Countering Biliary Atresia: Sarah’s Story

Five-year-old Sarah Sirpenski loves watching TV shows about baking and can’t wait to give it a try herself. She enjoys spending time in her family’s kitchen, measuring imaginary cups of sugar and other ingredients, say her parents, Kate and Dan. One of his doctors even calls him “corn muffin” – although that fancy nickname has a far more serious inspiration than just a sweet treat.

“You would never know what she went through just by looking at her,” Dan says.

From zero to sixty

When Sarah was born she seemed a bit jaundiced, a common occurrence in many infants.

But when a blood test revealed high levels of bilirubin – a yellow substance found in bile – his pediatrician recommended further evaluation to assess the health of his liver and gallbladder. Ultimately, the family had a probable diagnosis: bile duct atresia.

In biliary atresia, the most common cause of liver disease in newborns, a tube called the common bile duct is blocked or damaged, blocking the flow of bile. This can quickly lead to malnutrition and liver damage. Because biliary atresia can only be confirmed with surgery, Sarah had to undergo a Kasai portoenterostomy, a procedure that restores the drainage of the bile and connects the liver to the intestine. Her doctors referred her to the Center for Childhood Liver Disease at Boston Children’s Hospital.

“It seemed like one week we were talking to the Boston Children’s team and the next week Sarah was having surgery,” Dan explains. “Everything went from zero to sixty like lightning. “

I’m just trying to survive

There was a reason time was running out: Even with surgery, liver damage associated with bile duct atresia may ultimately require a liver transplant. The Kasai procedure – performed by Dr Heung Bae Kim when Sarah was barely 2 months old – went extremely well, and for a while it seemed like Sarah was in the clear.

But this procedure cannot cure bile duct atresia. Instead, many children who have had a Kasai portoenterostomy early in life will still need a liver transplant because the inflammatory process in biliary atresia involves the bile ducts inside and out. outside the liver. Children with biliary atresia are also prone to cholangitis, an infection of the bile ducts.

A few months later, Sarah herself suffered from cholangitis. She had to drain her bile ducts, needed antibiotics intravenously, and had a feeding tube to make sure she was getting enough nutrients. In an attempt to gain weight, she munched on high-calorie Dunkin Donuts corn muffins, which inspired her by the hepatologist Christine Lee’s nickname.

“This first year and a half has been really tough,” says Kate. “We felt like we were on a treadmill going nowhere – just trying to survive.”

Delay transplantation

All in all, Sarah spent about three months as an inpatient at Boston Children’s. Although she finally started to improve, the specter of a transplant loomed. “Dr. Kim was always trying to push the goal post,” Dan explains. “We all wanted Sarah to celebrate her third birthday before she needed a new liver.” When it seemed like a transplant was inevitable,

But even as Sarah and her family prepared for the transplant, her clinicians still had a trick up their sleeve. Dr Kim and his colleague, surgeon Dr Khashayar Vakili, believed they could use an innovative surgical approach that could delay the need for a liver transplant. Sarah’s parents agreed to proceed and the operation was performed successfully and worked as expected.

Beat the odds

So far the procedure has been successful and Sarah has been able to delay a transplant. She has an incredible memory, often citing movies and loves making people laugh. She also enjoys spending time with her older sister, Norah, playing with her dog, Grace, and learning to ride a bike. “She just wants to keep going faster and faster,” Kate says. Although the future is still uncertain, Sarah has already beaten all odds and her parents are grateful for her care.

“Boston Children’s has always been there to support us,” says Kate. “Some families travel around the world to come here. We’re lucky it’s in our own backyard.

Learn more about the Childhood Liver Disease Center.