The focus this month was on my reduced lung capacity.  My diaphragm is weak enough that I’ve started part-time use of two machines.  My overall strength is dropping but I’m still in pretty good shape.  We’re about to set off on a two-ish month road trip all round the States while I’m still strong enough.

August 7 – to my family

Six months ago my only serious weakness was in my mouth and throat muscles.  My diaphragm had begun to weaken but I wasn’t feeling it and I figured the 74% breathing capacity result I got then was artificially low because air was leaking through my weak lips.  It was only when I could barely get any reading from the spirometer at home last month than I recognized my diaphragm really had weakened.  That explained why I was getting tired so quickly when I did physical work.

So I wasn’t surprised that my breathing capacity this time we went to John’s Hopkins was down from 74% to 39%.  However, although I’ve lost muscle mass on my arms, shoulders, and legs, the technicians pronounced me strong.  I’m far from as strong as I was but I have no trouble balancing.

I asked how much longer they guess I will be able to walk — 18 to 24 months although of course “results may vary”.  That’s better than I expected and at the very least we should be able to enjoy our cross country road trip in the rv starting next month.

They’re getting me a BiPap machine to wear at night that will push more air into my lungs and a Cough Assist machine to clear secretions from my lungs.

I’m feeling less tired today than since I got home from the retreat.  I rarely had health problems in the past and I always recovered quite quickly when I did, which means I expect tiredness to be temporary.  More broadly, I expect I will recover from ill health.  I know I’m not going to recover from ALS and that I will grow more tired more of the time and I will continue to lose strength but I have no experience to set realistic expectations.  The lack of a crystal ball doesn’t bother me, I now experience how things really always were.  We never know for sure what will happen.

My Tibetan doctor has taken me off the precious pills and put me on a different regimen for the next month.  She’s had very intermittent internet access for the past few weeks while travelling in remote areas of Tibet so I don’t know specifically what these new meds target.

August 18 – to my family

The director of the John’s Hopkins ALS clinic arranged for me to visit a pulmonary doctor based on my 39% breathing capacity test result and he set me up for more breathing tests.  When we went for them on Wednesday I couldn’t get my lips round the spirometer mouthpiece so it was impossible to get a reading.

Next we went for my six month checkup with the neurologist who specializes in ALS.  All three of us enjoy these visits and find plenty to joke about.  He’s intrigued by my Tibetan Buddhist practice and medicines and because he’s Indian I greet him with namascar, hands together and raised in the blessing prayer.  He reciprocates and we repeat the gesture when we part.

He had me walk, balance on one leg and hop on one leg.  I had no difficulty doing that with either leg so he says the 39% breathing capacity result must have been falsely low.  Some of the air I breathed out must not have gone through the spirometer but escaped around it because my lips are too weak to make a tight seal.  Nonetheless, he says it will be good for me to use the BiPap machine.

I asked him about the conflict between two things I’ve read about ALS.  One is that an environmental factor triggers a latent defect in the genetic structure of motor neuron cells so when they replicate, the RNA in the new cell does not produce the correct motor neuron behavior.  The other is that motor neurons, unlike our other cells that replace themselves every seven years, do not replicate.

He said motor neurons and other cells that make up the central part of the brain are not replaced.  Most experts do believe ALS is triggered by environmental factors but there are many theories about what is triggered.  I forgot to confirm it, but the fact that motor neurons don’t replace themselves must mean that stem cell treatments to replace them will not work.

So, the neurologist is pleased with my overall condition.  His only suggestion was that I should get a Botox injection to cut the excess saliva I’m producing now, which medicines aren’t sufficiently reducing.  Felicity and the grandkids will be pleased about that 🙂

Yesterday I had my annual checkup with my highly skilled and delightful primary care doctor who is from Pakistan.  She noted that I am weaker than I was a year ago but am still in pretty good shape.  She prescribed for me to have shingles and flu vaccines because I must minimize the risk of getting things my body needs to put energy into fighting off.

I seem to be recovering from the exhaustion that left me unable to do anything but sit reading for the week following the Buddhist retreat.  We got our 23 year old RV (same age as Doma) back a couple of days ago from having its water and waste tank gauges replaced (the only defect I found when I was living in it at the retreat) and I was able to repair its ladder to the roof, which took a couple of hours and involved much climbing up and down a stepladder.  I had to rest for a few minutes near the end because I was short of breath but overall the project went much like before I had ALS.

