Living and dying with ALS

Slowly being robbed of the use of his body, Toronto Sun writer Jerry Gladman and his family come to grips with a frightening diagnosis

I have ALS.

There, I said it. I have ALS. God damn it! I have ALS.

Amyotrophic Lateral Sclerosis. Also known as Lou Gehrig's Disease, Charcot's Disease (for French Dr. Jean-Martin Charcot, who first wrote about it in 1874) or Motor Neuron Disease in Europe.

Most of us probably identify it with Lou Gehrig, the all-star New York Yankees slugger whose career was cut short by ALS and who made the famous retirement speech at home plate at Yankee Stadium in which he said: "I consider myself the luckiest man on the face of the Earth."

 ALS' CRUEL END

Jerry Gladman and his wife Norma pause at the top of the stairs in their Richmond Hill home after he stumbled while going up to take a shower, only one of many daily activities altered since he was finally diagnosed with ALS last April -- after nearly a year complaining of symptoms. Many of us also know it from years of watching the Jerry Lewis Muscular Dystrophy telethons during which ALS was a featured component, but took a back seat to the suffering of kids with MD.

Some of us recall it in regards to a British Columbia woman named Sue Rodriguez who challenged the constitutionality of the Criminal Code all the way to the Supreme Court for the right to take her own life rather than await ALS' cruel end. She lost, but later died of an overdose of drugs in the privacy of her home with the assistance of a doctor.

And others yet learned of it from a book called Tuesdays With Morrie, a beautifully inspirational story of a former student's time with his old sociology professor in the latter's last months battling the illness.

But for those who don't know of it, ALS is a disease of the nervous system. It attacks the motor neurons, which convey the brain's messages to the voluntary muscles throughout the body. When the muscles don't get these messages, they cease to function.

Over the course of the disease -- the average life expectancy at the time of diagnosis is only two to five years -- the muscles in the arms, legs, chest and throat weaken and then shut down totally. In the final stages, the body becomes paralyzed, although the mind typically remains active and alert, as do the senses. Some have likened it to being buried alive.

And, unlike Mr. Gehrig, most of us suffering with ALS do not consider ourselves particularly lucky in any way.

The cause is unknown, nor is there a cure. There are relatively few medicinal treatments, although scientists throughout the world race against time to develop those that might be effective. Those that do exist only promise to extend life for some months, which, of course, we will take.

Although my symptoms first began appearing in June 2002, I wasn't officially diagnosed until last April. With the majority of sufferers, the disease first appears in the limbs. Mine started in my throat, which is called Bulbar ALS, affecting my speech, chewing and swallowing. It's now progressed into my arms, hands and legs, limiting a good portion of my normal activities. As well, it's wreaked a fair amount of emotional havoc.

BROKE MY HEART

Because of the nature of my work and my function at The Sun (I do book reviews and write a weekly obit-type column called "One Life"), I am able to continue earning a living. But it's beginning to affect some of that. I can no longer take written notes, talk on the phone or walk very far.

However, the most tragic part for me was having to quit teaching. For the four years prior to my diagnosis, I taught news reporting at Ryerson University. Although I came to it late in my career, I loved teaching beyond compare and my plan was to take early retirement -- I just turned 60 -- and teach full time.

In January, when I realized I could no longer lecture or manage the workload along with my Sun duties, I decided to resign. It saddened my students and broke my heart.

As I write these words, tears freely and frequently spilling onto the keyboard, I know full well this will be the last large story I produce. It signals the beginning of the end of a career that began 44 years ago when I was 16, a Grade 10 dropout who was lucky enough to get into this racket as a copy boy, and who somehow managed to achieve a modest measure of success and write some fairly decent yarns.

But none of this comes remotely close to dealing with the true reality of ALS -- ending my life paralyzed, dependent on others for my every need from eating to personal care to passing the long hours to breathing.

And to the awful realization that I won't be around to see my children marry and shape their lives, my grandchildren flourish, to grow old with my brother and sister (close friends as well as siblings), or spend the next 20 years or so, as planned, enjoying every minute with my life's partner, the incredible woman who has made each day a joy for me for more than a quarter of a century.

