We were skeptical at first because the neurologist considered my allergy to the brown-tail moths irrelevant but the progression of symptoms during these three months persuaded us his ALS diagnosis was correct.

When I got the diagnosis I could not speak clearly.  Three months later I could not speak at all.  At first I had trouble swallowing liquids, then swallowing anything became problematic.

Adjusting to the changes got me thinking about my future.  I’d trained myself to recognize the truth of old age and death but now I had a schedule.  I would most likely die within a year or two.  What’s more, my arms and legs could weaken very soon.

No need to worry about after I died because when I started long, high altitude wilderness treks in the Himalayas, I had written “Croak Notes” for Felicity so she would know what to do if I croaked.

The question was, how best to live my remaining months while I remained mostly strong.

I questioned if it was worthwhile to start new projects.  Then a dear friend asked: “Has anything really changed?  Could you ever be sure you’d live long enough to finish anything?  Isn’t the question whether you would enjoy doing what you do get done?”

What a blessing to have so wise a friend!  I began constructing a room for my meditation practice and considering how to renovate this barn.

April 3 – To my family

I’ll get the diaphragm strength test and the spinal tap next Monday, April 10.  The spinal tap will be checked for 15 indicators, one of which is the presence of Lyme Disease antibodies.

Felicity and I have been researching while waiting for more test results, and both of us are growing skeptical about the provisional diagnosis of Lou Gehrig’s disease (ALS).

It seems significant that my tongue felt odd at the same time I had the allergic reaction to the brown-tail moths on all my exposed skin.  That moth lives only in Maine and Cape Cod so doctors here in PA are unlikely to know anything about it.

My only symptoms are difficulty with speaking and swallowing liquids.  The main thing I’m aware of is weakness of my tongue and lips.  I have no difficulty chewing, just maneuvering food to where I can chew, and removing chewed food from between my gums and cheeks.  I have no trouble swallowing food, just gagging when I try to start swallowing liquids.

I am prone to making the snoring sound while awake.  I am also prone to coughing, mostly triggered by taking a sip of coffee or other liquid.  These symptoms are consistent with Lyme as well as ALS.

The blood-brain barrier allows oxygen and etc to cross into the brain but not bacteria and etc.  The barrier becomes permeable during inflammation, however, so mine would have been when the brown-tail moths were active.  In that situation, some bacteria and viruses can cross the barrier.  The spirochete that carries Lyme is one of them.  So if I had Lyme bacteria in my bloodstream at that time, they could have gotten into my brain.

My first test for Lyme last summer was positive, the second negative.  I didn’t notice any tick bites.  The test for Lyme in my spinal fluid is looking to see if those bacteria crossed my blood-brain barrier.  The problem is, tests for Lyme are prone to yielding both false positives and false negatives, so whichever result I get, it will not be definitive.

It’s also possible that although the tests I’ve already had are said to have ruled out myesthenia gravis, they may not have.  The more we read, the more apparent it becomes that each of the possible causes of my symptoms manifest in a variety of ways that mimic each other.

So we may not know if, for example, I really do have ALS for quite a while.  We would know for sure, if I understand correctly, only if my arms and or legs grow weak.  They’re fine so far.  I finished transplanting twenty cedars and good-sized shrubs a couple of days ago and spent yesterday felling dead trees, piling heavy garbage that I’ll take to the landfill, and clearing the area where all that was.  Now I can plant a peach tree there and I feel fine.

We’re hoping for a definite diagnosis and hoping there’s treatment for it, as there would be for example for myesthenia gravis, but growing reconciled to the possibility that there may not be one.  Meanwhile, we’re researching the possibilities but not obsessively, and enjoying the moments as they come.

May 2 – To my family

The results are in for all 17 tests I had done on samples taken on April 10th.

All the results are consistent with ALS and do not indicate any other diagnosis but we still don’t know if I do have ALS.

I still have no symptoms I can detect anywhere other than around my mouth.  I spent all day yesterday doing construction work with no ill effects, for example.

My speech has been a lot worse in the last few days, however, and my lips are weaker.  My speech continues to be worse later during every day and on days when I’m tired.

