ALS and Frontotemporal Dementia

Diseases can come with running mates

When I was caregiving my spouse for the ten and a half months that we knew he had ALS, no one ever mentioned frontotemporal dementia (FTD).

Yet even in the first two or three months, as I took over more of the household routine and business, I became aware that something had been amiss far longer than we’d thought. I found a bank account accruing NSF charges, month after month, and a number of credit card accounts as if every bank invite that had come in the mail had been duly filled out.

Even in week two after our GP gave a preliminary diagnosis (based on previous experience with three patients, and the simple question — “have your emotions been out of control lately?”) even then, my spouse admitted to being unable to come up with words, and how language facility felt to be slipping away. It had been too long since he could ably play the gypsy jazz guitar he loved so much; he’d thought it was the result of carpal tunnel or arthritis. But the cognitive was as messed up as the physical. Maybe more, in those early days.

No one said anything

True, the evidence does seem to be growing exponentially at this point in time. But even in 2015–16, when we were in the midst of living with amyotrophic lateral sclerosis, and going to hours-long clinic visits, no one ever spoke of the possibility of some form of dementia. The focus was on the physical, with the odd question focused on emotional well-being.

My understanding — our understanding — was that the emotional questions were about the undercurrent of depression and sadness, an unsurprising result of a terminal diagnosis. Anti-depressants were suggested, even as our GP made it clear that given the frontal-lobe reality of the disease, anti-depressants could not be counted on. Though he also said to go ahead and try. My spouse did, with no improvement.

My spouse passed away in mid-April, and the following Christmas our family GP phoned to let me know that the symptoms in my 85 year old father that appeared to be the ravages of a stroke were, in fact, ALS. My father passed away two years after that, and not once did his mind waver: it was so clear. I had moments of wishing his mind was a bit less clear, but was thankful he was not ever in the mental fog that my spouse had been. It made me suspect that there was something very different about my spouse’s ALS.

With my spouse, no one talked about the personality changes that might occur. We had a social worker as part of the Clinic team, but he seemed more intent on reminding my spouse that “whatever has worked for you in the past to get you through” should be a go-to place. Really? We haven’t been in this place before, and don’t expect to be again. Death is a once-in-a-lifetime experience. For the most part. From what I’ve heard. “Put away your textbooks,” I wanted to say to that social worker. Or had he come up with this on his own?

Most common form of dementia under age 60

In fairness to those who were working with us, my spouse’s form of ALS was rapid. Two to five years is listed as average, and yet he passed away in less than a year. He was not a big person; that was part of it, certainly. But changes occurred weekly, and it was a challenge to keep up. And while our GP worked to shift with us as we flew — fell? — through the stages, there did seem to be a lack of openness to our reality with other professionals. Truth is that I had to learn to hold on tightly as the ride took off. I couldn’t do much else. And toward the end, after months of trying to talk about the elephant dying in the room, when we reached a point of my spouse hardly looking at me, when the “stages” — denial, anger — were palpably surrounding us, I felt as if I was in a morass of emotion, none of it sorted through.

In retrospect, I wonder: what part of the morass was a human being who simply did not want to accept that at age 58 he was dying? What part of it was sadness? anger? And what part of it was the disease itself? Or some iteration of it? It would have been useful to have known and understood that. Reading through the list of symptoms of FTD (the “most common dementia diagnosed in patients under age 60”), and an unwillingness to talk, a tendency to anti-social behavior, depression, language difficulties, and more, are all part of the condition.

In the end there was love

It wasn’t romantic; it was simply human. The overwhelming sadness made it raw. Raw love, without the refining of a nice dinner and satin sheets. Raw love such as one might experience looking out over a subway train of early morning humans, or a newscast about some disaster: that sense of we are all here together, and we are all so vulnerable.

Humans need balance

We eat different food groups for the purposes of health. And when dealing with death, or life-altering times, we require a balance of heart, mind, and gut. We need our physical needs met, our emotional, and our intellectual. Food, exercise, sleep, a hug, and knowledge. Without any one of these, we start to list, and go sideways. Knowledge can be key, and takes time. Sometimes it feels as if you don’t even know what questions to ask. Sometimes you have to feel your way into such questions. Sometimes people need to let you know about frontotemporal dementia. Or about other places you’ve never been before.

Seek knowledge. And may someone be there for a hug.

You might want to check out my recent story on caregiving and boundaries.

Or what caregiving taught about living with COVID-19 times.