My ALS Adventure – July 2017

This month is when we realized that while ALS is my indicated diagnosis, ALS is less a definitively diagnosable disease than a label applied to a varying cluster of symptoms.

Re-reading what follows, I still believe this is true: “You can have a powerful influence over your health if you learn how to purposefully relax your mind and body” but I’m skeptical that we can “call on the memory of wellness and bring the state forth“.

For the benefit of other ALS patients, I’d like to be able to report sometime in the future that as a result of Tibetan medicine, nutrition changes and living in neither hope nor fear, my body has restored itself to full health.  There would then be a program others could follow.

But I’ll report whatever happens.  Meantime, I bless my parents and the culture in which I was raised for this:  “I’m very lucky to have read so many adventure books as a child because they accustomed me to experience life that way.  Unexpected things happen on an adventure.  You expect that and enjoy figuring out how to respond.”

July 17 – to my family

The brain MRI I had on May 18 that was sent to Johns Hopkins has been reviewed by a pair of experts.  I’ve asked my neurologist to explain it but he is away right now.

What we think the experts’ report says is there is no indication of ALS at this time.  There are:  “mild T2/FLAIT hyperintensities in the periventricular regions, which are nonspecific, however likely represent small vessel ischemic changes in a patient of this age.”

The report concludes:  “no focal signal abnormality in the brain parenchyma other than mild patchy areas of increased signal in the periventricular region compatible with chronic small vessel ischemia.”

Ischemia constricts blood supply so perhaps this means the motor neurons that send signals to my mouth muscles are not being actively killed by anything but are dying for lack of blood supply?  The process appears to be irreversible.  I don’t know if it is likely to occur also in other areas of my brain.

So the report seems to suggest I have Progressive Bulbar Palsy (PBP), not ALS.  Life expectancy for PBP patients is only 1-3 years because as swallowing grows increasingly problematic, food gets in the lungs, infection sets in, and the patient dies of pneumonia.

However…  If we understand this correctly, and if the diagnosis is now correct, presumably I could have a feeding tube surgically implanted to avert that problem.  I haven’t researched them but I understand that a fully active life is possible with them.

We will do some research into all those terms, which Felicity is better able to understand because of her training, but we’d have to do far too much to have any hope of real understanding.  Our focus is on getting a definitive diagnosis because that would tell us what, if anything, would be productive to do, and most of our effort is on dealing with the practical effects of losing my mouth muscles.

As I’ve said before, I reckon I’m very lucky to have read so many adventure books as a child because they accustomed me to experience life that way.  Unexpected things happen on an adventure.  You expect that and enjoy figuring out how to respond.

Being unable to speak is not a single experience, for example.  One on one conversation is entirely different from chat in a group.  I can manage the former but not yet the latter.  And there are, I’m recognizing, many aspects to food texture and configuration that impact swallowing.  Chopped tomato is great because it doesn’t stick to the roof of my mouth.  Black coffee is very hard to swallow and while coffee with thickener is more practical and it still tastes right, it doesn’t feel like coffee.

The unrelated bug that was making us both tired has finally gone away so I’ve started daily exercise on the cross-trainer again and that feels very positive.

Jul 24, 2017 – To my family

My neurologist’s opinion is that I have ALS presenting itself first as bulbar palsy.  He views Progressive Bulbar Palsy (PBP) as a subset of ALS.

What is becoming evident to Felicity and me is that very little is actually known about ALS, or PBP, or autoimmune disease in general.

[Jan 27, 2018 –  When I wrote this I thought ALS is an autoimmune disease.  It isn’t.  WebMD clarifies the difference: “MS is an autoimmune disease that causes your body to attack itself, ALS, also called Lou Gehrig’s disease, is a nervous system disorder that wears away nerve cells in your brain and spinal cord.”  What follows is not wrong but it is inapplicable to my condition.]

