It was my mother’s voice that I could no longer remember, even weeks after her death at the age of 67 from amyotrophic lateral sclerosis — my mother, who had taught me language.

I don’t know why that should have surprised me, because her voice, with its distinctive Carolinian lilt, was the first thing to go. During the fall of 1989, 15 months earlier, she had been diagnosed with the disorder — also known as ALS or Lou Gehrig’s disease, after the baseball titan who was struck down by it at the age of 36 in 1939 and died two years later.

My mother was one of the unlucky 10 percent of ALS patients whose symptoms first appear in the bulbar muscles of the throat. For some time, she’d had a persistent scratch in her throat, hoarseness, and difficulty swallowing. When she drank liquids, she sometimes partially inhaled them. The doctors called this phenomenon “aspiration.”

To what? she used to wonder in the family’s lighter moments. To her credit and in the face of unremitting misery, my mother managed to retain her wry sense of humor long beyond the point when she had lost forever the ability to speak or drink or swallow.

ALS is a progressive, terminal disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. As the upper and lower motor neurons degenerate, they stop sending messages to the muscles, which begin to weaken. Unable to function, the muscles develop “fasciculations” (twitches) and eventually atrophy.

If they are cursed to live long enough, some ALS patients ultimately lose the ability to initiate and control all voluntary movement, with one pitiless exception: They can still move their eyes. This barbarous affliction often suffocates its victims when their lungs are crushed by the weight of their own wasted muscles.

 

ALS leaves the mental capacities of its victims cruelly intact

Mama’s disease advanced relentlessly after an initial period of hopeful remission. By the early spring of 1990, her arm and leg muscles had begun to weaken and her speech deteriorated over the summer into an unintelligible croak. By late August, Mama was communicating with us by writing shaky, brief notes on little pads she carried in her lap, pocket, or purse when out of the house. She could still walk, but not far without becoming exhausted, so she had to use a wheelchair in public.

It made her furious that, because she couldn’t talk, some people assumed she was mentally disabled and addressed whoever was pushing her chair. My mother’s mind — by a ruthless cosmic joke, in her reckoning — remained keen and observant until the end, locked in the prison of her failing body.

We talked, after a fashion. I called her long-distance and she listened, answering in inflected grunts. I visited her home in western New York as often as I could from 300 miles away, and we laughed, read books, and played cards — a feat she could still manage because my stepfather, Jim, had made her a little wooden stand in which to arrange her hand, since she could no longer hold the cards. We played Uno, a game that my young son, Gabriel, loved, too.

One time when we visited her, she wrote me a note asking me to help her by straightening up the sewing room that Jim had built for her in the previously unfinished basement of their house. My mother had loved sewing and knitting her entire adult life, and now she could no longer do either one.

 

The day of Mama’s final Thanksgiving, she could no longer eat

The last Thanksgiving I spent with my mother was marred by a particularly cruel circumstance: She could no longer eat. Within 10 months of my mother’s diagnosis, the doctors had to implant her with a stomach tube. While my stepfather, my sisters, my son, and I ate Thanksgiving dinner, Mama sat alone in the living room. By then completely detached from eating and its rituals, she stared listlessly out the sliding glass door at the remnants of her vegetable garden and the withering lawn.

During my last visit, two weeks after Christmas, 1990, she was noticeably worse. Her strength was ebbing, and the formula she consumed through the feeding tube made her chronically nauseated. The quality of life, she wrote to me, made it scarcely worth prolonging, though she had hoped to live to see my sisters finish graduate school, to see my son, then 11, reach his teens.

Would she have wanted her life prolonged on a ventilator, if a crisis should occur and she could no longer speak to protest? No, she could still insist with an emphatic shake of her head. Her premature silence, though harder to listen to than any audible thing in my life, spoke volumes.

I was going through a crisis of my own, slated for surgery that spring. The morning I had to leave her house to drive back home to the Hudson Valley, I helped her put on her socks. “I’m sad, Mama,” I told her — and wrote later in a poem for her memorial service:

I’m at the end of a long tether, a ribbon
of disconsolate days, a ceaseless
slumber. Unable to speak,
she scrawls a note, a loose leaf
from the heartless trunk of grief.

“Wear a bright color,” she wrote on the notepad. It was her panacea for all of life’s insoluble dilemmas.

Two weeks later, on January 25, 1991, my stepfather called to say my mother had died quietly in bed that morning during a gentle snowfall, just after he had come in from shoveling the driveway. She was fully conscious, he said; she had squeezed his hand. There was no final message for me, my brother, or my two sisters, but Jim insisted Mama was smiling.

I still don’t know whether Jim’s implausible version of her passing was merely a merciful invention, related for the benefit of her children. I could never bring myself to ask him.

 

Silence sometimes can speak louder than words

What has memory taught us,
mother of the stilled tongue
who broke up the harshest lessons
with the curved face of a spoon,
the first syllables of a lullaby
our hearts will surely break in?

Only, I suppose, that silence sometimes can speak louder than words. In the years since my mother’s death, I’ve read with a combination of hope and chagrin the announcements of new studies and possible treatments for ALS. In 2010, researchers from Johns Hopkins and the National Institutes of Health used a new sequencing method to discover a gene that appears to cause the familial form of the disease, which affects 5-10 percent of ALS patients.

“If you look at the spectrum of diseases caused by dysfunctional genes, our knowledge of almost all of them has grown out of the familial form of those diseases,” said Brian J. Traynor, who led the study. “By finding the genes associated with those diseases, researchers can insert the causative genes in animals, creating models that can help them decipher what takes place to cause pathologies and develop ways to stop them,” he explained.

In addition, the California Institute for Regenerative Medicine has funded several research projects investigating the causes of ALS and developing stem cell-based therapies for the disease.

Two different nights in late 1995, I watched Nightline programs in which Ted Koppel interviewed Morrie Schwartz, an ALS patient and retired Brandeis sociology professor who gave his last interview on October 13, 1995. “I’m going to find a way to take advantage of silence,” Morrie said in May 1995, “because maybe that’s the way to really hear yourself.”

Swallowing hard, I listen still for my mother’s wordless counsel.

* * *

Note: The preceding essay first appeared in the March, 1996, edition of Inside Health magazine, published monthly at the time by The Times Herald-Record. It accompanied an article written by our medical writer at the time, Beth Mullally (later, Beth Quinn), titled “Battling Lou Gehrig’s Disease: A New Season of Hope.”