his suppressed immune system is more susceptible to infections. The most curious restriction for him will be decaying plant material. This means that stirring the compost bin, raking dead leaves in the fall, and even gardening could be restricted. We will also be implementing a strict hygiene routine in the house after the transplant. Oh yeah, and school will be fun!

Then, we met with the transplant team pharmacist who explained the regimen of medications required following the transplant. Most importantly, Prograff, the anti-rejection drug that will keep Porter's immune system in check and keep organ rejection to a minimum. Yes, that's right, not zero rejection, but minimum rejection. We learned from the pharmacist and doctors that transplant patients basically live with a constant low level of rejection. Having absolutely no immune response to a donor graft is essentially not possible. But, keeping the immune response at a minimum encourages the integrity of the graft. It's tricky business titrating the Prograff to appropriate levels as a young patient grows and matures with a donated organ. Oh yeah, and Prograff has side effects, most notably, high blood pressure, which of course is treated with....more drugs.

Next, we took a short jaunt up to the Genetics and Metabolim clinc to meet with Dr. Amy Kritzer. It was great to meet her and chat about Porter's metabolic disorder. Since UCD's are a rare disease it is always refreshing to talk to someone who has a deep understanding of them and a lot of experience treating patients like Porter. She said she has been in charge of many UCD patients through their liver transplant surgery and stay at BCH. Porter will be on Ammonul infusion before and after the surgery and ammonia levels will be monitored throughout. Seems straightforward enough. It's reassuring to know there is an experienced metabolic team at the ready to help Porter.

Heading into our final segment of day two and just hours away from departing back to Vermont, we received news that Porter's ammonia level from the lab was 128! We couldn't believe it. Porter hadn't shown any ill signs that morning and was currently happily chasing his sister around the conference room table. This was not sick Porter. So, while Katie and I grabbed some lunch in the cafeteria, Sarah took Porter for another lab sample. While we ate lunch, we learned his ammonia was down to 40. A bit confused, we conferred with With Dr. Burke,  then cut his protein down for the rest of the day and continued on to our last appointment.

Our final bit of learning for the trip was about the logistics of what it will be like when we "get the call." After 30 days on the transplant list, Porter's "need score" will increase and he will be given 1B status which is very high on the list. This means that if we list him soon, he very well could have a new liver within a few months. Our transplant coordinator explained that when we get a call that a donor organ may be available for Porter, two things will happen: we will depart for the hospital ASAP and Dr. Kim will depart Boston and travel to wherever the donor is. Once we get to the hospital, it may be a "hurry up and wait" scenario as Porter is prepared for potential surgery and we wait for the confirmation that the donor liver is fit for transplant. If the team decides the liver is good, then Porter will be prepped for surgery.  They will do all but remove his liver before Dr. Kim walks into the OR with the donor organ. There is always the possibility that the donor liver will not meet the transplant standards.  If that is the case then we will go home.  They call that a "dry run" and it happens more than half of the time.

Well, that was about all we could handle in two days! We feel encouraged by what we learned, and will find out next week whether the transplant team will accept Porter into the program. We still feel strongly that Porter will benefit from this transplant and look forward to learning more and finally putting him on the list.

Thank you to Lindsay and Steve for some quality Boston hospitality!

  

Porter Street is just down the road from Lindsay and Steve's apartment!