My ALS Adventure – January 2018

This was when I had a feeding tube installed.  I explain how it was done.  Next month I’ll write about adjusting to the new way of eating.

Jan 3 – To contributors to Doma’s college education

Note:  Doma is an amazing young woman from Nepal whose education we’ve sponsored for the last ten years and who will graduate from Hampshire College in MA this May.

I haven’t said anything about myself during the long campaign on Doma’s behalf because I wanted the entire focus to be on her but now the funding is complete I’ll give you a quick update.

We’re happy here in farming country outside Gettysburg, close enough to visit our kids and four young grandchildren.  The pre-Civil War brick farmhouse, outbuildings and seven acres of land were extremely run down when we bought the place but we liked the area — looks a lot like where we grew up in England – and I thought I’d have fun doing a lot of the renovation myself and managing the work I lack the professional skills to do.  I was right: it has been very satisfying — and there’s more yet to keep me happy.

The negative news is, I was diagnosed with ALS at the start of this year.  It began in my mouth and throat but it hasn’t yet progressed to other muscles.  Because it’s now more than 18 months since the first symptoms, it’s increasingly likely it won’t progress, and a few months ago I was introduced to a doctor of Tibetan medicine who has cured many MS patients and more recently also cured a growing number of ALS patients.  ALS is incurable by Western medicine but perhaps the Tibetan treatment I’m getting will work for me, too.

I feel blessed to have been doing my Tibetan Buddhist practice for long enough that when I was told to expect rapid weakening of all my muscles and death within one to two years, I saw it as a great opportunity to train myself in equanimity.  Now it seems likely I will live longer, I’ve grown more frustrated by my loss of speech and the enjoyment of food, the amount of time and effort eating takes, and so on, which makes this a new opportunity to train in equanimity as well as in relishing my blessings.  If I recover, I can practice extreme gratitude 🙂

Jan 4 – To my Tibetan doctor

I don’t think my tongue has shrunk further and Felicity agrees, so the change in how things taste presumably does, as you suggested, result from the Riluzole saliva reduction medicine.

I slept 10 hours last night and woke feeling fine.  I haven’t had a recurrence of waking for no apparent reason as I did for a few days right after Christmas.  I felt on the verge of mild despondency and suspect it resulted from processing my isolating experiences at Christmas and perhaps fighting off a heavy cold from which Felicity is still recovering.  My morning hour of practice today felt restorative and illuminating in the same way as before Christmas.

Difficulty sleeping is an example of how each of the bulbar-controlled muscles has to work well for the entire system to function effectively.  I can’t blow my nose because my cheeks and lips are weak.  I can’t generate much pressure and my lips can’t contain it, anyway.  That results in my nose getting clogged, but I can’t breathe through my mouth without dribbling.  I can’t lie on my side because the upper cheek sags down between my teeth and gets bitten.  The only way I can sleep is on my front with one hand under my face applying pressure that keeps one nostril open.  So long as I keep my head elevated a bit, the saliva drains down my throat so I need to stay still, which usually isn’t a problem.

It takes a while to figure out solutions like that, especially because what worked a few months ago might stop working if the muscles weaken more, but it’s interesting to someone like me who enjoys finding solutions to problems.

Jan 17 – To my family

You know I’m getting a PEG (feeding) tube installed next Tuesday but I don’t think I’ve said much about it. I was recommended to get one several months ago because the big risk for patients with swallowing problems is food getting into the lungs and causing pneumonia, but I was always bad at taking advice 😉

I’m pretty sure I haven’t been getting food in my lungs but the weakness in my tongue, lips, cheeks and throat has made eating increasingly difficult.  I can take only small spoonfuls and I must wait for the last one to get to my throat before I can take the next.  Drinking is even more difficult and, like food, it must be a specific viscosity.  Also, for reasons I don’t understand, drinking provokes coughing fits that persist for up to an hour after I finish a glass of Ensure.

Eating has become all too much like working on the cross-trainer.  Pretty soon after I start, a voice inside my head starts saying, “You’ve done enough.  You can stop now”and I have to keep replying, “No, I haven’t finished yet.  I can handle another 5 minutes”.

Figuring out what to eat and how that changes is somewhat interesting.  I’ll probably have to start all over again with the tube.  ALS patients need more than the usual number of calories, presumably because of the subtle muscle twitching.  I don’t have that but I need 2,500 to 3,000 calories a day to keep my weight at 140 lbs and that is a struggle.

I didn’t feel hungry at all yesterday morning, for example, so I blended my usual 3 chicken patties and a cup of rice for breakfast but I didn’t add the usual two eggs that take the meal up to around 1,000 calories.  It turned out to be much harder than usual to swallow so I had to add a bunch of olive oil.

