[Mark S]

<p style=”text-align: left;”>I had long consult at the neuro-muscular clinic and Cleveland Clinic yesterday.</p>
The first thing the resident said was “wow, you have a chart.” I said yes it is a mess and we talked extensively about it.

Then, he gave the most organized and complete Neuro exam I have ever had.

Surprise! It confirmed everything I have been telling (and being ignored) the neuro-muscular specialists at UPMC. The thing about physical exams is you have to actually do them, not cut and paste notes from other exams that were not done.

The previous EMGs did not test areas of primary weakness, so yet another complete study, the one that was ordered 22 months ago and never done.

I get so stressed for doctor’s appointments, especially new Drs because you have no idea if they are going to do their job and if they don’t, you really have little recourse at the time.

While the progression/outcome would have been the same, I really would have liked to live aspects of the last two years differently.

 

 

 

 

[Mark S]

The exceptions often prove the rule itself needs to be reconsidered.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8151104/

[Mark S]

IMO it is so hard to diagnose because modern medicine is nearly 100% dependant on technology.

Doctors are losing the ability to do physical exams and don’t take the needed time to do histories.

Research shows they are not great at testing reflexes if they even test them at all.

In all my exams only one Dr ever tested the jaw jerk reflex, only a few Hoffman.

Quick “don’t let me pull you” only check a single initial muscle activation, and not relative or sensitive to say 2 months ago.  3/5 bicep is observable without testing and 8/10 when you don’t know what 10 was is impossible.

Also language.  Numb means you can’t feel a pin prick or vibration. However ALS creates a weird feeling in muscles that feels “numb”ish. There is also pain and sensory abnormalities.

Then EMG, read the literature, it’s not great and has to be guided by the initial exam.  My super EMG Drs didn’t even do the needle placements my neurologist ordered.

Lastly, there is a major incentive to avoid a false positive.  So they default to letting patients decline where no tests are even needed, because a 1st year could see it.

I don’t like being so cynical, but ALS dx requires both skilled primary care and general neurology.  Two specialty care areas that are becoming harder to obtain.

What should happen.

Quality Neuro exam. If UMN signs are observable, scans and blood work to rule out list. If everything ruled out, honestly say, we don’t know but symptoms are consistent with early MND and we should see each other every 3-4 weeks to see if progressive.

Anyway, my point is we could do much better with what we have until there are biomarkers.

 

 

 

[Mark S]

I am going to assume after 20 months, Drs have ruled out stroke, tumor, Lyme, viral syndromes….

And the question is it non-progressive bulbar palsy or bulbar onset ALS.

Are those  correct assumptions?

Unfortunately ALS is known by time and exclusion.  Hang in there but don’t just wait and see until after every thing is ruled out.

It took me over 2 years to rule it all out.

 

[Mark S]

Yes the tasty stuff and x-ray is the same.

I suspect a large survey would show the same thing, no PT and SLP dropouts.

Here is a video, don’t watch, just listen to it, don’t even try, just listen, if your tongue is fasciculating, just feel it against teeth.

I don’t know your range and ease, but allow you tongue to rest in a spot and feel your breath pass over and any open and closing of throat.  It’s hard, only a few minutes to start a few times a day.

You try to increase time and focus not effort.

https://youtu.be/LAIFC8CrYCk

[Mark S]

In my continued efforts to see what support people are receiving, has anyone been introduced to exercises of the sphincter muscles. I know the line of “these are not effected” but I had swallowing test under fluoroscopy which showed weakness, esophagal and Pharyngeal. If my pelvic floor muscles were tested, they would show weakness as well.

 

I had major lung problems last winter and best guess is I aspirated because of reflux. I was treated aggressively with steroids and did get better. I treat the reflex but also do exercises.

As I am developing a program for myself, which I will share, it would help to know if this is completely foreign of if your PT and SLP showed you exercises that help.

 

 

[Mark S]

Jim gave an excellent professional legal view.

When I have helped the dying, I asked questions and listened.  What do you think it will be like? What is your view of afterlife? What do you need? Who do you need to be with? Any people you need to reconnect or reconcile with? What can we do for you so you are supported?

I know what I would think I want, but that could change in a second.

In having these discussions with many patients, families and professionals, I absolutely could have never predicted the endless range of needs and beliefs.

The commonality is no one wants to die in a hospital. Yet, 60% do and another 20% in LTC facilities.

I know the focus is on medical breakthroughs, but the absolute most important moments in my life were with the dying. Some in months leading up to and some where I held their hand at the last moments. We need to be so much better in this regard.

 

 

 

[Mark S]

Amanda asked this in April and 4 months later there is a wave of media stories.

I want to watch the Ady Barkan documentary and the stories of Chris Snow are inspiring to me.

