Living with ALS: One Man's Story

Below is a copy of an online article that is in the latest issue of Men's Health.  If you substitute the word, girl for boy, left arm for right arm, and wife for mother, you will have a very detailed description of my experience with ALS.  I am roughly the same age as the subject in the article and have shared many of the same experiences, thoughts and emotions.  In fact, I see the same neurologist mentioned in the article, Dr. Elman, at the University of Pennsylvania. 

As you read through the article, I have highlighted areas that I have personally experienced to give you an idea of some of the things that I have dealt with.  For those of you that have been following my blog, you may notice some similarity in my initial reaction to diagnosis (Post 20 - Happy Birthversary).

Living with ALS: One Man's Story

http://www.menshealth.com/health/living-als

When a Man's Body FailsGary Temoyan is a young, fit father of two. A guy just like you, perhaps. Except his body is systematically shutting itself down. Temoyan is one of thousands of young men—soldiers, athletes, cops—who suffer from Lou Gehrig's disease. There is no known cause. There is no treatment. There is no cure. How do you explain that to two little boys?

By Bob DruryGary Temoyan soars above me, dropping from 13,000 feet. The red streaks on his tandem parachute stand out sharply against a bold blue sky. I glance at his parents, my eyes lingering for a moment on his mother. She's craning her neck, her eyes glistening. I've seen Joan Temoyan cry before, but not like this: These are tears of happiness. My gaze wanders next to Temoyan's two young sons, 9-year-old Joey and 6-year-old Tyler. Beaming at their dad's flight, at his momentary escape from his wheelchair. I think of what might have been—for all of them.

It all began rather innocuously for Gary Temoyan. The death sentence, that is.

A winter's day 3 years ago. He'd dismounted from his Highway Patrol motorcycle to write a speeding ticket. A twinge of numbness gripped his right thumb...as if the digit wouldn't cooperate, wouldn't hold the pen. He shook the hand, hard. Just the morning cold, he told himself. He gripped the pen tighter. Wrote the ticket. But still...

Temoyan was so proud of that motorcycle. It validated all the years of cramming for tests, of footing his own bill through the police academy. Afterward he'd applied to nearly every PD in southeast Pennsylvania, even a few across the river in Jersey. And when Lower Merion, at the rump end of the Philadelphia city line, finally called, there was pride in the Temoyan family. Gary was the first to choose a career in law enforcement. And it was all to mount that motorcycle. Even the years of community policing, of pedaling his mountain bike on patrol 10 miles a day, the joy of "going to work to work out," were merely steps toward the "real" bike patrol. When the day arrived, when Lower Merion had two Harley Davidson Road Kings to issue, he was beside himself. "Please-please-please let it be me," he prayed. It was.

He would continue to exercise and work out, of course. He was a buff 37-year-old, handsome in a Steve Carrell kind of way, with a strong Armenian nose and a thick head of wavy black hair, gelled just so. He continued to surf, fish, and wakeboard, to coach and play lacrosse, to ride dirt bikes with his sons. But as much as he enjoyed the rush of the mountain bike duty, the motorcycle patrol was his dream come true. And then it happened again. The thumb. The pen. The clipboard slipping out of his hand in the station house for no apparent reason. It happened until he couldn't ignore it any more. There was no pain, just weakness. Also, it seemed to be spreading through the rest of his right hand. And the twitching in his right arm. What was that?

He saw all the right doctors. The first, his regular physician, pegged it as a pinched nerve, perhaps from that motocross spill he'd taken a few months back while off-roading with Joey. But the doctor sent him to a hand specialist, who ordered an MRI and an electromyogram to measure the electrical impulses of his arm muscles. The tests found nothing unusual. His spine and neck were "perfect." Then, the dreaded words: "Maybe you should see another specialist."

He did. There were more tests. A hematologist did blood work. He had a full-body scan with barium; certain cancers can mimic the symptoms he was presenting, he was told. Temoyan thought about a couple of colleagues who'd battled the Big C. They were back on the job. Not so bad. There's hope. But the tests were negative; it was something else. He noticed the hematologist looking at him strangely as he recommended yet another doctor, a neurologist in Philadelphia. Temoyan stared back at the man and thought, He knows something.

By this time he had difficulty even holding a pen, much less his sidearm, in his right hand. His arm was twitching more often, too. And now there was something, well, off with his right foot. Sometimes it wouldn't cooperate when he told it to take a step. Told it to take a step? Who has to tell their feet to move? The Philadelphia neurologist ran more tests. A week later he called. "Gary, can you come down?"  He went alone. It was better that way. He and his wife were on the rocks again. The neurologist asked him to sit. Told him they'd reviewed the tests and ruled out Parkinson's disease and multiple sclerosis. That's good news, right?