Anyway, I finally wised up.  The only reason not to get a PEG is because, who wants a tube in their belly?  I don’t, but it will be better for me than the way I eat now, and better for both of us because it will be so much easier to be away from home.  We can go on trips again 🙂

A PEG tube is a small plastic one through which I will be able to inject food and drink directly into my stomach.  The surgeon (who insists the procedure is not really surgery) snakes a camera and light down my throat into my stomach and uses the camera to maneuver the light into the center of the stomach, well away from other organs.  The light is visible through the belly wall so once it’s in position, he in essence stabs a little hole in its direction and inserts the plastic tube into the hole.  A few days later I can start injecting boluses of food down the tube.  “Gin and tonics, too, if you like” he said.

The risk of damage during the surgery is very low.  Infection is possible but unlikely.  I can shower and do anything else I want once I have the tube.  Because I will regain so much time that I now spend preparing food, eating and clearing up afterwards, I can start an exercise program again and begin regaining strength.

It’s a good decision.  Better if I’d made it a few months ago but I wasn’t ready then.

The only other thing I have to report are a couple of dreams.  The night before last I was playing on the floor with a composite of my grandkids.  The thing we were playing with had a tiny whistle that (s)he demonstrated.  We knew I couldn’t do it but we wanted me to try, so I put the whistle between my lips, pressed them together with my fingers and blew.  Air escaped but there was no whistling sound.  We smiled at each other.  It’s very cool that I know what’s going on with my body in my dreams.

Last night I was surprised to notice that I was replying to someone.  It was only a whisper but so encouraging.  Maybe I can do better than that, I thought.  I put my mind to it and rapidly regained normal speech.  That seems pretty unlikely now that I’m awake but it’s good to know my optimism remains deep rooted.

It should take only a few days to get used to the tube so I’m looking forward to seeing you all again soon.

Jan 23 – To my family

The surgery went well.  I’ll keep notes while I recover and send you an update in a week or so.

My ALS Adventure – December 2017

This month I decided I would definitely get a feeding tube installed.

The way some foods taste had changed.  I’d postponed giving up bagels until they became almost impossible to swallow because I enjoyed chewing them so much along with their taste.  Now I’d had to give up on lamb because it tasted gross and on red wine and dark beer because they tasted sour.  I would have had to give them up, anyway, because I could only swallow more viscous liquids.

Christmas presented some emotional challenges but I was still almost always happy.

December 12 – To my Tibetan doctor

My PEG tube consultation is on the 19th.  At the time we scheduled it I only wanted to ask questions and make sure I understood all the ramifications.  Now, I’m sure I should have it done, so we’ll schedule the procedure as soon as we can after the 19th.  It’s hard for us to go anywhere because of my eating and drinking difficulties, and they take up a lot of time every day, anyway.

Returning to the one-precious-pill-every-three-day routine went smoothly and was welcome because after sleeping an average of 11 hours a night, spending an hour to an hour and a half on each of three meals, and doing two hours of practice, there’s not much time left for other things.

My attempts to gain a few pounds weight in case of an everyday illness are not working.  I’m stable at 140 lbs despite an average of 2,500 calories daily and sometimes more.  Felicity is envious of this side effect of my ALS 🙂

I’m not aware of any changes in my body.  I don’t think anything is getting worse.  I did feel a couple of times that my tongue was ready to start regaining strength but there was no specific basis for the feeling.

I feel fairly tired by evening time but usually okay during the day.  I have 2 or maybe 3 hours in the afternoons when I can choose what to do.  The weather has been good most days so I’ve been working on renovating the summer kitchen, taking it slowly, not making myself tired.  Progress is slow but very satisfying.

December 20 – To my Tibetan doctor

The PEG tube consultation was helpful.  I’d already realized I should have it done but it was good to have the procedure explained and learn a bit more about living with one.  I’m having it done on January 23rd.

Now it’s scheduled, I realize I’d have been smarter to have had it done before.  Missing one of my three meals yesterday because of the appointment left me very tired, and it’s always hard (for which read, pretty much impossible) to drink enough because it takes so long and causes so much coughing.  I’m eager for an end to that!

My sleep has been a bit shorter the last few days, 8 or 9 hours, not 10 or more, and it has been interrupted more.  I don’t know why.  I feel it’s a transitory problem.  My gut felt a little odd for a couple of days when the sleeping change started.  I probably ate something that didn’t agree with me.  My gut feels okay again now.  I felt tired when I woke today but I’m fine now after lunch.

I forgot to mention that the way things taste has changed.  I assume that’s because my tongue shrank.  I’ve always loved red wine and dark beer but they both taste sour now.  I had to give them up a couple of months ago.  I gave up coffee, too, around that time because it just doesn’t feel right with thickener.  Savory foods still taste fine,  Chicken, turkey and to a lesser extent pork taste ok.  Lamb, which I used to love, tastes gross now and I haven’t tried beef for quite a while.  Pear smoothies are good.  Chocolate Ensure tastes good but gives me coughing fits.