There is also a documentary about Selma Blair and her life with MS.

How do the celebrity spokes patients make you feel? I have very strong and mixed emotions about what is becoming a bit of a distinct genre.

 

[Mark S]

Lisa,

June 5th was too long to wait. Talk to the doctor who ordered it, but I would ask to get a referral to a Pulmonologist (assuming that it was ordered by someone else)

 

 

[Mark S]

Thanks for the encouragement.

There is such a disconnect between from a pure neuro-physiological frame work of what “should happen” and what is clearly happening.

I try to look at myself as a person I am treating. What would I do if I walked in?

For example over last week, with my left hand palm down I can’t  spread my fingers. If I really try for a while. I can move them a little and they shake.

If I turn my palm up I can do it. This makes no sense from the nerves in arm/hand perspective. The start position shouldn’t matter. But it does.  So I create all sorts of novel exercises to see if I can get the function back.

The first thing I would do is ask person how they experience a loss of function. For me it’s like a very specific data file was deleted.  So I try to write out a new program.

If anyone wants to write out, as the remember, how would you describe the “feeling” of physical loss, it would be very helpful to my understanding.

 

[Mark S]

Dagmar,

I was looking for something else and ran across this.

One of the things that I practiced at University of Pittsburgh Medical Center was the Feldenkrais Method.

I am going to “flex” while I still can and boast. I was one of the best practitioners ever. The last treatment I gave was a couple of years ago, I had to quit when my hands couldn’t work the way they needed to. It is how I knew I was going down this path long before anyone could see.

When my kids get back to school, if I have the energy I’m  going to make a website with writings and videos so I can try to help myself and others in a different way.  I have been teaching over 25 years, and will continue as long as I can.

Mark

[Mark S]

John, thank you, I can’t tell you how helpful this is.  About a year after first EMG, I finally got appointment with amazing Neurologist, (he moved and semi retired that month) that was certain. But the EMG was done by top MND specialist.  At the end he said coldly “it’s not ALS maybe LEMS” and walked out and wouldn’t even answer any questions.

So it became a telephone game where that one test was the “truth.” I was recently hospitalized and one neuro Dr. Said, “Dr L said you don’t have ALS and if you did you would be in a wheelchair or dead by now.”

So now I have a new Dr.

I worked in healthcare all my life, mostly in Neuro and oncology, so I am knowledgeable and can digest Reserach.  I have only known one person with ALS. Everyone thought his illness was his diabetes, until another PT friend got him to see Dr L.

Pete died last year after 18 months of rapid decline.  In my research over the last two years, it seems that the slow onset, the 10% or so that live over 10 years have a very difficult time with early care.

No matter what, I always keep telling myself, one thing at a time, but it is psychologically difficult.

Mark

 

 

[Mark S]

I am in this situation, so hesitant to post, but I have reached the 95% confidence level.

Briefly, my first neurology visits were 2 1/2 years ago and first signs of weakness 3 1/2 years. I realize this would be a long duration onset.

I definitely present clinically with ALS and everything has be “ruled out” (including ALS until my next visit where the testing starts over again) but in a few weeks I have my 4th EMG/NCS because my symptoms continue their progression.

So I am not asking for an opinion, because I have top notch specialists, and they don’t have answers either, however, I was wondering if anyone has so many EMGs before 100% dx.

Thanks, Mark

 

 

 

 

 

 

[Mark S]

Carol, I was concerned about that because I have dangerous reflux and esophagial weakness but there must be something in the processing that makes it unlike a standard herb/spice.

 

[Mark S]

I have been taking Theracurmin.

It without a doubt changed GI function but I don’t sense any direct neuro-muscular effects.

I will keep taking it (less frequently) because any improvement is good.

 

[Mark S]

Until established test it doesn’t exist and even then…

It took me well over a year to get the full MG blood panel. My mother had MG and it should have been fully tested on the first visit when I complained of jaw weakness when eating.

Ordering tests is point and click.

 

[Mark S]

As I mentioned in another post, acupuncture and energy medicine doesn’t and really can’t conform to western diagnosis.

If you go a skilled practitioner, they will feel your energy/pulses.  You feel where there is too much, too little, out of balance and treat to bring into balance.

You assume that will relieve symptoms improve function.

IMO after working with acupuncturists and having a lot of “energy” based therapy, if your interested find qualified people and sense who you trust and try it.

The biggest red flags are people that are obviously anti science, selling all sorts of stuff, make any promises.

When I treated people, the approach I always used was, “I don’t know, but will try my best.”

My best friend came to visit and treated me for a few days.  I am more alive, breathing and walking better.

I don’t know how long it will last, but the treatment effect should be noticable and in one or two treatments.

If not try another, maybe another after that.

