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Children's has triple transplant into 3-year-old

Saturday, December 12, 1998

By Anita Srikameswaran, Post-Gazette Staff Writer

Surgeons from Children's Hospital announced yesterday that they have performed the world's first transplantation of a heart, liver and lungs into a child.

Bremdpm Ednie, 3, at Childrens Hospital 

The 20-hour surgery, which was developed to meet the unique needs of 3-year-old Brendon Ednie, started on Aug. 21 and finished the following morning. Doctors waited for the boy to be medically stable before making the procedure publicly known.

Brendon, of Daytona, Fla., has a hereditary disease called Alagille Syndrome that made his liver, heart and lungs fail. He was born without a pulmonary artery, which is the large vessel that channels blood from the heart to the lungs for oxygenation. His liver lacked small bile ducts, leading to high blood levels of bilirubin and jaundice.

"The first time I saw Brendon in the office before the transplant, I was shocked," said Dr. Frank Pigula, who performed the boy's heart and lung transplants. "He was green. I've never seen anybody this color."

The bilirubin elevations also made the boy extremely itchy. "He had scabs all over. It was heart-rending to see him," Pigula said. Brendon first came to Pittsburgh in October 1997. He spent the next 10 months waiting for suitable organs to become available.

Just before the surgery, Brendon was playful, cruising around his home and building a vocabulary, despite the fact that the dark yellowing of the "whites" of his eyes signaled an urgent need for transplant, said his father Bryon Ednie, a correctional officer for the state of Florida.

"He was complex from the moment he was born," Ednie said. "We had to learn to deal with it because that's what we were given. We had an unhealthy child and we decided to try and make him as healthy as possible and enjoy him as long as we had him."

Brendon's best bet was the unusual multi-organ transplant.

The surgery was done two years ago for one adult in this country, according to the United Network for Organ Sharing. A few more adults have received the same multi-organ transplant in the United Kingdom. Davina Thompson, of London, received a heart, lungs and liver in 1986, and was the longest-living survivor of the procedure until her death in August.

"We don't know how long he is going to live for. It's too early to say," said Dr. Jorge Reyes, who performed Brendon's liver transplant. "Our expectation is he will have a long life."

The surgeons transplanted the heart and lungs first, followed by the liver. All the organs came from a single donor. Brendon's liver transplant could not succeed without support from a healthy heart and lungs. If the heart and lungs had been transplanted alone, there was a high risk that his diseased liver could not make the clotting factors needed to stop bleeding during the operation. So, all of the transplants were performed in one long procedure.

The operation went smoothly and Brendon was awake and sitting up a few days later. He has not rejected any of the organs, but he did suffer a setback shortly after the surgery because of a bowel infection, his father said. Now, with some oxygen and respiratory support, the boy is breathing on his own, playing and watching "Teletubbies."

He will likely be discharged from the hospital and entering the rehabilitation program in the next week to 10 days, the doctors said. Multiple organ transplants require even more care than the sum of their parts would dictate, so Brendon will be watched closely for a while.

"We're on our toes and we will be working with Brendon and his family to get him through this with a normal life," Reyes said.

The doctors don't know if they will need to do a procedure like this again, but they said they would not hesitate if an individual required it to get well.

Fewer than 100 children and adults have been diagnosed with Alagille Syndrome worldwide. Along with Brendon's problems, the genetic defect can also cause eye and kidney abnormalities, butterfly-shaped vertabrae and developmental retardation. Some affected people may have a prominent, broad forehead, deep-set eyes, straight nose and small pointed chin.

According to Dr. Carolyn Bay, a geneticist at Children's, the responsible gene has been recently identified and localized. The syndrome has been a research focus at the Children's Hospital of Philadelphia during the past year. A mutation in the Jagged1 gene on chromosome 20 can create a range of problems, from mild to severe, in the affected person, she said.

With Alagille Syndrome, only one parent has to pass on a defective gene for the child to be affected. A child with a disease such as cystic fibrosis inherits two copies of the problem gene, one from each parent.

Genetic tests to identify the disease are currently used only in research settings, Bay said.

Ednie has not been tested for it, but he already knows Brendon's disease comes from his side of the family. One of his sisters died from liver failure and another had a liver transplant here 16 years ago.

"She's fine now," he said. But "they never diagnosed her with anything. That's why we didn't know it was hereditary." The Ednies also have a 5-year-old daughter, Rachell, who is healthy.

Ednie went back to Florida yesterday to see his daughter, now living with her grandparents in Orlando, and to return to work, while wife Rebecca remained with Brendon.

They intend to keep Brendon here as long as necessary, Ednie said.

"We just want him to come home the healthiest he can."

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