That my speech is worse when I’m tired is more consistent with myesthenia (weak signal reception) than ALS (brain cells dying) so although the nerve tests seemed to rule out myesthenia, the neurologist has now prescribed meds that would counteract it.  If they have no effect, that will rule out myesthenia, which would be unfortunate because its effects are reversible while those of ALS are not.

The neurologist is also going to schedule another brain MRI because my previous ones were last July and August.  Nothing unusual was visible then.  Maybe it will be now.

The results of brain cell death in the medulla, which controls the mouth, might be visible but that would still not tell us the cause.  ALS would be likely but there are other things that kill brain cells.  Ultimately, we have to keep waiting for changes that are definitive.

Whether or not changes are visible in the medulla, there should still be nothing unusual in the rest of my brain because my only symptoms are around the mouth.

Meanwhile, I continue to enjoy life and be especially grateful for Felicity’s presence.  The only painful result of whatever is going on is I can’t talk much with my grandchildren.

Jun 15 – To my family

It feels like time for an update although there’s not much definitive to report.

It’s not certain but very close to it that I have Progressive Bulbar Palsy (PBP) aka bulbar-onset ALS.  What happens is the Motor Neuron (MN) cells in the brain stem that send signals to the mouth and related muscles die and are not replaced.  That results in signals no longer coming to the tongue, lips and etc, so they progressively lose function and atrophy.

I have now pretty much lost the ability to speak, and swallowing is problematic.

I think, but am not yet certain [I know more now — see What is ALS?], that the MNs are not replaced in the same way our other body cells are every seven years or so.  That means something is killing them, but the cause is not yet known.  The only treatment that looks promising is to replace the MNs with stem cells that can morph into any other cell with specific functionality.  Clinical trials are going on.  Hospitals in China and Thailand already advertise stem cell treatments but it’s not clear if they really are using stem cells.

So there is at this time no known cure for PBP or any way to significantly slow its progression.

Around 95% of patients with PBP go on to develop progressive loss of muscle function throughout their body, i.e., full-blown ALS.  They live for around two years on average although some live much longer.  Steven Hawking is the famous outlier.

There is no known cure for ALS and the only drug available, which I am taking, only extends life by a couple of months on average.  A new drug is scheduled for approval this August but I don’t know anything about it yet.

So let’s hope I’m among the 5% whose ALS does not spread throughout their body.  But let’s also bear in mind that we will all die sometime and my body is already 73 years old.

I feel very fortunate to have started Buddhist practice long enough ago that I feel equanimity about all this.  I will do everything I can to remain healthy while accepting that I cannot control what happens, only respond in a mentally healthy way.

My only symptoms so far are PBP-related.  I finished constructing my practice room and the adjoining bedroom.   The air conditioning is working well even in the almost 90 degree weather we’re having now.  I’m also continuing my work outside to transform the derelict yard into a welcoming place to walk and sit.  I enjoy both the work and the result.

Learning how to respond to the practicalities is an ongoing process.  Since I can’t talk now, I type on my phone to tell folks what I want.  That and my long hair and the fact that my lips are now so weak that I tend to dribble leads some to think I am mentally defective and possibly dangerous.  I shall have to make more effort with my appearance so I look well groomed.  But many people respond with a kindness that I’m not used to, which is heart-warming.

My father would not talk about it even when my mother’s leukemia was far advanced.  She had great courage and accepted that, but her last months must have been desperately lonely. 

It was the same a few years later when my step-mother died of colon cancer.  My dad cared for her physically but he would not talk with her about her fear.  

Englishmen of my generation, too, were taught that feelings are never to be acknowledged.  I’d seen the suffering that creates and wanted to do better for my family.

Nonetheless, while what I wrote at this time about recognizing the imminence of my death was true, my awareness of it was almost certainly more intellectual than I imagined.

March 1 – To my family

I wish I could have seen the Johns Hopkins neurologist in the first place.  She is so thorough and expert.

She says there are three possibilities.  There’s a series of tests where electrical currents and pin pricks are applied to see well how the nerves transmit signals.  Each of the diagnoses has a different profile of test results so once I’ve had those tests we’ll know which one is right.  I hope to have the tests soon.