"Tap your foot," the neurologist said.  He tapped his right foot, practically willing his toes to rise from the carpeted floor.  "Can I be frank with you?"  "Shit, absolutely." It was no time for subtleties.

"Have you ever heard of amyotrophic lateral sclerosis?"  Temoyan stared back, a blank expression.


"ALS? Lou Gehrig's disease?"


The walls closed in and Gary Temoyan's world went gray. The neurologist's voice seemed to issue from afar, as if the words were spoken under water: Quit your job. Get your affairs in order. Write a will. "Live what's left of your life the best you can..."

He was numb on the drive home from the downtown Philadelphia hospital. His doctor said 2 to 5 years. How long had it been since his thumb first twitched? How long did he have left? He tried to run the days, the weeks, the months, in his mind. But they were in a jumble. Joey and Tyler's faces floated before him. I'm not going to see them go on their first dates. Go to college. Start a family. His life, their futures, wiped away in a doctor's sentence.

He pulled his car over to the side of the highway. He grabbed the steering wheel with both hands and squeezed it so hard he thought it would crack. Then he wept. Wept and wept. Until, he thought, he had no more tears left. He was wrong.

Indulge me with a brief physical experiment. All you need is a second hand on a timepiece within your line of sight. Now prepare to put this magazine down and remain perfectly still for 90 seconds. Don't flex or twitch a single muscle in your body. Don't turn your head or wiggle a toe or scratch an itch or run your tongue over the roof of your mouth. Don't sniffle or swallow. You may breathe and you may blink. Ready? Go.

And there you have amyotrophic lateral sclerosis. Times infinity.

ALS is a progressive neurodegenerative affliction that, in the simplest terms, attacks and kills specific nerve cells and pathways in the brain and spinal cord. These cells, called motor neurons, are the longest ones in the human body, and carry messages from the brain and spine in order to control all the body's voluntary muscles. When these motor neurons degenerate and finally die in the rapid course of the disease, they take with them our ability to move. Forever.No one knows precisely why this happens; no one knows how to stop it. Roughly 10 percent of ALS sufferers appear to have inherited a triggering gene from a close relative; the other 90 percent of those diagnosed—15 people a day, 5,600 a year, with roughly 30,000 Americans afflicted at any given time with what is medically classified as "sporadic ALS"—run a racial, ethnic, and socioeconomic gamut. Like most diseases, ALS preys predominantly on older people; the average age of onset is 55 for both men and women.

Except, that is, in the case of fit young men—soldiers, athletes—men who, researchers have recently become alarmed to discover, are contracting it with more regularity. Fit young men like Gary Temoyan. It is still too early to account for the causes of this phenomenon, although many organizations and researchers, including the nonprofit ALS Association and various arms of the United States military, are racing to find answers. Do the multiple hits to the head that athletes receive spark an onset? The fertilizers spread over the fields they play on? The vaccines given to soldiers deploying overseas? We do not know. Yet, as one ALS clinician tells me, "Just from anecdotal experience, I think any physician dealing with ALS will tell you that there is some eerie predilection among young men who are physically fit to get this disease. As a general rule, young, male ALS patients tend to be leaner."

Joel Kupersmith, M.D., the chief research and development officer for the U.S. Veterans Administration, has noticed the same disturbing trend over the past two decades, since soldiers returning from the first Gulf War in 1991 began in clusters to display symptoms of the disease. The VA is recognized throughout the ALS community for its cutting-edge research, and it counts among its members approximately 1,000 sufferers, the largest registry of ALS patients anywhere. This allows the program to work with an extensive resource base. Dr. Kupersmith's medical teams are conducting experiments on mice found to possess a genetic defect similar to the human gene that triggers the disease in the 10 percent or people who contract familial ALS. VA scientists and researchers have completed a number of studies on the changes in biochemistry and physiology that may indicate the onset of sporadic ALS. "We hope that someday this will all lead to treatment and perhaps a cure," he says. "But unfortunately, that day is a long way off."

