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Got a bucket list? I have one. Well, thanks to COVID-19, mine is a flexible bucket list.

A list of things that one has not done before but wants to do before dying

The doctors say I’m dying. My body tells me every day that I’m dying. So, if I’m dying then I need a bucket list.

What do I want? More than a few things have come to mind.

First, KFC original recipe fried chicken skin. Uh huh. Just the skin. The chicken is OK. The skin is great. 11 herbs and spices will do that.

What else?

Second, I put drive a Tesla on the list. That’s not likely to happen. What else? My wife and I planned to add a few vacations to the bucket list, too. Las Vegas, San Francisco, Napa Valley, maybe even Maui and a drive up the 10,000 feet of Mt. Haleakala (and, hopefully, back down again).

One last visit to see family.

Thanks to COVID-19, KFC chicken skin prevails. Travel does not.

What else?

Frankly, not much. Dinner out with my wife or friends? COVID-19. Cruise through the shopping mall in a new motorized wheel chair? COVID-19. A summer driving excursion, over hill, over dale? See above.

Perhaps I’m not imaginative enough. Perhaps I have most of what I already need and want.

My bucket list isn’t very long.

In college you get courses with names. Psychology 101. English 101. Gym 101. OK, maybe not that last one, but you get the idea.

What about Symptoms 101?

That seems to be the number of symptoms associated with ALS and ALS-like neurological diseases. From Mayo Clinic:

Signs and symptoms of ALS vary greatly from person to person, depending on which neurons are affected.

Signs and symptoms might include:

• Difficulty walking or doing normal daily activities
• Tripping and falling
• Weakness in your leg, feet or ankle
• Hand weakness or clumsiness
• Slurred speech or trouble swallowing
• Muscle cramps and twitching in your arms, shoulders and tongue
• Inappropriate crying, laughing or yawning
• Cognitive and behavioral changes

That’s the short list of symptoms from Mayo Clinic and the most common symptoms for what might be typical ALS. I had none of those symptoms in the beginning and only one common symptom two years later— doing normal daily activities— that one related to a weak diaphragm and very weak back muscle.

That does not mean everything else is normal. It. Is. Not.

My smaller list of ALS-like symptoms are those that occur much later in ALS, specifically the respiratory problems that require ventilation. Add to that some weird muscle cramps, more fasciculations than those I have had for over 30 years, weakened voice, lack of appetite, weird movements, and a very wacky autonomic system which adds to the misery.

I haven’t done a full count of symptoms yet, but I’m sure the list would get to about 101.

Amyotrophic lateral sclerosis— that lengthy, almost unintelligible descriptive medical term which most of us translate as ALS— is a nasty, debilitating disease.

ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size. It may begin with weakness in the arms or legs, or with difficulty speaking or swallowing. About half of the people affected develop at least mild difficulties with thinking and behavior and most people experience pain. Most eventually lose the ability to walk, use their hands, speak, swallow, and breathe.

As miserable as that list appears, the one that gets most ALS victims is the one that arrives last. The inability to breathe. Most people with ALS die of respiratory failure. They cannot breathe.

A very small percentage of those diagnosed with ALS have what is called respiratory onset whereby most symptoms are confined to core muscles, diaphragm, intercostal muscles; those used to breathe.

My version of ALS is considered to be respiratory onset.

While those with ALS have a life expectancy of 3 to 5 years after diagnosis, and many live beyond a decade, those with respiratory onset are rare; they have less than two years. The verdict is in.

My two years are up.

Almost.

Neurologists use a rating scale to determine ALS disease progression.

The progression and severity of ALS is rated by doctors on the ALS Functional Rating Scale, which has been revised and is referred to as ALSFRS.

Since ALS was on my future radar for more than a year I walked through a number of rating scales to see how far my symptoms had progressed. One is a revised version of ALSFRS.

ALSFRS-R includes 12 questions that can have a score of 0 to 4. A score of 0 on a question would indicate no function while a score of 4 would indicate full function. This scale has been useful for doctors in diagnosing patients, measuring disease progression…

I did not find the scale useful until recently.

Why not?

The ALSFRS-R scale has some limitations though since it is not useful to compare scores of people who present with different onset. In ALS the main type of onset is bulbar, followed by limb-onset; which describes the region of motor neurons first affected. Individuals may also present with respiratory onset, but this occurs very rarely.

Respiratory onset.

Many other symptoms have begun to appear, however, breathing is the most prominent, obvious, and debilitating symptom.

Thanks to very weak core muscles, especially breathing and back, I cannot walk.

Yet, I can speak and type.

Go figure.

They say hindsight is 2020 vision. I’m old but my last test at the eye doctor confirmed 20/15 vision. Two cataract surgeries and a slight magnification of the new lenses probably helped with the score.

I’m farsighted.

Anything from outstretched fingertips to eyes is a bit blurry without glasses. Yet, I, as with many of us, suffer from hindsight bias.

Hindsight bias, also known as the knew-it-all-along phenomenon or creeping determinism, refers to the common tendency for people to perceive events that have already occurred as having been more predictable than they actually were before the events took place. As a result, people often believe, after an event has occurred, that they would have predicted, or perhaps even would have known with a high degree of certainty, what the outcome of the event would have been, before the event occurred.

