I AM AFRAID

My outwardly cocky attitude helps me get through the average day, but in the depth of the night, during those occasional moments of wakefulness that people of my non-tender years suffer, I admit to myself: I’m afraid.

Do I fear the Big Sleep? Of course I do, but no more than I did before my diagnosis. Before it became a less, ahhh, distant concern. That’s something we all must deal with, anyway.

I’m afraid of encroaching paralysis, that slow death of a thousand little cuts. I’m afraid of the eventual morning when I can no longer take a shower... shave myself... stand up from the Porcelain Throne... roll myself out of bed... count out my assortment of pills... brush my teeth.

I am afraid of losing my physicality, piece by piece.

Walking is a massive chore now, but I fear the day I will be forced to give it up. Falling is not an option: It is waaaay too hard on my Thinky Parts. Oops - something else to worry about.

I can eat just fine today, thanks to a (mostly) trainable left hand. But there will come a day when my arms and hands become too incompetent for the task, and I will have to depend on others to pack my pie-hole with provender. Already, Dee must cut my steak for me, it being a job for two hands. The idea of being unable to eat unaided terrifies me... and when my capability to swallow becomes compromised, that’s when the real horrorshow will be upon me.

Losing independence. Losing agency. I fear these things. I can drive now, but how much longer can I do it safely?

I am afraid of being a non-person, someone who can no longer look at others performing the myriad actions of work, of leisure, of life, and think: I can do that.

Yes: I am afraid.

But I have too many things to look forward to. And as a good friend has said to me, pain is the sensation of fear leaving the body. Perhaps fear is the sensation of pain leaving the body.

THE MIRACULOUS MEAT-MOBILE

Many of us older folks will remember a Milton-Bradley board game entitled “The Game of Life,” a pleasant way to pass a few hours in those days before Ars Electronica ate the world. You would select a little plastic car and push it along the convoluted pathway on the board according to the spin of the Wheel of Fortune. Along the way you would pick up a Significant Other and a pack of children after landing a job. At the end of the game you would either land in Millionaire Acres or the Poorhouse... the end of Real Life’s game having been considered a little beyond the intellectual capacities of its young players. Nevertheless, Milton-Bradley’s automotive analogy was - and still is - is a valid one.

Every single one of us has a consciousness - a soul, if you will - bound up in the flesh of our brains and carried around by our bodies. I think of it as driving the Meat-Mobile.

Each of us pilots a Meat-Mobile along the highway of life. There is room for only one passenger, although other Meat-Mobiles may drive alongside us for long stretches of our journey.

Some of us tool along at a leisurely pace. Others drive like bats out of hell, running off the road and ending their trips prematurely. If we are fortunate, we get to look at beautiful scenery along the way. Others drive along with gritted teeth and hands gripping the wheel, navigating through the traffic with singular intent.

The road goes on forever, but our fleshy vehicles do not.

Despite regular feedings of the finest fuel and careful maintenance at the recommended intervals, the miraculous meaty machines that transport our souls will break down. Sometimes the problems are easily remedied with a quick fix along the shoulder or a few days in the shop, after which we resume our journey. Inevitably, though, the machine – as do all machines – will no longer function. This is a rule of the road, a rule of the Game of Real Life.

Alas, we have not yet figured out how to escape our individual Meat-Mobiles and hop into new ones when the old ones no longer serve. This is an inconvenient fact, especially considering that my Meat-Car has thrown a rod.

But, damn it, I’m coasting as far as I can while the scenery is so wonderful!

THE SHOPKEEPER

I am like a shopkeeper these days.

Every day when I wake up, I do an inventory. It’s not about how many sacks of flour or bars of soap occupy the shelves: It is about what abilities are left to me.

My inventory-taking begins when I lift both of my arms straight up. I can still do this while I lie in bed. I wave them to and fro, back and forth, to keep the muscles – the ones that remain – as limber as possible. I do not want them to freeze in place.

I lift up my legs and move them up and down, side to side. I stretch them. After I get out of the bed and haul myself to the bathroom, I will grab the edge of the counter and do some squats. I will stretch my hamstrings. I really don’t want my legs to lock up.

And now we take out the checklist, thereupon to tick off the Items of the Day.

