Fighting ALS

By BRENDA SCHORY - bschory@kcchronicle.com


Bob Schmehl sits in his kitchen with his wife, Edie, at their St. Charles home. Schmehl has ALS and is fighting to support an ALS registry for doctors and patients. (Rob Winner photo)



<!--[component:schoolicons]<br>-->CAMPTON HILLS - The wheels on Bob Schmehl's walker move slowly as he takes tiny - though determined - steps through his living room.

His wife, Edie, 75, helps him navigate a short distance from the walker to settle in a chair at the dining room table.

For dinner, she has adapted a fork that fastens to his hand so he still can feed himself. Schmehl demonstrates that he can only lift his hands halfway to his mouth, and his fingers can no longer curl around a fork very long. He leans into his plate so he can eat.

The couple are contemplating when Schmehl will graduate from the walker to a wheelchair, or when he no longer can speak, move or swallow.

They don't say out loud what will happen, but as amyotrophic lateral sclerosis - also known as Lou Gehrig's disease - continues its deadly progression, Schmehl could die within two to five years.

"Lou Gehrig's is a horrible disease that attacks the nerves and muscles, causing total paralysis and death in a short time," Schmehl, 65, said.

Despite the prognosis, Schmehl is fighting to create a national registry to help research the disease and eventually find an effective treatment or cure. Even as the nerve signals to his fingers are fading, he hunts and pecks keystrokes to type e-mail letters seeking U.S. Senate support for the ALS Registry Act.

"What we're trying to do is get a body of doctors and researchers and just plain patients who can be on this registry," he said. "Not only the demographic and geographic, but environmental. They [want] to track it by race, by time, by area and genetics."

For example, Schmehl has worked as a salesman all his life. Someone else with the disease might have been a farmer and exposed to fertilizers and pesticides. Another person might have a genetic connection. Vitamins might help one patient, while Schmehl finds some relief from massage therapy.

The ALS registry legislation passed the U.S. House by a vote of 411-3 in October at a proposed cost of $75 million through 2012. The Centers for Disease Control and Prevention in Atlanta would manage the registry.

But in the U.S. Senate, the bill is being blocked by U.S. Sen. Tom Coburn, R-Oklahoma. Jim Manley, spokesman for Sen. Harry Reid, D-Nevada, who introduced the bill, said Reid is working to combine it with other bills to dodge Coburn's block.

ALS research and support agencies also are critical of Coburn; they say he does not understand how important the registry is.

The Federal Drug Administration considers ALS an "orphan" disease with 5,600 diagnosed each year, or about 2 to 4 people per 100,000 population, said Jeffrey Deitch, managing director and co-founder of the ALS Hope Foundation in Pennsylvania. About that many die each year as well.

"Epidemiologists want to understand things like where you were born, what work you did, maybe what other health conditions you had that correlate with ALS," said Deitch, who has a doctoral degree in neuroscience. "We actually have no idea how many in the U.S. have it. We think about 30,000, based on research academics. We're just piecing this together from reports."

A spokesman from Coburn's office did not return messages seeking comment. But according to a letter posted on his Senate Web site, Coburn says scientists, rather than career politicians, should be making decisions about medical research.

"As a result, scarce dollars would not necessarily go to the most promising areas of research, but to the disease with the best lobbyist or celebrity advocate," Coburn's letter states.

Schmehl has harsh words for Coburn:

"This hold tactic is a callous, selfish act," he said. "ALS is very hard to diagnose, but with a registry we might be able to find common factors and eventually a link to so many unanswered questions about this disabling disease. Research is being done, but without data to support the studies, a cure may never be found."

Amyotrophic lateral sclerosis is a degenerative neuromuscular disorder that causes progressive paralysis and ends in death.

Resources for ALS information, services and the registry act:

- Les Turner ALS Foundation, Skokie, 847-679-3311 or http://www.lesturnerals.org/.

The organization not only supports research, but patient services, such as transportation to the Lois Insolia ALS Center at Northwestern Memorial Hospital, in-home services, support groups and equipment loans.

- ALS Hope Foundation in Pennsylvania supports research, http://www.alshopefoundation.org/

- ALS Association, a national organization with a network of chapters for patient services, community support and research, Greater Chicago Chapter at 312-932-0000, http://www.alsachicago.org/ or the national Web site, http://www.alsa.org/

- Agency for Toxic Substances and Disease Registry is studying both ALS and multiple sclerosis and their relationship to hazardous waste sites, more information at www.atsdr.cdc.gov/dhs/ms_fact_sheet.html

- Patients Like Me at http://www.patientslikeme.com/ is a Web site that allows patients with difficult-to-diagnose illnesses, such as ALS, to share information.


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