IT TAKES EVERYTHING: Living with ALS after the ice melts

By Tobias Ma, September 11, 2014 —

He’s wearing sunglasses and has his head tilted back against the seat, motionless, as his brother unloads a wheelchair from the minivan. You would think he was only sleeping, until the door opens and his brother lowers him gently from his seat to his chair and you can see the flaccidness of his rag-doll limbs as they dangle into his lap. His name is Kyle Ruppe. He’s 32 years old and he has had amyotrophic lateral sclerosis for three years. The average survival rate from diagnosis is three to four years.

Kyle’s brother Justin is the elder of the two. He wears his hair long under a baseball cap, cocked upwards, sporting the lean, muscular physique that I imagine Kyle once had. I ask Kyle,

“How did the disease progress?”

He’s responsive and can clearly make sense of everything I’ve told him. But when he opens his mouth I can’t understand anything he says. His voice is slurred and hollow, like slow-motion air forced through a too small tube.

Justin says, “It’s okay. Just let him talk and I’ll tell you what you can’t make out.” He waits for Kyle to finish speaking. “Everything happened fairly quick. It started with his hands.”

Kyle was diagnosed with ALS within the year that his first symptoms began. In that year he lost the ability to walk, and the remainder of his functional muscles were severely weakened.

“Just started with his hands,” Justin says again, “Then he lost the use of his muscles. From the wheelchair on, he lost the use of his mouth, talking, eating. He’s on a feeding tube now.”

“Are you in pain?”

Kyle grunts a vehement yes.

“Chronic,” Justin says. “His liagaments and tendons are exposed due to the loss of muscle. He’s really sore. Shoulders, back, neck are really bad. The pain is chronic.”

When I imagine the gradual loss of limb function, I’m reminded of escape attempts in nightmares, the sensation of being trapped in a pool of quicksand that traps every muscle fibre. I ask if ALS feels like this. Justin stares at me coldly and says,

“Imagine having 50 pound weights tacked onto you, trying to lift your arm and realizing you’re only holding a toothbrush.”

We’re standing outside the Saddledome to watch several prominent members of the Calgary Flames association perform an ice bucket challenge in Kyle’s honour, an event organized by the ALS Society of Alberta.

Kyle is a diehard hockey fan, having played defence at the junior level. An odd mix of Kyle’s retinue as well as employees and bigwigs of the Calgary Flames gather to watch. The ritual is completed, ice water is dumped and photos are taken. President Brian Burke is there and has his tie undone as if he’s going to take the challenge. Burke appears to change his mind after watching one of his colleagues get doused, screaming in shock. He signs Kyle’s jersey and takes off. The other suits disappear.

Kyle’s family heads inside for a tour. I ask who Kyle was banking on to take home the Stanley Cup this year, between Los Angeles and New York.

“Neither of them,” Kyle says, straining with effort to make himself audible this time. He’s a Flames fan all the way. The brothers grew up playing hockey. Their father practiced with the Calgary Flames on occasion, and still plays with the Stampeders alumni.

“Hockey gave him everything,” Justin says. “Work, friends. Memories.”

“Does he still have those people?”

“Yeah. Tight-knit guys.”

“Did you ever play together?”

“Not besides pick-up shinny. I was older so we were never in the same division.”

Kyle bursts into protest at this.

“True,” Justin says, smiling. “You got called up sometimes.”

We’re standing in the Flames trophy room. About a third of the plaques in here have Mikka Kipprusoff’s name on them. Another third are for some guy named “Jarome Iginla.” Kyle is smiling. Then his smile gets wider. I turn around and Martin Gelinas has just appeared out of nowhere, hair flecked grey but still powerfully built, one of the heroes of the 2004 Cinderella story that nearly landed Calgary a Cup. He comes charging up to the first person in line, me, and shakes hands with everyone in the room. If he’s still mad about the referee throwing out his overtime goal against Tampa in game six of that playoff run, he doesn’t show it. I’m temporarily possessed by an insane scheme of hiding in the building after the interview is over and asking Gelinas to help me improve my wrist shot. He chats with the brothers intensely for a few minutes, tells everyone to make themselves at home, and then vanishes. As Gelinas is leaving, Kyle says, “Thank you.”