What is autoimmune disease?  Our bodies have specialized cells whose purpose is to recognize and kill cells that don’t belong in us, ones we think of as infections.  If I understand correctly, the defensive cells first recognize newly arrived ones that may be harmful, then program themselves to recognize that specific kind of foreign cell more quickly.  Something goes wrong in the case of autoimmune disease — the defensive cells mistakenly start recognizing cells that are part of one’s healthy body as if they were foreign.

The mistake may have originated in my case when I experienced the moth allergy.  But that is unknowable because the origin of autoimmune diseases is unknown.  And there is no way to diagnose ALS or PBP.  Other things, e.g., myesthenia gravis can be ruled out, but definite evidence of ALS is only available by means of autopsy when Motor Neuron cells can be examined.  I’m not certain even that is definitive, though, because I’ve found no information so far on whether it’s apparent if the MN cells died before the body overall died.  It’s definitely not known what triggers the killing of the MN cells.

About the only thing we’re sure of is that whatever disease I have is not hereditary.  That’s a relief because a small percentage of ALS patients do have a genetic variation that is associated with the disease.

Turning to the current state of my health…  I’m still fine everywhere except for my mouth and throat and I restarted work on the cross-trainer with good results.  On rare occasions I can form sounds that are recognizable as words but really I can no longer speak and swallowing continues to grow more difficult.  I’m deeply grateful that Felicity works so hard and is so good at finding new ways to prepare food to minimize my difficulty.  Puree with the right viscosity always works.  Anything scratchy is a problem because it gets stuck in my throat and triggers explosive coughing.  Drinking can have the same effect.

Moving on to something I found yesterday that may explain why I’m doing well overall… https://www.baar.com/atk_rept.htm  There’s a lot of interesting information there, including this:  “You can have a powerful influence over your health if you learn how to purposefully relax your mind and body, and to use your own personal beliefs – whatever they may be – to help reduce unpleasant symptoms and enjoy good health. We all have patterns in our brain of the memory of what it is like to be well. We’re wired for these memories. We can use our beliefs to call on the memory of wellness and bring the state forth. Our thoughts and feelings and beliefs are actually patterns of brain cells, a physical phenomenon. It’s possible to use brain cell patterns to promote health. This is a scientifically proven tool, shown in more than 200 studies to be effective. The scientific evidence is here.”

So my Tibetan Buddhist Practice, which is based on visualization and other techniques mentioned in that article, and the understanding of existence I’ve arrived at based on those teachings look to have been perfect preparation for the negative aspects of my current situation.

It is in fact very interesting to see how things are changing.  I can still have deep conversations one on one, for example, but I haven’t yet figured out how to participate in fast moving group conversations.  I’ve been happy to experience joyful experiences but I’ve been suppressing my awareness of sadness and that’s a mistake because it is a real experience.

In conclusion:  We don’t really know much more about what’s going wrong in my body than we knew a year ago, it does seem I’m doing the right things about it, and there’s a not insignificant chance I could remain healthy overall for quite a while.  I almost certainly will need to have a feeding tube implanted, though.

Jul 24 – From Felicity

Just one slight addition. Dr Chaudhry explained that bulbar palsy is a description of a specific symptom, the way Martin’s ALS has presented.  ALS is the description of the disease process itself.

PBP is the failure of the muscles of the tongue etc that are controlled by nerves coming from a specific part of the lower brain.  It is the effect ALS is having in this case, as opposed to affecting the nerves that control limb movement for example.

As Martin says, we are hoping his will be one of the cases that never progress past that stage, or at least the progression will be slow enough that he dies of old age first.  If not, we will deal with whatever comes as we always have, a new challenge to overcome with love and humor along the way

P.S. Our California trip was fun except that Dad’s box of puréed dinner stowed next to his computer tablet caused great consternation at the security check at Oakland airport.  It had to be examined by many serious faced officials and needed much explaining on my part before it was deemed safe to travel.

My ALS Adventure – April-June 2017

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 ALS Adventure – Telling My Children

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.

My ALS Adventure – What is ALS?

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.

The entire structure and function of our body is governed by the proteins it synthesizes and that operation is controlled by genes.  Proteins are both building blocks for muscles, connective tissues, and other structures and they also, in the form of enzymes, carry out nearly all chemical processes within the body.  Our body produces thousands of different enzymes.

