In May 2003, Jakob was evaluated for liver transplantation at Pittsburgh Children's Hospital (PCH). After a long road of disappointment, we found our way to PCH's doorstep. The evaluation process was relatively easy, but two very long days for a 3 year old little boy. Meeting the surgeons was an emotional, validating experience for me, and one that I will remember for the rest of my life. For the first time I felt as if a team of doctors really listened to my concerns about raising a child with Maple Syrup Urine Disease (MSUD), and the life-long difficulties we faced relying on dietary therapy to manage his disease. The treatment options, dietary therapy versus liver transplantation, are complex and personal decisions that should remain private family matters. However, suffice to say, after evaluating our own family situation, Jake's future, and his potential for a viable life as a full functioning adult, we decided to go ahead and list him for transplant.
PCH is the most respected hospital for transplantation in the country, and had performed numerous transplants for metabolic diseases, but Jake would be their first for MSUD. As such, PCH took every precaution to ensure the safety of transplanting a child with a disease as volatile as MSUD. Early on, the transplant team partnered with the Clinic for Special Children (CSC) to learn more about MSUD risk factors and to develop a protocol, the first of its kind anywhere, to transplant children specifically with MSUD. The PCH protocol incorporated rapid turnaround in the lab for results of amino acid levels, educating nurses and support staff about the effects of MSUD, and having MSUD TPN available if needed. Developing a protocol of this nature was no small feat and it took the hospital, along with the assistance from CSC, eight months to establish. Meanwhile, Jake was listed on the United Network of Organ Sharing (UNOS) list in May.
At the time Jakob was listed, the UNOS ranking system spanned from negative 10, meaning non-critical and a likely extensive wait for an organ, to plus 40, which is a priority, to Status 1 meaning emergent. Jakob was initially listed at a negative 10 while PCH continued to develop the protocol. For us, his listing was a test of our patience since Jake would remain in a hold status until the protocol was in place. I made countless phone calls to our coordinator, Lynn Seward at PCH, to see how the protocol was progressing. It was frustrating to be listed knowing this wasn't even the real wait for the transplant, but I knew it was best, not just for Jakob, but also for the safety of everyone transplanted in the future with MSUD. While Jake was not in liver failure, his need for a liver to stop brain deterioration was still regarded as a priority given the fact that once brain damage occurs it cannot be reversed. Prior to completion of the protocol, PCH met with the UNOS board and successfully petitioned to get Jake's score elevated to a plus 40 status. This now meant our wait wouldn't be very long. Jake's score also established the precedent for other MSUD patients to receive an equally high score and to help our battle with insurance coverage, as it demonstrated just how perilous it is to live with MSUD.
In January of 2004 the protocol was complete and Jake came off the "hold" status... finally! Now the real waiting would begin. I can't fully explain to anyone who hasn't gone through this process just how tough the waiting is, and I feel for everyone that will go through it. Deciding to transplant is hard enough, but then to wait for it to happen is the most stressful ordeal I've ever faced. On Mother's Day 2004 we received the call we'd been waiting for. PCH had a liver for Jake. At that moment I went into what I'd rehearsed in my head for so many months, but it didn't really go the way I had planned. I remember feeling slightly disoriented like I couldn't quite get my act together. I called the charter flight company we had chosen to fly us to Pittsburgh and told them to fuel up the plane.
On our way out the door to drive to the airport, roughly an hour after the first call, we received a second call canceling our trip. PCH determined the liver was too small for Jakob so they passed it on to a better match. My husband and I sank on our front steps in shock, while all the adrenaline plummeted, but we agreed that things happen for a reason, and it was not Jakob's time.
At Midnight On May 29th 2004 we again received the call from PCH and this one proved to be the one that would end our waiting! Jake and I were visiting relatives in Charleston, South Carolina while my husband was at home in Virginia. This time everything went smoothly and it only took us 20 minutes to get ready and out the door to the airport! My mom, Jake, and I boarded a charter plane at 1:30 a.m. bound for Pittsburgh. The flight was frantic due to some bad weather but we made it to Pittsburgh by about 5:30 in the morning while my husband arrived by car shortly afterwards. When we arrived at the hospital, our coordinator, Lynn Seward, who by now had become a family friend, was standing there to greet us. It would take some time, and sedation, to get an IV started but once it was in we waited for them to tell us it was "time". I was thankful for the sedation because Jake was resting peacefully and he didn't notice me pacing the floor with tears in my eyes. Around 2:30 p.m. it was finally "time", so we walked, with Jake on his gurney, down the hall where we said our good-byes. Luckily he was really sedated so that part wasn't bad, but watching him go behind closed doors was now the most difficult thing I've ever experienced. In fact, writing about it now still makes me emotional.
Since the surgery happened on a holiday weekend, we didn't get updates like we normally do when the hospital is fully staffed, so every once in a while I would ask a nurse from the PICU for information. Everything was going well and by 9:00 p.m. Dr. Sindhi came in to the family surgical waiting area to tell us the surgery was a success! We would be able to see him. Jakob looked great right after his surgery. I was actually surprised at how good he looked. It was while looking at him in that PICU bed that I realized how really brave and strong he is, and all I could do was thank God for sharing him with me!
Jake's recovery went relatively smooth. He stayed in the PICU for 2 days, and then onto the transplant floor for another 8 days before being discharged. Every day he gained a little more strength and began to resemble the Jake I knew before surgery. We had some obstacles with recovery, but as each one would come up the Pittsburgh team would address it and we'd move on. I could list the issues we encountered but I think it's best not to get into the particulars. Let it suffice to say that everyone handles the surgery differently and the transplant team is the best source of information on how to best address any complications that may arise post-transplant.
A year after transplant our lives are totally different, and Jakob is doing better than we expected. We've noticed a great deal of improvement in his ability to focus, his speech, and lately, his gross motor skills. The medications have tapered off and I'm thankful he's already been weaned to such a low dose. I don't want to gloss over the trials of a transplant but I want to be honest about the changes that have occurred in our lives. Since transplant we've seen the progress in Jake intellectually. I watch him eat anything he wants with other children and he's now able to keep up with them physically. I can now rest assured knowing his future is brighter in regards to his physical, mental, and emotional well-being. Speaking for myself, and I know many of you can relate to this, ...now I can breathe!
Mom to Jakob, successfully transplanted
May 30, 2004
- Written by Susan Jasin