I’m not suggesting that I’m recovering or even that my strength is no longer declining, just that my exhaustion last week may have been an anomaly.

My visit with the pulmonary doctor will be at the very end of the month by which time I should have gotten used to sleeping with the BiPap machine.

I’ll let you know how I get on with the machines and what the pulmonary doctor says at the end of the month.  Then we’ll set off on our great road trip.

August 20 – from my Tibetan doctor

The Basam Lhalung and Samnor formulas are both aimed at regenerating your kidney function and overall capacity to generate the bodily constituents (cells and tissues of all constituents of the body).

Basam Lhalung rehabilitates the kidneys particularly after excess stress and load causing inflammation. This helps the kidneys to regenerate and then focus on proper filtration, function and mobility of chuser and neural flow.

Samnor supports the rlung flow and vitality particularly from the rlung generation region of the pelvis.  It cleanses the chuser related to immune and neural function, cycling and signaling, and helps rebuild the foundation for their function.

Both of these formulas are aimed at rebuilding your energy, stamina, kidney function and overall bodily constituent base.

August 31 – to my family

Our first appointment at Johns Hopkins yesterday was for a botox injection to control my dribbling.  It seems odd to administer a powerful neurotoxin to a patient with a neurological disorder but it makes sense.  The injection is given from outside the mouth into the salivary glands.

It stung quite painfully for a very short time on the right side of my face but not the left and I was not aware that anything had been done after a minute or two.  They start with a very low dose so I’m to continue with the tablet I’ve been taking.  It will take a week or maybe more before the botox begins to work and it will take longer before I know if the dose should be increased.  I’ll need to have the injection renewed every 3-5 months.

The second visit was for more tests of my lung capacity and functioning.  The technician this time was more persistent than the one who gave up earlier in the month.  We got a reading of 38% capacity breathing in and 35% breathing out.  You’re recommended to use a BiPap machine at night if your capacity is below 50%.  He also took an arterial blood sample.

I’ve been getting used to the BiPap for a few days.  Breathing with its help is easy enough but my mind isn’t used to having something strapped round my head when it goes to sleep.. The cough suppression machine is more challenging physically.  It blows air forcefully into my lungs, then sucks it back out with what feels like even greater force.  I look like a bullfrog.

My first visit with the pulmonary specialist at the end of the day was reassuring.  The oxygen and carbon dioxide levels in my blood are perfect so my lungs are working well.  He asked many questions about my activities and listened carefully with a stethoscope then told us the 38% and 35% lung capacity readings are falsely low, just as my neurologist said.

ALS patients with bulbar onset typically get artificially low lung capacity readings partly because weak lips mean air escapes around the side of the test instrument and also because muscle signalling weakness makes it hard to push and pull one’s breath as forcefully as the diaphragm muscle could really accomplish.

So, since I’m pronounced healthy enough to enjoy our maybe two month long road trip, I’ll now go back to packing 🙂  Here’s the RV.  We’re not calling it The Leisure Seeker haha.

This month was thought provoking.

June 26 – to my family

I was surprised when I tried to use the spirometer the ALS At Home study gave me along with other tools to monitor my health.  To use it I must blow through a tube at the end of which is a fan to measure the strength and duration of my out-breath.  I knew it would be hard to stop some of my breath from escaping around the tube because my lips are so weak.  What surprised me was discovering I can no longer take a deep breath or exhale forcefully.  My diaphragm is weak.

When I was thinking about it a few days later I remembered having to pause to catch my breath when I was using the cross-trainer.  The first time it happened was less than three minutes after I started.  I had to stop and pant for a minute or two.  After that I was careful not to push myself when I started and I worked my way up to 30 minute sessions that were fairly vigorous.  I imagined the problem had been weak leg muscles that rapidly regained strength.

Now I realize my diaphragm was already weakening.  That was over three months ago.

It looks like my breathing muscles are on the same trajectory as my mouth and throat muscles followed.  There was a long period where the decline was quite slow, then the pace accelerated.  Or maybe the weakening didn’t accelerate but just reached the point where the result was a serious loss of function.

There are external devices that enable one to keep breathing when the diaphragm can no longer do the job.  When they no longer work you can have a tube installed in your throat and have air pumped in and out that way.  I’m guessing life would continue to feel worth living with an external device although it would severely limit what I could do.  I don’t think I’ll want the throat tube but I’ll keep an open mind and see how I feel as my symptoms develop.  The advanced directives we signed years ago specify no heroic measures to preserve a non-functional life.

