Children’s Hospital liver recipients give world of thanks

Still confined to a bed Wednesday at Children’s Hospital of Pittsburgh, Ali Al-Garni met the boy who saved his life.
“Every day when I take my medicine, I will remember you, because I’ve got your liver,” Al-Garni, 24, of Saudi Arabia, told Johnathan Devantier, 9, of St. Louis.

“Thank you.”

Last month, Al-Garni and Devantier underwent the world’s first “domino” liver transplant involving a child with maple syrup urine disease. Devantier suffered from the genetic metabolic disorder, which can make eating protein fatal.



The transplants, performed June 23-24 at Children’s Hospital in Oakland, were led by Dr. George V. Mazariegos, director of pediatric transplantation. To cure his disease, Devantier received a liver from a deceased donor. In a domino-effect, Devantier then gave his liver to Al-Garni.
Sixty-seven domino liver transplants have occurred nationwide -- only three involving patients with maple syrup urine disease, according to the United Network for Organ Sharing.

Maple syrup urine disease does not originate in the liver but is caused by a lack of enzymes throughout the body. A liver transplant allows patients to make enough enzymes to overcome shortages.

It also means that Devantier’s liver is suitable for someone without the disease. The liver accounts for just 15 percent of the flawed enzyme production in people with maple syrup urine disease; therefore, their livers are healthy enough to place in people with end-stage liver disease.

Al-Garni, who suffers from familial cholestasis -- a genetic disease that causes liver failure -- first came to Children’s in 1998, when he was 15. He returned to Pittsburgh four months ago because his condition deteriorated, and doctors in Saudi Arabia couldn’t help him. He was a perfect match for Devantier’s liver.

“As a mother, that is the best option possible,” said Dana Devantier, Johnathan’s mom. “Not only did someone else’s gift save Johnathan, but he was able to save another person.”

Al-Garni and Devantier are expected to recover fully.

Devantier was discharged from the hospital last week, but will be in Pittsburgh through August for monitoring by doctors at Children’s.

Al-Garni’s recovery is slower because he was much sicker than Devantier. He expects to be in Pittsburgh for several more months.

After Al-Garni thanked Devantier, there was an awkward pause. Then Devantier broke the ice.

“This is my scar,” he said, lifting his shirt to show the three long, red lines intersecting in the middle of his belly.

Al-Garni lifted his blue hospital gown, revealing the same red lines. Smiling, he said: “This is mine.”