ALS has its diagnostic roots in athletes. Lou Gehrig was an American baseball player renowned for his ability to smash balls into orbit. He had set several Major League records, including 23 career grand slams. His endurance was legendary — he also held the record for most consecutive games played. At age 34, seemingly at the peak of his career, he started to feel tired all the time. Perhaps he was simply slowing down. Professional sports take a toll on the body. But the deterioration didn’t stop. His power and speed decreased in a terrifying fashion. Gehrig kept meeting the ball squarely with his bat and it simply “wasn’t going anywhere.” With the baseball world watching him riveted and horrified, Gehrig announced his retirement at age 36. Two years later, he was dead.

Gehrig’s illness and death shook the public. ALS is known colloquially as Lou Gehrig’s disease, a designation that reflects what makes the disease so merciless and feared. ALS can strike the fittest, healthiest man without warning or exemption. Ninety per cent of ALS cases have no discernible background, meaning that physicians cannot explain the cause at all. About 10 per cent of the cases are hereditary, but even if you knew you were at risk you could do nothing to protect yourself, as there are no preventative measures in terms of diet, exercise or medication. Those who develop ALS face a limited, debilitated lifespan with near one hundred per cent certainty. Unlike cancer and heart disease, ALS cannot be fought with radiation and surgery, and treatments are mainly palliative. It’s nature is that of a lightning storm striking a crowded desert.

We are in an elevator. The way Justin maneuvers Kyle’s chair in without running over anyone’s toes suggests that he’s done this many times before. I ask Kyle if there are any misperceptions about ALS. I expect something cautious, yet inspiring, like “ we are people too,” or “we just want to be perceived as normal.” Kyle replies but his voice cracks. Justin steps in.

“What people need to understand, what they need to understand, is that it takes everything. Everything. It is a fatal disease. But in the meantime, it takes everything.”

The only freedom ALS has left Kyle is the freedom to admit what he has lost.

“I understand cognitive function is spared.”

Justin answers, quick and defiant, “Yes.”

“So how does he keep his mind active? How does he stay sane?”

“Just normal human interaction, having the kids around helps a lot, having people over and around.”

“TV?”

“We’re relegated to a bed and chair, so, yeah, TV and movies.”

I ask what their thought process and plan was when Kyle was first diagnosed.

“We took every possible treatment,” Justin says, shaking his head, “Everything. Diets . . .”

Later, Justin mentions, “Food was a big thing for him. Taking him out to eat nice places, good meals.” He lowers his eyes, his voice thick with bitterness, “Now even that’s been taken away from him.”

“Are there any days where you’re mad all the time?” I ask Kyle.

“Every day,” Justin chuckles softly. “No, no. We’ve definitely been through that.”

“Treatments that help?”

“Nothing for the ALS. For complications there are painkillers, muscle relaxants. He’s got spasticity issues. He gets cramps, bad leg cramps. Anxiety sometimes.

“Fear?”

“No,” Kyle croaks.

“He’s scared,” Justin says distractedly, not hearing him, “and there are anti-depressants, but . . .” he trails off.

Kyle looks at me pointedly and says something that sounds like “No, I’m not.”

I nod back, recalling how scared I’d been during a time in my life when I was in and out of MRI machines, when every month held a calendar date for some new nerve test, or improbable treatment.

When Justin is distracted by something, or absent, it would seem that Kyle is powerless to communicate. The bond of trust between the two brothers is apparent. It’s a bond that appears to have been strengthened rather than weakened by Kyle’s complete dependence on Justin to interact with the outside world.

“You two must have a good relationship.”

“Well…” Justin says, catching Kyle’s eye. They both start laughing. They’ve lived together for thirty years, and have only been separated during Kyle’s time in college. They’ve never been in a fist fight, an anecdote even more amazing given that both are hockey players.

Anyone who has not experienced the physical sensation of being chained up in their own body the same way Kyle has could not accurately describe the frustration of losing all means of interacting with the world. The sad irony of this is that Kyle cannot communicate this frustration to me without the help of his brother. But like most ALS patients, his mind has remained perfectly cognizant of its limitations.

Although the final stages are consistent, ALS does not always begin the same way. Usually patients will experience weakness and awkwardness in a limb, such as a dragging foot or difficulty turning a key in a lock. Sometimes, speech and swallowing are affected first, known as bulbar onset. A minority will initially experience breathing problems. Respiratory illness is often what claims ALS victims in the end — the lungs cannot muster the energy to flex properly and the patient asphyxiates.

“I am someone,” Kyle wheezes, straining to make himself heard, “who wants the option.”

