Battle agaist Batten's disease goes on Parents of Howard twins ask for more research funding.

May 20, 1991|By Kate McKenna | Kate McKenna,States News Service

WASHINGTON -- Members of the House health appropriations panel remember when Ian and Joey Joyce, 6-year-old twins from Ellicott City, came to Capitol Hill last year to help plead for federal aid for Batten's disease, the degenerative brain disease that had crippled and nearly blinded the once-healthy brothers.

Last year the twins' eyes had darted around to see with theiremaining peripheral vision. Now, 12 months later, the brothers sat in total darkness during panel testimony May 8. Nor could they really speak out. While Joey still has some verbal skills, Ian has been unable to speak since suffering severe seizures a few weeks ago -- a typical sign of the disease, which destroys brain functions one by one.

"They could see the deterioration in them over one year," saiMichael Joyce, who has two other children. "Of course, we see it every day."

Batten's disease occurs in one in every 12,500 births. Of all thmedical causes for which requests for funds are brought before congressional panels, the need to provide research funds for Batten's may have been one of the least familiar. Yet, interest in Batten's was strong enough last year for the House Appropriations Committee to break a cardinal funding rule: Work with all causes, but favor no one disease.

Stating in its report that the panel "is not satisfied with the pacof research in Batten's disease," the committee earmarked $2 million in grants to study the disorder.

But this year may be a different story, according tcongressional staff members and those familiar with the struggle for funding.

"Last year was tight; but this year is extremely tight," saiCharles Siegel, aide to appropriations health subcommittee member Rep. Steny H. Hoyer, D-5th.

"Mr. Hoyer is still concerned about Batten's disease this year abefore," added Siegel, "but he's also had to push for more attention to things like women's reproductive diseases."

Aware of such increased pressures, Michael Joyce and Dr. JAlfred Rider of the Children's Brain Diseases Foundation this month reminded the House health appropriations panel how swiftly last year's $2 million in grants had resulted in more than two dozen research proposals.

"It is obvious that when meaningful money is available, first-clasinvestigators will respond," said Rider, whose 43-year-old son, Charles, is believed to be the oldest living victim of the disease.

"It is imperative, therefore, that the committee again adopt similar wording as last year in order to be sure that these grants will continue to be funded."

More than 440,000 Americans carry the recessive gene thacauses the disorder, the most common neurogenetic disease in children. No test has yet been developed to detect carriers of the disease -- a priority for Batten's research.

The disease's most common form can strike children ages 5 t10, slowly attacking the brain, function by function. Such patients die by their late teens or early 20s after living many years in a vegetative condition.

"This irreversibly severe illness constitutes an enormous nursinand financial burden to families with afflicted children," said Rider. "Patients may live, if that is the proper term, in this deteriorating state from 10 to 25 years."

Michael and Rosemarie Joyce did not know they were carriers oBatten's disease until their twins were diagnosed in February 1989. An older son, Conor, 8, is healthy. Their youngest child, Andrew, 3 1/2 , may be too young to display early signs of Batten's: loss of vision, seizures, irreversible behavioral and intellectual changes.

As for Ian and Joey, they spend their days learning some skills athe Baltimore office of the Maryland Foundation for the Blind, and being cared for by their parents on alternating schedules. Michael Joyce is a technologies specialist for Bell Atlantic, and Rosemarie Joyce works in the labor and delivery room at St. Agnes Hospital in Baltimore.

"My own particular hell is that I know that one of them will gbefore the other," said Michael Joyce. "The good news is that they probably don't know what is happening to them. And, of course, it helps that they have each other."

Although Joyce and Rider hope that their recent pleas fofunding will keep Batten's disease out in front as the subcommittee marks up the bill within the next few weeks, they feel the loss of the panel's ranking minority member, Rep. Silvio Conte, R-Mass., who recently died of cancer.

It was Conte's personal promise to the Joyce family last year "to get some money" for Batten's disease that many believe tipped the scales.

But congressional aides have said that even if an increase iBatten's funding is not feasible, a continued $2 million grant allocation is possible even in this year of increased fiscal austerity.

"A lot of what we do on the appropriations committee is not on technocratic model," said one committee staff member, who noted that emotional appeals like those of the Joyce family affect members of Congress. "Remember, money for cancer is not driven by bureaucracy -- but by fear of cancer."

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