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US News

GENE FIND IS RAY OF HOPE FOR KIDS WITH RARE ILLNESS

New York families with children afflicted with a rare genetic disease are cheering the discovery of the gene that causes the illness.

Scientists say one in 30 Ashkenazi Jews carries the gene for familial dysautonomia, a disorder that strikes the autonomic and nervous systems, impairing swallowing, breathing, heart beat and sensations.

For Steve Kietz, 42, the scientific breakthrough – announced last week by researchers at Massachusetts General Hospital – means his son Josh, 9, who suffers from FD, might one day be cured.

“For Josh, we all dream of genetic therapy,” said Kietz, who lives in Syosset, L.I.

“Finding the gene was our only hope.”

Josh cannot swallow food and must receive all his nutrients through a feeding tube connected directly to his stomach.

Children with the disease struggle with a variety of physical disorders which can also cause social and learning problems.

Many also suffer from chronic respiratory infections that can cause death.

When the disease was discovered in the 1950s, only 50 percent of those with FD lived past the age of five. In recent years, early diagnoses and medical advances have improved the quality of life of children afflicted, with half living past the age of 30.

Though only 500 people worldwide suffer from FD, a small group of families has galvanized much of the genetic research and innovations in treatment, through fund raising and advocacy.

“I think this is going to open up new ways of approaching treatment and give new hope to the individuals who are affected,” said Dr. Felicia Axelrod, director of the Dysautonomia Treatment and Evaluation Center at NYU.