Boston, Day 1

Author:  Chris

"On a scale of 1 to 10, it's a 10.9". Those are the words of Dr. Kim. Heung Bae Kim, MD at Boston Children's Hospital. "The heart and lung transplants get a lot of attention. But, a ventilator or external pump can suffice to keep a patient alive for weeks. There isn't a machine on Earth than can do what the liver can do."

Dr. Kim is the surgeon. He does pediatric organ transplants. He's pretty good at it too. Dr. Kim's perspective on the reality and gravity of the liver transplant was just one of many things we learned while visiting Boston Children's this past week.

We would classify our current management of Porter's OTCD as "exhausting." We are thrilled that he hasn't had any catastrophic ammonia levels, but we still experience some scary moments. We still visit the emergency room periodically. We are still constantly doing oral washouts, denying him protein for a day or two here and there to help manage his ammonia levels at home. Porter has made little progress in eating by mouth and managing all his nutrition through the G-tube is exhausting for us. Our main concern is sustainability. We thank our lucky stars that Porter's particular mutation and disorder doesn't necessitate an immediate transplant, but how sustainable is our current regimen? For him and for us? With the support of our team at UVM, we have decided we are ready to seriously consider a transplant option for Porter. While an organ transplant isn't free of its own complications and life altering consequences, our initial education and input from Dr. Burke, Dr. Korsen (in Boston) and others, has helped us learn that a transplant will eliminate Porter's hyperammonemic crises. His metabolic disorder would essentially go away. He may be able to eat what ever he wants with no metabolic dysfunction. For the rest of his life.

The transplant process can be a long one, so we needed to start somewhere. Dr. Burke helped us schedule a consultation visit to Boston Children's Hospital. So,with both kiddos in tow, we drove to Boston to meet with the team of clinicians representing the Liver and Multivisceral Transplant Program. It's good fortune that my sister, Lindsay, and her husband Steve live just minutes from the hospital. Katie spent our first day in the city with Lindsay and Steve at their downtown gym, AMP Fitness!

Katie helps out at AMP Fitness!

We stayed with them and spent two jam packed days navigating BCH and consulting with the transplant team. The team consisted minimally of a financial advisor, social worker, hepatologist, surgeon, pharmacist, and the transplant coordinator. We also met with a dietitian and an infectious disease specialist.

Our first morning in Boston, we dodged morning traffic and arrived early to our first appointment. One other young family sat with us in the waiting room. They had a young girl who seemed about Katie's age. Sitting on his mother's lap was her younger brother who looked about Porter's age. After a few minutes, the mother asked, "Did he have a liver transplant?" Sarah and I looked at each other, then Sarah said, "No, but we're here for a consult. How did you know about the liver?" The mother said, "Well, its Monday. Monday is liver day. Gavin [the boy] had a transplant three weeks ago. We are being officially discharged today!"


Sarah and I couldn't believe what we were hearing. You mean we just walked into BCH for a liver transplant consult, and we run directly into a family with an 18 month old boy who just received a new liver!? You've got to be kidding me. Before we were called to our first appointment we learned that Gavin had Biliary Atresia and had received his new liver after waiting for 12 months. Gavin was doing great, the transplant was a success, and they were on their way home. Turns out they live in upstate New York, about an hour's drive from our house in Vergennes, Vermont. As we exited to our appointment, Gavin gave us a huge smile and waved good bye. Another fortunate circumstance for sure, and an encouraging introduction to Boston.

The most influential interview that morning was with Dr. Kim. He drew us a diagram of how the transplant is done. He explained which blood vessels must be severed to remove Porter's liver, and how those same vessels are later connected to their counterparts in the donor liver. He helped us learn about the possible complications from such a surgery and how those complications are treated. Children's blood vessels are small and harder to work with than adult vessels. But, Dr. Kim specializes in children, and he is experienced with the small plumbing. He explained the various options that come with various types of donor organs. A live donation would be a small child-size portion to transplant. A deceased adult donor organ would also likely be divided, with the small portion reserved for someone Porter's size, and the larger portion could go to a second recipient. Finally, Dr. Kim described that he would travel to the donor location and personally evaluate the donor organ and then harvest the organ himself and transport it to Boston. Understanding the severe scrutiny the donor organ receives before it is considered for transplant is reassuring. Dr. Kim and others on the team we met that day helped us understand that there are discrete characteristics that can discount an organ for donation, and that Dr. Kim and his team will never use an organ that doesn't meet their high standards.

The transplant team really asked more questions of us than we did of them. This is because their goal is to gather information in order to thoroughly discuss and decide whether Porter is a good transplant candidate, whether our family is ready for something like this, and whether to accept us into the progam. Once we get the go-ahead decision from the team, we will have the opportunity to put Porter on the transplant waiting list.

Day 1 was exhausting! But, it would turn out to be a walk in the park compared to Day 2.