Parents: Transplant for disabled girl now a possibility at CHOP

Amelia Rivera, 3, with her mother, Chrissy, at home in Stratford.
Amelia Rivera, 3, with her mother, Chrissy, at home in Stratford. (MICHAEL BRYANT / Staff Photographer)
Posted: January 31, 2012

The parents of 3-year-old Amelia Rivera, the disabled girl who was initially rejected for a kidney transplant at Children's Hospital of Philadelphia, now say the hospital is willing to consider such an operation.

The Riveras said they met with medical personnel at the hospital for one hour Friday, after which they were given instructions on how to proceed with a possible transplant - including how to have family members tested as potential donors.

Chrissy and Joseph Rivera said they were not told whether their daughter, who goes by the nickname Mia, would be medically eligible. But the couple, of Stratford, Camden County, said Monday that they saw the meeting last week as a step forward from what happened earlier this month.

"As of now, we're taking this as a positive sign," Chrissy Rivera said.

According to the parents, a physician told them Jan. 10 that the hospital would not perform a transplant because of the girl's mental disability.

Amelia, whose condition is called Wolf-Hirschhorn syndrome, became the focus of national attention after her mother wrote of the initial rejection in a blog post. As of Monday evening, there were 37,000 signatures on a petition at change.org urging the hospital to change its initial decision.

On Monday, hospital officials said they could not comment on the case, citing patient confidentiality rules. The hospital has said it does not make decisions on whether to do a transplant based on a patient's intellectual ability.

Chrissy Rivera said her daughter's chart initially had the words mental retardation listed as a reason not to perform a transplant, and that those words had been removed. She said Amelia's case must now be further reviewed by specialists to determine whether she is a good candidate.

"If there's a medical reason" that a transplant would be a bad idea, she said, "of course we're not going to do it."

She said the family had health insurance but declined to be specific.

The cost of a kidney transplant can exceed $100,000, but in the long run is cheaper than dialysis, according to the American Society of Transplantation.

Wolf-Hirschhorn syndrome, caused by a missing piece of the fourth chromosome, results in cognitive delays and other developmental symptoms. It is estimated to occur in one out of 50,000 children, said geneticist John C. Carey, professor of pediatrics at the University of Utah School of Medicine.

Roughly 20 percent of children with the condition die in the first year or two, but once past that hurdle, they often live for decades, said Carey, who treats patients with the syndrome. One of his patients is 62, he said.

About 30 percent of children with the syndrome have some degree of kidney trouble, and maybe a third of them end up needing dialysis or a transplant, he said.

Carey said he was not aware of any patients who had received a transplant but said if one were to take place, a potential issue is that most such children also suffer from seizures and take anti-convulsant medicines. Amelia is among them, according to the family's blog posts.

Anti-seizure medicine can speed up the metabolism, making it harder to calculate the right dose of the immune-suppressing drugs that must be taken to prevent rejection of a transplanted organ, Carey said.

"To me, that just presents a bigger challenge," he said. "That doesn't present a reason not to do the transplant."


Contact staff writer Tom Avril at 215-854-2430 or tavril@phillynews.com.

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