On the other hand, the VA has made great strides in experiments that have shown to ease the suffering of ALS victims—more on that later—but until someone comes up with the elusive cure, ALS victims must continue to live with the disease's cruelest irony. Patients in the late stages of ALS eventually, inevitably, experience total paralysis, with their eye muscles and sight the last to go. And yet—a spike-through-the-heart "yet"—in nearly all these cases the afflicted person's mind remains sharp and alert. This condition is referred to as "locked in"; sufferers are conscious and aware of their environment, but cannot interact with that environment in any way, as they are force-fed nutrients and oxygen into the shell of their inert flesh.

It's a few weeks before his jump, before he would cross skydiving off his personal bucket list, and Gary Temoyan is recalling his not-infrequent "Why me?" moments. How he lost 25 pounds because he would not eat; how his wife left him; how his depression nearly destroyed his relationship with his sons. "Sometimes I'm bad now, but I was a monster in the beginning," he says. "Mad, screaming all the time. I won't say the diagnosis caused the divorce—we were going downhill anyway. But it certainly hastened it. All I felt was the unfairness of it. I've led a healthy life. An athletic life. I eat well. I've got great kids, wonderful boys..."

At the mention of his sons, Temoyan's face crinkles. His eyes close and the part of his body that he still controls trembles as he sits in his motorized wheelchair. He covers his eyes with the good left hand, the hand he can still move, and takes a deep breath. The tears fall through his fingers.


Temoyan's right hand, as if made of rubber, droops off the arm of the wheelchair. I reach out and run my fingers over his index finger and thumb. It's warm to the touch.


"I can feel that," he says. "I just can't do anything about it."

The tears stop. It was with great effort, I can tell. "All the parts of my body that don't work, I can still feel. I can tell you that right now a fly is crawling across my left foot."


I look down, shoo the fly. He tries to smile. He offers to fetch me a beer. He doesn't need his mother for that. Joan and her husband, Armand Temoyan, a former Philadelphia rug merchant, have only this week moved back north from their Florida retirement home to live with Gary and the boys. To help with the cooking and the cleaning, to pull on Gary's socks and Velcro his sneakers, to zip his fly when his fingers fumble, to turn him in bed when he cramps. But Temoyan doesn't need his mother to fetch us a beer. He has installed ramps in his doorways, and he can do that himself. Still the tough cop. He lifts his right hand with his left arm, the only working limb he has left. "But for how long?" he says.

Now there are two cans of Coors Light before us, and Temoyan jerks his chin toward the involuntary muscle twitching in his left bicep. He has long since learned the technical name for the twitches. "Fasciculations." All humans experience these, which are usually so benign that they go unnoticed. An ALS sufferer's skin fairly jumps. "That's my motor neurons firing the nerve for some reason. They don't know why. It's coming up the left arm now." He lifts his full beer can. "If I pick this up I can feel it. I have that memory of what it was like, how it started, in the other arm. So I know this is coming. Hopefully slower this time."

There is no history of ALS in the Temoyan family. And though the disease's symptoms initially present themselves in one of the four neurological quadrants of the body—the bulbar segment for the head; the thoracic segment for the spine; the lumbosacral segment for the legs; or, as in Temoyan's case, the cervical segment for the arms—they all lead to the same conclusion. "Some people, such as Gary, are slow progressors," says his neurologist, Lauren Elman, M.D. "They plateau for a certain amount of time, but that plateau will not continue forever. You can make predictions about where the disease is likely to go, the most common patterns. But none of us can tell for sure what a patient is going to look like a week from now, a month from now. In the end, however, it is all ALS."Temoyan himself is all too aware of what he calls his own roller-coaster ride. "Am I on the upslope right now?" he says. With great exertion he shows me how he can use his left arm as a lever to help him wobble on his withered legs for a few seconds. "Or am I just going steady?" He sits back down, his exhaustion palpable. "Or is the big drop right around the corner? There's never been a documented case of ALS stopping, much less going into remission," he says.

Temoyan has written his will, but contrary to his original neurologist's recommendation, he did not quit the Lower Merion police department right away. He trained himself to fire his gun with his left hand in order to pass his annual firearms test, and he threw himself into the desk job his superiors provided. He refinanced his home to pay off his wife's share when she moved out, and gained back the weight, all 25 pounds, with burgers, milkshakes, and cheesecake. He knew that the weaker he became, the sooner he would die. As the disease spread to his legs, he learned to walk with a cane. He showed up at the station house 5 days a week that first year, then 3, then 2, and now, today, he tells me next Wednesday will be his last shift. "I stopped carrying my gun—I didn't want to hurt anyone. But when it comes to the point of having to ask other cops to help me into the toilet stall, it's time to get out," he says. "Plus, I want to use what energy I have left to spend with my kids."

