Who said: don't bother testing patients?

Another way the stigma continues, is pwME are so wary of mentioning symptoms, and facts of this illnesss that we may keep shtum about ME. We miss educating about it because we don't want to weather the crap and abuse that may be sent our way.

 

Oh my god, @JaimeS ! That is grim and disgusting! In some ways we have come a long way, and in others not!

 

I also think there is another subtlety amongst all this. When you look at all the other medical conditions that can lead to extreme fatigue, exhaustion, etc, I think I'm right in saying that these symptoms are invariably secondary, they are never the illness itself. So I think people are conditioned to expect there to always be some other primary condition to point at, that explains the extreme fatigue. When there isn't one to see, people default to assuming the person is pulling a fast one. The fact that extreme fatigue, PEM, etc are the primary symptoms of an otherwise invisible disease, is something people are not well geared up to sensibly interpret; we are conditioned to presume the person is making excuses, unless we can see "what is really causing" the person to be that way.

 

There's a snopes.com article that talks about the photo that @JaimeS posted: https://www.snopes.com/fact-check/1930s-housewife-smile-therapy/

The article is long and kind hard for me to follow (brain not doing well today). From what I could tell it's not clear where the photo is from. But the idea of that it's a photo from some kind of "smile therapy" appears to be false.

Either way, it is definitely a creepy photo.

 

It's been hypothesized that damage to basal ganglia is what causes 'central fatigue'. But even then, this is still secondary. What's damaged the basal ganglia?

 

Here's probably the most relevant passage:

 

I've been ill for fifty years now and I am still hard pushed to describe my symptoms. Every time I put something into words it doesn't come out right. We latch onto words like fatigue or PEM but the length of posts discussing exactly what they mean shows how inaccurate they are.

I am waiting for the day I can simply say we have an autoimmune disease that attacks pyruvate dehydrogenase or whatever where the listener will nod sagely because it is too difficult to understand.

To say we just stop for a bit is impossible for people to comprehend because it is not something they experience. They latch onto words like fatigue and think they know what it means and that what helps them will help us.

HIV attacks the part of the body which protects against disease so the patient gets lots of strange diseases - who would have predicted a disease like that? We have a disease that attacks the energy producing system, but now we know that viruses will happily evolve to use any part of their host so that central premise should not be beyond comprehension and the people who claim to treat us should not be falling back on banal explanations.

 

And how varied. A few months ago at #MEAction, I said "we all know the definition of PEM, it's just really not well-described in the literature" and Jen was like, "you think so? What's PEM?" and I described my sepsis-like, shock-like experience.

She said, "that's not PEM to me".

This bowled me over so I asked four or five other pwME.

As a writer -- and as someone trying to 'pin it down' -- I went into great detail. No one had my same experience. If we were all to describe it phenomenologically -- e.g. what is going on in the body right now -- we would report different experiences.

We all experience/describe PEM sufficiently differently that I now have to say that the definition is "a notable and prolonged worsening of symptoms and/or the appearance of new symptoms post-exertionally".

Which is actually how clinicians who've seen the disease often and in many, many different people have ended up describing it.

 

This is one of my favorite sentences I've read in awhile @Mithriel , because it takes such an unexpected swerve at the end with "...because it is too difficult to understand."

 

Sorry I'm addressing every bit individually, but this is a very cool post and I had separate points to make about each bit.

This is really smart and you're exactly right. It's that there are no words for our experience so we fall back on these ones that are kind of, sort of, not quite, but maybe right. And the use of these words for common experiences makes people feel like a) they understand the symptom and b) they understand how to make the symptom stop. That's a really interesting observation.

 

[

I think that knowledge and understanding of the condition has been set back by the simplistic notion of PEM which developed. I recognise in myself two sorts of PEM in addition to a relapsing/remitting element which seems unrelated to exertion, or lack of it. These may occur in different combinations, and with differing degrees of severity and duration. It is doubtful whether a questionnaire could be developed to tease this out.

It probably took about 20 years to untangle this. I suspect that many patients will only recognise what goes on when they no longer have meaningful contact with doctors.

 

My daughter was found to have antibodies to the Pyruvate Kinase receptors in the Basal Ganglia.

She was tested for these because she had a high ASO (Anti Streptolysin O) titre. She has no motor tics, just significant impairment in cognitive function.

 

OMW, fascinating @MEMarge . I also found a paper implying that dopaminergic receptor dysfunction, particularly D2 receptor, can lead to downstream damage of basal ganglia.

I have dopamine receptor (D2) dysfunction. Genetic.

https://t.co/XKRE6eMGin

 

Ohhhhh here we go.

