Problems arising for pwME from additional diagnoses of MCAS, hEDS and POTS. Advocacy discussion.

[DHagen]

I'm not sure if you read my paragraph? I don't think our biggest problem is that lay people believe the pseudoscience. And, no, I don't think lay people should have to be experts in their own disease. I guess I was trying to push back against the 'lay person can't identify pseudoscience/medical professional can' dichotomy. The reality seems more complex than that.

I did read your paragraph. You wrote:

(And frankly, I agree that it's usually pretty easy to make a good guess about what is and isn't pseudoscience, for a bullshitometer to turn red, although being completely certain is a lot harder.)

In addition to the comments by @Jonathan Edwards, I was responding to your agreement that "it's usually pretty easy to make a good guess about what is and isn't pseudoscience."

While it can be easy to think a treatment sounds unlikely, the inability to be completely sure that something is pseudoscience is diabolical for people who are utterly desperate for some improvement. We often see on the forum that people thought some treatment sounded unlikely, but with their doctor, let alone the Mayo Clinic, promoting the therapy, and the desperation to be well, they gave it a go anyway.

I do not have a diagnosis of POTS or hEDS or MCAS, though I know many people who do. I do not know if these labels have any utility - I am able to appreciate that the labels are misleading or even counterproductive because they suggest a particular mechanism that may not be relevant to understanding the underlying pathology. That's fine. For people who are suffering, if these labels are done away with, they need to be replaced with something. I have not seen any viable alternatives suggested. That's something that can be worked on. That is terrible, but it may simply be where we're at.

I still take issue with the idea that it is easy to tell that these diagnoses are "imaginary," "pseudoscience," or "quackery." When confronted with how upset some people have gotten in this thread, the response has been that "no one is blaming patients." To me, stating that it is easy to tell that these concepts are "ridiculous" either puts blame on patients for accepting these diagnoses or implies that the only reason the patients cannot be blamed is because they are self-evidently too stupid to have figured it out.

That referring to hEDS patients as "the bendy people" who can be dismissed as full of shit on the basis of some instinct apportioned only to those privileged few who hail from Yorkshire should be understood as an expression of contempt should, I would hope, go without saying.

Looking over the information provided by the Cleveland Clinic (which I cite not because they hold some special authority or are even a worthwhile institution when it comes down to it, but because patients in the US will commonly be directed by their physicians to their resources) for Ehlers Danlos Syndrome and POTS, I am still not certain at what point it is meant to become obvious that this is "pseudoscience." Especially when compared with decsriptions of ME/CFS and the fact that that's the one I am supposed to accept as valid. Except for all of the bits that are also pseudoscience, which are easy to pick out.

 

[Hutan]

To me, stating that it is easy to tell that these concepts are "ridiculous" either puts blame on patients for accepting these diagnoses or implies that the only reason the patients cannot be blamed is because they are self-evidently too stupid to have figured it out.

I've thought a lot about what you have written.

I've searched this thread, and no one has used the word 'ridiculous' to describe the MCAS, hEDS and POTS diagnoses. I don't think anyone here would want to suggest that a patient believing in any of those diagnoses is stupid or they are to blame for accepting them.

For one thing, ideas can look plausible. Even if parts of an idea are in fact ridiculous (such as the originator of the idea having a background in tarot card reading or believing mast cells cause spontaneous combustion), some parts can remain plausible. As I've said, even though someone who has spent a lot of time looking at these ideas may be able to quickly see some of the problems with them, it can be hard to completely rule out all aspects of an idea.

There is nuance - someone really can have orthostatic tachycardia, someone can have food sensitivities, someone can be flexible. The problems, the uncertainties, come at the next step - so what does that mean about the cause of the disease and about the treatment? My joints are flexible, I could get a diagnosis of hEDS if I wanted one - but does the flexibility mean anything in the context of my ME/CFS? My son, who got ME/CFS at the same time as me, who has much the same symptoms, is not flexible.

Circumstances matter a lot. If someone we trust is telling us something, and especially if we want something to be true, it can be easy to believe it, no matter how clever we are. Wanting to be well in order to keep your job, your family, your plans, your identity is a strong reason to believe something.

It isn't reasonable to expect most patients to put a lot of time in to evaluating ideas related to their illness or to developing the knowledge needed to do the evaluation well. They have to decide who they trust. And that's where the patient charities and the commentators come in - we need them to be a lot more cautious about promoting ideas and treatments than they have been. They have to be worthy of the trust patients put in them

If someone is going to offer advice and influence others, in my view especially a clinician, they do need to put time into being sure about what they say. Unfortunately, when it comes to our illness, it's not enough to see that the person speaking comes from the Mayo Clinic or Harvard University, a lot more work has to be done.