Am I standing up? Yes. So far, so good. Can I still stand up to pee? Yes. So far, so good.

There are a lot of tasks involved in getting ready for life amongst civilized humans. I take a shower. I shave. The first is a monumental challenge, made slightly less so thanks to the seating area in the shower stall. The second takes longer than it used to, owing to the fact that I must now do most of it left-handed. I am happy when I finish the job without having slashed any major arteries.

Wrapping myself in a towel is no longer a job that requires no thought – It’s surprisingly hard work. Nevertheless, I manage it.

The other little jobs proceed according to their pace. Taking the morning assortment of medicaments. Brushing and flossing the teeth - thank Gawd for the electric toothbrush and the water flosser. Applying deodorant and inserting hearing aids. I can accomplish all these unaided, although with a remarkable degree of clumsiness. My left hand was never my dominant hand, but it has become surprisingly useful out of sheer necessity: Mister Right has become that unemployed friend camped out on the couch. He doesn’t do much.

Getting dressed gets more difficult by the day. The little things we take for granted – donning our trousers and shirts, socks and shoes – now are exacting and difficult work. Depending on how fast I need to accomplish the act of clothing myself, I sometimes will need help. Thank Gawd, Dee is there to provide it.

My daily inventory continues. How hard is it to stand? How hard to walk? How long before I slouch over?

No inventory is complete without checking the valuables in the safe. In my safe are the big three items: my ability to speak, to swallow, to breathe. So far, these are still pretty good... but when they start to go, things will get serious in a big hurry.

I am a diligent shopkeeper, one for whom the process of taking inventory has pretty much become continuous. I do it every morning, and I keep doing it all throughout the day.

Yet despite my diligence, the items on my shelves are disappearing, one by one.

IN WHICH THE AUTHOR EXERCISES A RARE DEGREE OF RESTRAINT

“Sorry, I can’t make it. It’s just my ALS again. It’s fine.”

There’s a tiny part of me that wants to trot this line out – but it would be a major dick move, don’t you think?

WHY I LOVE WARREN ZEVON

Warren Zevon was a musician - a rock singer-songwriter who died of pleural mesothelioma at the ripe young age of 56. He had a rollicking case of OCD, a sardonic sense of humor that some have described as “mordant,” and a life-long dislike of doctors. The latter probably accounts for his premature demise.

He has been gone almost fifteen years now, but some of his songs still bring a lump to my throat and tears to my eyes.

I am in no way an expert on his work. I don’t own a lot of his music, Life’ll Kill Ya being the only complete album I have out of the fourteen out there. But that one gets the job done.

When you listen to Life’ll Kill Ya, it’s easy to get the impression that he wrote it after he discovered that he was terminally ill: It is packed with musings on death and gallows humor. But that impression would be wrong, despite the masterful (and obviously NSFW) tune “My Shit’s Fucked Up,” possibly the best description of being afflicted with a terminal illness I have ever heard.

Well, I went to the doctor 
I said, “I’m feeling kind of rough” 
“Let me break it to you, son - 
Your shit’s fucked up.” 
I said, “My shit’s fucked up? 
Well, I don't see how...” 
He said, “The shit that used to work - 
It won’t work now.”

Exactly.

Then there’s “Porcelain Monkey,” a satirical take on a Elvis that also has a grinning death’s head lurking in the background.

But the song that stabs me in the heart – a song that is still difficult for me to listen to – is “Back in the High Life Again.” Written by Steve Winwood and Will Jennings, it was happy and upbeat, a huge hit for Winwood in 1986. Fourteen years later, Zevon covered it and turned it into the cri de coeur of a delusional lost soul. It was heartbreaking. It was perfect.

Owing solely to coincidence, that song will forever be associated in my thoughts with the passing of a dear friend. So effectively does it bring back those feelings of grief that I can barely bring myself to listen to it. But I love it nonetheless – and, by extension, I love its creator.

And as for me? My shit’s fucked up.

WALKING ON JUPITER

“If I forget thee, O Jerusalem, may my right hand forget her cunning.” - Psalm 137


People ask me, “How do you feel?”