December 31  – To my Tibetan doctor

I’ll start blogging about life with ALS in January, hoping it may be helpful to others.  I’ve noticed a few stages so far, each offering a somewhat different opportunity to train in equanimity.  Christmas included traditional foods that we only have at such times, which I remember as delicious, and which I can no longer eat.  I felt a little regret that I couldn’t have them this year but mostly I enjoyed memories of happy previous times with my family, and I enjoyed their delight in the foods now.

A bit more difficult was being in groups of friends and family chatting and joking.  I can have good conversations with one person at a time using a notebook but that really doesn’t work in a group.  It breaks up the flow.  So I felt isolated at times.

A benefit of travel is the way it highlights differences.  I realized that although the change is so slow I don’t notice it from day to day, swallowing has grown more difficult.  I have to get the viscosity of my pureed food just right.  My tongue is very weak but the greater difficulty seems to be in my throat.  It takes quite a few spoonfuls of food before I can get a flow going.  And I get acid reflux sometimes now which I never had before.

But there’s still no sign of weakness in other muscles and I’m looking forward to having enough free time again once the PEG tube is in so I can resume work on the cross-trainer.  I feel good but I am much less strong than I was.

February 11  – Another recent photo

Baby Eleanor again, this time with her sister wearing a colander hat while studying a picture of her uncle and one of her cousins.

My ALS Adventure – November 2017

After three months of taking a precious pill once every three days Dr.T had prescribed a program of three different medicines per day for two weeks after which I returned to a precious pill program.  I was providing her with more details of my symptoms during the transition.

I had transitioned to the recommended diet but realized this month that I wasn’t getting enough calories.  We still haven’t figured out why, but ALS patients need more calories.

Swallowing was growing more difficult, I was coughing a lot, and my discretionary time was greatly reduced for several reasons, in particular because eating was now such a slow process.  I was beginning to recognize that I would have to get a feeding tube installed.

At a seminar on ALS and similar diseases I recognized that Western medicine aims to kill things to effect a cure while Tibetan medicine aims to get the body back in balance so it can cure itself.

November 8 – To my Tibetan doctor

Today is my eighth day on the new medicine.  Details from my diary since I started them:

I felt fine all day the first day but slept poorly that night because my nose was plugged.  I can’t blow my nose now because my lips are too weak.  I had a slight pain in my belly when I woke which disappeared after breakfast.  I coughed a lot in the morning and my throat was sore (I think one of my Western meds had dissolved in my mouth) and I felt tired all day, probably because I’d slept poorly, but I felt okay by bedtime.

I slept well and felt fine for the next three days.  On the sixth day I slept poorly and my lips felt swollen when I woke.  They felt as if they were stuck together.  I coughed a lot all day and had low energy.  It was difficult to swallow saliva that night and I ended up sleeping on my side with my head raised several inches.  Much coughing that day, too.

I slept well last night in the same position and feel fine.  Felicity says my lips do still look swollen but maybe less so now.  I ‘m still having periodic coughing fits but less often today.  What seems to happen is, saliva builds up in my mouth, something happens (presumably it starts sliding into my throat), that alarms my throat and a violent coughing fit ensues.

Overall: My energy has continued to be good except for the two days when I slept poorly.  I’m guessing the problems sleeping were unrelated to the medicine.  My bowels had returned to normal some time before I started the new medicine.  The only change I’ve detected is that my cheeks have been getting caught between my teeth more often for quite a while.  It’s a slow weakening of the cheek muscles.  I might be slowly having a little more difficulty swallowing but I’m not sure.

I’m following the diet chart fairly well.  Carbs are difficult because oats, which would be best, are too fibrous and irritate my throat.  I mostly use white rice because it blends to a good consistency.  I have potato less often because it’s less versatile.  Pretty much all my meat is chicken.  I’m making more fruit smoothies.

I wondered why I had to eat so much just to maintain 140 lbs weight.  Felicity read a couple of days ago that ALS patients need substantially more calories and then we remembered being told at Johns Hopkins that I should eat 3,000 calories per day.

I had a few short spells of feeling a bit dispirited by the amount of time it takes now just to stay alive, two hours longer sleeping, three full meals per day that take about an hour and a half each, hor-mey and meds, and so on.  Having ALS isn’t interesting now, I thought, just inconvenient.  Then I realized this phase is a new opportunity to practice equanimity and I felt fine again.

November 9 – To my family

It’s about six weeks since my last update so it feels like time for an update even though I have no real news.

The Tibetan approach to medicine is slow-acting.  To over simplify, where Western medicine aims to kill whatever is causing the symptoms, the Tibetan approach  is to re-balance the body’s workings so it will heal itself.   My Tibetan doctor is super responsive.  I feel blessed.

The regimen I’m on is not expected to show even subtle signs of improvement for at least three months and I’ve now been on it for just about that time.  That means we should expect my symptoms to have worsened during the first three months of treatment.  The hope is they would start to improve sometime after that.

