A more cruel killer

Is there any more poignant moment in the history of American sports than Lou Gehrig’s farewell speech at Yankee Stadium on July 4, 1939? As the Yankees’ first baseman, Gehrig had seemed indestructible, playing in 2,130 consecutive games over twelve seasons, earning him the moniker “The Iron Horse.” To the tens of thousands of people on hand that day, the scene must have been exceedingly incongruous. This man, the closest thing in Depression-era America to a Greek god, was announcing that he, too, was mortal. “Fans,” Gehrig began, amid the echoing P.A. system, “for the past two weeks you have been reading about the bad break I got. Yet today I consider myself the luckiest man on the face of this earth.” Two years later he died at thirty-eight of the disease that, informally, still bears his name.

The medical term is amyotrophic lateral sclerosis, ALS for short. According to the ALS Association, the term roughly translates to “no muscle nourishment.” As the disease advances, “Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost.”

Like Alzheimer’s, ALS is a disease without a cure. But there is a critical distinction. While Alzheimer’s often takes a decade or more to run its course, ALS does its dirty work, on average, in three years—though a small minority will live significantly longer. And, unlike with Alzheimer’s, people with ALS are more likely to be diagnosed in middle age or younger. Approximately 6,400 people are diagnosed each year—roughly two people, on average, out of every 100,000. Veterans are said to be at twice the risk of the disease, particularly veterans of the 1991 Gulf War.

In the last couple years, two people we were acquainted with in Somerville died of the disease: one a writer of fantasy novels and science books for young readers, the other an acclaimed painter.

The writer, Ann Downer, died just shy of her fifty-fifth birthday, but it appears that she packed much into those years. Her obituary reads like an adventure story itself:

“Ann was born in Falls Church, Virginia, and grew up living in the Philippines and Thailand, traveling in Asia and Europe, before returning to Virginia. A typhoon in the Pacific, a conical volcano in the Philippines, Buddhist temples and crocodile farms in Thailand, geckos and snakes in the gardens, and the classic children’s fantasies by Madeleine L’Engle and Ursula Le Guin: all fed Ann’s rich and voracious imagination.”

Ann’s friend Sarah Fishman noted that in Ann’s case, the disease ran a quick course.

“It took slightly more than a year for doctors to reach the ALS diagnosis, and she lived another year and a bit after that. Apparently the disease moves faster if it starts with losing your voice, which was the case with her. It was hard for her, I think, to be a woman of words, though usually written ones, and not be able to speak. As her emotions and thoughts got further boxed into her body, she felt great frustration. She didn't feel (much) physical pain but experienced the agony of not being able to communicate, except for with her eyes. She couldn't talk but could still understand almost everything, including what was happening to her.”

Jon Imber, who spent his summers painting in Maine, died in April 2014 at sixty-three. Over the last two years of his life, he was often filmed while painting, and, as the disease advanced, he could no longer paint with his right hand. As the documentary film Jon Imber’s Left Hand makes clear, the artist’s final paintings pack a visceral punch. A reviewer for the Post-Press Herald in Portland noted, “For Imber, the diagnosis is an urgent challenge: It motivates him to spend every last moment living.”

To view Imber painting, do a search for “Imber’s Left Hand.”