Community Rallies as Girl Fights Rare Illness

One twin dies of a rare condition; other faces long odds at survival.

ByABC News
April 14, 2009, 5:13 PM

April 15, 2009 — -- As Darlene Colclasure waited for her student Taylor Ramey at the local shopping mall in Asheville, N.C., she considered what kinds of shoes the two might look for for Taylor's upcoming prom.

"Silver, I think," Colclasure said. "To match the sparklies on her dress."

As one of Taylor's dedicated assistants at Clyde A. Erwin High School in Asheville, Colclasure is doing her best to offer some support to the 16-year-old and her family after the death of Taylor's twin sister Brittney last Friday.

Colclasure is just one of many people in the Asheville community to rally around the Rameys and offer help and support at a time when the outlook for the family is bleak.

Brittney died after a six-year battle with a rare genetic disease called myoclonic epilepsy with ragged red fibers, or MERRF. And as rare as it is, it is a disease that will almost certainly claim Taylor's life as well in about a year.

"She was an extremely courageous girl," said their father, Gary Ramey, 61, of Brittney.

"She had a sharp wit. ... She didn't ask 'Why me?'"

MERRF is a disease that affects the mitochondria -- the energy factories of the cell. The condition inspires questions because it is rare. The disease affects only two to 15 people in a million, making it difficult to gather a large enough sample size to study.

And while mitochondrial diseases may not be serious if only a small percentage of the body's mitochondria are affected, after a critical mass of mitochondria are mutatated, the disease can progress rapidly and is fatal.

"The severity of the disease varies from mild to terrible," said Dr. Salvatore DiMauro, professor of neurology at Columbia University Medical Center in New York.

The Ramey family experienced the full range of MERRF.

"Until they were 10, medically speaking, they had a perfect existence," said Ramey, who could not recall the twins having so much as an earache as children. But one evening Britney complained of a "jerking" sensation in her head.

"Over the course of four months it amplified into grand mal seizures every four days," Ramey recalled.

Brittney was treated for epilepsy but she did not respond well and the family's neurologist suggested a genetic test. Several months later, the clinic called to explain what MERRF was.