In fact they have worsened in the last three months, but despite my best efforts to keep track I don’t really know how much they’ve done so in the last three vs the last six.  The rate of worsening has definitely slowed, and I still have no noticeable problems anywhere other than in the muscles around my mouth and throat.  This may mean the Tibetan medicine is getting my body back to full health.

The main problems I experience now are, (1) my lips are weak so I have to press them closed with a napkin when I eat or drink, I dribble at times, and I can’t blow my nose vigorously, (2) my cheeks sag between my teeth and I can’t always avoid biting them, (3) my throat doesn’t swallow so well, it gets alarmed sometimes by the first signs of saliva or food coming its way and coughs violently, and I have to keep clearing my throat although I’m not sure why.

When we ordered an arm band for me about being an ALS patient I joked about having it say: “I make funny noises and dribble but I’m not dangerous”.  I’m a slow and noisy eater now but I’ll try not to be too bad at Thanksgiving.

Some foods and drinks taste different.  Sadly, all red wine tastes bitter.  I still have quite a bit left in the basement that I hope you’ll try.  I’ve no idea how much is still good to drink, but it’s okay in coq au vin.

It was easier in an odd way while my symptoms were growing noticeably worse because it was interesting figuring out how to do things in new ways.  A core aspect of Buddhist practice is training oneself in equanimity.  During the first stage when I was losing the ability to speak it was fairly easy to avoid having feelings of anger because it was a learning opportunity.

A few days ago, I started to feel frustrated because there’s been very little change to respond to for quite a while and the extra two hours I spend sleeping, the hour and a half or so every meal takes, the time I spend on the medical regime, and the two hours of Buddhist practice my teacher told me to do leave me much less time for things I choose to do.  Then I realized this is another opportunity to train myself out of responding to emotional scripts and into more perfect equanimity.

So, it continues to be good to be alive.

My latest project is renovation of the summer kitchen.  The posts at the back had rotted out and a couple of beams cracked as the back wall subsided.  I’m slowly jacking the structure up so it’s straight before I replace the footing and the rotted out parts of the posts and repair the cracked beams.  It’s interesting work, not very hard yet, and it’s going well.

November 11 – To my Tibetan doctor

I was very tired yesterday, which is unusual even after a precious pill, and my throat was extremely sore after I took the morning medicine.  I was producing huge amounts of slimy saliva and having frequent coughing fits all morning and into the afternoon.  I decided to skip the other meds yesterday and this morning’s dose so I’d have skipped a full day, and resume at lunch today.

I feel a bit tired this morning and my throat still feels sensitive but I no longer feel as if I’m getting a bug as I did yesterday.

The timing is unfortunate because we’re going to an ALS conference in Lehigh this morning where there will be a presentation about swallowing.

Felicity is concerned that my swollen lips could be a precursor to a new stage of weakening, similar to the way my swollen tongue was my first symptom of ALS.  I have an open mind — we’ll see what happens next.  My lips feel okay again although I’ve been having increasing trouble biting my cheeks and the inside of the front of my mouth.  If I had to guess, I’d say the ALS effects are continuing to increase but still at a much slower rate than before we began the Tibetan medicine.

November 16 – To my Tibetan doctor

I had never tracked calories I eat.  My metabolism is fast and I’ve always been quite active so I didn’t gain weight until I was over 50 when I was working very long hours at a desk.  Then I cut back on the quantity of food.  Looking back over what I’ve been eating in the last couple of months and having looked at a calorie counter I’m guessing I’ve been getting around 2,000 calories per day from three large bowls of different foods.

I started tracking calories in my health diary yesterday and chose what I ate so it would total around 3,000 calories.  I can do that every day by incorporating things like fish fingers and other fried foods which I’ve only eaten rarely in the past but maybe it would be ok if I also drink enough fruit smoothies and so forth.  It takes a long time to eat one of those bowls of food so it doesn’t seem feasible to get 3,000 calories from a fully healthy diet.  I’ll experiment.

I think swallowing is slowly growing more difficult and Felicity is concerned that I may aspirate food into my lungs and get pneumonia so we’re scheduling to learn more about PEG (feeding) tubes.  I don’t want to have one, of course, but it may become essential and it would mean I could pack enough calories directly into my stomach and eat just enough at meal times so I could finish in the same time as Felicity or guests.  It would also make it more practical to be away from home.

No other changes or news.  I feel good, just wish the extra sleeping, longer mealtimes and etc left me more time for other things.

November 18 – To my Tibetan doctor

The coughing fits lasted only a few days.  I’m okay again now.  I think what happens is, because my entire mouth, throat and related parts are weak, any additional difficulty has exaggerated results.  This morning, for example, it was unusually hard to eat breakfast because my nose was plugged.  I can’t clear it in the usual way because my weak lips mean I can’t blow my nose with enough pressure.  I just have to be patient and wait until I’ve been vertical for long enough.