Our genetic system is highly complex.  Its operations incorporate error-correction mechanisms but it can go wrong with an enormous variety of results.  It amazes me it works at all!

How might errors (mutations) occur?  They can occur spontaneously.  When a cell divides, for example, it makes a copy of its DNA, the molecule that carries the genetic instructions controlling its operation.  Sometimes the copy is not quite perfect.  Environmental factors such as radiation, chemicals, bacteria and viruses can also cause DNA to break down and when it does, the cell may not repair it perfectly.

How does the body defend against environmental risks carried in the blood?  The walls of the blood vessels in the human brain are high-density cells that limit what substances can pass from the bloodstream to the brain more than the cell walls of capillaries do elsewhere in the body.

Severe inflammation such as I had in reaction to the Brown-tail Moths can temporarily weaken the bond between those cells making them a less effective barrier.

We know far more about how our genetic system works than we did even ten years ago but not yet enough.  Western medicine has no cure for ALS because its cause or causes are at this point still a mystery.  The only existing medication that slows its progression extends life by only a couple of months.  I take it.

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.

My ALS Adventure – June 2016 to April 2017

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.

Who Does Trump’s Tax Plan Benefit?

 

Who does the Republican tax proposal aim to benefit ?  Let’s examine its intentions, then look at an approach that extends some of its features and is progressive.

Clear intent:  Give more to the heirs of the very wealthy, e.g., President Trump.  The estate tax is eliminated entirely in six years, which greatly benefits the wealthiest 0.2% of the population.

Clear intent:  Continue to take less from wealthy hedge fund managers and private equity executives who will continue to be taxed at capital gains rates that are about half the ordinary income tax rate on profits they pay themselves via the carried interest loophole.

Clear intent:  Give more to the owners of partnerships, S corporations, and sole proprietorships, which are overwhelmingly owned by rich individuals like President Trump.  Income from them that is returned to the companies’ owners is now taxed at the same rates as wages and salaries but it would be taxed at a new 25% rate.   The Tax Policy Center (TPC) finds that the top 1% would receive 88% of the benefit with the 400 households with the highest incomes getting an average annual tax cut of $3.7 million.

Clear intent:  Give all those with high incomes more by eliminating the alternative minimum tax and raising the threshold for the 39.6% top rate on income (which was 70% in 1980) to $1 million for couples, up from $470,700 today.

Clear expectation with unclear intent:  Add at least $1.5 trillion to the federal debt over a decade (many economists think it will be more) by cutting the corporate tax rate from 35% to 20% without raising other taxes.  The cost of this debt will grow as interest rates rise so the intent could be to “starve the federal government of revenues, setting the stage for a frontal attack on core social programs such as Social Security and Medicare.”

Unclear intent:  The corporate tax rate is cut from 35% to 20%, which will save US corporations $2 trillion over the next ten years.  The great majority of economists believe shareholders would be the primary beneficiaries but Republicans say employee salaries would rise.

Treasury Secretary Mnuchin claims: “many, many economic studies show that more than 70% of the burden of corporate taxes are passed on to the workers.”  Congress’s non-partisan scorekeepers — the Congressional Budget Office and Joint Committee on Taxation — as well as Treasury’s Office of Tax Analysis have all assessed the empirical research as showing that only about a quarter or less of corporate taxes fall on workers, meaning that they would receive a quarter or less of the benefit of corporate tax cuts.

The Tax Policy Center estimates that over a third of the benefit would go to the top 1% and 70% to the top fifth.  By 2027, low- and moderate-income families with children would receive little or no tax cuts, and many would see tax increases while 80% of benefits would go to the top 1% of households and after-tax incomes of the bottom 80% would rise by less than half of one percent.

Unclear expectation:  The effect on middle and lower income families.  The plan roughly doubles the standard tax deduction but it repeals or reduces current tax deductions that chiefly benefit workers and middle-class people, including mortgage interest, state and local taxes, student loans, medical expenses, moving costs, and tax credits for retired and disabled people.

