LEXA AND HER HUSBAND, PETER, wanted a second opinion. A death sentence had been pronounced on her, and they hoped I would be able to repeal it. Alexa was an elementary teacher in her early forties. In the year preceding our meeting, the small muscles in her hands had begun to shrivel up and she had increasing di culty grasping objects. She also su ered inexplicable falls. She sought advice from Dr. Gordon Neufeld, a noted developmental psychologist in British Columbia whom she had come to know through his consulting work in the school system. Believing it was “only stress,” she avoided considering a medical explanation.
Alexa forced herself to carry on with her professional duties; she struggled to maintain her routine beyond any reasonable point, well past the line most people would draw in taking care of themselves. “She worked incredibly long hours and was overextended,” Dr. Neufeld recalls. “I’ve never seen anybody push herself to the extent that she did.” Because she could barely hold pen or pencil, Alexa often stayed up long after midnight to complete her daily marking of student assignments. In the morning she would arise at ve-thirty, in order to arrive at school early enough to scrawl the day’s lesson on the blackboard, the chalk gripped in her closed st. As her condition deteriorated further, she nally accepted a referral to an international authority on amyotrophic lateral sclerosis, Dr. Andrew Eisen.
Electrophysiological testing and clinical examination left no doubt
in Dr. Eisen’s mind that the patient had ALS. At this point Peter and Alexa asked me to review the medical evidence, hoping I would discover something to challenge the specialist’s opinion—or, more precisely, hoping I would support their belief that the symptoms were purely stress related. The diagnosis was irrefutable
—as Dr. Eisen said, “a classic case.”
In ALS the motor neurons, nerve cells that initiate and control muscle movement, gradually die. Without electical discharges from the nerves, the muscles wither. As the Web site of the ALS Society explains: “A-myotrophic comes from the Greek language. ‘A’ means no or negative. ‘Myo’ refers to muscle, and ‘trophic’ means nourishment—‘No muscle nourishment.’ When a muscle has no nourishment, it ‘atrophies’ or wastes away. ‘Lateral’ identi es the areas in a person’s spinal cord where portions of the nerve cells that nourish the muscles are located. As this area degenerates it leads to scarring or hardening (‘sclerosis’) in the region.”
Initial symptoms depend on the area of the spinal cord or the brain stem where the disease rst strikes: people may experience muscle twitching or cramps, loss of normal speech or di culties swallowing. Mobility and limb movement are eventually lost, as is speech, swallowing and the capacity to move air in and out of the lungs. Despite a few reported cases of recovery, early death is usually inevitable. About 50 per cent of patients succumb within ve years, although some may survive much longer. The British cosmologist Stephen Hawking, author of A Brief History of Time, has lived with the diagnosis for decades—for reasons that may emerge when we come to study his example. In contrast with other degenerative diseases of the nervous system, ALS patients lose muscle control without su ering intellectual decline. As a research paper by Suzannah Horgan, a Calgary psychologist, puts it, “Most stories from patients convey the strains of having to manage the combination of an intact mind and an impaired body.”1
What causes the neurological degeneration from ALS is not known. There is some evidence there may be immune system
involvement, including a dysfunction of the cells in the nervous system that have an immune role. A class of cells called microglia serve a protective role in the brain, but when hyperstimulated they may become destructive. An article in Scienti c American in 1995 cited tantalizing preliminary data pointing to microglia as possible participants in multiple sclerosis, Parkinson’s disease and ALS.2
Alexa and Peter were striking in their desperation to think their way past their tragic situation. Peter, a retired engineer, would get bogged down in arcane details of muscle electrophysiology, quoting research of dubious signi cance and proposing theories that would have made an expert’s hair stand on end. He would often interrupt his wife when I asked her a question; she, in turn, would cast sidelong glances at him, as if for approval, as she gave her answers. It was evident that he found the prospect of Alexa’s death unbearably frightening, and also that she appeared to deny the diagnosis more for his sake than her own. I felt as though I was engaged in conversation not with two separate individuals, but with one possessed of two bodies. “Alexa could not a ord to think a separate thought,” says Dr. Neufeld. “She could not a ord to say anything about Peter that would have indicated she was a separate person from him.”
