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New tool launched in fight for ALS cure

By Gabe Semenza
Dec. 18, 2010 at 6:18 a.m.


LEARN MOREIf you suffer from ALS, you can participate in the National ALS Registry by visiting www.VictoriaAdvocate.com/ALS.

Here, you will find a link to the registry, as well as an Advocate documentary movie and special series about Victoria ALS sufferers.

Joan Kagler does all she can to stay busy this time of year.

The 74-year-old started dating again, attended a Christmas party on Tuesday and landed a part-time job at a Victoria antiques shop.

While Kagler copes better than she did in 2007 - the year her longtime husband died of ALS - she struggles still to make sense of a mysterious and deadly disease.

Until now, many researchers felt similarly helpless. They hope a new national registry, launched for the first time three months ago, could lead to better treatments and even a cure.

This optimism, however, begs a cynical question: Why did the country lack a national ALS registry until now?

ALS, or amyotrophic lateral sclerosis, is commonly known as Lou Gehrig's disease. It progressively destroys the brain's and spinal cord's motor neurons, crippling sufferers.

The disease has no known cure and treatments are often ineffective.

The National ALS Registry, launched in October, is the first federal program to collect, manage and analyze widespread data about Americans with the disease - using multiple sources.

The registry includes information from existing Medicare, Veterans Administration and other files, but also gathers data online from current and participating sufferers.

"We hold out a lot of promise for this," said G. David Williamson, a director at the Agency for Toxic Substances and Disease Registry, the federal group that maintains the registry. "Hopefully, the registry will help us determine whether or not we can find a cure."

Although researchers have for years studied ALS and collected data, the focus was typically on specific people - military veterans or athletes, for example. Thus, broad determinations about the disease were virtually impossible.

To complicate the research, no one group collected data from the various studies, reports and doctor's offices. Because of this, scientists don't know how many new cases occur each year.

Estimates suggest 30,000 Americans, mostly men, suffer from the disease today, but that figure is not definitive.

So, why did the country lack a national registry until October? Doctors, after all, diagnosed Lou Gehrig with ALS almost a century ago, and a French neurologist first described the disease in 1869.

No one interviewed for this story could pinpoint one explanation. While opinion varies, most agree:

Because such a small population of the country has ALS, a registry was never a national priority.

No independent organization could build the registry because the law forbids private groups from collecting sensitive medical data for these purposes.

Patients live on average two to five years with the disease - and so researchers have only a small window in which to capture data.

The federal government does not require ALS patients to report the disease, as it does with cancer patients. Researchers can only hope current sufferers volunteer to include their information in the registry.

Lucie Bruijn, the chief scientist with the ALS Association, discussed another snag in collecting ALS data. The disease is difficult to diagnose, she said, and thus complicated to track and analyze.

"Even with the registry, it's still a challenge to capture data from all American ALS sufferers," Bruijn, pronounced "Bru-in," said.

Despite these challenges, Bruijn and her colleagues view the registry as a substantial victory in the pursuit of a cure.

The ALS Association and ALS patients began lobbying Congress for the national registry in 2004. Former President George W. Bush signed the ALS Registry Act into law in 2008.

Work to refine the registry and determine its efficacy ensued until its October launch - six years after lobbying efforts began.

Williamson, the registry director, said his office is forbidden from releasing information about the database until it has ample records - about two year's worth, he estimates.

It is unknown, then, how many people the registry includes and what, if any, patterns exist.

The Advocate this week filed a federal Freedom of Information Act request to obtain the database, less records that name sufferers or otherwise broadcast their identity.

Williamson said he's pleased so far with the patient turnout. His office is in the midst of launching a national ad campaign to make doctors and ALS sufferers aware of the online registry.

Patrick Wildman, director of public policy for the ALS Association, emphasized the importance of ALS sufferers contributing to the registry.

"The more info we have about the disease, the better," he said. "Science relies on data."

The registry is bittersweet for Kagler, whose husband of 52 years, Jim Kagler, died a week after being officially diagnosed.

The husband suffered for years without a proper diagnosis, and lost 85 pounds before dying. Had doctors recognized his symptoms, the 73-year-old might have lived his final years in more comfort.

"I wish the registry had been there sooner, of course, but not even I was familiar with the disease until Jim was hit with it," the widow said. "It's just one of those things not many people knew about. People are still searching for answers. If this registry helps, it's better late than never. I just hope people are aware of it."

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