Girl Recovering After First Ever Quadruple Organ Transplant At Holtz
CBS Miami (con't)
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MIAMI (CBS4) – Her ruby red slippers and sparkly skirt glitter from across the room. Six-year-old Angela Bushi looks like a real live fairytale. In many respects, her life has been saved by a fairytale like rescue.
A team of doctors at the Holtz Children’s Hospital studied Bushi’s genetic composition and discovered she had an extremely rare condition called W.R.S or Wolcott-Rallison Syndrome. It is an illness that has killed just about every child that has it. But Bushi could beat it thanks to a quadruple transplant surgery that doctors performed on her, the first of its kind surgery ever done in the world.
“This is a miracle what they did to her and save her life,” said Valbona Bushi, Angela’s mother. “So I really appreciate it. All the doctors, they did the best job ever.”
Wolcott-Rallison Syndrome claimed the life of Angela’s infant sister, Lisa, after it went undiagnosed. Valbona Bushi said she pleaded with doctors in her hometown of Jacksonville to dig deeper and investigate. She said she knew something was wrong with Lisa’s organs but medical experts didn’t believe her until she came to the Holtz Children’s Hospital in Miami. That’s where genetics specialist Dr. Olaf Bodamer discovered the unusual genetic condition in Angela.
“It’s a very rare genetic syndrome. Last count in the medical literature last night was 82. So 82 published cases,” said Dr. Bodamer. “I think there are many more cases that go unreported and there are probably more cases that go undiagnosed. But still in 15 years of doing genetics in pediatrics and adult medicine I’ve never encountered a case before.”
Once diagnosed the team of surgeons strategized what to do about it. They decided to give Angela a new liver, pancreas and two kidneys, a total reconstruction of the child’s abdomen that left most of the old organs in tact as well.
“By replacing these organs, Angela is protected from liver failure and kidney failure,” said Dr. Andreas Tzakis, Director of surgery.
After 14 hours in surgery the team breathed a sigh of relief.
“We have a guarded optimism for Angela,” said Dr. Tzakis.
He said she can play and have fun like other six year olds. But she needs to stay away from children with viruses and infections as her little body continues to heal. Angela has an oxygen tube in her nose. Doctors said they don’t expect that she will need other surgeries in the future.