Hope can be wonderful. It can be quite helpful, not only for happiness, but in providing the motivation to hustle your butt, get done what needs to be done. Hope is often declared to be an absolute necessity, something that must be given to people, even in situations where a bad outcome is quite certain. 

The dying of motor neurons in people with ALS is a bedeviling puzzle. It is as if a bomb went off somewhere in the nervous system, eventually rippling its way through the entire network. The loss of function, especially for an athlete who has devoted so many years to successfully gain that function, then the diagnosis, is also like a bomb going off in the system, only this one causing numbness of emotion and loss of care about much that used to feel important. Fortunately, the bomb in this second anecdote does not necessarily continue to ripple through the system. The crater of it can be nicely paved over, after a while, for much of the time, though cracks and potholes in this adjustment can be expected to pop back up on a regular basis, especially since the first event continues to grow and presents a constantly moving target.

Though a lot of exciting things are being learned, by wonderful, devoted and brilliant researchers, most varieties of ALS are stubbornly elusive to any effective treatment. Cures being even slipperier little devils. It may be likely that ALS will be found to be more than one particular disease, though all varieties seem to have something to do with the shut down of the cell’s sanitation services, allowing the normal byproducts of a cell’s work day to build up. Somewhere, a motor neuron’s janitorial staff decided that they weren’t being treated so well. Perhaps it was a group of them. Never quite sure how these revolts get started. Revolts happen a lot in the human body. The most familiar kinds involve traitorous mutineers, like cancer cells, reproducing themselves and going on the march. Obviously, they must be put to the sword! Though the battles are often epic, the cells who must be killed are obvious and clever ideas as to ways to do it are countless. In someone with ALS, however, the motor neurons stage no campaigns, conquer no new territory; they’re more of a martyr’s brigade, harming only themselves. Same as humans who feel they have no value, although a trillion times bigger than a tiny motor neuron, I wish that motor neurons also knew just how much they will be missed, every last one of them! I also wish that there wasn’t such solidarity among the cleanup crews of motor neurons. A little union busting would be nice, in this one particular case.

Hope often seems to come with rigid assumptions. Sometimes it can be a little troublesome by causing a rigidity of thinking – chicken or the egg – which one came first? It so often gets tied up into an all or nothing activity. At this time, most varieties of ALS have little to nothing in terms of treatment or cures. Morgan Freeman put it well in the movie, ‘Shawshank Redemption’ – “Hope is a dangerous thing.” He went on to explain it well, though the movie ended up proving him wrong, in the end. I liked that. 

My diagnosing doctor is a brilliant researcher, though we thought that his bedside manner left a little to be desired. At that time, there was only one medication for ALS that had made it through the approval process, and that happened twenty some years prior. Not only is that an awfully long time to go without a breakthrough, the drug, Riluzol, was not earth shattering in its effectiveness, extending life by 3 months on average. My doctor seemed in no particular hurry to start it. After a few weeks, I remember calling his office in anger. I could detect my decline on a weekly basis. “This loss is important to me”, I barked. “We need to stop it now!” I continued. He prescribed it. I still take it, largely because it’s easy and doesn’t have any noticeable side effects. It stopped nothing in regards to disease progression; however, my life span with ALS has been longer than average. It’s my guess that his lackadaisical nature in writing the prescription was a reflection of his opinion about it. I too, share his opinion, now, but I had grabbed onto the hope that it was going to save me, working better than the data showed. No harm done, in this case.