Uh, say what?

In simpler terms, we knew something would happen a certain way but only after it already happened. It’s much easier to figure something out after we know what’s going on than it is before we knew it.

Does that make sense?

Hindsight bias may cause distortions of our memories of what we knew and/or believed before an event occurred, and is a significant source of overconfidence regarding our ability to predict the outcomes of future events.

Sometimes we think more of ourselves than we should.

I did not predict the outcome of our search for whatever was causing my symptoms. ALS is a disease of exclusion and I spent months pouring over hundreds of research papers and articles in a curious but perhaps vain attempt to help with the diagnosis.

The trip to Mayo Clinic was eventful in many respects. Mayo Clinic’s methodology is unlike anything I have experienced. Their entire staff was warm and friendly yet professional and very efficient. My neurologist looked over the detailed records I offered and asked me what I thought was going on.

I had a list.

ALS was on top.

Examples of hindsight bias can be seen in the writings of historians describing outcomes of battles, physicians recalling clinical trials, and in judicial systems as individuals attribute responsibility on the basis of the supposed predictability of accidents

That was not hindsight bias. It took nearly 18 months to reach a conclusion of three possibilities. ALS. Paraneoplastic syndrome (I have an overactive immune system. And a dark horse called thoracic radiculopathy.

If I could back up my hindsight about a year we would have lived it differently, but that’s another issue. Hindsight is 2020 vision. It’s easier to make judgements and rewrite motivation after the fact.

What is time? There are many concepts but this seems to be the most understood definition of time:

Time is the indefinite continued progress of existence and events that occur in an apparently irreversible succession from the past, through the present, into the future.

We measure time backwards and forward but do not yet have the capability to go backwards or forward in time.

Time travel:

Time travel is the concept of movement between certain points in time, analogous to movement between different points in space by an object or a person, typically with the use of a hypothetical device known as a time machine. Time travel is a widely recognized concept in philosophy and fiction.

The reality of the present is what we deal with, though we can plan for the future, and take lessons from the past. Have you ever said or thought, “If I only knew then what I know now?”

If I could back up only 24 months in my life, back to when ALS symptoms began to appear, and knowing nothing could be done, I would have lived those 24 months differently. Certainly there would be far less effort trying to track down the disease and we would have saved a trip to the Mayo Clinic in Minnesota.

In the middle of winter.

We have not devoted much time to the thought of time travel, but certainly we would have devoted less time to the search for a disease and more time to the pleasure of living. As of now, 24 months later, there is little pleasure in living.

For me, sleep is the only escape from a nasty reality. Pleasant moments are few and far between. What I f I knew then what I know now? More intimacy, more family, more preparation. Now, the big sleep is dead ahead.

Literally.

Our family lives far away from where we live but close enough to the Mayo Clinic that we could book a flight from there to visit family before heading back home.

Mayo Clinic conducted two more weeks of tests to rule out other disease possibilities and the various motor neuron diseases to end with the ALS diagnosis.

It was time to say goodbye.

There isn’t much to say so we kept it simple. This, to paraphrase the Mayo Clinic’s lead neurologist, likely is ALS. It’s time to get affairs in order. While ALS was on top of our list of potential diseases, prayers were there, too, and we kept family informed of progress.

It was time to say goodbye.

We live in a different age. Nearly everyone has a cellphone and that means FaceTime, Skype, Zoom, texts, email, and all the tools of modern communication keep the lines of communication open all the time; a new era far removed from letters and postcards of yesteryear.

We took a plane and rented a car to visit relatives and to say our last face-to-face goodbyes. The trip was pleasant and uneventful although everyone took a measure of hope that my ALS would fall in one of the 10-percent groups.

10-percent groups?

Most people with ALS die from respiratory failure, usually within 3 to 5 years from when the symptoms first appear. However, about 10 percent of people with ALS survive for 10 or more years.

That’s one 10-percent group, although I would rather not extend the current state of misery and torture for another eight years, but I understand the sentiment.

ALS is a diagnosis of exclusion; all other causes and diseases are eliminated first. That’s what Mayo Clinic did. Interestingly, there are more ALS mimic diseases which means around 10-percent of all ALS diagnoses actually turn out to be something else.

While it might seem good to be in that group, many of those mimic diseases are debilitating and deadly, too.

So, we said goodbye.

I describe ALS as a slow motion death sentence. Here’s how it works:

Amyotrophic lateral sclerosis (ALS), also known as motor neuron disease… is a disease that causes the death of neurons controlling voluntary muscles. Some also use the term motor neuron disease for a group of conditions of which ALS is the most common. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscles decreasing in size.

After at least 24-months since probable onset and the first breathing problems, and over a year since the original diagnosis, I have all of that. Some people with ALS live for a decade or more; some only a few years.

But it’s more complicated than that.

No cure for ALS is known. The goal of treatment is to improve symptoms.

Since there is no cure and those with ALS live only a few years, it can be described as a slow motion death sentence with relatively rapid debilitation.

