Pediatric Liver News
February 14, 2013

Hearing their infant daughter needed a new liver was the scariest thing the Sindlers had ever faced. Then they learned about Hopkins’ living related transplant program.

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 is common in newborns. Levels of bilirubin, a byproduct of old red blood cells that the liver breaks down, are typically higher in the first few days of life, causing benign temporary neonatal jaundice.

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 blockage in the ducts that carry bile from the liver to the gallbladder. Isabella’s liver was damaged and she would likely need a transplant. 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.”

When could be delayed with a Kasai procedure to surgically bypass the ducts and allow bile to drain from her liver, allowing Isabella to grow bigger and her immune system to get stronger. While not a cure for biliary atresia, Kasai does delay—and in about 25 percent of cases eliminate—the need for a transplant. But the earlier the procedure is done—preferably within the first three months of life—the better the outcome. Isabella was on the cusp of that window and, unfortunately, the Kasai failed. The Sindlers were back on the rollercoaster.

“We wanted to cry but we also wanted to be strong and get our daughter and family through this,” says Stephen Sindler. “The time for facing a liver transplant was now.”

Adding to their anguish was the fact that there are fewer donor organs for pediatric patients than adults. And getting an organ didn’t mean the battle was over as patients face organ rejection and other complications. But then they learned about the living related liver transplant program for children at Johns Hopkins and its excellent outcomes—a five-year survival close to 100 percent.

Pediatric transplant surgeon Andrew Cameron, director of the program, attributes that success to the high level of expertise and experience of Hopkins’ pediatric liver specialists, transplant surgeons and staff. He also notes that the program is “aggressively expanding,” potentially increasing the availability of organs 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.

Feeling more optimistic, the Sindlers elected living related transplantation for their daughter. Stephen’s blood type matched Isabella’s, and in February 2012 he donated a portion of his liver to her. 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 pediatric hepatologist Kathy Schwarz, director of the Pediatric Liver Center, 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.”