The first new drug in twenty plus years came out in 2017. Called Radicava, it produced a lot of excitement. Hordes of people were saying, “after all this time, ALS patients now have a new hope.” TV news trucks parked outside the hospital that housed the ALS clinic that I now went to. Chop chop, things move fast in news land. Breaking! Exclusive! You won’t see this anywhere else! The reporters started with little knowledge of the science of ALS in the morning, but they had until later that afternoon in order to beat their rivals to it, looking the smartest. It’s a tough business. Their job has so many boxes to check; They must be fast, first, entertaining, showing serious concern, perhaps even with a tear, give the illusion that they’ve been tracking this for years, waiting to spring forth with all of the tantalizing details, now that this final piece of the puzzle has been put into place. This would explain all of the excitement and sense of awe written across their faces. It’s beamed back to HQ, via the giant telescopic fishing pole erecting up out of the top of their van. News like this can’t wait. The signal is received back at HQ in the nick of time. There is no time to waste. Five o’clock news is nearly under way. TV viewers on the edges of their seats are accustomed to, even expecting, stories triggering certain emotions to be tucked into their proper slots. “After a drought of over twenty years! ALS patients finally are given new hope!!!” With palpable excitement, they smartly interview a couple of doctors, with wonderfully educated questions, then onto an excited ALS patient (me). In my former job, I had gotten on TV many times in these slots. I guess they figured that I’d be good, except that I already knew that this drug was being way over sold, and wanted to be realistic.  ‘Shit! Who gave us the wrong patient?’ they must have been thinking as they gleefully ignored and spun my ‘wet blanket’ with all of the skill that gets them to where they are – cue next, seventy-year-old man in front of wrecked trailer, explaining how the tornado sounded just like a freight train, with reporter wearing a face of sad, endearing empathy. Done! Giant fishing pole deflates back down into a van and off they go in a rush.

I took Radicava for eleven months. Cycles of daily intravenous infusions, to continue forever. It stopped nothing, but a single anecdote is nearly worthless in science. The efficacy in studies started to look thinner and thinner. Costing an awful lot of money and impacting our quality of life, it didn’t seem like a thing to continue. This was our opinion. 

But this essay is not about that.

Now by this time, you might be asking yourself, ‘What’s wrong with this guy? Has he no hope? And why is he saying these things?’ My answer is – I have a lot of hope, most of the time, but it is OK to ponder the question – ‘What exactly is it that you are hoping for?’

It takes a little time, but perhaps hope comes in an array of choices…choices as to what we hope for. One must be rather fluid in those choices, as the situation changes. At my rather late stage of ALS:

I hope to make it long enough to witness an event that I might be looking forward to. 

I hope to enjoy the next day, and that the night goes well enough for my wife to get much needed sleep. 

I hope that our morning newspaper has an exceptionally good David Brooks article for us to read together over breakfast. 

I hope to be able to be of some help to my step kids, if they need it. 

I hope that random funny things happen that day, and we laugh, a lot. 

I hope that the next episode of a sad, and sweet television show makes Mary and I cry again tonight. 

I hope to see various loved ones from time to time. 

I hope that I can be clever enough to make them laugh. 

I hope to be creative enough with my writing as for it to be useful and entertaining. 

I hope my blog chronicles the lives and events of many, giving a little witness to a time, a place, a people, without the sugar coating. 

I hope that an understanding of us humans is forwarded by me, a little.  

I hope that these stories long outlast me. 

I hope to be, relevant.

I am brimming with hope, much of the time. I have enough hope, some of the time, just, the word “cure”, is not on the list, especially at my stage in the game. 

Cure, however, is certainly on the list for others, some day. Let us keep working very hard to find it, and not drain off time and resources for drugs of limited value.


Disclaimer: These thoughts are narrowly focused on a terminal illness, of which the outcome is quite certain. For something like cancer, which one might have a reasonable possibility of beating, fight like hell, and hope, seems quite fitting advice, but I’ve never been in that situation. Many others have. Just take care to do it in a helpful way, I would say. Following statistics, not anecdotes with compelling characters, is a method of going about it that I prefer.

Disclaimer number two: Just because “cure” is off our list doesn’t mean that we don’t take advantage of modern medicine to fight like hell to compensate for the symptoms and stay alive, for now.

Disclaimer number three: We don’t have all of this figured out. Nobody does. We do the best that we can. Sometimes, self comparisons to the advertisement worthy, public projections of people “beating” ALS can cause a little angst. We’ve learned to be comfortable with our own definition of winning. 


Extra writing, I, and others have done on these topics:

 FDA Approved Alzheimer’s Drug? This Has A Parallel

Radicava Medication Experiences (I have not updated for some time)

Three Days into New Treatment and First Quandary

Article on the new drug, Rodicava, as seen by an early patient, me, Saint Louis Post-Dispatch, November 30, 2017, by Michele Munz