Toddler with a Rare Liver Disease Thrives After Lifesaving Procedure

March 24, 2017 7:00 AM

It took a nearly seven-hour surgery (called a Kasai procedure), but one tiny patient avoided the need for a liver transplant in his first year of life.

William Jouppi is known for dancing to the “beat” of the coffee grinder, singing in public and cuddling with his stuffed monkey.

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The 1 1/2-year-old also loves to eat, especially favorite foods like berries, eggs and crackers — all things his body couldn’t digest without a complex, lifesaving surgery at University of Michigan C.S. Mott Children’s Hospital.

Because a year ago, William’s liver was failing him.

“William was such a happy and calm baby, never fussy. You would never know something was wrong,” says his mom, Shelby Jouppi. “We didn’t know he was dying.

“It hit us like a ton of bricks.”

When William was born in fall 2015, doctors thought he had a bad case of jaundice. But he continued to turn yellow and became increasingly lethargic.

High levels of bilirubin in William’s blood prompted his pediatrician to send him straight to a hospital near the family’s home in Detroit. The 3-month-old was diagnosed with biliary atresia, a rare liver disease that blocks bile flow from the liver to the intestines.

Without bile, which breaks down fats and is key to digestion, William’s body couldn’t absorb necessary nutrients to live. The bile that can’t drain backs up into the liver and leads to liver damage. The condition affects about 1 in every 10,000 babies and leads to progressive, life-threatening liver disease.

His family had two options: a liver transplant or a complex surgery that could reduce the need for one. After researching several providers that offered the rare procedure, the family chose to travel from Detroit to Mott Children’s at Michigan Medicine.

“There’s no cure for what William has. All we could do was hope this surgery would improve his quality of life and make his liver work longer,” Shelby says.

“We did extensive homework on where to go, and there were some places where there was a likelihood the surgeon and team had limited experience with this procedure. We heard Mott had a first-rate pediatric liver transplant clinic. Thinking long term, we chose Michigan.”

William and his mother, Shelby Jouppi.

The surgery

In January 2016, a team led by Mott pediatric surgeons Erin Perrone, M.D., and James Geiger, M.D., spent nearly seven hours confirming the diagnosis of biliary atresia and rerouting William’s digestive system.

SEE ALSO: Tiny Heart Repaired While Baby Still in the Womb

During what’s called a Kasai portoenterostomy, surgeons removed the atretic biliary system and connected William’s intestine to the liver bed, allowing bile to drain directly from the small ducts at the edge of his liver straight into his intestine.

The procedure is successful in nearly two-thirds of patients, but the other third will require a liver transplant as an infant. Of the two-thirds of patients who have a successful Kasai, about a third of them will continue to have long-term success. The other two-thirds also eventually need a liver transplant, although sometimes not until they are adolescents or young adults.         

For William, the procedure was a success. While there is still a chance he will someday need a liver transplant, his hepatologist, Jacob Bilhartz, M.D., thinks a possible transplant would be years into the future.

“We put great trust in our surgeons, and we are so thankful that he was the recipient of so many years of expertise among everyone who took care of him. We feel very much at home at Mott and feel very loved and served by our nurses and doctors,” Shelby says

Today, the bubbly, blue-eyed toddler is “thriving,” his family says.

“William is living a normal, brilliant childhood and is so full of life. His first year has been full of joy and discovery, and he has brought so much love to our lives,” Shelby says.

“He has such an amazing quality of life, and we really see that being a direct result of the skill, dedication and hard work of the teams that took such good care of him. It’s an incredible gift, and we are so grateful.”

Top photo: Courtesy Shelby Jouppi - NOT FOR REUSE