I knew even before I was officially diagnosed that I would write this story. What I didn't know was that it would take me so long. I found every form of procrastination -- short of knitting outfits for my computer -- to avoid writing this piece. Deep down I knew it was some element of denial keeping me from it, but then I hadn't yet fully accepted it.

I guess I was thinking that if I went public with it, I would be removing the final barrier. I just wasn't quite ready to tap out that sentence that led off this article, the three little words that would seal my fate for all to see.

And then one afternoon, hard into a peaceful nap, I woke suddenly with an epiphany as clear as a neon marquee.

If I don't soon write this story, someone will have to write it for me.

When ALS first stole into my life -- in the form of a periodic nasality in my speech -- it was misdiagnosed as a stroke. Little did I know then I would gladly have accepted that diagnosis along with all it brought to the party, as opposed to what awaits me down the line.

The speech difficulties would crop up more and more, especially when I got angry, excited or emotional. My family doctor, Joel Abrams, a splendid and caring physician, thought, as I did, that it might be something involving my sinus. But never one to let anything slip by, he arranged for appointments with an ear, nose and throat specialist and a neurologist.

The ENT guy went down my nose and throat with a tiny little camera, searching hither and yon, and confirmed it was nothing in his bailiwick. The neurologist, a highly regarded practitioner with whom I was never comfortable, asked my history, gave a quickie exam, and suspected a minor stroke. He then set me up for an MRI (two months down the road).

The stroke made sense. Two years before I had suffered a mild heart attack, and I also had a long tussle with high blood pressure. And even though the MRI was inconclusive, mild stroke seemed like a suitable diagnosis. So they upped my medicine, prescribed diet and exercise and I began taking speech therapy for the nasal problems.

When I resumed teaching that fall, I greeted my new class with the news of my illness and the hope I would be able to work through my speech problems. I got around it by typing out all my lectures and having a student step in and read them when I had difficulties. They seemed to be enjoying the class so we carried on.

When I began to suffer new symptoms -- problems swallowing, sneezing and periodic choking -- my speech therapist, a gifted young woman named Leeanne Rabinowitz, thought there might be something else going on and suggested I revisit the ENT guy. He checked me again and found nothing, but when I told him my new symptoms, he felt it was neurological and sent me back to the neurologist.

"Aha, I know what it is," he postured. "You have myasthenia gravis."

It sounded a little ominous, but it was only a name I recalled from some TV commercials. He explained that it was a chronic auto-immune neuromuscular disease characterized by varying degrees of weakness in muscles, including those that control speech.

"Will it kill me?" I asked half joking.

"Hardly," he said. "It's treated with medication. I am going to book you into the hospital as an outpatient for a tensilon test, inject you with some stuff (edrophonium chloride), have you talk for a minute and if it is myasthenia gravis, your speech should clear up right away."

I did a little checking and learned most MG patients were treated with medicine and went on to lead fairly normal lives. It sounded even better than a stroke so I was ready to become poster boy for myasthenia gravis.

Except my voice didn't clear up. I talked for a full minute and the nasal problems remained. The frown on the neurologist's face triggered some sudden and heavy heart pounding. He said they would have to do further testing but he suspected something neuromuscular.

The words fell out of my mouth before I could check them. "Could it be ALS?" That fear had grown inside me ever since neurological was first mentioned by the ENT guy.

"I don't think so," he said. "It usually starts in the arms or legs."

He began testing the strength in my limbs, then stared for a time at my hand. "I thought I saw a twitch." He didn't explain.

I asked him again. "Could it be ALS?" He paused, looked into my eyes. "It could be," he said softly. "And if it is? What can you do for me?"

He paused again, shook his head and then said: "Not much."

When he heard the breath go out of me, he added: "But let's not jump the gun. There are all sorts of treatable neuromuscular conditions. Let's do some more tests and we'll see."

I would await the tests, but from that moment there was a gnawing at my gut I knew wouldn't go away.