I continue to feel blessed to be alive now with you as my family.  I paused work on the summer kitchen so I could sheet rock the porch wall with its new windows and I’ll start installing the trim today.  It looks much better already.  Next month I’ll go for three weeks of teaching and practice in Cooperstown, NY and when Felicity returns from celebrating Megan’s 90th birthday at the end of August we’ll go on a road trip in the RV for a month or two.  The adventure will continue.  We don’t know for how long but we never did.  That’s in the nature of adventures.

July 1 – my current thoughts

It’s a blessing that ALS progresses slowly, doesn’t impact cognitive function and that my only pain is from biting my cheeks and tongue.  I have time to notice the results of losing muscle strength, and reflect on the implications.

Wearing shorts these days, I notice muscle twitching in my legs, like eddies in a river.  My leg muscles are still my strongest ones but the motor neurons that control them are dying.

An apocryphal and a true Buddhist story have been on my mind.

The Buddha, on his travels one day, met an old man beside a river.  “I’m so happy to see you again, master,” said the man.  “I have practiced and practiced since you taught me so many years ago.  Finally, I succeeded!  Yesterday I walked across the river.”  “Oh, you poor man!” the Buddha exclaimed.  “There is a boat just round that bend.  It would have taken you across so easily.”

I never wanted to do miracles.  In High School my life’s goal was to attain wisdom, but how to start?  Decades later I started trying to become less selfish.  Now I just want to grow more aware and more kind.

The true story is about a revered Tibetan master who had diabetes.  Leading a ritual practice one day, he picked up his damaru drum that you hold with thumb and first finger.  His fingers could no longer hold the drum upright.  What did he do?  He laughed.

Acceptance of negative circumstances is not doleful for those who have sufficiently trained their minds.  Surprises of any kind are an occasion for joy.  That’s worth practicing.

Where I am now, almost all the time, is in interested equanimity.

Earlier this month our middle son was married in a beautiful ceremony framed by events to celebrate the occasion.  Their photographer captured it perfectly.  It was a joyful time that also provided me  an insight.

Some who die in villages in the Himalayas are said to cause trouble, especially those who die in an accident.  They don’t realize they’re dead.  They want to communicate just like before but they can no longer be seen, heard or touched.  Nevertheless, their family members feel their attempts to communicate.  I didn’t believe that explanation.  I figured the trouble arose in the minds of the grief stricken living and wondered why it was attributed to the dead ones.

I saw why at the wedding when I experienced a weak form of what the troubled dead are said to feel.  I can communicate one on one by typing on my phone but that’s not effective in a group.  Because I cannot drink or eat by mouth, I could only watch others.  To a much more limited extent I was like the dead villagers, present but separate.     It was an interesting feeling.

Meanwhile, I’m so lucky that I can still do things!  The porch looks so much better already, our terrific painter will hide the gaps, and then it will be perfect.

Living with ALS is the same as it was before in that I can’t know what will come next.

This month I noticed twitching in my right side.  Since I have ALS, that probably means the motor neurons that control the muscles there are dying.  My mouth and throat muscles have continued to weaken for almost two years but all the others seemed okay.  That seems to have changed.

The most likely future suggested by the twitching is I will lose the use of my legs and most of what I can do with my arms during the next year although other alternatives are also possible  I might regain some vigor by working outside this summer, or I might stay as I am for quite a while.

The only thing I can be sure of is my body will shut down sometime.  I feel blessed to be alive right now no matter what my future.  I’m so lucky to have lived long enough to reach that degree of wisdom and to still have the potential to wake up more completely.

May 11 – to my family

There’s been no great change in my health since the last time I wrote but there are a few things I’m tracking.

The Tibetan Kundey medicine seems to be reducing my mucus production, I’ve started applying a nasal spray before going to bed and I’ve had less nostril blockage while trying to sleep.  I need to see this go on longer before I declare victory, of course.

I’ve also been coughing less often although I do still cough more than in the past.

I had a couple more incidents late in the day when I suddenly began coughing violently and had great trouble drawing breath.  It didn’t feel life-threatening, just something I must quickly overcome.  The bad part was Felicity’s distress because I couldn’t explain that I felt sure it was a transitory problem.   I imagine it resulted from weakness of my epiglottis letting saliva drain toward my lungs instead of my stomach.

I haven’t yet overcome excess salivation.  Atropine drops seemed to increase salivation so I went back to the pill three times a day.  Most recently I’ve continued with the pill and applied atropine before going to bed.  That seems to be helping.  I haven’t yet figured out what triggers excess salivation.  I get spells of high production while the rest of the time it’s not a problem.