[Mark S]

Note to moderators.

I know trying to help people over forums is problematic, sorry if inappropriate. I can’t imagine thinking ALS when it was an unfortunate complication.

Cranial nerve palsies are a rare complication of spinal anesthesia. Most of the time they resolve by themselves or with interventions.

However, sometimes they don’t.

As always not medical advice, please talk to doctors. I would also see a Physiatrist and be very clear about onset of issues and ask if they have any suggestions about symptom management.

https://pubmed.ncbi.nlm.nih.gov/28343842/

[Mark S]

Trevor,

Your path is very similar to mine minus the genetic testing.

I think with greater awareness comes more people and an greater understanding of the chaotic nature of the disease. There is this history of an adherence to a rigid linear path and presentation.

I remember early in my career I treated a desperate woman that was diagnosed with Limb Girdle Muscular Dystrophy. She was unable to stand at all.  I was 27 and had no idea what I was doing with her. Looking back, she had no atrophy and I could get her to engage muscles of legs. I really thought I was going to get her walking.  It didn’t go well.

She like so many others I saw in Integrated Medicine Clinics had Non-typical presentations of whatever they had. (I made the point I couldn’t legally dx anything). 90% of people in clinic were women and another large cohort were dx’d with depression and while Drs didn’t put it on chart, conversion disorder. I read my notes and I see the code words and boilerplate.

Anyone that spends more than a few months in dx hell will have more than a few interactions when you know the Dr is thinking “they crazy”. Trust me that is a technical term they use when you are not in room.

When they let me in the physicians cafeteria and treat in hospital, I saw behind the doors and it isn’t great. I don’t like complaining about Drs, because we need them, but we need them to not be so certain all the time ever after so many of their “certainties” prove to be wrong.

 

 

 

 

 

 

disorder.

 

 

[Mark S]

Bill, sorry about the falls.

There are good PTs that are skilled with balance and fall prevention. Most of them work in-patient Neuro rehab units.  While it is a little “off label” many hospitals have vestibular rehab programs.  I would also seek out PTs that work with Parkinson’s patients.

 

[Mark S]

Bill,
<p style=”text-align: center;”>I have generally had the progressive week after week weakness and then just in the last two weeks my fingers just stopped.</p>
I am testing strength every day or so and don’t have great expectations of improvement, but I do know with me repetitive movements with resistance are not helpful.

I recently had a Neuro PT eval and we decided that it wouldn’t be advisable because the exercise intolerance is too great.

I have seen people riding stationary bikes, etc and that would be impossible for me and yet their motor functions are far more impaired than mine.

PT over the last decades has been largely absorbed into sports med with strength and endurance training.  It is hard to find trained manual therapists that teach movement efficiency and mobility.

 

 

 

[Mark S]

I wrote Dr B over 2 years ago and he responded personally and I intend to try to connect with him.

I worked professionally with a number of acupuncturists and the important thing to know is there are many different styles and nuances and with all physical intervention it is VERY dependant on provider.

It is what makes research so difficult. We would create research protocols for an illness/problem that were repeated every time.  One time I volunteered to be patient while we were developing protocol for needle placement. It felt horrible because it was wrong for me and really turned me off acupuncture research.

Thats not how it should work. Good treatment is in the moment and may be similar over multiple visits, it is seldom the exact same and never across multiple people.

Good luck with it.

[Mark S]

In general you are correct about the movement being based on functional intentions.

However, because of extreme training over the course of 35 years, I developed refined ability to activate/effect specific muscles or physiological functions.

When I was 16 I was in a biofeedback study.  I could control things like surface temp, heart rate, reduce muscle activity in any part of body. Much later I used EEG feedback.

I also have spent decades moving joints one by one every day though gentle range of motion movements.

My daily practice is how I knew I had a problem and how I manage.

If it was up to me there would be an intense program in the first month post diagnosis. Most people have never given any time to understanding their physiological function.

I have great insurance, so last December when I had a sudden voice issue, I referred myself to a voice clinic and got speech therapy before I really needed it and it helped a lot and now I have a relationship with clinic.  The SLP taught me things I had not considered and they caught my swallowing issues so I am much more careful and work on the issues.

One of my goals is to create a diagnosis survival guide. Learning breathing, relaxation, gait training, transfers, vocalization, swallowing skills right after dx would be extremely helpful and empowering.

[Mark S]

I had my NCS/EMG today.

I like my new Neurologist. He is clearly skilled and compassionate.

This one felt different, it wasn’t as intense, I don’t know what that means but I do know I couldn’t activate the muscles as I could 18 months ago.  After my leg was needled, it visibly fasciculated for 5 minutes.

Now I have to wait 3 weeks for my appointment to discuss the results.  It is so slow.