And then the twitching started.

I noticed it first in my left arm, one night while watching TV. I could actually see pulsating beneath my skin. I recalled the neurologist staring at my hand and I could clearly see the twitch between my thumb and forefinger that he only thought he saw.

They're called fasciculations. I know that because I looked up involuntary twitching and neuromuscular on the Internet. A fasciculation is an involuntary, spontaneous contraction of one or several motor units resulting in a muscle "twitch."

Anyone can have this jumping of muscles -- sometimes likened to a bag of worms -- and they are benign and often induced by exercise, lack of sleep or too much caffeine. But in motor neuron diseases, they are a hallmark of motor nerve irritation, particularly in ALS. Their presence may also help in reaching a diagnosis, although they are not specific to any one disease.

Norma leaves the house on an errand while Jerry works at his desk, now set up in the living room. The next day, with both arms jumping and twitching, I raced to my family physician and told him my new symptoms. I was frantic. He examined my arms and then told me to stick out my tongue. The look on his face told me the news wasn't good.

"You also have them under your tongue," he said, holding up a mirror to the shimmering bag of worms at the centre of my mouth. "I wish I could tell you different, but I think you may have a progressive neurological disorder."

I hit him with my now stock query. "ALS?"

"It could be anything. There are all sorts of treatable neurological diseases that have the twitching. It's just a sign. Let's wait for all the tests and we'll see. It may be something harmless."

But the pounding in my heart as I drove home to delight my bride with the latest somehow said otherwise. It was also accompanied by a feeling of anger. Why did the neurologist not look at my tongue? Why was this left to a general practitioner? And worst of all, why would I now have to wait weeks, maybe months, for testing, as I was earlier told?

All of that became academic that evening when I received a call from my aunt, Dafna. One of the leading specialists in Canada in the fields of rheumatology, arthritis and lupus, she was also an angel to all of our family members in any medical emergency.

Concerned about my situation, she involved her close friend, Peter Carlin, a leading neurologist who had diagnosed my mother with Alzheimer's disease. He asked a bunch of questions related to my symptoms and then he and Dafna decided they would hijack my case and have me treated at their home base at Toronto Western Hospital.

His concern was the most crucial test, the electromyography (EMG), be done by an actual neurologist as opposed to a technician, who might miss something. In hindsight, as will soon be clear, I would have preferred a plumber.

The EMG is actually part of a three-pronged test consisting of a directed history and neurologic examination, nerve conduction studies (NCS) -- basically shock treatments to test nerve function -- and the EMG, in which small needle electrodes are inserted into muscles in the arms, legs and back to determine muscle function.

The results of these three tests are examined to reach a precise diagnosis. While there is actually no diagnostic test for ALS per se, they can get close enough with the EMG and NCS, as well as ruling out any similar conditions, to determine if it is a progressive neuromuscular illness.

Without going into great detail, let me just say this battery of tests was fairly uncomfortable, particularly the shock treatments and the jabbing of needles into muscles. After the tests were completed, the neurologist -- a woman with genuinely reprehensible bedside manners -- spoke to Norma and me. This was the general thrust of her conversation:

"You have something not very good. It could be ALS, but we want to do further testing to rule out other similar conditions. If it is ALS, you should be aware that in cases where it starts in the throat people generally pass away within two years. There might be some treatment for what you have, but you shouldn't grasp at straws because it would only delay progress for a short term."

HORROR AND PAIN

I asked her a question relating to ALS. At this point, her tone sharpened. "You weren't listening. What I said was ..."

It didn't matter what she'd said. What we heard was ALS and two years. I looked at my wife and saw the same agonized expression of horror and pain that I'm sure decorated my face. We both knew at that moment we were beginning a journey into a place called hell.

As for the doctor who gave us the news, we saw her for maybe a total of five minutes. Her manner was cold and hard-edged, there was no compassion, no comforting hand on the shoulder of someone she had basically given a death sentence, and she seemed to be in a hurry.