Felicity asked me to tell you that I sleep much more calmly these days.  I’m only aware of the occasions when I wake and my throat, irritated by saliva, makes growling noises that I try to keep as quiet as possible.  She sleeps through those 🙂

You asked how I feel after a day of higher calorie meals.  Better, in a word.  Responding to your concern about fish fingers and whatnot, I’ve always eaten a pretty healthy diet and I’m not going to start consuming a lot of unhealthy food now.

You also asked about my lungs.  They’re still working fine as far as I can tell but there are a few times when in-breaths stop before my lungs are full.  I think that’s related to temporary problems in my throat and/or nose but I’m being watchful.  I’ve never felt short of breath.

We have an appointment a month from now with the surgeon who will install my PEG tune if and when.  The little research I’ve done so far makes me think it probably would be better on balance to get one installed.

But that reminds me…  The experts we met at the Lehigh conference do not expect to reverse the symptoms of ALS, only slow or ideally stop its progress.  I asked about replacing dead motor neurons with stem cells and was told that would not be effective because the nerve from motor neuron to muscle will also have died and cannot be regenerated.  Since the body is constantly regenerating (most of?) its component parts I don’t see why that would be true.

The more encouraging thing I was told is, not all ALS patients with bulbar onset progress to other parts of the body and the longer there is no progression, the better the odds there never will be.  Overall, my conclusion is, there’s very little understanding of ALS so far by Western medicine.

My ALS Adventure – October 2017

Although my mouth and throat muscles were continuing to weaken, the progression had slowed.  My other muscles still seemed okay but they, too, were weaker.  I was having very little difficulty accepting my new limitations.

I continued to feel I was in an equanimity training program.  I was very lucky, though, to be experiencing no pain.  Suffering is something we create.  Pain is something that happens to us.  We can train ourselves not to create fear but very few of us can transcend pain.

October 1 – To my Tibetan doctor

I recognized quite by accident during a break that I can no longer move my jaw from side to side.  I had suspected the area around my lips was weakening, making it harder to keep food in my mouth even with a napkin pressed against my lips, but I wasn’t sure.  I was also unsure whether drinking has grown harder — needing to take smaller sips and hold them in my mouth longer before swallowing.

Then I noticed toward the end of my first ten hours of practice yesterday (Felicity is away from home for a few days so I can do more) that yawning is a problem because my cheeks now sink in between my molars which means I must be very careful when I close my mouth.  I’d gotten used to biting my lips closer to the front of my mouth.

So I now recognize that all my facial, mouth and throat muscles have continued to grow weaker although the rate of decline has slowed.  It’s impossible to gauge the rate of change, or changes in the rate, since I began having to puree all my food.  Because I’m not making enough use of the muscles, it’s hard to detect changes in their functioning.

The muscles everywhere else in my body seem to be okay but they are weaker.  I started doing 12 minutes on the cross-trainer on the days when I don’t take the precious pill, not pushing hard but pushing enough so the muscles do some work.  It feels good, but it’s far from enough to rebuild the muscles’ strength.  They would be weakening if the ALS has spread to other muscles, but they would also be weakening because I haven’t been using them so much.

I continue to be happy 🙂

That’s all I can think of to tell you about my health.  I believe the way ALS progresses when untreated varies from person to person and I imagine the rate of improvement in Dr Lobsang Dhondup’s patients also varies so I won’t ask what to expect in my case.  I’ll just do my best to keep reporting what happens.

About nutrition:  I’m maintaining 140 lbs weight and I feel okay at that.  I haven’t been able to increase it.  I’ve pretty much completed the transition to alkalizing foods using this chart when I have questions http://greenopedia.com/alkaline-acid-food-chart/  I love pork and haven’t yet totally given it up but almost all my meat is now chicken and a little turkey.  My evening meal varies quite a bit.  Scrambled eggs, a chicken patty and rice all blended together are my staple for breakfast.  I usually have lentils and rice with blue cheese dressing for lunch.  I supplement with Ensure during the day.

October 9 – To my Tibetan doctor

I recognized again this morning that I really know nothing for sure about how my sickness is progressing.  Most days all seems the same as I think it was a month ago or more.  Occasionally, I notice there has been a decline, like the inability to move my jaw sideways, but I don’t know how long ago that happened.  This morning and a couple of days ago I have the sense that I may be getting better.  But as I said, whatever changes are taking place are too slow acting to notice.

I’m fine with not knowing what’s going on.  I’m totally confident that I’m doing what’s best to do and it’s all good training to grow more patient.  I’ve had no difficulty so far accepting the limitations — but I’m very lucky because there’s no pain.

Note: Feb 3, 2018 – I have no limitation at all with Eleanor – neither of us can speak 🙂

October 30 – To my Tibetan doctor

I’m feeling good today.  I spent the morning practicing and reading and this afternoon I enjoyed working (not very strenuously) on renovating the old summer kitchen that came with this house.  I was feeling a bit grumpy yesterday, not tired, just lower than usual energy.  The day before, I was feeling good.  I can’t identify a reason for the differences.  I almost always do feel good.  I’m getting used to being able to do less because eating takes so much longer now and I’m doing more practice.  Equanimity.

