Adam was born August 21, 2008, in Ottawa, at the Queensway Carleton Hospital (QCH). He is the younger of our two boys. Within a few days of his birth, our very sharp pediatrician (Dr. James McConville) noted that Adam’s bilirubin levels were off but not in the typical way that is seen with neonatal jaundice. Further testing ensued and Adam was diagnosed with biliary atresia. A condition in which one or more bile ducts are non-functional or absent, biliary atresia is a progressive disease in which bile backs up and leads to more and more damage to the liver leading eventually to liver failure.
We were referred to Dr. Carolina Jimenez, a member of the amazing GI/liver team at CHEO and we learned that to manage this condition, surgery of one form or another is required. At 6 weeks of age Adam underwent the Kasai procedure, a surgical attempt to find an alternative way for the bile to leave his liver by using part of his small intestine. The surgical team at CHEO was incredible, the procedure looked positive at first but as the statistics show, the Kasai procedure just wasn’t enough.
However, at that time, our little guy was pretty stable and a relatively happy baby but we knew his condition was progressing. Unfortunately, at 4 months, we were told by the medical team, “Adam is in liver failure and Adam will need a liver transplant…” and, “…since both of you have a blood type compatible with Adam, either one of you can be considered as a live liver donor.” We determined that, as a 6 month old, Adam would do best with his mom by his bedside and so I, Adam’s dad, went through a battery of tests to ensure he was otherwise suitable to be a live liver donor. He was found to be a suitable match. In came the amazing teams at Toronto General Hospital for dad and The Hospital for Sick Kids for Adam.
The liver transplant ended up taking place on Feb. 12, 2009 when Adam was 6 months of age. After hours of surgery, both patients recovered smoothly. Adam’s jaundice started to disappear the next day and he became a bright smiling charmer at Sick Kids. He did so well that he was discharged two weeks post transplant.
The first year was a bit rough, including many medications, challenges on his bloodwork and many trips to Sick Kids and CHEO. However, eventually all but one daily immunosuppressive medication was required and Adam was thriving.
The second part of our story began in 2013, when Vicky Ng, an amazing doctor and researcher at Sick Kids approached us with the idea of recruiting Adam into a study called the iWith study (Immunosuppression Withdrawal for Stable Pediatric Liver Transplant Recipients). The purpose of this study was to determine if it is safe to slowly reduce and stop the immunosuppressive drugs in some children who have had liver transplants. After numerous tests, including a liver biopsy, Adam was accepted into the study. The study began with a slow reduction in the dose of Adam’s medication along with Adam having bloodwork every two weeks to monitor for signs of rejection. After a year of slowly decreasing his immunosuppressive medication he was completely off of it. Follow-up biopsies were performed to ensure his liver remained healthy and showed no sign of rejection. Adam is now a liver transplant recipient free from all medication. This is incredibly rare and an amazing example of future possibilities in liver and transplant medicine.
Heather – Mom & Caregiver