Liver comes through for Spence

Published 12:00 am Wednesday, June 21, 2006

Sixteen-year-old Evan Spence was flown to Children’s Hospital of Pittsburgh Wednesday morning for an immediate liver transplant.

Late Wednesday afternoon, the news was confirmed.

“He is having the transplant and is in surgery right now,” Marc Lukasiak, public and governmental affairs representative at Children’s Hospital of Pittsburgh, said.

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Evan suffers from a rare and life-threatening disease called Crigler-Najjar Syndrome Type 1, which necessitated the inevitable need for a liver transplant, according to medical experts on the rare genetic disease.

At press time, Evan was said to be out of surgery and in the Intensive Care Unit recovering at the hospital in Pittsburgh.

Although the transplant is said to be complete, according to sources, Evan will still be in the hospital for a considerable amount of time.

“They said once he has the surgery, we will not leave for three or four months,” Joan Spence, Evan’s mother, said in an earlier interview.

According to the Crigler-Najjar Syndrome Association, there are less than 200 cases reported of the disease out of the 6.5 billion people in the world.

An enzyme in the liver that helps excrete a byproduct from depleted red blood cells from the body called billirubin, is completely absent or is extremely scarce in someone who is born with Crigler-Najjar Syndrome Type I.

Why is the enzyme so important?

Someone with Type I of this disease stores billirubin in their body – starting in places including the liver and gall bladder.

When the amount of unconjugated billirubin reaches toxic levels, the body can begin storing it in the brain.

This can occur when it is on-set by kernicterus, which can cause brain damage and severe harm to the nervous system, and can be fatal.

“God has his hand on him,” Joan Spence said.

Evan will begin his junior year of high school at Demopolis High in the fall of this year.

“I want to be an audio engineer or be in a band,” Evan said in an earlier interview. He plays the electric bass in the DHS marching band.