At birth, Isabella Sindler’s skin had the characteristic yellow color of jaundice. Her parents, Stephen and Teresa Sindler of Hampstead, Md., were told by doctors at the local hospital where Isabella was born not to be alarmed because the condition was common in newborns. Over the next two months, however, the jaundice persisted and Isabella had trouble gaining weight, which led to a feeding tube, more tests and, ultimately, a diagnosis of biliary atresia—a rare condition marked by abnormally developed and non-functioning bile ducts.
Isabella underwent a Kasai procedure to surgically bypass the ducts and to allow bile to drain from the liver. However, the procedure failed to normalize bile duct function, which happens in about half of infants undergoing the procedure. Isabella’s liver was damaged by bile build-up and she would likely need a transplant, the doctors told the Sindlers. The parents, both paramedics, were stunned.
“At first we were on a rollercoaster of emotion,” says Stephen Sindler. “We knew nothing and did what every parent would do—we searched the Internet for everything we could find out about biliary atresia and what to expect. We knew she would need a liver transplant—it was only a question of when.”
Adding to the parents’ anguish was the scarcity of donated organs for children, a far more acute problem in pediatrics than in adult medicine. And getting an organ didn’t mean the battle was over as patients face organ rejection and suppressed immunity, among other complications. Then, just as things were beginning to look dismally hopeless, the Sindlers found out about the living-related liver transplant program for children at Johns Hopkins. Nearly all patients survived past five years—an excellent outcome by any metric. Pediatric transplant surgeon Andrew Cameron, the program’s surgical director program, and Kathy Schwarz, director of the Johns Hopkins Pediatric Liver Center, attribute that success to the high level of expertise and experience of Hopkins’ pediatric liver specialists, transplant surgeons and staff. They also note that the living related liver transplant program is “aggressively expanding,” potentially increasing organ availability for patients like Isabella.
“We’re very lucky to have a sophisticated and dedicated senior group of pediatric gastroenterologists and hepatologists, a real asset as we move forward,” says Cameron, citing Schwarz and pediatric hepatologists Wikrom Karnsakul and Douglas Mogul, one of whom is always on call for transplant.
Feeling more optimistic, the Sindlers elected living related transplantation for their daughter. Stephen turned out to be a close match, and in February 2012 he donated a portion of his liver to his daughter. The operation was a success, though Isabella did experience some complications, not uncommon in these cases. This is where experienced and multidisciplinary post-operative management, Cameron notes, comes into play.
“We have a fantastic pediatric intensive care unit here, and the ability to integrate pediatric hepatologists in immune suppressive management,” says Cameron.
The Sindlers were further reassured by Schwarz, who walked them through the living donor transplant process.
“She put everything on the table, made it easy to understand, which was very calming,” says Stephen Sindler. “She kept us in the loop, treated us 100 percent as part of the team, encouraged questions anytime and was always accessible, even on a Sunday night.”
How is Isabella doing today?
“She’s finally doing fantastic,” says Teresa Sindler. “She’s still a little peanut, but she’s eating and gaining weight and no more feeding tube. I give a lot of credit to the physicians, who made Bella confident in herself. She walks into Hopkins Children’s Center like she owns the place and everyone eats her up.”
The Sindlers were also very helpful to a family who were new to the Pediatric Liver Center. In May 2012, Schwarz saw Abigail Bahr for nosebleeds, referred by an otolaryngologist who initially saw the child. A low platelet count signaled possible liver disease and Abby was referred to the Liver Center. The physical revealed enlarged liver and spleen and subsequent blood work and liver biopsy revealed autoimmune hepatitis as the culprit. Abby began treatment, to which she initially responded well but eventually started to develop fevers and ascites. It was time to talk about a new liver. Schwarz carefully reviewed the types of transplant (cadaveric and living donor). Anxious to avoid further complications of end-stage liver disease, the family proceeded with the latter option. Mom Jenni was a match, and the transplant was planned.
The opportunity for the Bahrs to meet the Sindlers and to see Isabella bright eyed, adorable, and healthy assuaged their worst fears and made the journey much smoother. On Oct. 28, Abby underwent liver transplantation. Both mother and daughter did well. Three months later, Abby’s main problem is being bored silly, and she can’t wait to get back to school and start playing soccer again!
Abby’s and Bella’s experiences are illustrative of a bigger trend. A recent report from Johns Hopkins published in the journal Pediatric Transplantation revealed that over the last 20 years, patient survival rate following living donor liver transplants at Johns Hopkins jumped from 82 percent to 100 percent, a momentous achievement in and of itself, made even more remarkable by the fact that the perfect survival scores were achieved without a notable increase in cost.