After she bustled out, leaving us shaken like two shell-shocked infantry soldiers following an enemy bombing, her young associate, who had been in the room and appeared extremely uncomfortable, asked if we would like an ALS pamphlet. We declined politely, but assured him we would get one when we knew for sure it was ALS.

Can you imagine? We had just been given some pretty awful news, news that would change our lives so drastically, and the best they could come up with was a pamphlet. A freaking brochure.

Unfortunately, we weren't done with this particular physician, although we would never see her again in the flesh. The date was Jan. 14. It would be another three full months before I was finally diagnosed, and, thankfully, it wouldn't come from her or from a pamphlet.

But come it would.

If I've learned nothing else from my experiences with the medical profession, one expression probably describes it best: Hurry up and wait.

The majority of the three-month period between initial testing and diagnosis was all about waiting. Waiting for people to return from vacation, waiting for tests to be set up, waiting for test results, waiting for doctors to return calls, waiting to find out whether I would live longer or die sooner.

Jerry's son and grandkids play basketball on the driveway while proud papa Jerry watches, right. He is surrounded by family and friends as he fights a deadly battle with ALS.

It was agony, for two main reasons. One is that waiting for results, as we all know, always messes horribly with your mind. It's a roller-coaster ride of emotions from hopeful to fretting to maybes to certainty to positive to dread and back again.

The other reason was I was getting worse. Whatever malady I had was marching onward and it scared the hell out of us.

Initially, it involved primarily my speech, chewing and my partially paralyzed tongue, which made moving food around prior to swallowing an Iditarod-type challenge while, at the same time, contributing to regular, albeit minor, bouts of choking.

Then came breathing difficulties where my throat seemed to close (caused, I later learned, primarily by stress), buildup of excess saliva at night that could also choke me, sensitivity to strong odours and emotions close enough to the surface to produce periodic crying jags.

Then it invaded my arms and hands, ever so slightly at first (although magnified by stress or tiredness) and then more noticeably. I had trouble holding on to things and lifting objects, my fingers were clumsy at manipulating items like keys and knobs, and the muscles in my arms would get fatigued fairly quickly. Crikey, I couldn't even pick my nose properly.

At the same time, stress aside, I felt pretty good. My appetite remained solid, I had energy, and my attitude was fairly upbeat. I knew I was treading in dangerous waters, but I was determined to carry on with my life for now.

What kept me up for so long was the support of my family and friends. Norma was a rock, as always, and ran wonderful interference for me. Once the kids -- Lee, from my first marriage and a father of four himself, and Jesse, now 22, and Caitlin, 19 -- accepted the seriousness of the situation, they were there for me every inch of the way. And as for friends, I never realized I had so many and all of them wonderful.

By this time, we had made the decision that I stop teaching so I could concentrate my energies on the job that provided the most finances. The Ryerson people were very supportive, and the genuinely touching e-mails and calls I received daily (and still do on a regular basis) from students stretching back four years were truly humbling.

Over the three-month period, I had a clutch of tests. There were barium swallows for my throat, a genetics blood test, a lumbar puncture (delightful, where they stick a syringe in your spine and withdraw fluid) and the best of all, a nerve biopsy (to determine if it was a nerve disorder rather than muscular), in which they removed a small piece of nerve from my left ankle, rendering the area dead for all time.

The waiting was excruciating. We were told it would take two to six weeks for the results of the nerve biopsy, which in itself seemed like cruel and unusual punishment. You could write a book in six weeks. Meanwhile, our emotions continued their relentless roller-coaster ride.

Several calls were made to the neurologist's office by ourselves, Joel Abrams and Dafna, but she, personally, never once returned calls to any of us. Can you imagine a professional not giving another physician the courtesy of a return call in such a serious situation?

Then came the final blow. After the six weeks were up, we were told by the neurologist's secretary that once the results were in, she would need another two months before she could get around to assessing them and providing a diagnosis.

Two months! What part of progressive disease did this individual not understand?

Less than two weeks later, I was told my absolute fears were confirmed -- I have ALS.