This is a little different from the “Hi, how are you?” conversation opener we’re all familiar with. “How are you?” is the kind of question people ask without really expecting (or wanting) a lengthy disquisition by way of a response.

Sometimes, though, people really want to know what it feels like to be me these days. Assuming you are one of those people, I will try to tell you.

The best description is that I feel as though the gravity knob has been cranked up. Ever since I first noticed that things weren’t quite right four months ago, it just seemed as though I was heavier. Walking - even simply standing - took much more effort. Today, I feel as though I’m walking on Jupiter (if you can imagine Jupiter having a surface, that is). It takes all my effort to get from Point A to Point B, and those two points are growing closer together daily.

Bending over and picking stuff up? Not fun, but I can do it. Cleaning out the cats’ litter box has turned into a Chore rather than a chore. Jumping? Fuhgeddaboudit.

My right arm, ahh, there’s another story. It was three months ago when the weakness in my right arm and the diminution of the fine motor skills in my right hand began to be not merely noticeable, but downright annoying. We were in that phase of our relocation in which our main activities involved hanging pictures and mirrors, assembling bookcases and furniture, and just plain schleppage: moving a lot of stuff around.

What became obvious was that my right arm was Not Being Helpful.

Picking up an electric drill, nailing picture hangers into walls - all of the myriad, routine tasks of setting up a new residence were becoming agonizingly, frustratingly difficult. And it was getting a little worse every week.

Now, in mid-July, my right arm is still functional, but only kinda-sorta. It depends on what I ask it to do. Position, time of day, and goodness knows what other invisible factors all play into whether it will perform a given task. But increasingly, it is going on strike. It is insubordinate, lazy. It is Beetle Bailey, taking a nap while Sarge shouts orders at him. I give it a command; it does not obey. Despite the fact that I remember Jerusalem, having been there almost exactly six years ago, my right hand is definitely in the process of forgetting her cunning.

Sometimes lifting a simple coffee cup feels more strenuous than bench-pressing 100 pounds used to be - the cup might just as well be welded to the table. Fortunately, my left arm can pick up the slack... for now.

I am gradually learning to function as a lefty. That’s hard work, too. Try shaving while (mostly) using your non-dominant hand. Don’t forget the styptic pencil.

The good thing? It’s not painful. Not crampy. My muscles twitch pretty much all over, but that’s mostly noticeable as I lie in bed at night. These gentle muscular fasciculations aren’t a source of discomfort - just a reminder that, gradually, the nerves that connect my voluntary muscles to my brain are quietly dying, so those muscles just talk to themselves.

So: it doesn’t really hurt, mostly. It’s just damned difficult.

A FAREWELL TO IRONS

I play my pestilential game
Without a single speck of shame.
I hack my way around the course
With absolutely no remorse.
The fairways, I have rarely seen —
I struggle once I’m on the green.
My drives will hook, or maybe slice.
They do not follow my advice.
My shots all seek the woods and water.
They do not travel where they orter.
O, I’d forgo all worldly goods
If I could play like Tiger Woods
For just one game. ’Tis not to be;
I guess I’ll have to play like me.


I learned how to play golf under my mother’s exceptionally patient tutelage over a half-century ago. Since then, I have played at places ranging from municipal courses that were not much more than cow pastures to some of the most exalted cathedrals of golf: the Black Course at Bethpage, Pinehurst Number 2, and Medina, all of which have hosted the US Open.

This is not to say that my game was anything to write home about. My scores typically exceeded 100, peppered by the occasional pleasure of a round in the mid-90’s. Nevertheless, any given day taking what Twain has been credited for calling “a good walk, spoiled” would have enough beautifully executed shots to keep me coming back again and again. It’s the same type of variable-ratio reinforcement that keeps gamblers at the tables.

Earlier this week, we went to Top Golf with Dee’s brother and his family. Great fun. Nominally a driving range, the place bears as much resemblance to an old-school driving range as does an Indy race car to a horse and buggy.

Whoever designed Top Golf seems to have taken a Japanese concept, run it through the British business model development machine, and popped out a golfy catchpenny engine of monumental proportions. If you don’t pay attention, you can run up a bill the size of which used to be associated with Hong Kong hostess clubs. But it’s enjoyable nonetheless.