The possibilities are, most likely to least likely:

1. Bulbar myesthenia
2. Bulbar myopathy
3. ALS – Lou Gehrig’s Disease

The first is a specific form of myesthenia gravis, where nerve receptors lose sensitivity and the associated muscles get a weaker stimulus.  Another form is ocular myesthenia which the neurologist in Maine thought I might have.  Bulbar makes more sense because it refers to the mouth and throat whereas ocular refers to the eyes.

We debated whether to tell you the other possibilities because there’s no treatment for them but I think the best way to live is in awareness that anything can happen, some preparation for disaster makes sense, and we should focus all the rest of our time on living happily.

I will not be worrying about the diagnoses I don’t want. Please don’t you worry either.  I’ll let you know when the tests are scheduled.

March 22 – To my family

We just got back from getting my tests done.

A technician applied voltage with a pen-like device to various parts of the surface of my limbs that were picked up by sensors on the skin in other places.  The results were stored and displayed in graph form on a computer.

Then a doctor applied voltage using a needle that pierced the skin, which wasn’t bad until he poked it up from under my chin deep into my tongue muscle. He had a younger resident with him who applied the needle to my left leg after she observed him do it to my right, and to my left arm after seeing how he did it to the right.  She went deeper than he did 🙁

The doctor then had a technician apply voltage to the surface of the left side of my face.  The technician told us he didn’t know how to interpret any of the results but we might be able to get something out of the doctor.  He suggested we hang around until the doctor finished with another patient.

The only definite thing we learned is the rest of my body is fine.  It feels the same as it always has but it’s good to know it really is all ok.

The doctor would not speculate about what could be wrong, saying these test results showed only mild results and he would need to know more of my history and see my MRI, blood and other test results before he could make a diagnosis.

The doctor who ordered today’s tests will arrange to see us.  Felicity will call tomorrow to get that scheduled as soon as possible.

So, although we were hoping for a diagnosis today, that was unrealistic because the doctor who ordered the tests was not the one doing them.  Nonetheless, it’s disappointing to have to wait longer to know what’s wrong.

March 24 – To my family

The neurologist called.  She says the tests I had a couple of days ago are not conclusive but they do rule out bulbar myesthenia, the one that’s treatable.  It’s likely I’m in the early stages of Lou Gehrig’s disease (ALS) but she will arrange more tests to confirm or change that diagnosis in the next couple of weeks.

Specifically, testing the strength of my breathing will reveal whether my diaphragm is strong.  It is as far as I can tell.  And a spinal tap will show if anything’s there that shouldn’t be present in the fluid around my spinal column.

I’m eager for the spinal tap result to see if there’s evidence from when the brown-tail moths triggered my rash last summer along with the feeling that my tongue was enlarged.  There is, as far as I’ve found so far, no known trigger for the onset of ALS yet my speech and swallowing problem began when I had that allergic response.  Both my neurologist and the doctor who did the tests a couple of days ago dismiss my brown-tail moth rash as a coincidence.  I don’t.

The other reason to be somewhat skeptical about the provisional diagnosis is it relies chiefly on tests done a couple of days ago by a doctor whose specialty is ALS.  We humans tend to see what we expect to see.  ALS is what he expected to see.

But I’m assuming it probably is ALS so I’ve started research.  About 25% of ALS patients start with Progressive Bulbar Palsy (PBP), which affects only speech and swallowing.  They are controlled by the medulla, which used to be known as the bulb.  A drug, Ritulek, is indicated for PBP patients in several articles I’ve read but I haven’t yet explored what it does.  Speech and physical therapy are also recommended, so I’ll resume my speech exercises and get back on the cross-trainer.

Some articles say the life expectancy for PBP patients is 6-24 months, however a doctor at Rutgers has many patients who lived 2-5 years with PBP before it progressed to ALS and some folks, like Steven Hawking, live with ALS for a very long time, 50 years so far in his case.

I mention these numbers so you won’t waste time researching them and because, if there is anything you’d like me to do while I remain healthy, please let me know.

My feelings about all this may change, but a fundamental Buddhist practice is training oneself not just to know intellectually and ignore, but to really feel the truth that every one of us will die and, more importantly, that it could happen at any moment.  I’ve been feeling increasingly okay with that for quite a while now.