The news was given to me in a compassionate, straightforward manner by yet another neurologist, Dr. Ralph Kern of Mt. Sinai Hospital, whose wife is a colleague of my aunt. He found out about my case while they were away at a medical convention and said he would see me as soon as he returned.

A few days later, in the midst of the SARS epidemic, Dr. Kern, an amiable, kind and communicative specialist, arranged to see me in a special office set up across from Mt. Sinai. He spent well over an hour examining me and talking to me.

While I still held out a little hope the diagnosis might be less threatening and not so terminal, I felt instantly with Kern I would get the straight skinny and he would answer all my questions. And I was right.

He got all my history right down to the tiniest detail. And then he gave me a thorough physical exam, similar to the one the previous neurologist gave me but quite a bit slower and a little more exacting.

"Look, I don't need to see those test results, although I will arrange to get them today. I have seen enough patients to know with fair certainty that you have ALS. I don't think those tests will tell me any different."

I'm sure I don't have to tell anyone who has ever received bad news what those words did to my heartbeat and blood pressure. Thankfully, I was able to go into shock mode and retain sufficient composure to get the rest of the story. It wasn't pretty, although it was a tad more heartening than the bombshell I'd received three months before.

The first thing he wanted to make certain was that I fully understood the seriousness of the disease and that it was terminal. "You have to start thinking in a different way. You have to get your house in order and there are issues you should deal with along with your family."

As an example, he talked about how I would deal with pneumonia, a common life-ender for people with ALS, and with the possibility of being put on a breathing machine. I told him I was against this type of artificial breathing device and I'd decline.

"All right, that's something you have to make clear in discussing this with your family. When the time comes, you may change your mind -- a lot of people do; I probably would -- and that's your decision. But this is an issue that should be discussed now.

"Meanwhile, the first thing I suggest you do is get a copy of Tuesdays With Morrie and read it. You'll find it very helpful."

I recalled seeing the movie, although that was before I'd become a member of ALS-R-Us. Mostly I remembered Jack Lemmon's sterling performance. (Later that same day I bought a copy, read it, underlined a million lines that now applied to me, and knew it would become a bible of sorts. I also believe everyone should read Tuesdays With Morrie.)

I asked him what I could expect. His eyes were serious (the rest of his face was covered by a SARS protective mask, as was mine). "There will be some bumps in the road, I won't kid you." He knew I knew what he meant and added it was something we could discuss later. I think he wanted me to do my own research, which, of course, I later did.

What I didn't know was just how soon I would be hitting those bumps and what he truly meant by bumps.

I asked him how much time I had left, holding my breath. He said the answer to that is always between two and five years. "That's what we know. What we don't know is why a Stephen Hawking (Nobel Prize-winning physicist) has lived 20 years with it, some have lived 10 years and others five. There is no real way of gauging it other than the averages we have. But everyone is different."

I would find out later just how right he was. All my research, including talking to others who deal with ALS, more or less said the same thing. No one really knows how long anyone with the disease will live.

When I told him what the other neurologist said about two years max because it started in my throat, he shook his head emphatically. "No. It doesn't matter where it started. True, it most often starts in the limbs, but some 25% start in the throat. It doesn't matter."

I asked what was available to treat it and he said not a hell of a lot. I mentioned the AIDS-like cocktail that was being used and getting a lot of attention in the media. He said it was still in the testing stage and there was no hard proof it worked. He also said the side effects were extremely harsh and toxic, often making people worse.

I mentioned some other promising treatments I'd read about. He said that's just what they were -- promising -- but there was no hard evidence yet. He gave some examples complete with facts and figures to show he was up on every aspect of ALS.

He said there was one drug that has proven successful in slowing down the disease -- Rilutek -- with minimal risk and side effects. He said he wanted to get me into the Sunnybrook clinic where he would arrange for me to get the medicine.

"Look, I'm under no delusion about a miracle cure," I said. "But if there is a drug that will slow down the disease for any time at all, I want that drug. The more time I have the more chance there is that something will come along and change everything. All I want is a shot."

He looked at me and nodded. "I absolutely agree. I always believe in erring on the side of the patient if there is even the tiniest chance something will help."

