Triple organ transplant saves young girl with rare disorder

by Symptom Advice on March 24, 2012

Jackson Health System

Angela’s father Petrit Bushi, Angela and her mother Valbona, smile after the 6-year-old girl received a new liver, two kidneys and pancreas.

By Rita Rubin

Valbona Bushi, 28, knew even before the doctors did: her daughter Angela’s liver was failing, just as Angela’s younger sister’s had.

At first, emergency room doctors dismissed the kindergartener’s flu-like symptoms as nothing serious. but the next day the 6-year-old’s abdomen was slightly swollen. Bushi, whose 18-month-old daughter Alicia had died of liver failure just a few months earlier, rushed Angela back to the hospital near their home in Jacksonville, Fla. after tests revealed the little girl needed a liver transplant and she was referred to Holtz Children’s Hospital, affiliated with the University of Miami Miller School of Medicine.

A new liver would save Angela from imminent death, but doctors wanted to do more — to try to actually extend her life. Angela, whose parents had moved to Jacksonville from Albania seven years ago, had been newly diagnosed with a rare, fatal genetic disorder called Wolcott-Rallison Syndrome. only about 60 cases of the disorder – which causes infant-onset diabetes and liver failure, as well as bone fractures, intellectual impairment and frequent infections — have been reported. only one of those children lived into young adulthood. It had killed Angela’s younger sister and damaged Angela’s organs.

On December 29, in an unprecedented multiple-organ transplant on a child, Angela received a liver, two kidneys and a pancreas, doctors said.

 The operation – the first time a transplant has been used to treat the disorder —  “might offer some hope for these children with this rare syndrome, that life can be prolonged, hopefully in a very meaningful way,” Dr. Andreas Tzakis, transplant surgeon, told Thursday. Tzakis is the chief of the liver and digestive tract program at Jackson Memorial Hospital.

A viral infection, the kind most kids easily bounce back from, caused Angela’s liver failure, said Dr. Olaf Bodamer, director of the pediatric genetics program at Holtz Children’s Hospital. In children with Wolcott-Rallison, a protein doesn’t function normally. When patients contract a virus, their cells are unable to clear the resulting waste products, Bodamer explained. “Theoretically, any organ could be affected,” he says, but the liver, pancreas and kidneys are most vulnerable.

Angelad was diagnosed with Type 1 diabetes as as a baby, but, since her transplant, is no longer diabetic.

Her post-operative course “was tougher than expected,” says Tzakis. but she recovered and was discharged from the hospital on Feb. 23.

Now, Angela can’t wait to return to kindergarten which she had attended for only two weeks before becoming ill, her mother said.

“Every day she says, ‘I miss my school,’” Bushi says. Angela’s return to the germy world of elementary school will have to wait until she’s more stable, but Tzakis has “guarded optimism” that the transplant operation will halt or slow the normal course of her disorder.

“they saved my daughter’s life,” Angela’s mother Valbona told

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