 

My ALS Adventure – September 2017

This month was uneventful.  Our main focus was on dietary changes that are an important aspect of Tibetan medicine.

September 23 – To my family

It’s a while since I sent my last update because I’ve been hoping to tell you something definite.  I’ve been keeping detailed notes for a little over 3 weeks about what I eat and do and how I feel.  They will reveal in the end how I’m progressing, but they haven’t yet which might mean my symptoms have stopped getting worse, or at least that they are getting worse more slowly.

Part of the difficulty is the dietary aspect of my Tibetan medicine treatment.  The doctor first wanted to make sure my body tolerates the “precious pills” because they are powerful.  At that time she gave us only a little guidance about foods that are good for me and ones to avoid.  There were no ill effects from the first pills.  They make me tired but that’s expected because they are getting my body to work on healing itself.  So she sent more pills and emailed several academic papers about Tibetan medicine.

There’s not much published research about Tibetan medicine and what there is focuses mostly on treatment of cancer or the results of testing to see if any of the ingredients are harmful.  They aren’t.  My doctor’s mentor in CA who has been treating ALS patients successfully hasn’t yet published anything about it, and my own doctor’s experience is chiefly with cancer patients.

What the papers did make us realize, though, is that diet is a big part of the treatment, which is good because it’s something we can control.  The papers didn’t give specifics, though.  I asked the doctor and she sent us quite a bit of guidance, but of course we now have more questions.

The overall dietary theme seems to be to eat mostly foods that have an alkalizing effect and avoid ones that acidify.  That’s not as straightforward as it sounds, though, because lemon, for example, which tastes acid, is an alkalizer.  And like anything else, it takes a while to make the adjustment.  I had, for example, been having a can of corned beef hash for lunch quite often as part of the effort to keep my weight at 140 or above but beef is acidifying so I cut that out and lost 5 lbs in a week.  I’m back to 140 now and still experimenting to find things I can eat that are okay with the treatment and also taste okay.

I feel happy but this is an ongoing process of adjusting to negative changes so I’m probably creating suffering for myself that I’m not noticing.  I’m not worried about what might happen next, I just get frustrated by new difficulties.  Nothing new there, right?  We all experience that.

I feel frustrated sometimes because I have less discretionary time.  Eating is a slow process, there’s quite a bit of medicine taking and treatment stuff to do, and I committed to the two meditation sessions of an hour each every day.  All those things feel productive most of the time, but they do end up taking a lot of time, so I have less time for other things.

The other challenge is I’m losing muscle mass because I have to avoid tiring myself so as not to interfere with the Tibetan treatment which makes me tired, anyway, so I’m getting much less physical exercise than usual.  I’ve been doing more recently, aiming for a better balance.

I’ve learned how to act in shops so I can communicate without much difficulty and in a friendly way.  Being in a group is disappointing because I can’t really participate.  I can Skype pretty effectively by holding notes up to the camera.  Emailing and participating via social media is good because that works just the way it always has.

Now it’s time for lunch – lentils and rice today – followed by some gentle exercise then clearing up my tarpaulin tractor shed that was destroyed by the amazing windstorm.

Reply from a friend

It doesn’t surprise me that diet plays a big role in the function of Tibetan medicine.  When I was studying Chinese medicine so much of what we were looking at was diet-related.  It was part and parcel of the use of medicinal herbs, etc.  Many of the Chinese stews and soups, for instance, had medicinal herbs in them, and food was related to season, the presence of certain “humors” in certain places/times, geography, etc.  It formed one holistic system, of which the human body was microcosm.

In Chinese medicine, palsies are either related to wind or phlegm, which type you suffer from being a combination of the two (shaking being more wind, and blockage being more phlegm).  The combo of wind/phlegm can be particularly tricky since usually those two forces are opposed to one another (movement and blockage).  I don’t know if any of this is resonant with the Tibetan forms of diagnosis and treatment, but the broad outline of what you describe sounds very familiar to me.

 

My ALS Adventure – August 2017

This was the month when my teacher, Phakchok Rinpoche, introduced me to my wonderful Tibetan doctor and I began a course of treatment that has cured other ALS patients.

I do not yet understand much of the theory on which the treatment is based but I will keep studying and I will write about it when I understand enough.

From here on, I will include some of my emails to my Tibetan doctor.  They include references to information from her, but I will not include her emails because communications from any doctor are confidential.

August 14 – To my family from my Tibetan Buddhist teaching retreat with Phakchok Rinpoche

I should start by explaining that Rinpoche is extremely kind and observes his students closely.  That explains why things happened as they did.  I had told the retreat manager and Rinpoche’s secretary ahead of time that it would be an entirely silent retreat for me because I cannot talk, but there’d be no need to do anything special for me.  I told them I’m happy and consider what’s going on to be a wonderful learning opportunity.  I just didn’t want them to be shocked by the change.