After watching everyone else flail away with an assortment of drivers, mashies, and niblicks, I took my turn at the tee, not really expecting to be able to execute what used to be a powerful, reasonably coordinated, surprisingly inconsistent golf swing. Yet even given my low expectations, I was nevertheless surprised at how difficult it was to draw the club back.

My favored Vardon grip was now beyond the capabilities of my right hand, which provided the barest hint of guidance as my left arm controlled the swing in a strangely foreshortened arc. I was able to hit a few balls off the mat, but it was obvious that crisp, 160-yard seven-iron shots were now forever beyond my reach. For me, the driving range was now the chipping range.

It was a sobering reminder that I was entering a new world. At the same time, it was thoroughly enjoyable to watch our niece and nephew whacking golf balls into the three-story-tall void. And the beer was cold, so, WINNING!

My bag of clubs went back to Texas with Dee’s brother. They will have a happy new home there where they can be put to good use. As for me, my days of playing golf are over... and that’s OK.

WHA’ HOPPEN? (PART 3)

It’s hard to imagine that Dee and I were looking forward to our appointment at Emory. You would think, given our nervousness at the potential of receiving bad, bad news, that we would've been dreading our visit there. But we weren’t. Perversely enough, we were almost eager.

The fact is, having the Damocles’ sword of uncertainty hanging over us was beginning to wear. It is nerve-wracking to know without question that something is seriously wrong, while at the same time to be unable to put a name on it... and to chart a forward course. Could whatever this thing was be fixed somehow?

We had, by now, pretty much completed our move into our new house in Woodstock, just seven miles up the road from our old neighborhood. We had also taken a ten-day road trip to visit our daughters in the Northeast. The driving was lengthy but manageable, and the only real difficulty I had was with walking any distance over a half-mile. Center City Philadelphia would have been completely unmanageable had it not been for the ease of getting Uber rides.

At last came June 19, the day of our appointment at Emory, a day both eagerly awaited and dreaded in equal measure. We arrived; we signed in; we were ushered into an examination room, and - following the collection of a few vital statistics, I enrobed myself in a gown and waited for the Neuro-Sawbones to show himself.

Show himself he did, and after a clinical examination that seemed to be both lightning-fast and to take forever, he looked up at me and said, “Well, there’s no point dragging this out - you have ALS.”

“Well, this sucks,” was my first thought as adrenaline squirted into my bloodstream. For it is one thing to suspect that there is something Very, Very Wrong with you, and quite another to have it confirmed.

Of course, my first verbal response was to make a semi-serious jape.

And then I looked at Dee, whose eyes had flooded with tears... the tears that should have been in my eyes but were not. For months I had told her not to worry, that there were other, much more likely answers than the Worst Possible Thing. And, as so often had happened through the course of our long years together, I was wrong and she was right. I held her in my arms, as much to comfort myself as to comfort her. Yet I was more concerned for her than for me. She had dealt with so many crushing events in her life, and here I was, adding to the pile.

Then came the process of being sucked into the System. Blood samples from both of us: why had I gotten ALS while Donna had not? Questions about family, about lifestyle. I was now officially a pALS - a Patient with Amyotrophic Lateral Sclerosis. Would I consider enrolling in this or that clinical study? Well, why the hell not? Thick folders full of information about support organizations, about what to expect over the road ahead, were thrust into my hands.

And now I have a better picture of my future than most of us.

Like all of us, I still buy my lottery tickets every morning. The Getting Hit by a Bus lottery. The Heart Attack lottery. But my odds have shifted ominously. For me, the overwhelming likelihood is that, sometime in the next two to five years, the creeping paralysis that already has made itself known to me in so many insidious ways will stop my breathing. And then, I must rely on your memories to keep me alive.

And I no longer buy green bananas.

WHA’ HOPPEN? (PART 2)

[When we last left our intrepid narrator, he was just beginning to experience that nervous, asshole-puckery feeling that comes with knowing that something is seriously wrong, but the wrongness does not as yet have a name: an inchoate sensation of dread.]

My internist had suggested that I visit my neurologist and get a nerve conduction test. OK, fine.