Also painfully obvious was Alexa’s inability to speak emotional language. She simply had no vocabulary to express her feelings directly: any question related to emotion would be answered by thoughts, delivered in a hyperarticulate but confused fashion. She seemed to perceive the world through abstract ideas instead of felt experience. “All of the emotions seemed completely frozen,”
con rms Neufeld.
What froze Alexa was her overwhelming fear of abandonment.
Given up by her birth parents, she had never established a connection with her adoptive mother. “There was nothing in that relationship; it never worked,” says Dr. Neufeld, who came to know Alexa closely in her last three years of life. “The adoptive mother had another child whom she favoured, and there was nothing Alexa could do, try as she might. She became estranged as an adolescent, nally, because she gave up. Until then, she worked
desperately to make a connection with her adoptive mother and couldn’t. It was a total vacuum. Alexa felt like there was a huge cavern where the sense of self should be.” Her rst marriage quickly fell apart. She grew up believing she had to take care of everybody. “There was never any respite in her,” says Neufeld.
“There was no internal resting place.”
In a 1970 research article, two psychiatrists at the Yale University School of Medicine, Walter Brown and Peter Mueller, recorded dramatically similar impressions of ALS patients. “They invariably evoked admiration and respect from all sta who came into contact with them,” wrote Drs. Brown and Mueller.
“Characteristic was their attempt to avoid asking for help.”3 This Yale study of ten patients employed interviews, clinical evaluations and self-administered psychological tests. The authors concluded that people with ALS seemed to have two lifelong patterns distinguishing them: rigidly competent behaviour—that is, the inability to ask for or receive help, and the chronic exclusion of so- called negative feelings. “Hard, steady work without recourse to help from others was pervasive,” the study notes. There seemed to have been a “habitual denial, suppression or isolation of … fear, anxiety, and sadness…. Most expressed the necessity to be cheerful…. [Some] spoke casually of their deterioration or did so with engaging smiles.” The conclusions of this 1970 Yale paper were not con rmed by a study seven years later, at the Presbyterian Hospital in San Francisco. One might say the jury is out, except that the Yale study is consistent with everything that can possibly be read about ALS patients, observed about them, or told by clinicians working with them. Studies in psychology—an art trying desperately to dress itself up as a pure science—often
nd only what the particular researchers have the eyes to see.
“Why Are Patients with ALS So Nice?” was the title of an intriguing paper presented by neurologists from the Cleveland Clinic at an international symposium in Munich a few years ago.4 It discussed the impression of many clinicians that people with Lou Gehrig’s disease nearly all seem to “cluster at the MOST PLEASANT
end” of the personality spectrum, in contrast to persons with other diseases.
At the Cleveland Clinic, a major referral centre for amyotrophic lateral sclerosis, the protocol for suspected ALS patients begins with electrodiagnostic testing (EDX). By measuring electrical conductivity, EDX detects the viability or death of motor neurons, the nerve cells that act on muscle bres. Niceness is commonly perceived by sta to be a feature of the ALS personality, reports Dr. Asa J. Wilbourn, senior author of the paper. His article noted:
“This occurs so consistently that whenever the EDX technologists have completed their work and deliver the results … they usually accompany it with some comment [e.g., ‘This patient cannot have ALS, he (or she) is not nice enough…. ’] In spite of the briefness of their contact with the patients, and the obvious unscienti c method by which they form their opinions, almost invariably these prove to be correct.”
“The interesting thing in Munich was that when we presented our paper, everybody came around,” says Dr. Wilbourn. “‘Oh yeah,’ people commented, ‘I’ve noticed that—I’ve just never thought about it.’ It’s almost universal. It becomes common knowledge in the laboratory where you evaluate a lot of patients of ALS—and we do an enormous number of cases. I think that anyone who deals with ALS knows that this is a de nite phenomenon.”
