A 6-year-old girl from Jacksonville, Florida is on her way to full recovery after receiving the first triple organ transplant operation performed on a child.
Doctors at Holtz Children's Hospital and the University of Miami Miller School of Medicine had performed a “combined” transplant of a liver, kidney and pancreas on Angela Bushi, who was born healthy but had developed insulin diabetes when she was just a year old which had eventually progressed to a rare autosomal recessive disease called Wilcott-Rallison Syndrome (WRS), according to the National Institutes of Health.
There are fewer 60 known WRS cases in medical literature worldwide, and the deadly disease is characterized by many health problems like diabetes, liver and kidney failure, bone and growth problems, repeated infections and intellectual impairment.
"This is a miracle what they did to her,” said Valbona Bushi, Angela’s mother had told the NBC Miami. Valbona said she was grateful because her other 18-month-old daughter had died from liver failure which was caused by the same disease a few months before.
In September 2011, Angela was starting to show signs of liver failure, and doctors had spent months trying to uncover Angela’s medical mystery. Testing later confirmed that Angela, like her sister, also suffered from WRS.
"She had the same symptoms as my other child who died, so we knew," Valbona said.
"Most children actually died during liver failure or complications due to liver failure,” Dr. Olaf Bodame, director of the pediatric genetics program at Holtz Children's Hospital told NBC Miami.
"Based on the syndrome, we knew that she would always be at increased risk for liver failure and renal failure, and her pancreas had already suffered some injury due to diabetes," said Dr. Bodamer, according to ABC News. "By replacing the liver, it could reduce the risk to zero for her to have future episodes of liver failure. It functions normally and does not carry the genetic defect."
Angela underwent the first-ever multiple organ transplant ever performed to treat Wolcott-Rallison syndrome on a child on Dec. 29. Surgeons had removed Angela's damaged liver, left all her other organs and transplanted the healthy organs on top.
"We remodeled the abdomen," Dr. Andreas Tzakis, one of Angela's transplant surgeons told ABC. "The other organs will stay, because if we had to remove them, it would have made the operation bigger.
The only signs of Angela’s December operation are a yellow feeding tube and a protruding stomach due to having two sets of organs, and her doctors said she is has been progressing well and on her way to full recovery.
"All the organs are functioning normally, and she has not needed any insulin," said Dr. Tzakis, according to ABC. "We are cautiously optimistic that she can grow to be an old person with her existing organs.
"She's back to normal like before. She's a normal kid," Valbona told ABC. However, Angela cannot go back to school and she has to be careful when interacting with other children who could be sick because her immune system is compromised.
Doctors will still monitor Angela's organ function, and even though they are optimistic they cautioned that there are no guarantees.
"We are taking it one day, one week and one month at a time," said Dr. Tzakis to ABC. "This is uncharted territory, so we don't really know exactly how to react. We have prepared for as much as we can."
Doctors said that Angela’s surgery might benefit other children in the future who might also need the same type of operation.
“It might give hope to children with this syndrome - that there might be at least one solution that can prolong their life. For the first time, we have a sense of enjoyment about this whole process. Seeing the child looking well and the family being relieved, this is the first time I feel joy for having done this procedure,” Dr. Tzakis told Fox news.