Another thing we do is try to act on the truth that every circumstance is an opportunity to practice acceptance.  I was shocked into recognizing that by a fellow student in Kathmandu whose husband had died within three months.  That was only six months before the class and they were both in their mid-30s.  I said I hoped her grief would not be too great or too long.  She said she was not trying to overcome her grief but to learn from it.

That may be the most helpful thing anyone has ever said to me.

So, the diagnosis is not yet definite but the provisional one is likely to be right.  I’ll keep you posted, of course.  Meanwhile, please do not be downhearted.  This will develop however it does and we will all respond well.  Mom says she will treat it as our next big adventure.

March 26 – To my family

Hi again, everyone.  No news, I just want to give you — with permission 🙂 — a reply to my last email:

I’m not sure how to react – or rather, I feel as though there are many different reactions occurring in me but that they’re all almost…subsurface.  Like maybe I could look inward, take hold of any of them, focus on it, and it’d be amplified. 

Naturally, I hope that the actual causes are not as serious as ALS seems to be.  There are many thoughts.  Of how others of us will experience this, if it is serious.  Of what I ought to do, or say.  Thoughts that your calm and accepting views, inspired by your practice, are a stabilizing and helpful influence while discussing this provisional diagnosis, certainly for me and I suspect also for others. 

It’s odd, the provisional diagnosis feels like I ought to think of it with great gravity, but that reaction also feels like clothing that doesn’t fit quite right, like an actor playing the role that’s written for them.  Like that’s how I think I’m supposed to react but the reaction is a story.  I don’t know.  Certainly it’s serious, but as you say, all our lives will end.  I hope yours doesn’t end soon, deeply hope this, but.  It will or it will not. 

The idea that there’s time that could be taken from you and us is only harmful.  There’s no future time that can be taken, only time from the present moment that we can get, all we can ever get is another moment.  A truth my own practice teaches me to accept but not how we instinctively understand things, which maybe is why I feel this strange sense of much movement beneath the surface of my mind. 

Do you also have that sense of confusion?  I do.  Questioning how I should react to this, and wondering how I am in fact reacting?  The last few nights I’ve found myself waking several times, not feeling anxious or thinking about anything and returning to sleep quite quickly, but I usually sleep like a log.  There’s something going on under the surface.

It feels rather like a trip to Nepal.  Perhaps I will recover as mysteriously as I got sick, like if my trip ended for some reason before anything new happened.  But more likely, I’m going to experience things that are new to me.  It will help me to write about them every so often if that happens.  Don’t worry, I won’t write daily!  I hope doing that will be helpful to more than just me, and I hope you’ll chip in periodically with your own reactions, too.

I feel very lucky that we’re a family of good individuals who work seriously at behaving well, and that we each have a way that suits us.

I believe you all know that while training with a form of Tibetan Buddhism and reflecting on quantum physics helps me grow more aware and less selfish, I would never try to persuade anyone else to adopt my method.  I just think it’s good to share how we’re dealing with our feelings and ideas.

Finally, I was especially struck by this:  The idea that there’s time that could be taken from you and us is only harmful.  There’s no future time that can be taken, only time from the present moment that we can get, all we can ever get is another moment.  That is so right.  I enjoyed transplanting three shrubs this morning and, assuming I get more moments tomorrow, I shall enjoy transplanting three more then.

What is this malady of mine, Amyotrophic Lateral Sclerosis (ALS)?  I’ve researched it not to find a cure — none is known at this time — but so I don’t waste time on what cannot be a cure.

ALS is a progressive neurological disease in which motor neuron cells deteriorate and die.  Motor neurons send signals from the brain (upper motor neurons) via the spinal cord (lower motor neurons) to muscles throughout the body.

Motor neurons control all voluntary muscle movement including walking, talking, chewing and breathing.   As the signals they send grow weaker, the muscles they control waste away until at last the brain can no longer initiate and control voluntary movements.

Different groups of muscles are controlled by motor neurons in different parts of the brain (see illustration below).  About 70% of patients first develop symptoms in their arms or legs.  About 25% first notice speech or swallowing problems (bulbar onset) — I’m one of those — and about 5% start with symptoms in their trunk.

No matter where the first symptoms appear, the great majority of ALS patients end up losing function in all muscles.  We lose the ability to speak, eat, move, and even breathe.  Most of us die from respiratory failure, usually within 2 to 4 years, although about 10% of us survive for 10 or more years.

