December, 14 2005

By COLLEEN LONG.

HARRISON, N.Y.

See the story and photo gallery here.

Jacob Sontag is 9 years old. The blond, blue-eyed boy wears glasses and has a wide, happy grin. His mom and his teachers say he's one of the most popular students in second grade.

But Jacob can't talk much. He's wheelchair-bound and can't hold his head up, so it's strapped to the chair. Jacob has Canavan disease, a rare genetic disorder that eats away at his myelin, white brain matter crucial for development. Without treatment, kids with the disease lose sight and their ability to swallow, and eventually die. The disease affects only about 1,000 children in the United States.

"A rare disease is only rare until it happens to you," his mother Jordana Holovach said. She couldn't sit by and watch her son die, so she created a nonprofit, Jacob's Cure, which aims to fund research to help her son and other afflicted children grow white matter again. Fortunately, Jacob received gene therapy a few years ago that stopped the deterioration.

Holovach's home is squeaky-clean; she's worried about germs that could make her son sick. Their rapport is easy, a normal mother-and-son act, though often Holovach is speaking for two of them.

"You think mom's loosing her marbles, don't you?" she says with a laugh to her son as she wanders the kitchen tying to remember where she put the syringe for his medicine. "You're just laughing away at me. I'm so funny," she says as Jacob grins huge, showing his crooked teeth. He takes several medicines daily and can eat some foods, but they have to be soft because his swallowing mechanisms are not that strong.

During the day when he's at school, Holovach spends time writing letters to members of congress, or plotting her next fundraiser. She's testified before Congress on the importance of funding stem cell research, met with Sen. Hillary Clinton about it and filmed a public service ad.

Holovach is doing a good job so far -- she has raised more than $2.5 million through Jacob's Cure. Now she's trying to raise a million a year so Jacob and others can take part in new gene therapy trial.

Holovach is hopeful her son will improve.

"He's getting the messages. Someday we're going to have to deal with the effects of being in a wheelchair. Not of the disease anymore, but learning how to walk. I can't wait."