Those problems and the cheek biting don’t bother me much because I’m used to them now.  I don’t like having such low energy, though.  Felicity asked if I think being 74 could be factor.  Of course not 😉

I haven’t felt like exercising on the cross-trainer recently but I spent most of the day a couple of days ago replacing the anti-rabbit fence around the vegetable garden.  That felt better than being on the cross-trainer, not boring.  My next project is to repair the bottom of the back wall of the summer kitchen.

My stomach was a bit disturbed while I was doing the fencing because I’d experimented with different foods the two previous evenings.  Felicity had bought salmon and I tried some along with my usual three kinds of veggies.  That had no noticeable effect so the next night I had salmon again but with broccoli and potato since I’d run out of my usual veggies.   I also added two scrambled eggs because I feel I’m not getting enough protein.  Oh, and both evenings I had a beer, for hydration, you know.

My gut continued to feel slightly distressed so I’ve eaten only formula the last three days but with the addition of a cantaloupe and yogurt smoothie in the mornings and more water.  I’ll go back to veggies instead of formula this evening.  I would never have given any of this any thought in the past.  It’s impossible to know how much attention to pay now.

It’s worth paying some attention.  Because of the discomfort and extra tiredness after working on the fencing I finally thought to look at where casein and soy, the primary ingredients of my formula, lie on the acid/alkali spectrum.  They’re both relatively acid-producing which my Tibetan doctor says I should minimize.  So I’ll start cautiously replacing more of the formula with foods that are more suitable.

I’ve been accepted into the “ALS At Home” study to assess whether ALS patients can monitor their vitals themselves and send the results to their doctor.  That means I’ll be able to monitor my lung capacity and other things.  I’m eager for that because I’ll be able to know for sure if there are any trends.

I’ve been doing a lot of reading while Felicity is away on her painting trip in France and doing some opera watching.  Coloratura soprano Kathleen Kim is spectacular and Sonya Yoncheva’s voice is so beautiful.  But try watching Yoncheva perform Casta Diva on YouTube then watch Callas who somehow goes beyond beauty and raises goose bumps.

Tomorrow I’ll drive to Hampshire College to take videos of Doma presenting her thesis to the Computer Science folks on Wednesday and the Natural Science folks on Thursday.  She’ll be presenting for parents of this year’s graduates on the day before commencement . Very cool!

May 26 – to my family

The end of pollen season along with the Tibetan anti-mucus medicine brought me relief from the blocked nose that was making it hard to get to sleep, and I have not had any more coughing fits that make it hard to breathe.

The ALS At Home monitoring equipment came a couple of days ago and I’ll learn how to use it this weekend.  I have to pass an online test before I can actually use it and at least some of the equipment uploads the results so I’m not sure if I’ll be able to access them but it’s a worthwhile program, anyway.

I haven’t yet changed my diet because I’ve been away from home quite a bit going to Doma’s graduation and helping her move out of college.

The negative news is I noticed twitching in my right bicep a couple of nights ago and again last night and there’s twitching in my right thigh  this morning.  That means the associated motor neurons are dying.  The ALS is spreading.  Felicity said she noticed twitching in my right shoulder a couple of months ago but hadn’t seen it since.

I’ve been feeling unusually tired for a while.  As I mentioned, I stopped exercising on the cross trainer.  I thought it might be a combination of age — I should expect to have less energy now I’m 74 — along with the wasting effect of getting little exercise over winter.  It looks like the greater factor is progression of the disease.

I’ll keep you posted of course and give a bit more thought to future plans.  Before I noticed the twitching I’d suggested we might take a road trip in the van and take the rest of Doma’s stuff to her in California where she will be living with David and Ilana before finding rooms near wherever her job turns out to be. I’m going for three weeks of practice with my Tibetan Buddhist teacher in July so we should probably take our road trip soon after that.

Please try hard not to be upset by my news.  We can’t know how long I will remain healthy enough to continue a relatively normal life, but we never could know that, anyway.  None of us can know our future.

I’ll continue doing things I enjoy and adapt to changing circumstances.  I’ll do less of what requires strength and more of other things.  In particular, we intend to spend more time with you 🙂

Speaking of things I enjoy doing, here’s how the summer kitchen is looking now.   Do you remember how it looked originally?  When it stops raining I’ll finish that wall and start on the floor.