The literature says all ALS patients develop symptoms throughout their body but the chief neurologist I met at Lehigh, PA who has worked in the field the longest told me that’s not correct.  Some patients with bulbar onset ALS do not develop symptoms in other muscle groups.

Up to 10% of ALS patients inherit it from a parent via mutations in any one of over a dozen genes.  The remaining 90%, of which I am one, are believed to have a genetic predisposition to the disease that is activated by an environmental factor.

Genes are contained in chromosomes located mainly in the cell nucleus.  Every chromosome contains hundreds to thousands of genes and every human cell contains 23 pairs of chromosomes.  We have about 20,000 to 23,000 genes all told, around 400 random ones of which are, on average, defective in any one of us.

Motor neurons are different from other cells because the genes that govern their operation are different.  Restoring their operation is not a matter of developing a drug to kill an external attacker such as a bacterium.  We’re not dealing with something that moves from one group of motor neurons to all others.  An environmental factor is triggering a change in our motor neurons’ genetic programming.

Curing ALS will require correcting the motor neurons’ genetic programming.  This December 10, 2017 report describes a potential approach based on CRISPR (Clustered Regularly Interspaced Short Palindromic Repeats), a naturally occurring bacterial defense system.

CRISPRs are repeating sequences of genetic code interrupted by “spacer” sequences – remnants of genetic code from past invaders.  The system of which they are part serves as a genetic memory that helps the cell detect and destroy invaders when they return.

CRISPRs can be programmed to target specific stretches of genetic code and edit DNA at precise locations, thus permanently modifying genes in living cells.  The first method to engineer CRISPR to edit the genome in mouse and human cells was published in January 2013.

In its current form, CRISPR isn’t technically gene therapy. Rather than replacing a diseased gene with a good one, it goes into the nucleus and directly cuts out faulty genes.

Meanwhile, since there is not yet a gene therapy for ALS, I am taking a treatment that has cured a significant number of MS patients and more recently, a number of ALS patients, too.  It is based on the idea that the body can, if treated in the right way, heal itself.

The treatment was developed by a doctor of Tibetan medicine who, earlier in his career, was sent by the Dalai Lama to reestablish Tibetan medicine in Mongolia and who is now based in San Diego, CA.  I am receiving this treatment from the first Westerner to qualify as a doctor of Tibetan medicine, a truly amazing achievement.  Her mentor is the Tibetan doctor who developed the treatment.

I will add comments to this post as I learn more about ALS.  I knew little about biology eighteen months ago and would be very grateful for comments by anyone who can correct or add to what I’ve learned so far.

I didn’t choose this as I did the experiences I learned so much from in business and trekking in the Himalayas but it is just as much of an adventure, a powerful opportunity for learning.

I hope my trip reports from this adventure will help others with ALS, those who care about them, and perhaps medical professionals.  The progression of symptoms is detailed enough to be useful to researchers.  How I have remained happy may be helpful to fellow patients.

These reports also benefit me in the same way as writing monthly status reports did in business.  They help me reflect.  It’s impossible from day to day to get an accurate sense of important changes that only become visible when you gain altitude.

This post tells how my symptoms progressed toward a diagnosis.  Next I’ll post about the nature of ALS.  Subsequent posts will be based on emails to family, friends and my doctor.

I’d prefer to write after I regain health as I did about depression.  There would then be no risk you’d be distracted by sympathy, but Western medicine has no cure for ALS.

I am receiving care from a wonderful Tibetan doctor who has cured other ALS patients but we can’t know if the treatment will work for me, which points to the overwhelming lesson from this journey — if I don’t write now, I may not have the chance to do so later.

So please, whatever is the most important thing you can do with your life, do it now.

Enough preamble.  Here’s what happened up until my ALS diagnosis nine months ago.

My symptoms began in mid-June 2016 when I was working under oak trees in Maine.  Their leaves were being eaten by Brown-tail Moth caterpillars to which many people are allergic.  An itchy rash developed on all my exposed skin and my tongue felt enlarged.

The rash was gone after two or three days but my speech was slightly slurred.  I could curl my tongue so it seemed I had not had a stroke.

