My Dad and ALS

My Dad was an intelligent and well-read man, active and physically strong, who loved to have long discussions on many topics.

At the age of 78, he began to slur his words. This first indication something was amiss was passed off by everyone who knew Dad as drunken slurring — he was, after all, a binge alcoholic.

The slurring got worse and he struggled to speak clearly. My brother, Jacques, who lived next door, persuaded him to see a doctor. He was referred to a specialist for testing. The diagnosis — amyotrophic lateral sclerosis (ALS), a progressive and usually fatal disorder that attacks the nerves and muscles was shocking.

For Dad, the first symptom of this insidious disease, and the most obvious, was the ongoing deterioration of his speech. As the nerves in his throat atrophied, his speech became more and more slurred. In conversations, he struggled to be understood, and often had to resort to writing short notes.

The loss of speech was a real blow for him. I remember my first visit after his diagnosis, when I hadn’t yet heard the test results. An article about the disease had appeared in a recent issue of the Globe and Mail, and he had kept it. He showed it to me, pointing to the article.

As I read it, I asked him if this was his problem, and he nodded, with tears in his eyes.

In some ways, Dad was lucky in how the disease progressed, since he lived alone. In 75% of cases, the motor nerves are affected first, and patients experience awkwardness when walking, or they notice that they are tripping or stumbling more often. Other patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock.

He never had those symptoms to any degree — his disease affected his throat and chest muscles and the nerves controlling them.

As the disease progressed over the next two years, his speech became completely unintelligible, and he resorted to communicating with writing and body language. The paralysis continued to get worse and swallowing became very difficult, causing him to choke. When swallowing became impossible, he had a feeding tube inserted. Maintaining his weight became a problem, and he grew increasingly gaunt.

Eventually, his breathing was affected as the diaphragm and intercostal muscles (rib cage) weakened, and his lung capacity and inspiratory pressure diminished. Bilevel positive pressure ventilation (frequently referred to by the trade name BiPAP) was used to support breathing, first at night, and later during the daytime as well. As his breathing got less and less effective, a tracheotomy was necessary. Throughout all this, he was able to remain at home, with regular visits from a nurse.

ALS mainly affects the motor neurons, and in most cases the disease does not impair a patient’s mind, personality, intelligence, or memory. Nor does it affect a person’s ability to see, smell, taste, hear, or feel by touch. Control of eye muscles is the most preserved function; and bladder and bowel control are usually preserved in ALS, although as immobility sets in and the diet changes, intestinal problems can require continual management.

I’ve often thought that his awareness and knowledge of what he could expect must have been one of the most difficult aspects of this disease for him to accept and live with.

The End

On his birthday, July 7th, the family gathered for his birthday (his 82nd) at his home. He couldn’t eat the cake, and he was gaunt and slow, but still upright, and able to enjoy his whole family. His mind remained sharp, despite the progressive degenerating condition of his body. Most people with ALS die of respiratory failure or pneumonia, not the disease itself.

It was the last time I saw him alive.

He was hospitalized in late July, needing permanent ventilator support, and three weeks later, he died.

ALS Awareness

About 25% of ALS cases are “bulbar onset”, the type that affected my father. Patients first notice difficulty speaking clearly. Speech becomes garbled and slurred, with an inability to speak loudly. Difficulty swallowing, and loss of tongue mobility follow. Eventually total loss of speech and the inability to protect the airway when swallowing are experienced.

Until my father was diagnosed, I’d heard of ALS, but never knew much about it. Since then, three more friends and acquaintances have suffered and died from this devastating disease.

This month, May, is ALS awareness month, bringing attention to the needs of people with ALS, and their families.

In August 2014, three young men living with ALS dumped buckets of ice water on their heads and inspired the world, creating a new awareness of the disease that was destroying their lives.

The ALS Ice Bucket Challenge has spread a new awareness and encouraged contributions to fund research into the causes of this medical condition. The campaign raised more than $100m in a 30-day period, and was able to fully fund a number of research projects.

One of these has identified a new gene associated with the disease, which experts say could lead to new treatment possibilities.

More about ALS

While most cases of ALS have no defined cause, about 10% are believed to arise from an inherited gene. Researchers believe other causes are chemical imbalances — specifically glutamate, auto-immune attacks, and the accumulation of abnormal proteins which can destroy nerve cells.

People with the familial gene have a 50–50 chance of developing the disease. Age also increases the risk — most ALS cases develop after the age of 40, with men more at risk than women. Environmental factors are also believed to trigger ALS onset. Smoking, chemical toxin exposure and even military service all raise the risk of developing ALS.

My father had most of these possible triggers — he was a life-time smoker, well over 40, and had served in the military during WW2. He lived and worked on a farm, so was occasionally exposed to toxic agricultural chemicals.

Despite ongoing research, the exact causes of Lou Gehrig’s disease are still unknown. Recently researchers have targeted specific genetic mutations that cause both ALS and dementia, and may be able to develop treatments for these diseases.

Because of the Ice Bucket Challenge, awareness of this insidious and fatal disease has grown. Donations in the hundreds of millions of dollars are funding research in universities and supporting families that must cope with the disease.

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Nicolette Goff — Teacher, gardener, author, painter — following my heart and expanding my awareness of all that is possible in life.