Similar patterns emerge from my personal encounters with ALS patients in private practice and in palliative care. Emotional repression—in most cases expressed as niceness—can also be found on exploring the lives of famous persons with ALS, from the physicist Stephen Hawking, the baseball great Gehrig, to Morrie Schwartz, the professor whose television appearances on Ted Koppel’s show made him a much-admired gure in the last months of his life and whose story and wisdom form the subject of the best- seller Tuesdays with Morrie. In Canada, Sue Rodriguez, a person with ALS, gained national prominence with her determined legal battle for her right to assisted suicide. In the end not even a
Supreme Court decision could deny her that right. Her story is congruent with what the lives of these others also teach us.
The life histories of people with ALS invariably tell of emotional deprivation or loss in childhood. Characterizing the personalities of ALS patients are relentless self-drive, reluctance to acknowledge the need for help and the denial of pain whether physical or emotional. All these behaviours and psychological coping mechanisms far predate the onset of illness. The conspicuous niceness of most, but not all, persons with ALS is an expression of a self-imposed image that needs to conform to the individual’s (and the world’s) expectations. Unlike someone whose human characteristics emerge spontaneously, the individual seems trapped in a role, even when the role causes further harm. It is adopted where a strong sense of self should be—a strong sense of self that could not develop under early childhood conditions of emotional barrenness. In people with a weak sense of self, there is often an unhealthy fusion with others.
The example of New York Yankees rst baseman Lou Gehrig is instructive. Gehrig earned the sobriquet “the iron horse” for his implacable refusal to remove himself from the lineup regardless of illness or injury. In the 1930s, long before the days of sophisticated physiotherapy and sports medicine, he set a record for consecutive games played—2,130—that would stand for the next six decades.
He seemed to feel that his prodigious talents and dedicated play when healthy were not enough, and he was too dutiful toward his fans and employers to ever take time o . Gehrig was caught up, according to his biographer, “in his self-designated role as a loyal son, loyal team player, loyal citizen, loyal employee.”5
A teammate recalled Gehrig’s participation in a game despite a broken middle nger on his right hand. “Every time he batted a ball it hurt him. And he almost got sick to his stomach when he caught the ball. You could see him wince. But he always stayed in the game.” When his hands were X-rayed, it was found that every one of his ngers had been broken at one time or another—some more than once. Long before ALS forced him to retire, Gehrig had sustained seventeen separate fractures in his hands. “He stayed in
games grinning crazily like a macabre dancer in a gruelling marathon,” someone wrote. The contrast between Gehrig’s unsparing attitude toward himself and his solicitude toward others was glaringly evident when a Yankee rookie was weak from a heavy cold. Placating the annoyed team manager, Gehrig took the young man home to be cared for by his mother, who treated the
“patient” to hot wine and put him to bed in her son’s room. Lou slept on the couch.
Gehrig has been described as a quintessential “mama’s boy.” He lived with his mother until his marriage, in his early thirties—a union the mother accepted only with marked ill grace.
Stephen Hawking was diagnosed at the age of twenty-one. His biographers write: “During his rst two years at Cambridge, the e ects of the ALS disease rapidly worsened. He was beginning to experience enormous di culty in walking, and was compelled to use a stick in order to move just a few feet. His friends helped him as best they could, but most of the time he shunned any assistance.
