For the Bushis it was like re-living their worst nightmare when just a few months after they lost their daughter Alicia to liver failure, their younger daughter Angela’s organs also began to fail. But thanks to doctors who conducted a first ever quadruplet organ transplant, the six years old is alive and thriving.
“When you see your children in this situation, and there’s nothing you can do, it is so hard,” said Angela’s mom, Valbona Bushi. “We went through a lot.”
Angela was born healthy but developed Type 1 diabetes when she was barely one. Gradually as the girl turned four she developed flu like symptoms which was soon found to be acute liver failure.
“This was kind of an unusual combination of acute liver failure and diabetes,” said Dr. Olaf Bodamer, director of the pediatric genetics program at Holtz Children’s Hospital, who eventually diagnosed Angela. “We knew that her sister had died under similar circumstances, so we assumed that her condition was genetic. When you go to the medical literature, the list of syndromes that came up to explain her condition was very small.”
The doctor’s persistence paid off and he was finally able to uncover what Angela was really suffering from – Wilcott-Rallison Syndrome (WRS), an extremely rare disease with only 60 known cases in medical literature worldwide. The syndrome led to many problems at once including diabetes, liver or kidney failure, growth problems, bone problems, repeated infections and impairment.
For most children the condition was fatal.
A team of geneticists and surgeons at Holtz Children’s Hospital and the University of Miami Miller School of Medicine/Jackson Memorial Hospital began to look for solutions to save the girl’s life.
The doctors quickly found that in Angela’s case it was not going to be enough to transplant just one organ.
According to Dr. Andreas Tzakis, chief of the liver/GI transplant program at UM’s Miller School of Medicine and Jackson Memorial Hospital, and one of the transplant surgeons who operated on Angela,
“The risk to her life was from three (different) organs – the liver, pancreas, and kidneys. If there was to be a solution, we knew we’d have to combine these (three different types of) organs into one transplant. We had several consultations within the team, outside the team, and the parents; and collectively, we decided the transplant was the way to go.”
Finally on December 29th Angela received the first ever quadruple organ transplant. All the organs came from a single donor to reduce complications.
Angela’s liver, pancreas and kidneys came as a ‘block’ as if they were just one organ. Medical experts say that previously, a liver transplant was considered as an option to treat WRS but was never done as the risk involved was great and there were chances that other organs could reject the treatment. But the transplant in Angela’s case was done with four organs at once which helped reduce the complications of the process and gave more chance of survival.
Angela not only became the first child to be operated for WRS, but also had a first ever quadruple organ transplant.
Angela was treated with anti-rejection drugs initially but now has fully recovered and was released from the hospital in February. She also does not need insulin anymore.
The only evidence of her innovative surgery is a slightly protruding belly, which houses two pancreases, a new liver and four kidneys.
“The surgery itself might benefit other children who might need this combination,” Tzakis said. “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.”
The Bushi family is just happy to have their little one back home.
“She’s a normal child again, and we can do everything like normal parents,” Valbona said. “She even wants to be a doctor when she grows up.”
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