Chapter 29: The Spoils of Victory

The protagonist in this loss filled saga finally steals a win! But is it too little too late?

Whenever there’s a substantial gap between my blog entries, you’d be correct to assume that I’m struggling to keep up with my progressive disability. But that’d not be the main reason. Reality would reveal an author far too apathetic to do to anything, let alone pen deep truths. Anyone who’s experienced the crippling agenda of clinical depression knows the void I speak of. Where hope once stoked the fires of passion, you’ll find only ashes. And they’re swirling in the winds of the forever looming storm.

I’ve been traveling to Washington University in St. Louis to receive lumbar puncture-administered doses of a highly regarded study drug that seeks to repair ALS-damaged motor neurons. Jamie and I were thrilled to get into the extremely competitive trial. For the double-blinded phase last year, we traveled all the way to Boston’s prestigious Healey Center at Mass General. Devastatingly, the drug did not change the course of the disease.

We traded in flights to Boston for round trips to St. Louis in this bad boy.

However, there was still that 1-in-4 chance I received placebo, and I was desperate to find out with absolute certainty. After lying untreated for many months following completion of that phase in Boston, I caught wind that the FDA finally did something right and had granted the drug’s sponsor an Open Label Extension (OLE). This not only extended my trial but also assured me I would receive the actual study drug, not placebo. We immediately contacted the research coordinator at the Healey Center and were fortunate enough to be transferred to a much closer site at Wash U.

Given the lack of benefit thus far, everything shifted from hoping the blinded drug was the real deal to praying it was placebo all along. In other words, I had a 25 percent chance of potential salvation if the drug worked as intended in my body. Not great odds; nevertheless, the dream was alive.

On my fourth dosing visit in St. Louis, my neurologist shared with us an entirely different take. He was skeptical I would see any benefit. In fact, he asked me to think long and hard about continuing the trial altogether. His chief concern was whether the cost of travel was worth it—not in the financial sense, but in time spent away from family and the unnecessary stress on my body.

He further buttressed his case for that particular line of questioning with the results of my serum neurofilament light chain (sNfL) lab results. As a plausible biomarker for neurodegeneration, sNfl is becoming much more widespread in research trial endpoints. The higher the number, the worse the correlated prognosis. Normal levels are around 30. Average ranges for ALS are between 80 and 100. Mine, we found out that day, is 160.
Upon hearing that revelation, I sat silent and stunned while the room burst into tears. It was diagnosis day all over again.

I had been progressing at twice the normal rate as the average pALS (on the functional rating scale) so it shouldn’t have come as such a shock that my blood was showing twice the neuronal damage. However, the most ruinous tell was the tone in which my doctor delivered his evidence for me to exit the trial. He clearly believed I was holding a losing hand. The correct move was to fold.

When you’re laser focused on beating ALS, you don’t give yourself permission to think about how you want to die. To accept what’s happening and redirect all your energies to living with ALS (sans hope) requires a whole different level of courage that I did not possess. But it appeared I had no choice. I was now face to face with the inevitable.

The life of acceptance proved to be an even bigger challenge than I anticipated. News of my latest prognosis spread like wildfire through my network, hence, a farewell tour of sorts commenced. One by one, from the entourage of my lifetime, friends came to see me, unsure of what they’d find. And one by one they left, having discovered the magnitude of my love for them.

If the average pALS dies 2-5 years after their first symptom, I figured I’d be lucky to see another year given the aforementioned data. It felt satisfying to say the things I needed to say to everyone. Yet there was still much for me to cross off my list before departing. That docket included letters to be delivered posthumously to the girls when they reached various milestones, business/legal loose ends, and a family trip to the ocean.

When suddenly (as if upon hearing that last line), the ocean found its way to us. Though it was no favor. For a wave of depression consumed me, and at the worst possible time, no less. All I could muster was a catatonic-like stare out my window at the plethora of able-bodied folks walking about. Anything more was too daunting a task. My surrender to ALS had officially sapped my will to live. Two precious weeks passed in this daze of despair.

On the tail end of that 14-day depressive episode it dawned on me that getting out of my chair wasn’t getting any more difficult (amazingly I’m still able to stand). For that matter, ALL activities of daily living hadn’t gotten any harder in quite some time. Not in the least. Even though I had "plateaued" in the past, never before had one lasted this long. As someone who was declining at such a rapid rate, that struck me as odd.

We arranged a teleconference with our research team at Wash U. The whole virtual roundtable was caught off guard when I demanded to press forward with the trial. Jamie was the epitome of supportive (she picked this trial after all). The lab coats were less so. Ultimately, everyone respected my wishes.

My reasoning to continue was equal parts finishing what I started, curiosity surrounding this recent "plateau," and guilt over giving up when our daughter’s name is Hope. But I had a fourth motivation. And in this case, it was to regain what I lost during my acceptance phase: A future in this family.

A few weeks later we returned to St. Louis for my fifth dosing visit. Before the needle was thrust into my spinal cord, we reassessed my muscle strength for the first time. I braced for those results harder than I did for the actual shot.

When the nurse practitioner returned with my numbers, in an attempt to prepare myself for the worst, I studied her thoroughly, searching for another tell. Alas, I detected nothing. She had a career in poker awaiting her should things not work out in medicine. I would have to settle for a surprise reveal.

Mind you, I can’t can’t speak of specifics regarding this trial. So let’s just say what I heard next from the future poker pro sent this Guinea pig on his way home sporting an ear to ear grin. Perhaps there WAS a way to win with a losing hand. You just have to be willing to push your chips all-in.

Unfortunately, it would be disingenuous to end the chapter there, for a new paradigm was about to become painfully evident back in Kansas City. I hadn’t taken into consideration that by potentially extending my life, I was simultaneously potentially extending the suffering of those who loved and cared for me. Thus, upon my triumphant return, a rare victory against an undefeated disease was cut abruptly short.

The life we had dreamed of is now a distant memory.

My current state of total dependence had already completely overwhelmed everyone in my orbit. Jamie’s world, in particular, had been revolving around mine for the past 18-months. And the gravity of keeping three humans alive (1-year old, 3-year old and husband) all while hemorrhaging our limited resources was not a sustainable environment to live in. It was inescapable that if ALS didn’t kill me, then it would surely destroy my wife.

When we mutually decided to accept my dire prognosis following that infamous fourth visit, Jamie was not prepared for me to suddenly about face and rejoin the fight. Her goal had already evolved from keeping me alive at all costs to ensuring I was comfortable and happy for the remainder of my days.

Now, it’s not my intent to lead you on. Though I feel things are looking promising at this admittedly early stage, I don’t know how this treatment will play out in the long run. There was a friend of ours who didn’t respond to this very same study drug and has subsequently dropped out.

But what I DO KNOW is there are ramifications for living your life in a frozen state of constant dependence. Because for me, tragedy isn’t being stricken down in your prime with a debilitating terminal illness. Tragedy is finally discovering your miracle drug and then finding out you’re too late.