Rinpoche invited me to join him and five others to lunch early on.  I later realized they’re all involved with Tibetan medicine.  One (Dr.T) is a highly trained Tibetan doctor, very smart and logical as well as kind.   Rinpoche told her to give me treatment.

Dr.T prepared four medicines for me to take at different times of day and, though I did not learn this until later, she initiated contact about me with her mentor, a Tibetan doctor who fled from Tibet with the Dalai Lama and who the Dalai Lama later sent to Mongolia to reestablish Tibetan medicine there.  He is now based in San Diego.

I took the four medicines for a week or so and she also prescribed a “precious pill” one day.  She and another woman who practices Tibetan medicine also applied heat treatment to the crown of my head, the base of my throat, my sternum and about four finger widths below that twice a day.

This treatment is for disturbed flow of the “rLung”, an internal wind about which I know nothing so far, and for malfunctions in the liver and kidneys, all of which Dr.T diagnosed from my pulse.

Then Dr.T’s mentor, who has many ALS and MS patients told her he used to treat ALS as a neurological problem but he later recognized that it’s more effective to treat the whole body.  He said to stop the four medicines and prescribed two new powders for me to take at the beginning and end of day in preparation for a course of precious pills that I will begin at 5 tomorrow morning.  I take one, go back to sleep, spend the day restfully and follow a pretty strict diet, rest and diet two more days, then take another precious pill and repeat the process.  After 5 pills and 15 days we will evaluate changes, if any, and proceed accordingly.

I’m skeptical about alternative medicine in general but open minded about Tibetan medicine because although the theories on which it’s based don’t make sense to me, the results have been studied very carefully over a very long time and I have great respect for results.

Also, Rinpoche is very smart and highly skeptical by nature.  I feel extraordinarily blessed to have met him and to have had the trust and discipline to practice sincerely, although not enough, in the ways he told me to.

Half way through the retreat I realized Rinpoche was treating me the same way as his most advanced students.  He invited ten of us out of the 80 or so to come for teachings at his house each afternoon.  Several  leaders of the community also commented that I am an inspiring practitioner.  I thought, oh how kind Rinpoche is to give me these teachings even though I am not a good student, he knows I may not have much more opportunity to receive teachings so he’s giving them to me now even though I won’t be able to understand much.

After a few days, when Rinpoche was describing a very advanced practice whose name had mystified me for several years, I realized I’ve been doing that practice for quite some time and my mind operates differently as a result.  Then I recognized that I had in fact understood everything Rinpoche told us.  I stopped feeling like a fake at that point.

So when one of Rinpoche’s most advanced students by far, who also teaches for him, presented me with 12 extraordinarily precious pills, one to be taken on each full moon and therefore enough to last until next year, and when the very advanced practitioner who lives permanently at the retreat center and teaches there gave me a fragment of Boudha stupa to place on my shrine, I felt authentically blessed.  They would not flatter me.  What they observe must, to my surprise, be real.

Many students know much more doctrine, and many know much more detail of ritual practice, but that kind of mastery was never my goal.  I simply want to be less selfish and more aware so I can be more kind.  Rinpoche is very open about his own difficulties with practice and how they have changed, the amount of practice he does is huge, and the results in him are so inspiring.

I am in no doubt that I will die and if I had to bet, it would be that ALS will make my body uninhabitable within a year or so.  Noticing and responding to the changes really is interesting, and I’m curious to see what does in fact happen.  I’m very lucky to have always been curious.

I’d prefer to live longer and remain active, chiefly I just realized, so I can produce more benefit by overcoming selfishness.  So I’m most open to the Tibetan medicine helping me as much as it has the man being interviewed here who was treated by Dr.T’s mentor, Dr. Lobsang Dhondup:

I also feel blessed by your presence.

August 15 – From a family member

It must have been a pretty wild moment when they gave you the fragment of the Boudha stoupa, and realizing that Rinpoche had included you because he believes you’re at a different level than you thought, and not solely out of concern for your health and well being.  It’s amazing to me how far you’ve come in the last few years through your practice.

August 18 – From a friend

I’ve read all of this with great interest and done a bit more research online.  I can’t begin to understand much of it but it certainly falls into a very wide category of “why not try anything that might work.”    Anything that may mitigate your symptoms and increase your feeling of well-being is helpful.  I’m impressed with the degree of progress that you have made with your practice that has been recognized by your teachers.  The fact that you didn’t realize this until now seems to be part of the essence of Buddhism where the ego isn’t getting in the way and one quietly goes along incrementally improving and deepening.   It also seems like good preparation for dealing with how the disease might progress whether or not you gain any sort  of remission.

August 19 – My reply

It would be so much better if we could talk about all this.  We must make an opportunity!

