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.