  • The standard deduction will be raised to $24,000 for couples and $12,000 for individuals but the $4,050 personal exemption  is eliminated
  • The mortgage interest deduction is unchanged for current homeowners, but is capped at $500,000, down from $1 million, for all future mortgages.
  • The deduction for state and local income/sales taxes would be eliminated.
  • The deduction for state and local property taxes would be capped at $10,000.

The Tax Policy Center (TPC) analyzed the change in after-tax income for the poor, middle class and rich and found the middle class would get a 1.2% boost to their after-tax income and the bottom 40% would get almost no benefit.  Their taxes would actually increase $10 to $20, on average, by 2027.

The Center for Budget and Policy Priorities assessed the intent of the plan in its original form when the top tax rate would have been 35%.  They estimated the overall results of that plan would be:

  • The top 1 percent, who make above $733,000 annually, would see average tax cuts of $90,000 in 2018, increasing their after-tax incomes by 5.9%.  They would receive about 45 percent of the total net tax cut.
  • The top one-tenth of 1 percent, who make over $3.4 million, would receive average tax cuts of $507,000 in 2018, raising their after-tax incomes by 7.2%.
  • This average increase in after-tax income for those in the middle fifth of the income spectrum would be 1.2%.
  • The bottom fifth would gain less than 0.5%.
  • People with annual incomes over $1 million would receive average tax cuts of $138,000 in 2018, compared to average tax cuts of $270 for households making below $75,000. Millionaires’ after-tax incomes would rise 6.4 percent in 2018, compared to a 0.9 percent increase for those making less than $75,000.

The Joint Committee on Taxation estimated the results of every detail of the plan but we do not need more detail to confirm that its purpose is to benefit the wealthy.

So let’s move on.  Is there a way to build on some of the Republican ideas to start reversing our society’s dangerously high inequality?

We use tax revenue to pay for services we want from our government.  The “we” who want those services is, to coin a phrase, “we the people” not also “we the legal entities such as corporations” so it would be more straightforward to tax the income and wealth only of people.

We could then focus just on the relative contribution each economic subset of people should contribute to the cost of government services that benefit us all.  We would still have vigorous disputes about relative contributions but we could better understand each others belief about what is fair.

Making that change might also encourage us to pay more attention to how much our government spends on each of its services.

We know what Social Security and Medicare cost us because we pay for those with dedicated taxes.  But most of us do not know that what we spend on our military activities dwarfs every other discretionary service and includes having our troops in 53 out of the 54 nations in Africa.

A better program might look like this:

  • Entirely eliminate taxes on businesses and tax only the income their owners derive from them.  Replace the lost revenue by:
  • Making income tax rates steeply more progressive, perhaps returning to the 1980 70% rate
  • Entirely eliminating preferred subcategories of income such as capital gains, pass-through and carried interest.  We would tax all income above, say, $12,000 per person, from all sources at the same rates.
  • Making the estate tax very steeply progressive on amounts above, say, $5 million (which my grandfather’s generation did to end the stranglehold of Britain’s aristocracy).

Some other things we could consider:  If we wanted to accelerate cutting the influence of our financial oligarchy we could also establish a wealth tax, similar to property tax but applied to all wealth.

We could also build on the Republican idea of cutting tax deductions that benefit some people over others. and entirely eliminate tax preferences, including mortgage interest, state and local taxes, student loans, medical expenses, moving costs, and tax credits for retired and disabled people.

We could raise the minimum wage if we believe the Republican theory that eliminating the tax on businesses would cause wages to rise.  We might raise them to the level where a head of household could support their family as they did before America needed to be made great again.

We could also explore new taxes such as this one and use those revenues to fund maintenance of the transportation and other infrastructure that we all depend on.

Summing up:  The Republican tax proposal contains some good ideas but the way they are structured would greatly benefit the very wealthy and do little or nothing for everyone else.  It would also greatly expand our debt whose cost will be born by us and future generations.

The promises made to sell the plan are spurious.  It can not be supported by anyone who understands it and is not wealthy and selfish.

