My ALS Adventure – August 2018

 

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.

2 comments on “My ALS Adventure – August 2018

  1. Martin and Felicity, if you travel to Maine, please do contact me. If you want to stay in a house for awhile, you are welcome here. Muriel

  2. Very interesting article. I did not know the magnitude of your illness until I read it. However, I am confident of your recovery through faith and perseverance! Enjoy your long drive from North East to South West!

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