Lou Gehrig's disease mostly rare, often fatal

By Shelia M. Poole

The Atlanta Journal-Constitution

The disease that is slowly draining the vitality of Jeremy Williams is relatively rare but usually fatal. Amytrophic lateral sclerosis progressively weakens muscle tissue, eventually imprisoning its victims in their own bodies.

Many people know ALS as Lou Gehrig’s disease, because it struck down the legendary Yankees first baseman.

“For the first time there was a public face, although we are aware that there were cases of ALS back to the mid-1800s,” said Candace Wood, who heads the ALS Association of Georgia.

ALS destroys brain cells that control muscle activity such as walking, speaking, swallowing and breathing, according to the National Institute of Neurological Disorders and Strokes. For instance, the signal from the brain that tells someone to lift a foot or raise an arm doesn’t work. That’s often how people first notice there is a problem. They may find themselves tripping a lot or being unable to perform simple tasks such as buttoning a shirt.

The cognitive and sensory functions of people with ALS remain intact; a person can feel an itchy nose, for instance, but can’t move his arm to scratch it.

ALS is sometimes known as an “orphan” disease, because it strikes a relatively small number of people — 5,600 a year in the United States. By contrast, the American Cancer Society expects 1.5 million people to be diagnosed with cancer this year.

Caring for someone with ALS can take an emotional and financial toll. Depending on the stage of the disease, the person may need round-the-clock care, including a ventilator to breathe.

“It’s a horrible disease,” said Dr. Jonathan Glass, a professor of neurology and pathology at Emory University and director of the Emory ALS Center. “This is one of those diseases that we see a lot of, we know a lot about, but we don’t know what causes it,” he said. “That makes it very difficult to develop a cure.”

One drug approved by the Food and Drug Administration has been shown to slow the progression of ALS and can add about 10 percent more time to a person’s life. But it isn’t a cure.

“We have a lot of work ahead of us to better understand this disease, develop treatments and, hopefully, cures,” Glass said.