The mystery of ALS
PROGRESS IN FIGHTING DISEASE HAS BEEN SLOW
By David Kiefer
Mercury News
Article Launched: 12/02/2007 02:33:15 AM PST



Charlie Wedemeyer has lived with amyotrophic lateral sclerosis for three decades, defying his doctor's prediction in 1977 that the coach would live only another two to three years.

Yet ALS still is considered fatal and incurable, and it has no known cause.

The degenerative affliction, also known as Lou Gehrig's disease, is a progressive weakening of the muscles caused by nerve degeneration. Early symptoms include weakness in the arms or legs, slurred speech and difficulty chewing and swallowing. Slowly, the muscles stop responding, even those needed to eat and breathe.

The disease does not affect the heart, but it does attack the respiratory system. ALS patients often die of pneumonia.

"It's a disease of loss," said Linda de Mello, executive director of the Greater Bay Area chapter of the ALS Association. "They're constantly losing things that we consider essential to the quality of our lives."

The disease usually is diagnosed about two years after symptoms begin, and 50 percent of patients die within three to five years of diagnosis. Twenty percent live five years or more; up to 10 percent will live more than 10 years.

About 30,000 Americans suffer from ALS at any one time.

Progress in fighting the disease has been slow. There are more than 15 strains of ALS, and the starting point for researchers - identifying mutations that cause it - has been found in only one strain.

Lucie Bruijn, science director and vice president


of the ALS Association, said she is convinced that breakthrough drugs will be created over the next decade to dramatically slow the progression of the disease. The ALS Association has spent more than $40 million on research grants, and recently partnered with two biotech companies on a stem-cell research project.
While the affects of the disease have already taken their toll on Wedemeyer, by now there is no reason to believe he can't have a normal lifespan. His biggest concern is the diminishing range of motion in his face, which he uses for communication.

Because Wedemeyer has a slow-growing strain of ALS, there was time to figure out ways to stabilize his health - often through seat-of-the-pants improvisation - and allow the family to develop a strong support system.

"It takes a community to support an ALS patient," de Mello said.

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