But it’s more complicated than that.

About 10% of all cases of ALS begin before age 45 (“young-onset” ALS), and about 1% of all cases begin before age 25 (juvenile ALS). People who develop young-onset ALS are more likely to be male, less likely to have bulbar onset of symptoms, and more likely to have a slower progression of disease.

I’m old already.

Late onset (after age 65) is associated with a more rapid functional decline and shorter survival.

Uh oh.

Respiratory-onset ALS is a rare variant that accounts for about 3% of all cases of ALS, in which the initial symptoms are difficulty breathing (dyspnea) with exertion, at rest, or while lying down (orthopnea). [Traditional] symptoms tend to be mild or absent at the beginning.

That is where my symptoms began. Respiratory onset. Other muscles are far less affected.

It is more common in males.

It sucks to be a male sometimes.

Respiratory-onset ALS has the worst prognosis of any ALS variant; in a population-based study, those with respiratory-onset had a median survival of 1.4 years and 0% survival at 10 years.

Uh oh.

I’m at the median already.

The first day of tests at Mayo Clinic consisted of a battery of pulmonary tests. The result was as expected.

Strong lungs. Weak muscles; specifically diaphragm and intercostal muscles.

Intercostal muscles are several groups of muscles that run between the ribs, and help form and move the chest wall. The intercostal muscles are mainly involved in the mechanical aspect of breathing. These muscles help expand and shrink the size of the chest cavity to facilitate breathing.

As with the more well known diaphragm, you need those muscles to breathe. For whatever reason, mine were weak.

The afternoon tests included the aforementioned EMG— electromyography. Think of being poked with dozens of needles in dozens of muscles (including the tongue); each needle an electrode of sorts used to measure electric impulses.

Electromyography is an electrodiagnostic medicine technique for evaluating and recording the electrical activity produced by skeletal muscles. EMG is performed using an instrument called an electromyograph to produce a record called an electromyogram. An electromyograph detects the electric potential generated by muscle cells when these cells are electrically or neurologically activated. The signals can be analyzed to detect medical abnormalities, activation level, or recruitment order, or to analyze the biomechanics of human or animal movement.

What I said but with more words, more syllables.

Here’s the clinical interpretation of the EMG test results:

This is an abnormal study. The electro diagnostic findings are most consistent with a length-dependent, large fiber, atonal predominant sensormotor peripheral neuropathy with superimposed polyradiculopathy versus polyradiculoneuropathy. However, the possibility of an early stage diffuse disorder of lower motor neurons with underlying peripheral neuropathy cannot be ruled out.

In essence, ALS is on the way.

It looks like a motor neuron disease; not many of those, and ALS is on the list.

My neurologist:

This likely is ALS. It would be wise to get your affairs in order.

I’m a curious person so my wife and I had already done a long and serious study of possible diseases over the previous year. After all, my first neurologist, a year earlier, had identified ALS as a potential disease. That was my conclusion, too.

We were ready. Almost.

Within a week I began to suffer very labored breathing within minutes of my morning run jog trot shuffle. It was so bad I could not finish a 15 minute walk, let alone the shuffle.

Resting helped but with almost any exertion or movement I would become completely out of breath.

What’s going on?

A trip to the emergency room yielded my standard test result. Nothing is going on. Blood work was normal except for slightly elevated CO2 (still within normal range and indicative of some unknown neuromuscular issues).

Test after test in ER confirmed the same thing. Something is going on but doctors couldn’t put a finger on it.

What’s next?

Travel.

The ER doctor put a call into my most recent neurologist who recommended a trip to Mayo Clinic in Minnesota. Yes, that Minnesota. Too hot. Too cold. Too wet. Too dry. Too many bugs.

I have a handful of phrases that I use to keep life balanced. Some I made up on my own. Here’s an example:

Nothing improves without change.

Here’s another one:

Everybody wants your money.

One more:

If everyone is out to get you, paranoia is a good attitude to have.

I like this one, too; often attributed to Mark Twain.

“Lies, damned lies, and statistics” is a phrase describing the persuasive power of numbers, particularly the use of statistics to bolster weak arguments. It is also sometimes colloquially used to doubt statistics used to prove an opponent’s point.

The phrase derives from the full sentence, “There are three kinds of lies: lies, damned lies, and statistics.”; it was popularized in the United States by Mark Twain and others, who mistakenly attributed it to the British prime minister Benjamin Disraeli. The phrase is not found in any of Disraeli’s works and the earliest known appearances were years after his death. The phrase was attributed to an anonymous writer in mid-1891 and later that year to Sir Charles Dilke, but several others have been listed as originators of the quote, including frequent erroneous attribution to Twain himself.

Numbers don’t always tell the whole story for your health. Check out these heart rate numbers from a recent morning run.

Looks good, right? What does it mean? Healthy runner? Maybe. Or, someone with a deadly disease? Possible.

My watch records my heart rate while exercising. 150 beats per minutes is maximum for my age and I had little trouble reaching that, despite the odd posture, mild fatigue, and breathing problems.

What’s going on? Whatever it was doctors didn’t know and it was about to get worse. Much worse.