About Lou Gehrig’s disease: It’s rare, debilitating and deadly — and every case is unique

(Christopher Cherrington | The Salt Lake Tribune)

ALS, or amyotrophic lateral sclerosis, was discovered in 1869 by French neurologist Jean-Martin Charcot, whose country still calls it “Charcot’s disease.”

It exploded into the American consciousness 70 years later with the announcement by baseball legend Lou Gehrig — who for decades held the record for the most consecutive games played — that he was retiring at age 36 due to a deadly disease.

“The past two weeks you’ve been reading about a bad break,” an already hobbled Gehrig told a packed Yankee Stadium on July 4, 1939. “Yet today I consider myself the luckiest man on the face of the earth. … I might have been given a bad break, but I’ve got an awful lot to live for.”

Two years later, the Iron Horse was dead.

And ALS became commonly known as Lou Gehrig’s disease.

The typical patient is between 40 and 70, but, like Gehrig, some younger people also have it. Men are 20 percent more likely to get ALS than women, and more than 90 percent of those afflicted are Caucasian, according to the National Institutes of Health.

Military veterans are twice as likely to have ALS as the rest of the population, says Emily Jordison, a coordinator with the ALS Association’s Rocky Mountain Chapter. A spike took place after Operation Desert Storm, the U.S. military’s 1991 war on the border of Iraq and Kuwait, leading some to consider environmental factors.

“We don’t know why it hits vets,” she says, “but the Centers for Disease Control [and Prevention] is exploring possible explanations.”

The CDC has sent thousands of surveys to build a massive national ALS database, Jordison says, asking questions about where patients have lived, what chemicals they may have been exposed to and what kind of unusual vaccinations they had (some soldiers were given anti-anthrax shots, for example).

It is examining numbers in certain regions to identify “clusters,” or places where there is a higher incidence than would be expected.

The national organization is seeing a potential cluster in Utah County, where ALS numbers have increased in recent years.

Some ALS cases nationwide involve athletes who might have suffered a head injury or others who were engaged in strenuous physical activities. But, Jordison notes, they did not cause their own illness.

“I’ve seen it hit zumba instructors and housewives,” she says. “One patient drank a lot of diet sodas and her family members were convinced it was the aspartame.”

It is not your diet, she tells them all. “It is nothing you did wrong.”

The vast majority of cases are classified as “sporadic,” which means it can affect anyone, anywhere. In about 5 percent to 10 percent of cases, it is “familial.” In other words, ALS can be inherited, which means there is a 50 percent chance each of your kids will inherit the gene mutation and may develop the disease.

Jordison has seen all kinds, and, while there are similarities, each manifestation is as unique as a single wild flower in a vast field of yellow.

ALS also encompasses an umbrella of variants. Some kill a person quickly, others go much slower.

As one patient put it, “If you know one person with ALS, you know one person with ALS.”