But we do need a better tax system.  What do you think of the progressive approach above?  Don’t worry if it seems impossible.  If we are clear on what we want, we can figure out how to get there.

We the Easily Bamboozled – Tax Reform

 

We do need to change how we fund our government’s activities and how our economy distributes the wealth it creates.  But the tax plan now being revealed is not what we need.

Our President and Republican leaders keep telling us it is urgently necessary to lower corporate tax rates so companies can stay competitive.  It has become the party line.  As my Republican Senator Toomey’s website says:

“Our country’s current corporate tax rate of 35 percent is one of the highest in the developed world, far higher than the average 25 percent rate of our economic competitors. Without a significant reduction in business tax rates, the U.S. will never be the best place to invest and create jobs … The best economic stimulus for the middle class, who have seen their wages stagnate and tax bills rise over the last decade, is a well-paying job. Tax reform will help deliver on this goal with its focus on lower rates, investment, and growth.”

Really?  The 20 US companies that accounted for 20% of US GDP last year, meaning all our economic activity, paid taxes totaling only 0.6% of GDP and only 3.6% of all federal taxes (Source: Forbes).

Businesses pay taxes on profits not revenues, of course, but my point is that our theoretical corporate tax rate is not making our businesses less competitive or stifling their job creation.   That is not the motive behind this tax plan.

But before we get to that, what would this budget cost?  The fiscally conservative Committee for a Responsible Federal Budget estimates $2.2 trillion in lost revenue over a decade.  The tax cuts would total $5.8 trillion.  New revenues totaling $3.6 trillion resulting from “base broadening” would allegedly reduce the net loss to $2.2 trillion.

Treasury Secretary Mnuchin told us “The plan will pay for itself with growth”.  But we have seen over and over again, most recently in a multi-year “experiment” in the state of Kansas which its governor trumpeted as a “shot of adrenaline” to the state, that a tax cut plan that will pay for itself is a fantasy.

That fantasy cannot and will not ever come to pass.

So, what motivates this plan?   Who would benefit?  The Tax Policy Center estimates that 80% of the $5.8 trillion in tax cuts would benefit the richest 1 percent.

And who would lose?  55 million Americans would lose their tax deductions on what they save in tax-deferred 401k plans and what they pay in state and local taxes.   They are our middle class.

Those who earn less would be hit harder, losing government services eliminated to pay for the tax cut, which look to include about $1.5 billion in Medicare and Medicaid.

Would anyone other than those with very high incomes benefit from this plan?  Yes — their heirs.

This is a good budget if you want your kids to live under an aristocracy of folks like President Trump whose inheritance installed him among the plutocrats who bought our political establishment.

We do not have the leaders we should want.  Their plan is very far from what we need.

The Wall

 

I love Pink Floyd’s “The Wall”, Trump’s is absurd, and this is about a different kind of wall altogether.

A Zen Buddhist was about to teach a class of six-year-olds meditation.

One of the boys asked: “Does meditation open your Third Eye?”

“Not exactly” the teacher replied.

“Does meditation teach us to levitate?” the boy asked.

“Not exactly.”

I’ve heard,” the boy said, “that meditation teaches us to walk through walls.”

“Yes, it does.”

“But what happens if you lose your meditation while you’re in the wall?”

“You get stuck.  It happens to me all the time.”

[Hat tip — Anam Thubten]

We the Easily Bamboozled – Part 1

 

To understand what’s going on in the USA now it’s helpful to go back a little way.  We could go back to Reagan and Clinton but his successor, G. W. Bush, offers the clearest illustration.

Nobody imagines President G. W. Bush was setting policy.  He was simply the lead spokesman for an agenda set by others.  That’s the important point

We do imagine President Obama set his policies, but let’s consider a few examples.

Obama inherited an economic collapse whose origin was financial deregulation introduced in Clinton’s time.  What was done by the Obama administration?  The collapsing financial institutions were bailed out. Not one of the people who acted criminally was punished.

