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Liver Transplant Cures Patient with Rare Congenital Disorder

AustinA dream came true for 11-year-old Austin Sprock.  All his life, Austin had to stick to a low-protein diet. He had Maple Syrup Urine Disease, a rare inherited disorder causing an inability to process protein properly. The name comes from the sweet odor of the urine. If untreated, the disease is life-threatening, and severe disability and paralysis can occur.

But on discharge day at St. Louis Children’s Hospital Feb. 13, all that changed. Austin got to eat his first bite of ice cream — and all he wanted from a monster bucket containing 20 scoops of Ben & Jerry’s.

Austin was effectively cured after receiving a liver transplant at St. Louis Children’s Hospital.  For the past two years, the family had been considering a transplant for Austin. After assessing the risks, it took just six hours for the donor organ to arrive.

The first documented case of such a cure involved an 8-year-old girl with the same condition who received her liver transplant in 1997 at another hospital. That transplant, however, was actually for an unrelated medical condition, but it stopped her symptoms. Clinical follow-up in subsequent years showed a positive outcome for her and other children with the disease who received liver transplants. Amino acid levels stabilized within hours of transplant and remained stable despite a substantially higher intake of protein.

Austin savored the flavors — chocolate, vanilla, peanut butter, banana — in front of a roomful of family and hospital staff. Child life specialist Stacy Sedlack, CCLS, CIMI, started planning the party after Austin’s transplant evaluation, where he said ice cream was the one thing he was most excited about eating. One day when Sedlack was checking on him in the pediatric intensive care unit, Austin, half asleep, opened his eyes, saw her and just said, “Ice cream.”

“It was pretty apparent he was excited about the party,” Sedlack says.

His whole life, Austin has had to drink a nutritional formula and maintain a low protein diet. “Now he can have anything,” Sedlack says.

Austin’s mother, Tina Sprock, says that diet, stress, illness, heat and other factors would affect Austin’s metabolism 100-fold, and he would have to return to the hospital for what would be everyday episodes for other children. With each illness and hospital stay, he grew sicker.

Now for Sprock, a cold or flu will no longer be cause for alarm — it can be managed at home.

“I have nothing but wonderful things to say about Children’s,” Sprock says. “It’s truly a team effort, and they include the parent every step of the way. If I ask for more information, they give it to me. The nurses here are the best they’ve ever been since we started coming here more than 11 years ago. They supported us and pushed Austin to get better and stronger.”

“Austin has been very involved in his own medical decisions, especially when it came to his transplant,” says Lisa Gildehaus, RN, BSN, liver and kidney transplant coordinator. “His parents and his extended family also provide a great support system, which will no doubt help Austin continue a successful recovery.”

Toward the end of the celebration, Austin and his family asked members of the hospital team if they wanted some ice cream, too. When Jeff Lowell, MD, Austin’s doctor, thanked the family for the serving, their immediate reply reflected their sentiments toward the team that helped them: “No — thank you.”

 

Pediatric Liver Care and Transplant Center