Parents Appeal For Research, Recognition Of Batten's Disease

Diseaserobs Children Overnight Of Life

September 15, 1991|By Jackie Powder | Jackie Powder,Staff writer

Six-year-old twins Ian and Joey Joyce can't see, speak, walk or smile. The boys require almost constant care, but the last thing their parents want is sympathy.

Since Ian and Joey were diagnosed in 1989,Rosemarie and Michael Joyce have been in the forefront of fund-raising efforts for research into Batten's disease, the incurable that is killing their sons.

The Ellicott City couple has testified before Congress three times to request more money for research and has worked closely with the Children's Brain Diseases Foundation.

But federal research dollarsfor the degenerative brain disease have been elusive, which angers and frustrates the Joyces as they watch Ian and Joey deteriorate.

"We don't want people to feel sorry for us, we want people to help us," said Michael Joyce.

"The only thing that makes this manageable is the fighting we're doing to put an end to it."

On Sunday, Sept. 22, the foundation will sponsor its largest fund-raising project -- the third annual Batten's Disease Bike Tour.

Proceeds from the event, which raised $12,000 last year, will go toward Batten's Disease research.

Established in 1967, the foundation was organized by Dr. J. Alfred Rider, a San Francisco doctor, to raise research money for the disease. Rider's son, who died last month at 43, was thought to bethe oldest living person with the disease.

Since 1967, the foundation has raised $1.5 million for Batten's disease research, an impressive amount for a small organization but hardly enough to do much more than scratch the surface, the Joyces said.

"It's almost an example of child abuse," said Michael Joyce. "Batten's disease was diagnosed over 150 years ago and we're not much further along in research."

The disorder is the most common degenerative brain disease in children today.

One in 12,500 births results in a child being born carrying the genetic defect.

There are 300 known children with the disease, though there are probably many more cases because misdiagnosisis common, the Joyces said.

Batten's disease usually strikes between the ages of 2 and 7, causing progressive blindness, seizures and mental retardation. The average life expectancy is 15 years.

Through efforts of the Joyces and the Riders, the federal government is becoming aware of the scope of the disease.

Last year Congress approved $2 million for the National Institutes of Health to conduct research on Batten's disease and recommended in May that another $2 million be set aside for research.

NIH is evaluating research proposals and will award grants later this month, the Joyces said.

Although encouraged, the Joyces wonder how much progress could have been made by now if Batten's disease had received more research dollars.

"Ifthey had treated AIDS research like they've treated Batten's research, they'd be so far out in left field now," said Michael Joyce.

Hesaid the main reason for the scarcity of research dollars is that people touched by Batten's disease haven't been as vocal as others competing for research money.

"For the most part these parents are so overwhelmed trying to care for these children and keep their familiestogether that there's not much of a lobbying effect," Michael Joyce said.

For the Joyces, the disease has become much more overwhelming in the past year as Ian and Joey lost many of their skills.

In April, Ian began to have seizures that left him unable to speak, walk,crawl or hold his head up by himself.

He was fed through a feeding tube for two weeks and now can eat only strained foods.

Joey developed the seizures last month and he, too, has deteriorated significantly.

"It's been really hard, even though you expected it, you don't expect it the way it starts," said Rosemarie Joyce. "One day Ian was fine, in the evening he had seizures and he woke up unable to speak.

"It was hard knowing that Joe was going to do the same thing eventually and not knowing when," she said.

Just last year the twins were riding horses at a therapeutic riding center, swimming at school and playing in their backyard.

In their class at the Maryland School for the Blind last year, the boys were the top functioning students. Now, they're at the lowest level in the class and they may not even be able to attend school this year.

As they watch Ian and Joey decline, the Joyces become even more determined to go after research dollars.

Thinking of their other two sons, Andy 4, and Connor, 8, the Joyces look forward to the day when a blood test can identify carriers of the disease.

"We were given Ian and Joey to put an end to something like this," said Michael Joyce.

"I look at them and think that if we don't bring this to people's attention, if we don't fight for what's right. . .

"It's time that people got their priorities straight and did something about this."


* Date: Sunday, Sept. 22

* Time: The tour begins at 9 a.m. Registrationis between 8:30 a.m. and 9 a.m.

* Cost: Free with $25 or more in sponsorship; $25 without sponsors.

* Route: Tour begins at the Miller Library in Ellicott City. Riders can choose from 5-, 10- and 30-mile routes.

* Information, registration: Michael Joyce, 461-6583.

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