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.
Dear Martin, My cousin Linda was diagnosed with ALS last spring when she could no longer speak. She was a math teacher and has 3 children and 2 grands. We didn’t know about her illness till July when she had trouble swallowing. I was busy getting ready for my daughter Jamie’s wedding October 22 and Linda’s daughter Jess had scheduled her wedding for Oct. 28th. Linda got progressively worse and wanted no hospital bed, intervention or feeding tube. She died the week before Jamie’s wedding. Three days before her death she insisted on mowing the lawn so it would hasten her death. She didn’t want to overshadow or interfere with Jess’s wedding. I don’t know where she got her courage to face her disease but she truly loved Jess. I wish you strength and send you love and a huge hug. Flora
I can tell from Facebook that you are surrounded with family and love.
Yes, I am. It was good to see your picture with your granddaughter. She is so cute!