For me, it's not about saying 'nothing about this idea can possibly be true and you are stupid to believe it'. It's more about looking at what we know and concluding that there isn't enough evidence for a responsible person to say about someone with the constellation of symptoms 'I know what is causing your disease and I know how to treat it'. As you say @DHagen,

I am able to appreciate that the labels are misleading or even counterproductive because they suggest a particular mechanism that may not be relevant to understanding the underlying pathology.

That is exactly the point. You've nailed it.

Looking over the information provided by the Cleveland Clinic (which I cite not because they hold some special authority or are even a worthwhile institution when it comes down to it, but because patients in the US will commonly be directed by their physicians to their resources) for Ehlers Danlos Syndrome and POTS, I am still not certain at what point it is meant to become obvious that this is "pseudoscience." Especially when compared with decsriptions of ME/CFS and the fact that that's the one I am supposed to accept as valid. Except for all of the bits that are also pseudoscience, which are easy to pick out.

Yeah... But the difference is that ME/CFS is a list of symptoms. Its validity should be in the lack of claims about causation and, currently, about effective treatment. I think the main pseudoscience of hEDS (importantly not EDS which are proven genetic diseases) and POTS is in the certainty of causal mechanisms and in the creation of disease entities out of symptoms that have many causes.

That Cleveland Clinic muddles up EDS with hEDS. It suggests that all 'hEDS' is caused by genetic mutations affecting collagen, but that just isn't true. Someone can have particular loose joints for many reasons. It makes no sense to talk about someone who is quite flexible and has some other symptoms that may but probably are not related to the flexibility and someone who has a specific life-threatening genetic mutation in the same article. I expect hEDS is quite a money-spinner for the clinic.

The Cleveland Clinic suggests that it can get people on the 'path of recovery' from POTS. Then they say there is no cure but offer treatments including talk therapy, biofeedback, exercise and nutrition guidance including increased salt intake. They say 'we work with you to create a treatment program that works for you'. They say 'Studies show that reclined aerobic exercise, such as swimming, rowing and recumbent bicycling, has the best results. Strengthening your core and leg muscles is also helpful.'

 

[DHagen]

Thank you for taking the time to respond at such length, @Hutan.

I've searched this thread, and no one has used the word 'ridiculous' to describe the MCAS, hEDS and POTS diagnoses.

This is true, and the scare quotes that I used muddied the waters in this regard. Other pejorative terms have been employed, however. In my mind, "woo" implies a degree of ridiculousness in as much as it designates something deserving of ridicule.

I don't think anyone here would want to suggest that a patient believing in any of those diagnoses is stupid or they are to blame for accepting them.

This, or something very similar, has been stated multiple times. To me, this claim rings hollow when it is followed up with statements indicating that it is easy to identify these concepts as being "imaginary," "pseudoscience," or "quack medicine" (all terms that have been applied to the diagnoses under discussion). That a repeated inability to correct make an easy identification equates to stupidity was my inference.

I also object to the usage of demeaning language with reference to patients.

I agree with a great deal of what you've written. As I indicated, I have come to appreciate the problems with these terms and with the way that they are used - that's thanks to the discussions here on S4ME. I am not arguing against the criticism you voice here (and I am certainly not defending the Cleveland Clinic). I referenced the Cleveland Clinic's documents because, without any additional research, I found nothing there that I would expect a lay person to identify as pseudoscience at first brush, and thus would neither blame them for accepting the diagnosis nor with failing to have spotted an obvious flaw. The argument is not that there are no flaws, merely that the flaws would not be obvious to someone newly diagnosed. They were not easy to spot for me. Maybe that's my failing.

My larger purpose in writing was to again call attention to what I, and I think others, perceive as a degree of contempt and disdain for the patients (here thinking particularly of patients with a diagnosis of hEDS, MCAS, or POTS without having also received a ME/CFS diagnosis); contempt and disdain which continue to manifest despite all of the professions of good intentions. This came up at the start of this thread, it was hashed out, and then nothing much changed.

Maybe I am being over sensitive. I can say that, whatever the intention, the people that I have tried to convince to read the discussions on this forum have come away feeling hurt and attacked, to the extent that it has changed the way that they interact with me. At the same time, I recognize that this is a long standing community, and asking people to constantly police their language in ways to which they are unaccustomed and to constantly reiterate positions that they have expressed multiple times over the years is not going to be especially conducive to discussion amongst the long-standing members.

I suppose the ground has been covered and seems unlikely I've added much value here, so I'll stop. Thanks again for responding.

 

[Hutan]

seems unlikely I've added much value here,

To the contrary, thanks for writing. I guess reducing the use of words like quackery and pseudoscience would help when communicating about these terms, but, I'm not sure it would be enough. I suspect that raising questions about the validity of the labels is at the core of the problem.

I'm really sorry that your suggestion of the forum to people you know has negatively affected your relationship with them.

