What IS ALS? Does it Have Any Bits of Silver Lining?

From my experiences of both spouse and parent living and dying with this disease

Look up ALS (Amyotrophic Lateral Sclerosis) and the definition you’ll find is:

a rare progressive degenerative fatal disease affecting the motor neurons, usually beginning in middle age, and characterized especially by increasing and spreading muscular weakness and atrophy called also Lou Gehrig’s disease)

Note that in the Commonwealth, the disease is known as MND, or Motor Neuron Disease.

Rare

Not so much anymore, it seems. When my spouse was initially diagnosed, and I began to research, the information floating about said “one in 50,000” people… but fast forward a few years, to the point at which my father was also diagnosed, and this is hard to believe: I don’t know 100,000 people. And since then I’ve come to know others, and know of others. So rare… not so much.

Progressive

Many diseases seem to stop and start, lurch on, come to a standstill. But not ALS. Rather, it has a pattern with each individual, and it sets its own pace, unique to that person. My father’s iteration of the disease was more “text book.” He showed symptoms (that we thought were possibly a series of small strokes) by mid-2015, was diagnosed at the end of 2016, and passed away in December of 2018 after nine months in a care-home. Three to four years is considered “normal.”

My spouse, on the other hand, exhibited symptoms in the late fall of 2014 (mostly, he appeared to have had a few too many beer post-golf games, and struggled to play his beloved Django-style jazz on guitar). He received a preliminary diagnosis from our GP, who had had several other patients with it, on June 1, 2015, and formal diagnosis from a second neurologist the third week of July, and passed away April 10, 2016. Ten and a half months. Very quick.

A book titled Motor Neuron Disease: A Practical Manual by a group of doctors led by Kevin Talbot at the Oxford MND Clinic, became my go-to source for real info. The book was published in 2012, and even now is still an invaluable resource, because — alas — research and understanding of this disease is painfully slow. This book will guide you to better understand the myriad possible sources of ALS/MND as well as any changes in treatment.

From this book, I came to know that this disease sets an unflagging pace for each person. From this book, I came to know that the rate of progression is relative to the rate of onset; in other words, if it takes a long time to diagnose the disease, it will progress slowly. If medical folks are quick to recognize and diagnose, the progression will move the same — quickly.

Our GP knew the one question to ask my spouse that would distinguish this disease from some other possibilities: How have your emotions been lately?

My spouse had been mortified to find himself bursting into tears when he said goodbye to his brother at an airport early in 2015. But he was even more risible than usual, too.

ALS does a number on one’s frontal lobe, which normally regulates emotions.

Degenerative

The disease has different points of onset. It can begin with a shift in breathing, swallowing, talking, which were the beginning points for my father. Or in the extremities, with a “dropped foot” or in one’s hands. With my spouse, it began with his hands, most noticeable as a musician and guitar teacher.

It then spreads. One of the earliest symptoms with my spouse was fasciculations — little twitching movements — in his upper arms. But some people do not experience this, including my father.

Again, for each person living with this disease, there is some variation. My spouse did not experience any pain really, until the muscles in one shoulder decided they were just unable to do their job anymore. Overnight, it seemed, his shoulder just seemed to hang, unsupported, and that hurt. We would support it with pillows. And painkillers became the order of the day.

As the disease progressed, and as a family, we learned to administer medication, we tried to keep to a tight schedule, and keep pain at bay as much as possible.

In the last months, morphine, and then fentanyl, were both blessings. Morphine in particular, as it is a miraculous palliative drug. It simultaneously alters the capacity for breathing and alleviates the anxiety about the same — a wonder drug. I used to cry with relief as I gave it to him.

Fatal

There’s that word. I’d watched The Lou Gehrig Story with my dad when I was not even twelve; I knew then that this disease did not come with Hope.

Yes, you can research all sorts on the internet, and there are those who claim they have battled and won. But there are no medically-recorded instances of “healing.” I’m not going to repeat that.

I’m not.

It is the hardest, to live with building a different type of hope. A Hope that has a strong and resilient partnership with Acceptance. I write about my own wrestling with these two in my memoir of that time, Dance Me to the End: Ten Months and Ten Days With ALS. And it was a most challenging piece. From the moment our GP said those letters, we were in a different place altogether.

