Saturday fund-raiser hits close to home for one of its organizers

August 07, 2002|by RICHARD BELISLE

BURNT CABINS, Pa. - Tawnie House knows the horrors of Huntington's disease, an always fatal malady that attacks families because it is passed down through generations.

House doesn't have the disease but her great-grandmother died from it, as did her great-grandmother's two sons. House's own mother died from it, as did her mother's brother. House's oldest brother committed suicide when he learned that the gene had passed down to him.

"When one member of the family gets it, the whole family owns it," said Mary Louise Franz, 77, of Baltimore. Before she retired Franz was a social worker at the Baltimore Huntington's Disease Center at the Johns Hopkins University School of Medicine.


After she retired, Franz kept up her interest in the families she worked with, including House's family and one other Fulton County family.

Last year she met them again while attending the annual Burnt Cabins Homecoming, which raises money for families in the area with Huntington's disease.

About 1,000 people attended last year, said House, one of the organizers.

This year's event will be held Saturday beginning at 4 p.m. at the Burnt Cabins Civic Association grounds.

It will feature country music, an auction, raffle and food. Auction and raffle items include two tires from NASCAR driver Rusty Wallace's race car, art work, a signed T-shirt from country singer George Jones and Shaker furniture. The fund-raising goal is $10,000.

Children of a parent who carry the disease have a 50 percent chance of inheriting it. If a child of an parent the the disease does not get it, their children will not either.

Because House has not been diagnosed with it, her children will be safe, she said. It cannot skip a generation.

Huntington's is a hideous disease, House said.

"In the old days, people with Huntington's disease were sometimes thought to be crazy. My great-grandmother died in a state mental hospital. One of her sons died from a beating in a jail after the police thought he was a drunk. His brother committed suicide when he saw what he was going to face," she said.

House began to cry as she related her family history.

"My mother was in a vegetative state for seven years before she died and my oldest brother committed suicide," she said. "This is why I work so hard on the fund-raiser, to help out families who have this disease."

Franz said a person can suffer with the disease from 15 to 30 years before death comes. It is usually diagnosed in the early 30s but can come on sooner.

Huntington's is progressive, according to information on a Johns Hopkins Web site. Early on, patients can still perform most of their usual activities.

They may still be able to work and even drive. Involuntary movements are mild and infrequent, speech is still clear, and dementia, if present at all, is mild, the site says.

They become more disabled as they age and need more help with daily activities. Involuntary body movements are more pronounced. In the late stages patients require the almost total care offered in hospitals and nursing homes. They can no longer walk or speak and have difficulty swallowing. They die without feeding tubes.

Huntington's disease patients are advised to make decisions about the end of their lives while they are still able to do so, the Web site said.

There is no cure or direct treatment although research is under way, Franz said.

To get to the fund-raiser, take U.S. 522 north from McConnellsburg, Pa., then east on Pa. 76 to Burnt Cabins.

For more information on Saturday's fund-raiser, call House at 717-987-3409.

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