Pursuing Progeria: Worldwide Effort to Find Children with Rare Condition

Aug. 27, 2010 -- December 13 marks a sad anniversary for Heather Barnett. Her son Ory died that day in 2006 after he suffered a heart attack during a minor surgical procedure.

"He loved Legos, Star Wars and cartoons, like other kids."

Ory was only 10 years old when he died, and by that age, Heather said he had already been through a number of health crises, including a stroke in 2005 as well as seizures and a series of mini-strokes.

Ory suffered from progeria, an extremely rare and fatal disease characterized by what resembles premature aging in children.

They look normal when they're born, but by age 1 or 2, children wih progeria start to lose their hair, fail to gain weight and start to become progressively more aged in appearance. They also tend to develop brittle bones.

Children with progeria generally don't survive past age 13.

"By age 10 or 12, there are signs of heart disease and hardening of the arteries," said Dr. W. Ted Brown, director of the New York State Institute for Basic Research, who has been studying progeria for the past 30 years.

The Progeria Research Foundation says that the disease strikes only about 1 in every 4 to 8 million newborns around the world.

Right now, there is no treatment for progeria and only as recently as 2003 has a gene mutation been identified as the cause. Because the disease is so rare and very little else is known about it, the Progeria Research Foundation recently launched a campaign called "Find the Other 150." The campaign's goal is to find the other 150 progeria children around the world that scientists believe exist so they can have access to care, participate in clinical trials and get support from other families of children with the disease.

Scientists arrived at the estimate of 150 based on the incidence of progeria and the overall world's population.

"If the incidence is really 1 in every 4 to 8 million, it comes out to be about 150 more," said Audrey Gordon, president of the Progeria Research Foundation.

So far, 67 children around that world have been identified and diagnosed, including 16 in the United States. The Progeria Research Foundation said that 13 of those children were identified as a result of the campaign.

"It's been more successful than we ever imagined," said Gordon.