On the way home, my brother, Mike -- he'd sat in on the session because Norma was away -- and I rehashed everything that was said. We agreed it was pretty well the same news I received in January only now it had a name, I would soon be treated for it and my demise wasn't quite so absolutely pencilled in on the calendar.

But as the conversation withered and we drew closer to my home, tears started to trickle down my cheeks.

This soon turned into a torrent as the tension of the past months came crashing down on me along with the reminder that I had yet to share this official news with my family. I have ALS and no matter how positive a spin we applied to it, chances were pretty high that I wouldn't be with them too much longer.

ALS VICTIMS

 

AN ALS PRIMER
Amyotrophic Lateral Sclerosis:

A = absence of myo = muscle trophic = nourishment

Lateral = side (of spine)

Sclerosis = hardening

Dr. Jean-Martin Charcot, a French neurologist, published the first full account of this disease in 1874. ALS, also known as Charcot's Disease, Lou Gehrig's Disease and Motor Neuron Disease, is a major neurological disorder. In Canada, it is the most common cause of neurological death on an annual basis.

ALS destroys motor neurons that are an important link in the nervous system, and through which the brain controls the voluntary muscles throughout the body.

Leg and foot muscles are controlled by motor neurons in the lower spinal cord. Arm, hand and finger muscles are controlled by motor neurons in the upper spinal cord. Speaking, swallowing and chewing are controlled by motor neurons in the brain stem.

ALS does not affect the five senses -- sight, hearing, taste, smell and touch -- nor does it normally affect the mind, eye muscles, heart, bladder, bowel, or sexual muscles. There is no possibility that ALS is contagious.

RARELY IN TEENS

ALS strikes about six people per 100,000 per year. Between 1,500 and 2,000 people in Canada currently have ALS. Although it most commonly occurs between the ages of 40 and 70, it can also occur in older people, and rarely in teenagers.

The most common form of ALS is called Sporadic ALS. In addition, about 5% of patients have an inherited variety called Familial ALS.

Symptoms are what you experience, while signs are what your doctor can see. The early symptoms of ALS may seem rather vague. They can include tripping, dropping things, slurred or "thick" speech, and muscle cramping, weakening and twitching. Some people with these early symptoms may assume that they are normal signs of aging. As the disease progresses, the muscles of the trunk of the body are affected. Weakness of the breathing muscles develops slowly over months or years.

For some people, the muscles for speaking, swallowing or breathing are the first to be affected. This is known as Bulbar ALS. The term "bulbar" refers to the motor neurons located in the bulb region of the brain stem, which control the muscles used for chewing, swallowing and speaking. ALS symptoms, and the order in which they occur, vary from one person to another.

The rate of muscle loss can vary significantly from person to person, with some patients having long periods with very slow degeneration. Although the average life expectancy of newly diagnosed people is between three and five years, 20% live more than five years, and 10% live more than 10 years. Being a progressive disease, ALS may spread throughout the body over time, and at some point in this process, may involve the muscles required for breathing.

ALS can be difficult to diagnose in the early stages, when symptoms may mimic other conditions. However, there are clinical signs that can be indicative of a wasting of motor neurons in either the upper or lower portion of the spine.

CLINICAL SIGNS
As such, clinicians familiar with ALS usually see the following signs of lower and upper motor neuron degeneration:


Some may assume these are signs of normal aging.
Over time, as muscles continue to weaken, and the weakening spreads throughout the body, it becomes more apparent that the cause is ALS.

In addition to a physical examination, people are often given an EMG, blood tests, an MRI (Magnetic Resonance Imaging), and other tests to search for the presence of other diseases that can look like ALS.

Doctors who do not usually see people with ALS may be reluctant to suggest it as a possible diagnosis. They normally refer the person to a neurologist.

Telling someone and his or her family that the person may have ALS requires a sensitive, understanding and compassionate manner.

People should also be told about the disease, current research and drug trials, as well as about support services and the ALS Society in their province.

Copy write: JERRY GLADMAN -- Toronto Sun

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