Obama also went along with the Fed’s ultra-low rate interest policy that destroyed the value of pension funds along with individuals’ savings.  That triggered new speculation, again favoring the financial institutions.  Meanwhile, pension funds that are now paying out more than they earn will fail.

At the start of Obama’s first term Democrats could have established lower cost single payer health care for all Americans.  Instead, we got the highly complex ACA which preserves the central role of insurance companies and perpetuates all other aspects of a system whose primary aim is profit.

Obama was enticed into  ruinous and utterly unproductive war in Afghanistan as well as the destruction of Libya.  He avoided overt war against Syria only at the last moment.

Obama genuinely wanted to make a better future for all.  So why did he lead us into all those disasters?  Because, like his predecessor, his advisers were from the institutions that are in fact in control.

Now we can see why the situation under the Trump administration seems so confusing.  Two things are going on in parallel, one masking the other.

Trump is motivated solely by self-aggrandizement and self-enrichment.  He is a bully and a liar.  He appears also to be to some degree mentally ill but we don’t know about that for sure.

The result of Trump’s personality is what we see, a constant flurry of inflammatory tweets, intemperate remarks and outright threats.

And the appearance that our President is deranged is alarming.  I’ve written elsewhere about the risk his threats may lead to nuclear war on the Korean peninsula and a nuclear arms race in the Middle East.

But, preoccupied by Trump’s incendiary rhetoric, we pay little attention to what’s going on in parallel, what the ever-present advisers to our Presidents and Congressmen are doing in the meantime.

We notice only fleetingly what is actually being done:

  • further weakening of banking regulations
  • destruction of environmental protections
  • astronomical increases to our already overwhelming military
  • additional tax cuts for the wealthy to be offset by cutting Medicare and further expanding Federal debt
  • and so on and so on

These changes are part of an orchestrated program that benefits only those who are in fact in control of our government.

President Obama naively went along with the advice he was given, imagining it was given in good faith.  President Trump has no principals of his own and seems easily manipulated.

But the different character of these men makes no difference to what is being done to we the easily bamboozled– except that Trump’s thoughtless belligerence  may blunder us into WW3.

Our Presidents are now simply figureheads of an oligarchy.

I expect in a future post or two to elaborate a bit on changes by the oligarchy that we should be resisting and I would be delighted to get help with that in any form; guest posts, comments, or emails.

I will also continue thinking if there’s anything we can do short of revolution, which I am not in favor of because, like the French Revolution, it could lead to even greater disaster.

Collective Unawareness

 

It is quiet this morning.  No howl of fast accelerating cars and trucks propelling their drivers to work.

Today we celebrate the accidental discovery of this land whose inhabitants we slaughtered and which we call our homeland.

It is not our true nature to do such things, to brutalize others.  We just have the habit of behaving that way.

So let’s change!  Let’s become who we truly are.  Let’s become more and more the good people we have often been.

We are now spending a trillion dollars a year on things we think of as defending ourselves.

We have for sixteen years been in a “war on terror” that can by definition never be won and which motivates terrorism against us.

We are at war in seven countries, none of which has declared war against us.

We are spending vast sums preventing people whose countries we help destroy from coming here.  We sing:  “This land is our land…”  We say:  “This is the land of the free” while giving up our freedoms.

This would be a particularly good day, since we will not be distracted by work, to take a deep breath, relax, and consider who we really are.

We are a people who desire happiness and often act generously but who have some very bad habits.

We entertain ourselves with spectacles of violence, we fear violence against ourselves, we think that fear justifies our own violence, and we imagine magical solutions.

Our leader told us he would build a huge and beautiful wall to keep immigrants out.  He said Mexico would foot the bill and too many of us pretended to believe him.

Our leader now tells the world we may utterly destroy North Korea.  We already did that to Iraq and Libya, just not yet with “fire and fury the world has never seen”.  I won’t go on with the litany.

What I’m saying is, we really are not bad people.  We are good people with bad habits.

So please, let’s spend some time today noticing our selfish, fearful violent habits and start to shed the collective unawareness that makes it possible for us to do terrible things.

Let’s resolve to become the kind and happy people we really are.  Let’s do it!