Using walls and objects as well as sticks, he would manage, painfully slowly, to traverse rooms and open areas. There were many occasions when these supports were not enough…. On some days Hawking would turn up at the o ce with a bandage around his head, having fallen heavily and received a nasty bump.”6
Dennis Kaye, a Canadian who died of ALS, published Laugh, I Thought I’d Die in 1993. His book has the reader doubled over with laughter, even knowing the author’s fate—exactly as Kaye had intended. Like several other writers with ALS, he remained undaunted by the exorbitant physical demands of writing without the use of his ngers or hands. “Let me start by saying that ALS is not for the faint of heart,” he begins his chapter titled “Lifestyles of the Sick and Feeble.” “In fact, I only recommend it to those who truly enjoy a challenge.” Kaye tapped out his volume with a stick fastened to his forehead. Here is his description of the “ALS personality”: “One seldom sees words like ‘deadbeat’ or ‘lazy’ used in the same sentence as ALS. In fact, one of the only traits ALSers seem to share is an energetic past. In almost every case, victims were either classic over-achievers or chronic workaholics…. I’ve
been called a workaholic, and I suppose if the work-boot ts … but technically, even though I worked all the time, I was never driven by an addiction to work so much as an aversion to, perhaps even a disdain for, boredom.”7
Another Canadian with ALS, Evelyn Bell, authored her book Cries of the Silent by wearing a laser light attached to a special glass frame, shining it on a spelling board, painstakingly pointing out each letter of each word to volunteer assistants for transcription. For her, too, such zealous dedication to a goal was not new. She relates that she had lived her life “at a feverish pace.”
She was the mother of three children while building a successful business career: “It was a challenge to juggle home-making, parenting, business, gardening, interior decorating and chau euring, but I loved the roles and performed them with great intensity…. During the years of raising a family, my Nutri-Medics business grew extensively and I enjoyed many company cars and numerous trips to foreign lands. I reached many levels of success in the business, being top achiever in Canada for a number of years. I felt I wanted to be a success at parenting and everything I did.”
With unconscious irony, Evelyn Bell reports all this just after writing that “we knew we could always replace money but not our health or our marriage.”8
Disease frequently causes people to see themselves in a di erent light, to reassess how they have lived their lives. A sudden realization hit Dennis Kaye one day as—with “glib satisfaction”—
he watched his father, and two employees, doing work that he, Dennis, had always unquestioningly performed on his own.
“Before long,” he writes, “satisfaction turned to frustration….
Almost all my accomplishments were in one way or another connected not to my aspirations, but to the aspirations of my father. I don’t want this to turn into an Oprah-style confession, but from the time I was a kid working my summer holidays, I’d been helping my father meet his goals and obligations. Except for a couple of years in my late teens, I’d spent the past fourteen years meeting someone else’s deadlines…. Suddenly, in the blink of an
eye, I found myself pushing thirty and facing a deadline of my own … the ultimate deadline.”
The same compulsive sense of duty to others is evident in Laura, an ALS patient I met recently. A sixty- ve-year-old former teacher of dance, Laura greets me at the door of her magazine-classic wood and glass West Coast home. Even leaning on her walker for support, she displays the grace and elegance of the ballet dancer.
She was diagnosed with ALS four years ago, while undergoing chemotherapy for breast cancer. “I went to a concert,” she relates,
“and I couldn’t clap all of a sudden. My ngers were cramping and they just weren’t as dexterous as they usually are. It seemed to get worse as I went through the chemo. I had several bad falls; one time I broke my cheekbone and my eye socket.” Laura’s speech is halting, but the cadences of lively humour and a love for life can still be heard in the near-monotonous ow of her delivery.
Laura’s medical troubles came on after a tense year during which she worked hard at the new bed-and-breakfast business she established in the home she shares with Brent, her second husband.
“I had always wanted to open a B and B,” she says. “I found this place, but there was stress because we had to come up with more money than we could really a ord. I felt guilty that Brent had to subsidize my nancial venture. That rst year was di cult, decorating the rooms. We built the carriage house. I ran the business, made the house, as well as decorating. It was practically a year to the day we moved in that I discovered the lump.” The ALS diagnosis followed a few months later.
Laura exempli es just how impossible people with ALS nd it to let go of self-imposed responsibilities long after their bodies have signalled rebellion. When we conducted our interview, the housekeeper for the bed and breakfast was away in Europe. “It turned out that 70 per cent of our clientele are repeats,” says Laura. “You get to know them as friends, you know. I’ve been feeling guilty because we said we are not going to take any guests for the month while Heidi was gone. But last weekend we had