If there is one positive Temoyan has taken from his affliction, it is a reaffirmation of human kindness. "As a cop, you become jaded," he says. "People lied to me on a daily basis. You ask them the time of day, they lie to you." No more, he says. He recalls the fundraisers held for him by various community organizations and police associations to build assets in the "Special Needs Trust" his older brother administers. It is that money that helped fund the ramps in his house, his wheelchair, and the small lift that rises to his second-floor bedroom. He speaks of the childhood friends who have reappeared in his life to proffer all manner of assistance. He mentions the woman, a complete stranger, who called one day to offer a donation—her late husband's specially equipped van, worth close to six figures. He'd died of ALS, she said.

Then our conversation segues to the "wonderful" Dr. Elman, who works out of Philadelphia's University of Pennsylvania Hospital, home to one of the most renowned ALS clinics in the country. Before he met Dr. Elman, he says, he felt he was being not so much treated as managed—"Diagnose and adios," in the medical shorthand. But the University of Pennsylvania hospital clinic is an experiment in multidisciplinary care, designed to address a myriad of physical, emotional, and real-life practical needs. It has mental-health experts, physical therapists, dietitians, speech and language specialists, pulmonary doctors, and social workers. Its goal, in Dr. Elman's words, "is to postpone the process of dying in order to continue the process of living."

Temoyan's most recent visit to the clinic was the first time he was unable to walk into the hospital on his own two feet. "That was hard," he says. "That was why my parents moved back. I've been cooking for the boys, actually teaching Joey to cook for himself. But, jeez, he's only 9 years old. It's good that he can go back to being a kid again. Plus, I'm finding..."—he stares for a moment at his lifeless legs—"I'm finding that I'm going to need a lot more help doing things."

Temoyan's voice trails off and he is silent for several moments. I bring up the BMW I noticed parked in his garage, a gleaming, black 2009 convertible he purchased last spring. His face brightens. "Part of my bucket list," he says. Like the deep-sea fishing trip he took to the Caribbean with his sons and his best friend; like the snowmobiling trip outside Aspen. Like the skydiving adventure he had just begun planning. His thoughts return to a recurring theme. "I used to be the fixer, the man in charge. Now I can't even lift an arm to put on deodorant. I wear a hat all the time because I can't comb my hair. I wear elastic-belted pants. I'm 40 years old, and I have to have my parents move back in with me."

He wheels across the deck and up the ramp to fetch us two more cans of beer. His good shoulder is visibly slumped.

I'm not going to lie to you," Dr. Elman says. "Right now the best we can do is look for the little, incremental clues to this disease, clues that probably won't help people now suffering from ALS."

This is a new experience for me. I've written about Parkinson's, cancer, all manner of post-traumatic stress disorders, heinous battlefield wounds, and traumatic brain injuries. In each of these stories doctors always held out a hope, however thin or distant, for a treatment, if not a recovery or cure. Not so with amyotrophic lateral sclerosis.

Lucie Bruijn, Ph.D., chief scientist for the ALS Association, describes the global effort to combat the disease among researchers in gene therapy, stem-cell biology, and genetics. The journal Nature reported in August 2010 on the discovery of a protein produced by the human body that may act as a deterrent to another protein believed to have a critical role in the spread of ALS. The italics are mine; the scientific literature on ALS is filled with such hesitant modifiers. "The breakthroughs that excite us won't lead to a treatment tomorrow," Bruijn says.

ALS research advocates also chafe at the disease's "orphan" status. As Bruijn explains it, patients contract ALS at nearly the same rate as other neurodegenerative disorders, but they die so much more quickly that pharmaceutical companies sense only a minor market for potential drugs. The only FDA-approved drug to treat ALS—riluzole (brand name Rilutek), an amino acid blocker—scarcely delays its progression. "The unfortunate truth about this drug," Dr. Elman tells me, "is that in clinical trials the margin of effectiveness was that it slowed time to death, or time to permanent ventilator support, by about 3 months."However, because of the clusters of soldiers returning home from the first Gulf War with ALS symptoms, there is some hope for patients enduring those "wasting" months. I recently viewed videos of paralyzed patients controlling computers and wheelchairs solely with their thoughts. The still-experimental technology, called Braingate, a collaborative venture between the VA and medical researchers at Brown University, involves implanting a baby aspirin–size electrode into a patient's lower cortex so that it detects brain waves and sends a signal to the motor neurons. Naturally, this process alone will not work in ALS sufferers whose motor neurons have ceased to function. But when this technology is combined with another process involving implanted wires in the body that serve a similar, impulse-transmitting function to motor neurons, locked-in patients have even been able to move previously paralyzed limbs.