I will repeat the word Hope. It’s just a different type of hope than what we are used to thinking, in our rather crazed world where we believe we have control over most everything, and all we need to do is believe…

Early on, our GP warned us that ALS is the “poster disease” for activism around assisted dying; portrayals of the worst-possible will be the norm. It wasn’t until more than halfway down this path, that a nurse took me aside, and laid out, in explicit detail, just how a person with ALS passes. Peacefully, for the most part. Usually in their sleep. Not with the gagging and misery that is evoked on the screen. (Don’t misunderstand me here, though — although her words truly calmed me, and at that very moment Canada was in the midst of decision-making over the proposal of assisted dying, I am on-side with individuals being able to make their own choices about the deeply personal.)

But plain language was and is invaluable, and I was grateful.

Motor Neurons

Are those brain-communicators that tell our muscles how and when to move. In ALS, these die off, and the communication breaks down. That’s it, in a small nutshell. I discovered that, after learning of their father’s diagnosis, this was what my children most wanted to hear: what — exactly — the disease was doing within their dad.

Why this happens, no one is exactly certain. And what to do about it, even less so.

Middle age

My spouse was diagnosed at 57, which is close to text-book onset. My father was diagnosed at 85. Not so text-book. Live long enough, and you’ll get something.

Should my children be tested? Familial ALS is but a small percentage of those who are diagnosed. Our GP said that even with both a father and maternal grandfather, their odds are no different. I suppose I could be tested too — after all, I share losing a parent to this disease with my sons.

But even if the markers show up, there is no way to know whether it could begin at age 21, as with Stephen Hawking (who had a truly rarer juvenile form, with which one can actually have a fairly “normal” life-span) or 57 or 85.

ALS affects more men than women, and more often than not, those affected are athletic or at least in “good” physical shape. It also affects a disproportionate number of military vets, especially those from the Gulf War.

Spreading Muscular Weakness and Atrophy

With the rapidness of my spouse’s illness, every week brought a new challenge. I knew, from reading the Oxford Clinic book, that the progression of those who are physically smaller at point of diagnosis is frequently quicker.

But the fact that he was 140 pounds, and 5'7" was a contributing factor to his being able to stay home. I was just 50, and in good physical shape — at least to begin with — and had the help of three sons, in their teens and twenties. (Though I was cautious with what I asked of them.)

Thanks to info from helpful workers at our local ALS Clinic, and family who jumped in to renovate a downstairs bathroom to create a working roll-in shower, the entire duration was spent at home, other than a 24 hour hospital stay to have a feeding tube. We never did require a lift or complicated equipment.

Silver linings

There are a lot of heavy clouds to this disease. But the silver linings have to be noted, and this — being able to stay at home — was a sizable one. Because there are so few answers and so little treatment, hospital stays and testing and poking and prodding… just doesn’t happen.

I mentioned risibility — a propensity to laughter. My husband loved a good laugh, and his brother came to visit several times throughout those months and worked to be even funnier than usual.

With the frontal lobe affect, there were many tears — they could come on quickly and uncontrollably, and be anxiety-causing and just downright embarrassing for him at times.

But the easy laughter that also welled up — also to the point of tears flowing, but oh what different tears — tried its best to make up for it. We had many good and deep laughs.

And my father, who had grown up in a most serious household, a home that questioned levity, he too was given to laughter in those last several years. He did not cry as my husband did. Not that he wasn’t terribly saddened at times — he worried about my mom and leaving her. But it was such a pleasure to see him laugh as he never had through his hard-working life, and to see an almost child-like grin take over his face often, with a twinkle in his eye. (Yes, even with lax muscles, I recognized the “grin.”)

And now

My fear — well, one of my fears — through those months of caregiving, was that I’d never be able to remember these two men who have been my family for thirty and fifty plus years without this horrific disease. I was terrified that I would only ever see them in my mind’s eye, pushing around that bloody walker, or attempting to smile with the sagging ALS muscles.

But Time passes. And as months and even years move, I now see them walking as they walked before. I see, in my mind, their smiles — full-on smiles. My husband always had a quickness to his step, a lightness to his feet. My father not so much, but how he loved to wield an axe over a mountain of cut logs, and split them for the fireplace, stacking them into order.

And that is what I now see.

I write this piece

In the hope that it will give you a deeper understanding of ALS/MND. I write for those who might be close to someone living with the disease. I wished that someone, anyone, could be honest with me and share in a real way.

And I wish for you, with understanding, some peace. A moment of stillness. Moments of looking into the eyes of a loved one and knowing that whatever you share with that person does not disappear. That does stay with you, always.