It was Rob Phair who first asked me this when I worked at Stanford. And I that I didn't think of this myself.

He said, "You've told me you can get a crash from cognitive effort. And from physical exercise. Do these differ at all?"

"LOL no," I replied, and then actually checked in with myself the next time each happened.

YES, darn it, they're different. And a sensory crash is something else altogether. (Don't worry, I reported back.)

Again, just my experience, but really interesting to see you say you have two crash-types, @chrisb. I don't think it's just contact with others that manipulate our thinking on this. It's also that we're cognitively compromised when it's actually happening. I don't know about you, but brain fog means that days when I'm truly sick all blend together. When I had really severe crashes, I lost time, like... thought it was Monday when it was Thursday. We're not exactly at our sharpest when this is going on!

I'm going to try and qualitatively describe each.

Sensory crash:
headache that built into migraine, sensory sensitivity ratcheted up to 11 until I was holding my hand over my eyes and flinching when someone closed a door anywhere in the house. And yet that's all I can recall of my last one because it was so bad. Less compromised physically: I was able to leave the house and walk around elsewhere even in the middle of the crash, even though I felt distinctly wobbly and desperately wanted to lie down, I had to go someplace to escape the sound. (There was a party at the house.) Getting up and leaving would not be possible with a physical crash of an equivalent awfulness.

Cognitive crash:
vision goes blurry & unable to focus. Word finding issues. When typing, switch words or letters or become unable to spell a simple word for several seconds until the cognitive circuits line up properly. More of a feeling of physical exhaustion than a sensory crash; but there's headache too. Eyes go weirdly dry and actually hurt a little.

Physical crash:
the worst. Legs and arms go numb, useless, and/or tingly. Feel weighted down as though wearing an X-ray smock. Brain fog, GI issues (gut stops digesting ANYTHING)

It's almost like the processes you used is what is most impaired.

Which makes total sense.

I guess it's still sensible we call it the same thing -- given that it's overexertion leading to worsened symptoms. And given that it occurs no matter what kind of process we try and engage in. There are commonalities for me: in each, my ANS dysfunction worsens (temp control, OI issues, dizziness) and I always have pain where my spine joins my skull. But note that the difference in symptoms could have the implication of different palliative 'fixes'.

And that's interesting -- and not something I've ever seen discussed.

I hope no one ever splits this thread. Even though we've changed topic, the context of where we started feels important to the conversation.

 

Actually, reading the above and thinking some more.

There are going to be a few big "ifs" in this statement, so bear with me.

IF I'm right and there are definitive differences in 'kinds of crashes'.

IF I'm right in that those symptoms directly relate to 'which part' of yourself you overexerted.

THEN this is definitively a disease of the CNS; or at the very least, PEM is a result of overloading the CNS. NOT merely a result of generalized metabolic dysfunction.

Hear me out.

Cognitive dysfunction and sensory dysfunction having cognitive and sensory sequelae at the very least strongly implies that it's those parts of the brain that help us carry out these activities are what's experiencing some overload or exhaustion.

And... it implies the fatigue is truly central -- that is, brain fatigue and not muscle fatigue at all. I mean, if you're severe, you could be deconditioned, but that would be on top of everything else.

It totally explains the relapsing remitting nature -- how you can go from moving around one day to entirely unable to sit up the next.

Please poke holes in this. I welcome it.

 

I can only speak from my own experience.

I agree there's absolutely a "brain fatiguability" componenent, but there's also a muscle component in the sense that they burn and exhaust very fast, independently from the brain. That is, the muscle itself; which feels like a metabolic thing.

If I exhaust from the "brain"/CNS, that leads also to muscle problems (like problems with control, "weakness", paralysis at times), i.e. the muscle itself could do it, but the control via nerves is "exhausted"; but it also includes symptoms after activity that I would call CNS (including ANS), like immense tiredness, headaches, sponge brain, OI, pain (especially from neuropathy then), other cognitive stuff and so on.

 

Very interesting .

I will discuss this with my daughter, but would agree from standing on sidelines that cognitive overexertion seems to have a different manifestation - subtle but different.

We are finding that pain is ramped up at certain times of monthly cycle, so PEM then is more acute , so perhaps this is a wider issue for some too?

 

Yes my daughter feels worse just before and during periods and they are somewhat erratic. They are more frequent ans sometimes longer.

 

Yes to increased pain/ symptoms before and during. Reported as feeling " ill" or as if about to come down with something.

Periods have been erratic and of differing length, but seem to have settled to within a week or so of previous cycle lengths- that said it could change again!