My slurred speech was a bit worse after about three weeks, too long for an allergic reaction to be the explanation, so I went to my primary care doctor who ordered blood samples and a brain MRI.

The MRI showed no structural damage to my brain.   We got the blood test results by phone and were told they indicated tick-borne disease.  We didn’t think to ask which one and the doctor’s office could not locate the results later but I was put on a three week course of antibiotics.

At the end of the three weeks, July 26, about six weeks after the first symptoms, my speech was worse and my tongue felt weaker.

I was then examined by a neurologist.  She detected double vision when I look toward the upper left.  I wasn’t too surprised because I also get double vision if my head gets very cold in winter.  The neurologist ordered an ultrasound for blood flow on both sides of my neck

The ultrasound results were normal.  On August 16 the neurologist ordered more blood work.  Everything was fine except for my usual high cholesterol.  The results for tick-borne disease were negative.  She ordered an MRI for blood flow in my brain.

My brain blood flow was normal.  On August 30 the neurologist prescribed pyrostigm because my symptoms could result from ocular myesthenia.

The pyrostigm had no effect by September 7.  I had been doing very heavy physical work and driving 11+ hours back and forth between Maine and Pennsylvania so I wondered if the medication had not had the right conditions to be effective.

The neurologist said I must have had a stroke that left no trace in the structure of my brain.  She ordered a barium swallowing test which I took on September 16.  No problems were detected.

We moved to Gettysburg, PA and on November 3 I visited a new primary care doctor who prescribed speech therapy.

After three weeks of speech therapy that taught me to articulate more deliberately but left my tongue weaker and my speech worse as I tired, the therapist told me to stop the exercises and recommended consulting another neurologist.  The exercises would have been beneficial if I’d had a stroke so she believed it must be something else.

It was now early December, almost six months since the first symptoms.  My speech was continuing to deteriorate, swallowing was growing more difficult and my nostrils kept getting blocked.  We scheduled a consultation with a neurologist at Johns Hopkins Hospital but it would not be until March 1st.

I went to Nepal for Tibetan Buddhist teachings for six weeks right after New Year’s day.

By the time I returned in mid-February, eating had become a struggle.  It was hard for my tongue to maneuver food between my teeth, food was getting stuck in my cheeks, and it was hard to avoid biting my cheeks when I chewed.  There was no problem with jaw movement and I could swallow food without difficulty but I could take only small sips of liquid and I would sometimes gag which led to coughing fits.

On March 1, 2017 the Johns Hopkins neurologist said my symptoms indicated either Bulbar Myesthenia, Bulbar Myopathy or Lou Gehrig’s Disease (ALS).  She ordered an EMG with Nerve Conduction Study.

The EMG was performed by a different Johns Hopkins doctor, one who specializes in ALS, on March 22.  The sensory conduction showed reduced right ulnar sensory amplitude (the ulnar nerve runs from the neck to the hand and is our largest nerve unprotected by muscle or bone so injury is common), the motor conduction showed reduced velocity across the elbow segment of my right ulnar nerve, repetitive nerve stimulation was normal and needle EMG showed neurogenic changes limited to the right side of my tongue.  No weakness was detected anywhere else in my body.

Two days later (Mar 24) the first neurologist called to tell me the EMG ruled out Bulbar Myestenia and her colleague had diagnosed ALS.  She ordered a test of the strength of my diaphragm and a spinal tap that would include tests for Lyme Disease because Lyme can cross the blood-brain barrier when there is inflammation as there had been in my reaction to the Brown-tail Moth caterpillars.

On April 7 my speech was very indistinct after a week of very hard work outside.  I rested for a day and my speech was clearer the next day.  My tongue felt less weak but my lips were always weak so I dribbled.  I could still swallow food without difficulty but I hesitated with liquids.

I had the pulmonary test, spinal tap and a consultation with the ALS neurologist on April 10.  He said we had eliminated every other possibility so I definitely have ALS.  Weakness spreads to all muscles of most ALS patients and they die, usually from breathing problems, within a couple of years.

This is long enough for one post.  I’ll describe what happened next in future chapters.

It’s worth noting now, though, that in the eighteen months since my symptoms began, I have experienced no weakness in muscles other than my mouth and throat.  That means I may be one of those whose symptoms do not progress.