Six-Year-Old Recovers from First-of-Its-Kind Liver, Pancreas and Kidney Transplant

For most children with Wolcott-Rallison Syndrome (WRS), life is short and filled with multiple problems, including diabetes, liver and/or kidney failure, bone and growth problems, repeated infections, intellectual impairment, and premature death.

Fortunately for 6-year-old Angela Bushi, doctors at the Holtz Children’s Hospital and University of Miami Miller School of Medicine diagnosed her extremely rare condition before many of the complications associated with the genetic disorder took her life, as they did her little sister’s. More importantly, just four days after Christmas 2011, transplant surgeons from UM/Jackson Memorial Hospital performed an innovative and unprecedented combined transplant of a liver, two kidneys and a pancreas while keeping her other healthy organs working well.

“This was the first time transplantation was used to treat this dreadful syndrome,” said Andreas Tzakis, M.D., professor of surgery, chief of the liver/GI transplant program and founding director of the Miami Transplant Institute. “It required a remodeling of the abdomen in a way never done before.”

A terminal disease that causes proteins to malfunction and keeps the body from fighting viruses, WRS is so rare that only about 60 children have been documented with it in medical literature. None has lived past 30, and because of concerns about complications with other organs, other centers that have diagnosed the syndrome considered but rejected liver transplants as treatment.

But by transplanting all four organs during the same operation, Tzakis and his UM/Jackson team minimized the complications and gave Angela a chance that may now be available to other children with this rare disease.

Born healthy, she developed type I diabetes when she was just a year old and living with her parents in Jacksonville. In September 2011, when she was five, she began to have flu-like symptoms. Ultimately, she developed liver failure, but her mother Valbona Bushi already suspected something was terribly wrong. Just a few months earlier, Angela’s 18-month-old sister Alicia had also developed flu-like symptoms, which quickly progressed to liver failure, ending her life.

With the doctors at two other hospitals stumped, Angela was transferred to Holtz Children’s Hospital, where she was determined to be too sick for a transplant. With a failing liver and no diagnosis, she was destined to die – until Olaf Bodamer, M.D., Ph.D., clinical professor of human genetics and UM’s director of clinical genetics, did the genetic detective work and pinpointed the culprit: WRS.

Soon Tzakis, Jennifer Garcia, M.D., assistant professor of pediatrics and a pediatric gastroenterologist, and Akin Tekin, M.D., assistant professor of clinical surgery, worked with the pediatric surgical, anesthesia, and critical care teams at Holtz to devise an innovative plan aimed at sparing Angela her sister’s fate.

On December 29, about a month after the child was placed on the transplant list, Tzakis and his team removed her diseased liver and gave her a new one from a deceased donor, plus a pancreas and two kidneys. The child’s own two kidneys and pancreas were left in place to spare her even more surgery.

Though she had a difficult postoperative course, Angela has completely recovered – she doesn’t require insulin anymore – and was discharged from Holtz on February 23.

Other members of the UM/Jackson team who made her discharge possible include: Barry Gelman, M.D., professor of clinical pediatrics, and Michael Nares, M.D., professor of clinical pediatrics, both of the Pediatric Intensive Care Unit; Stephan Klumpp, M.D., assistant professor of anesthesiology; resident Matthew Nunnelley, M.D.; and Tangilar Dorsett, RN, B.S.N., clinical care coordinator, Antoinette M. Behlar, RN, clinical transplant coordinator, and Judith McCullough, Ph.D., clinical psychologist, all of the Jackson Health System.

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