The VA has already begun experiments with ALS patients. "It is called proof of concept, and it is the closest we've come to helping people with ALS," Dr. Kupersmith notes. "It's not a cure for the disease, but I think it will help a lot of people stricken by it."

But this help, he admits, is still some ways off, which leads me to thoughts of Temoyan's family, especially his mother, praying for a cure but recognizing that one day her son will be locked in. He won't be able to tell her where she might soothe an ache, where she might scratch an itch. She'll peek her head into his bedroom in the middle of the night to listen for his breathing, wondering if his lungs have stopped working.

It is a horrifying future, one few diseases can match. Facing this, ALS patients tend to shut down and draw a thick curtain between themselves and the outside world. But not Temoyan, which Elman admits surprises her. "He's the kind of guy you wouldn't think would be able to handle this. Macho guy, with a macho job. He was all about his motorcycles and his gun, and when one by one these things are taken away, well, just looking at it on the surface you don't necessarily anticipate that most people will rise to the challenge as well as Gary has. He has really chosen to fight this disease. Just the fact that was able to walk for 2½ years after diagnosis, well, that in itself is extremely rare."

All serious diseases are hell on the sufferer's family. But ALS, with its certainty of permanent cognitive imprisonment, is particularly invidious. Joan Temoyan, in fact, had previously voiced her dismay to me that Gary had not yet sat down with his sons to explain what was truly happening to him—that he is dying. Joey and Tyler have attended ALS events with their father, including Phillies games. "They know Daddy's sick," Temoyan says, "that he has a disease that affects his arms and legs, that something's happening to Daddy's body."

The message he prefers to convey is, I still love you, and I'm going to be here. That "here," however, is becoming harder to maintain. By the time I meet Temoyan, roughhousing with the boys has already become a thing of the past, and even his occasional forays into their street hockey games—he and his wheelchair make a formidable goalie—last only a few moments before he is panting from exhaustion. "I don't want to upset them," he says. "I hope and pray for a cure in my lifetime, but I'm not stupid. I can see myself turning worse. Death is so hard for a young child to comprehend. But I also know I can't wait until the point where I can't speak anymore."

So on one unseasonably cold Friday night in October—2 months after Temoyan swooped to the ground in a parachute; 2 months after his mother again expressed to me her sadness that Gary had not yet spoken to the boys about his future—Temoyan, his ex-wife Layne, and their sons gathered in the living room of his home. A few days earlier the psychological therapist at the hospital had provided Gary and Layne with a list of questions that would help the boys open up about their father's deteriorating condition. Why do you think Grandma and Grandpa moved back here? Why do you think Daddy needs so much help doing normal things? Gary glanced at the list often as his ex-wife steered the conversation. "The key was to have them engage us," Temoyan tells me the next morning. "To get them to ask the questions. Layne was good at that. She was on the floor, curled up with Joey. Tyler sat on the couch next to me. It was hard, really hard. I was afraid I would break down."

Layne and Gary went through the process with their boys. Remember when Daddy limped? Then Daddy needed a cane? And now the wheelchair? Well, Daddy has something called ALS, and we want to explain to you what that is.

They told the boys that Daddy would be getting worse, and Gary joked that there would probably come a time when Joey might have to feed him, "the way I fed you for all those years." Then they told the boys that despite the scientists and doctors all over the world working to beat this disease, right now there was no cure. Nine-year-old Joey knew what that meant, but Tyler, just 6, was a little unsure. When Layne and Gary explained further—"that's why you see Daddy getting weaker"—Tyler began to cry. And Gary and Joey began to cry. Tyler left the room and returned with his two favorite stuffed animals. He placed them on his father's lap. "We hugged, and cried a little bit more. I told them how much their mother and father loved them, and I promised them that I would never lie to them, that I would tell them when I felt, when the doctors felt, that I was getting worse. They seemed to accept that. And, I swear, I feel like there was a 1,000-pound gorilla lifted from my shoulders. I think my sons felt the same way."
And when it was over, after they had all shed their final tears, Layne went home. Joey microwaved popcorn, Tyler put in a DVD, and the three Temoyan men settled in on the couch. Together they watched Cloudy with a Chance of Meatballs.

"Best damn movie I ever saw," says Gary Temoyan.