Upon his two-month wellness visit, however, Sam’s pediatrician noticed that his eyes looked “off”… not quite yellow, but not white. This was a symptom of jaundice, which is caused by the liver not removing bilirubin, a yellow pigment from the blood. After the results of blood work were in, Sam was diagnosed with biliary atresia.
Biliary atresia is a disease of the liver that affects newborn infants. It can lead to liver failure and the need for liver transplant within the first 1 to 2 years of life. It is the most common reason for liver transplantation in children in the United States.
The next step after diagnosis was the Kasai procedure, which gets its name from the Japanese surgeon who pioneered it. In the Kasai procedure, the bile ducts are removed and a loop of intestine is brought up to replace the bile ducts and drain the liver. As a result, bile flows from the small bile ducts straight into the intestine, bypassing the need for the larger bile ducts completely. The Kasai procedure can restore bile flow and correct many of the problems of biliary atresia. This operation is usually not a cure for the condition, although it can have an excellent outcome. Sam had the Kasai procedure done very soon after his diagnosis, and for the next several months, his condition improved.
The Grovers knew that the Kasai procedure was a temporary solution, however, and that a transplant was inevitable. As Sam started to feel better, they used that time to research transplantation centers. They began to hear more and more about Yale-New Haven Transplantation Center (YNHTC), specifically Sukru Emre, MD, and his team. The YNHTC expertise in both split liver and living donor liver transplantation was a particular draw, as was the excellent surgical outcomes.
Meeting the YNHTC team
According to Darien, “Our first visit with Dr. Emre and his team sealed the deal. In addition to feeling confident about their skill, we felt that Dr. Emre and the YNHTC team were a really good fit for us - we trusted them.” The fact that both Darien and Josiah had family in Connecticut was a bonus. Sam was listed for transplantation at YNHTC.
In anticipation of the eventual transplantation, Josiah, Darien and other family members underwent the extensive evaluation to see if any of them might be a match for a living donor procedure. Josiah was determined to be a match. In the event that a deceased donor liver would not be available at the time of the transplant, “there was not even a moment of hesitation on Josiah’s part that he would be the donor,” said Darien. The family anticipated that the transplant would occur in fall 2011.
But in August 2011, as Sam reached his first birthday, he developed a major gastrointestinal bleed. His condition accelerated the need for a transplant. A suitable deceased donor liver wasn’t immediately available, so Josiah became his son’s donor and the living donor transplant was scheduled for September 19.
On that day, Darien and Josiah’s parents and other family members were in the waiting area with Darien. They kept each other busy, playing cards and giving breaks to one another as they awaited progress reports. Darien said, “It was a very long day, but the YNHTC team, including the surgeons, transplant coordinators and social workers gave frequent updates and make sure that we were hanging in there. I had the same sense of security and trust envelop me as on the day we met Dr. Emre. I knew that we all were in good hands.”
The procedure went very well for both father and son. After the procedure Darien recalled, “Josiah’s recovery was particularly intense. With living donor transplantation, you’re taking a healthy person and putting them through a procedure that initially makes them feel like a train wreck. On the other hand, Sam had felt like a train wreck for so long before the surgery that he felt much better than Josiah did post-surgery.”
Today, both Sam and Josiah are doing fantastically. Josiah is back at work, and Sam is hitting all the important growth milestones he missed along the way, including learning to crawl and climb. Darien created a video slideshow to document Sam’s life-saving journey.
Darien considered what she would say to other parents in a similar situation. “One thing that helped us was being proactive. We knew that transplantation was inevitable, so we quickly began to research transplantation centers. There’s a point at which things can happen very quickly, so by putting ourselves in the right place as soon as possible, we felt well-prepared.”
Become an organ donor
And as they celebrate the outcome, the family is mindful of the importance of organ donor. “So many people suffering from disease that requires an organ transplant don’t have the blessing of meeting their match. That’s why organ donation is so important. I find that when I ask people if they are organ donors, some will say, ‘I will when my driver’s license is up for renewal.’ But the good news is, you don’t have to wait! Do it today, because organ donation saves lives.”
Learn more about how you can become an organ donor.