“A few weeks ago, he went in, couldn’t breathe, his lungs were full of fluid. He went in with pneumonia. Hours and hours of not being able to breathe. He told the doctor.” Justin stops pushing the chair and stares at me — “He told the doctor, don’t try anything if shit goes south. No resuscitation. And this usually afflicts people twice Kyle’s age . . . they usually don’t have his kind of support. I heard one story about a lady with ALS who drove her electric wheelchair in front of a train. If you were to have one thing, it should be the right to your life.” Justin pauses and watches his brother’s face, before telling him, “It should be your choice. Peacefully. Instead of gasping for air.”

Then he shakes his head and adjusts his hat, the creases on his face smoothing themselves out as we continue walking. We pass rows of twisted metal rods poking out of a black box.

“I don’t know what the hell that stuff is, man,” Justin says to me.

Kyle mumbles something.

Justin says, “Oh, right. Glove warmers.”

The debate over the right-to-die has flared up in several high-profile cases over the past decade. ALS has been one of the diseases that proponents of right-to-die point towards, as the fact that late-stage patients cannot control their own bodies, which means that they cannot commit suicide.

In 1993, Sue Rodriguez, a woman with ALS from Victoria, B.C., challenged the Supreme Court laws against euthanasia. She lost in a 5–4 vote, which upheld the ban on doctor-assisted suicide. Nonetheless, an anonymous doctor helped her end her life at her home in 1994, and other doctors have followed suit at the behest of terminally ill patients.

Since then certain European countries, such as Belgium and the Netherlands, have legalized assisted suicide, including for terminally ill children (with the consent of their parents or guardians). In Canada, however, assisted suicide has been illegal in all provinces except Quebec, which legalized it in June 2014.

Every once in a while, a Canadian doctor will be caught and castigated for their participation in a suicide, although this may change when Supreme Court hearings begin on the subject again in October.

“What would you say to someone who has just been diagnosed with ALS?”

“Go fucking do everything,” Justin almost yells, suddenly heedless of the children scurrying around. “You don’t have three to five years, you have six to eight months before this,” he gestures in front of him, “Before you can’t do anything. He still does trips. We’ve got a great friend who can take trips with him. I can’t take off, I’ve got these little idiots now,” he says, pointing to his kids and laughing with me. “Kyle still gets to go out and about but it’s not easy. While you can, do it.”

Justin took Kyle to Sedona and parts of the U.S. to participate in experimental treatments that people told him might prove of value. Nothing helped. He wishes they had gone cross-continent instead and forgone the treatment, and instead just tried to focus on seeing everything instead of participating in homeopathic, naturopathic and other alternative treatments that didn’t work.

Justin says, “That’s where this ice bucket challenge helps. Research. There’s absolutely nothing right now. If they could just find something . . .”

If we were still living in a tribal society, some people might see Kyle as diseased, contagious, his condition a punishment for a crime of his ancestors. Perhaps life was simpler in some ways before modern science, which defies our ability to explain why things happen as much as it supplements our understanding of how.

No one understands exactly what causes ALS, although there are all sorts of theories involving genetics, enzymes and proteins. But the possibilities of scientific cause seem irrelevant in the face of another, darker certainty, particularly given how remote any hope at a cure is right now. Kyle was a healthy, athletic man in his prime when this disease seized him by the throat. There was no rhyme or reason to nature’s calculus, nor regard for the aspirations and hopes he might have set for a future which would have been uncertain even for a healthy young adult. ALS simply did not care.

Later, Justin tells me that Kyle wishes he developed some other disease. Anything with a higher percentage chance of survival. He starts to talk quickly, getting red in the face,

“There’re diseases that raise so much money, like breast cancer, which doesn’t equate to the survival rate. Whereas heart disease kills so many more people and it brings in a fraction of that. And people argue against ALS—”

Kyle interjects, his voice is weakening from the length of the interview.

“No, I agree,” Justin says. “But people say, oh, ALS isn’t that bad, it doesn’t kill that many people. But is that how you equate the severity of a disease? When you rate the magnitude of suffering, ALS is at the top. But it’s so rare and so unknown.”

“Kyle,” I ask, “Do you have any regrets? Things you wish you had done? Girls you wish you had banged?”

The brothers laugh. Justin says, “He was a good-looking hockey player. That part wasn’t that hard.” I look at Kyle. His mouth twitches in a smile, but the eyes stay the same, brown as a marble, wide and sad. Justin has a beautiful wife and children now. Whether Kyle had desired that future for himself once I dare not ask.

“As for other regrets? I don’t think so.” Justin looks down at his little brother.

Kyle mumbles something to him, with a smirk.

Justin says, “Well, he’s got one.”

But that’s off the record.