It’s said that there are 84,000 different Buddhist practices because there are at least that many different kinds of people.  What I’ve observed is it’s really quite difficult for us Westerners to see the fundamental ideas accurately or how the practices work.

Doma and my friends  she’s spending the summer with in California were telling me by Skype about a seminar they attended recently where there were five speakers.  Two were Tibetan, the other three were famous American Buddhist teachers.  My friends were struck by the humility and lack of ego of the Tibetans vs the very strong egos of the Americans, who made things both too complicated to understand and sound too easy to do.

Doma was especially struck by one of the Americans saying, first feel compassion for yourself then, only when you have mastered that, begin to extend your compassion to others.  She said: “That’s exactly the opposite of what I was taught!”

My own experience is, first we need the humility to do what a teacher who resonates with us tells us to do.  We Westerners are trained to doubt, and we want things to be easy.  We want quick results.  But it doesn’t work like that.  Retraining ourselves is a slow process that’s only effective if we do enough work.  There are the 84,000 different kinds of work, so we have to find one that suits, and then we must actually do it, not for ten minutes every so often, but much longer.  Rinpoche told me to do two one-hour sessions every day and ten hours when Felicity is away.

A teacher really is necessary,  We can make a lot of progress on our own by reading, reflecting and sitting, but at some point we really need the guidance of someone who has done the work and can articulate what to do and what results to notice.  That’s very hard because our culture doesn’t have words for some aspects of existence and many words we do have suggest something different from the nearest equivalent Tibetan words.  Because we have a different understanding of words like “compassion”, understanding the Buddhist view of existence is not easy for us even though the view is quite simple.

And there’s an even more fundamental problem that’s illustrated by what Doma noticed.  The purpose of Buddhist practice is to develop undiscriminating compassion.

If that is not our primary purpose, which for many Western Buddhists it isn’t, we get caught up in things that aren’t helpful.  How to reconcile Buddhist metaphysics with Western science?  How to understand Buddhist metaphysics in perfect academic detail?  How to perform specific Buddhist ritual practices with perfect fidelity to all the music, hand gestures and whatnot?  How to gain the personal benefits of Buddhism with the least possible effort?  And so on and so on.

August 24, 2017 – To my Tibetan doctor

It’s impossible to be clear about what’s going on from day to day.  The weakness in my mouth and throat progressed very slowly for 6 or 8 months, then quite rapidly in the last 4 months or so.  I lost 25 pounds weight without really noticing as eating grew more difficult and I ate less as a result.  I’m now back to what I weighed in my early 20s, which would be okay except less of it is muscle.  But I’m paying attention now, regaining a little weight and hoping to resume moderate exercise soon.

That’s a long way round to saying that I’ve been more tired on the non-pill days than I was before starting the treatment but I don’t know why and I’m not worrying.  It could be that the ALS is spreading to my limbs but it doesn’t feel that way.  I’ll let you know what develops.

August 29 – To a family member

The founders of all spiritual practices teach us to be kind to one another and Buddhism does, as you say, focus especially on kindness.  The teacher at my first weekend of teachings, which were about how to perform a particular set of Tibetan Buddhist ritual practices, told us at the end: “Your friends might ask what you’ve been doing this weekend and you might wonder how to explain.”  He laughed.  “It’s simple.  Tell them you’ve been training to be more happy and more kind”.

What I’ve been focusing on over the last year or so is simple acceptance of what happens and responding to that with equanimity.  Very easy to say but it takes a lot of patient self-training to do.

We don’t accept things as they happen — we have emotions about them.  Some things we like, some things we don’t, others we ignore.  And then we get disappointed when things we like don’t last.  And we get angry or sad when things happen that we don’t like.  It seems natural because we always have done that, but we’re just making ourselves suffer.  Suffering doesn’t just happen to us.  Circumstances happen and we create suffering in response.

August 31 – To my Tibetan Doctor

I recognized yesterday that I can’t keep track of the details of what goes on by memory alone so I started a diary.

I had more difficulty than usual swallowing at breakfast on the days of the 4th and 5th precious pills, for example, but I was tired from all the driving and furniture moving and etc on the two days before the 4th, and I inadvertently had extremely spicy food the night before the 5th.  I was swallowing with no more difficulty than usual later in the day on both the 4th and 5th.

Without being able to look back over the detail I can’t correlate potential causes with observable effects.  That’s a long way round to saying that I don’t think there’s any improvement yet, it’s possible the bulbar palsy is slowly progressing but it may be stable, and I’m pretty sure nothing else is affected yet.

I’ve been feeling somewhat lethargic, which is most unusual for me, and I wonder if it’s related to my being careful to rest on the pill days, then not doing much on the other days just because I didn’t on those days.  I got the sense that I’d feel better if I resumed getting more physical exercise.  So I spent two or three hours yesterday and today cutting firewood, doing it by hand and taking it easy.  Too early to know the effect but I enjoyed doing it.

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.