I had heard numerous stories about these tests, most of which had described them as some sort of mash-up between Weird Science and medieval torture. As it happens, my test felt totally inconsequential. A little electrode, a little zap, feel the muscle twitch. Afterward, I asked my neurologist whether the Big Bad Scary Diagnosis was a possibility.

“Well, it’s a possibility, but it’s extremely unlikely,” she said. “What I’d like to do is to order up an MRI of your cervical and lumbar spine, to see if something’s getting pinched.  I’m going to put down a tentative diagnosis of cervical radiculopathy.”

Cervical radiculopathy. I couldn’t help but like the idea of a condition that seemed to borrow its name from the word “ridiculous.”

So I went for my MRI: an hour of lying in a giant metal doughnut while a symphony of metallic noises worthy of Edgard Varèse played around my head. It was, perversely, like a colonoscopy in reverse.

Then the results came back. A little arthritis here, a bulging disc there, a sprinkling of stenosis... but nothing that would explain the changes that were creeping over me day by day. My neurologist recommended that I seek a second opinion from the Emory Neuromuscular Clinic, and she gave me a name and a number to call.

“How about that Big Bad Scary Diagnosis?” I asked. “Is that still on the table?”

There was hesitation in her voice when she answered. “You need to see the people at Emory.”

Stay tuned, friends! More to come...

WHA’ HOPPEN? (PART 1)

For some time now, Dee had been subtly agitating for a household relocation.

We had been in the same oversized, overstuffed East Cobb residence for nearly two decades, during most of which time we were empty-nesters. There was more house than we really needed, except for those times when we would host family and friends for holiday or Sabbath dinners.

Two years ago, Dee took a bad fall, cracking her left hip and shattering her wrist. Suddenly, negotiating the stairs between the main level and our bedroom became a painful, physically challenging task to be undertaken twice a day: down in the morning, up at night. The idea of a house with a Master on the Main, in realtor parlance, started making a lot of sense. Who knew when the next physical challenges would be coming along, and who could tell how much warning we’d have?

I resisted the move. The whole idea of moving – selling the house, packing up our monstrous mass of detritus, finding another place to live – horrified me, especially as all our previous relocations had been at employer expense. Now we would be on our own during the entire obnoxious process.

And yet, Dee was right. Getting out of the big house and moving to something a bit more sensible was what we needed. Many of our friends were already doing it: moving out of expensive east Cobb and downsizing. And so, in late 2017, we did the necessary repairs and painted the entire damned interior, all preparatory for staging the house and putting it on the market.

The entire nerve-wracking process of disposing of our former residence and purchasing our new one took roughly three months. Three months of cramming crap in boxes. Three months of nervously waiting for bids. Three months of schlepping stuff around... of jettisoning the useless miscellany that accumulates over the span of decades... of online garage sales and giveaways... of deciding what we really wanted to take with us as we relocated a mere seven miles north by northwest, just over the Cherokee county line.

Around the time of our move, I noticed something just wasn’t right. Somehow, my standing posture had changed, devolving from a normal erect stance to that of an old man, with slumped shoulders and bent knees. I saw that I had begun walking with a forward lean, and those boxes - all those damnèd boxes! - had gotten awfully heavy. Having been in reasonably good condition after years of weight training (my trainer would refer to me as The Beast-O), this was... weird. Maybe I had wrenched my back out. Yeah, that was it.

Then, one day, I was hanging up a pair of shorts on one of those hangers with the spring-loaded clips... and I couldn’t squeeze those clips open with my right hand. Now I knew something was wrong.

I had, just a few weeks earlier, begun taking a new medication. Could that be the problem? I called my internist, and the sawbones on call for the weekend suggested stopping both that drug and the statin I had been taking as well. “Come in Monday and we’ll order some blood work,” he added.

The results confirmed what I already knew: my muscles were literally melting away. But it was not myopathy. My muscles were not the problem. “See your neurologist,” my internist said. “Get a nerve conduction test.”

And that, friends, is when I began to feel that sensation familiar to so many of us: the dreaded Anal Pucker. Because I had had the bad habit, as a child, of reading my dad’s copy of the Merck Manual, over two thousand eight hundred pages of onionskin paper on which was delineated every affliction humankind is known to suffer. I had my own copy now, and all manner of Nasty Things began to suggest themselves...

...but we’ll save the rest for later, shall we? (Hey, it can’t be a cliffhanger - we know how it turns out!)

GALLOWS HUMOR

You should be aware that the very first thing I said to my neurologist after he pronounced my doom was, “This may sound about as important as a fart in a hurricane, but... can I get a handicapped placard?“

Gallows humor? I has it.

Making fun of the unspeakable is, for me, a defense mechanism. It’s the way I cope with the inevitable feelings of sadness and fear that come with my condition. I’m not sure whether other people appreciate it, but it comes with the territory.

And you’ve got to have a sense of humor to deal with some of the bizarre synchronicities this universe throws at you.

F’rinstance: The very same day we got the Bad News, I found a message on my phone from the local cemetery, where my wife and I have a little bit of real estate. “Give us a call when you can, nothing urgent.“

Timing is everything.

Indeed, it was nothing urgent – just a request to set up a meeting to discuss their conversion from paper to digital record-keeping. But the timing was exquisite, amIright?

NAMING CONVENTIONS

When I decided to start this blog - my third! - I had to select an appropriate name for it. I decided on the one you see above, but several others were under consideration:

ALS well that ends well - Well, there’s only one way this ends – and it ain’t too good.

The Gehrig Chronicles - Lou Gehrig is the name Americans most often associate with ALS. But these are my chronicles, not his.

The Lame Duck Diaries - The term “lame duck“ refers to someone who is still in office but whose successor has already been selected. You’re still around, but you can’t accomplish much.

Writing a blog in these days of Facebook‘s ascendancy seems like a futile and stupid gesture. Nevertheless, I think there’s value in blogging. I have written on various electronic pages for fourteen years now, and I have my own opinions and motivations for doing so, not least of which are self-aggrandizement and time-wastage. But unlike people’s Facebook pages, blogs must seek their own audience. And I think there will be an audience for this one.

I’m writing this because I am one of the tiny percentage of humans who has ALS: amyotrophic lateral sclerosis.

Many people in the US know this disease by its other name, Lou Gehrig’s disease. Gehrig was a famous baseball player - the legendary Iron Horse of the New York Yankees - who resigned in 1939 after having been diagnosed with the mysterious ailment that had caused his formidable batting average to melt away. His farewell speech was a model of humility and strength, a tearjerker unto this day.

ALS got a lot of attention four years ago when people started posting videos of themselves on the Internet getting buckets of ice water dumped on their heads, with the objective of raising money for medical research. Lou would probably have chuckled over those shenanigans.

Here’s what you need to know about ALS: It is incurable, and it is inevitably fatal. The only question is how long it takes to kill you.

Once in a while you’ll hear about an outlier: someone on the far side of the survival curve. Stephen Hawking was one such person, having lived over 40 years after being diagnosed. His was an unusual case, and he put up with a lot of quality-of-life impairment to get there. As for myself, I’m not going to be walking the planet 40 years from now. Of course, that’s no big surprise, given that I am 65 today.

Ten years? Maybe. I’ve spoken with a guy who’s been around that long. But that’s also pretty far out on the curve. It’s much more likely that I will be shuffling off our mortal coil sometime between two and five years from now. I’m not happy with those odds, but facts is facts.

There’s a lot of exciting medical research going on right now, much of it involving stem cells. Some of the studies even indicate that the progress of the disease can be reversed. This is awfully good news, but probably not for me. The likelihood of a working stem cell therapy being available to me in time is not a proposition that any betting man would take.

I’ve often said that we buy a lottery ticket every day when we get out of bed. In fact, we buy a whole bunch of them, and there’s a way higher chance of us getting a winner than if we buy one of those Mega Millions jackpot ducats. I’m talking about the “Getting Hit by a Bus” lottery. Or the “Kidney Cancer” lottery. And I, friends, have won the fucking jackpot.

I’m planning to write here until I no longer am able to do so... and I hope that’s a long time. But my mad typing skillz, always questionable at best, are now being challenged in all kinds of new ways. So, as Timothy Treadwell famously said, bear with me. Because this is my last and most personal story.