 

[Jonathan Edwards]

Excuse me? We're now mocking people diagnosed with hEDS as "bendy people?"

I realise that there may be major cultural differences here and that being old and tired I am sometimes too informal with words but no, I am not mocking anybody diagnosed with anything. And I actually think it is reasonable for me to expect people to se that and not to accuse me of being prejudiced, unsympathetic or antagonistic.

By bendy people I was referring, as the context suggested, to medics who sell 'hEDS' rather than 'BPS'. I have in mind a young female psychiatrist who I think uses the email name 'bendybrain' and seems to believe that there is some mind-body link between hEDS, fatigue and mental state. In other words mixing the pseudoscience from both ends.

 

[Jonathan Edwards]

I do not have a diagnosis of POTS or hEDS or MCAS, though I know many people who do. I do not know if these labels have any utility - I am able to appreciate that the labels are misleading or even counterproductive because they suggest a particular mechanism that may not be relevant to understanding the underlying pathology. That's fine. For people who are suffering, if these labels are done away with, they need to be replaced with something. I have not seen any viable alternatives suggested.

Being hypermobile to the extent measured on the Beighton score used to diagnose hEDS is very common and normal. Something like 40% of young women will qualify at some estimates. There is no reason to think it has anything to do with any pain or fatigue. Large studies of young people show little or no relation between hypermobility and symptoms.

People who get diagnosed as hEDS presumably have pain or fatigue for another reason. The job of a doctor is to give them the most well-informed opinion they can of what that other reason is, rather than give them a name that explains nothing. Sometimes doctors don't know - and for ME/CFS and similar patterns of chronic symptoms we don't really know anything much, except that a lot of people suffer and there are patterns to the way they do. The more that paeople insist of having a diagnosis and an explanation the more they will find doctors prepared to sell them made up stuff.

 

[Jonathan Edwards]

I think maybe there is a big cultural shift going on, related to what I was saying about the change in medicine, particularly in the USA. Fifty years ago the Mayo Clinic could be relied on to provide rigorous science-based medical information. People had the idea that medicine was a profession devoted to the common good and people's health and which strove for high quality and honesty.

In those days that was true. I was humbled by reading US textbooks for my higher degree in medicine, seeing how much more rigorous they were than what I had been taught in the UK.

But that has changed out of all recognition. Medicine in the US is now a largely unregulated business venture where clients are there to be made money out of just like selling cosmetics or video games. The public probably have not appreciated what has happened. So, now, information from the Mayo Clinic may as well be an advert on the TV for dog vitamins.

In other words the public's bullshittometers need to be drastically recalibrated. To the extent that you assume that information is made up stuff unless you can detect a reason to think otherwise.

Why has this happened? I guess it is part of the progressive commercialisation of everything. But I also think it has quite a lot to do with patterns of social mobility in the mid twentieth century. In the 1970s mdeicine was populated by a lot of rather earnest, highly intelligent people with a sense of vocation, many of whom either came from rural or uneducated family backgrounds, having got scholarships to university, or had migrated to the UK and US in the face of fascism in continental Europe. Some were remembered in a book called 'Hitler's Gift'. I think that sort of mobility has changed. There are people from ethnic minorities, often women, going in to medicine maybe for rather similar reasons now but intelligent young people are mostly channelled into finance. Science and medicine are no longer considered good careers for bright kids.

The tide needs to turn back it seems to me, but it seems ever more popular to vote for cutting public spending on health. Maybe all one can do is to say 'caveat emptor'.

 

[Hutan]

I've been reading a couple of genetic studies on hEDS.

Spoiler: my summary of the results from the two studies

In one, they found two families with a particular mutation in a kallikrien gene that was associated with some connective tissue pathology. In those cases, the disease could probably be described as a new form of EDS.

They also found some rare variations in the kallikrien genes at higher rates than the general population in a sample of a couple of hundred people with a hEDS diagnosis. About 30% of the sample had one or more rare variations versus about 12% in the general population. All sorts of things could be happening there. For example, each variant could be contributing to increased flexibility and some other symptoms, or contributing to increased flexibility only with the other symptoms people have completely unrelated to the variant. Or the heterozygous variant might be having no effect at all.

In a fairly small GWAS of a hEDS sample, there was overlap with genetics findings for joint mobility, which isn't surprising. But, the strongest overlap was actually with ME/CFS, suggesting that at least some of the sample might just be people with ME/CFS who happen to be flexible and went to a doctor who likes to diagnose hEDS.

The GWAS found a couple of locations enriched in the sample, but it's hard to know what to make of them, especially given the small size of the study. Of course the variations only occurred in a percentage of the participants, probably a small percentage.

Clearly, things are complicated. I think there is almost certainly a small percentage of people diagnosed with hEDS who have a monogenic disease that would fit under an existing or new EDS group. There are probably some people with a polygenic reason for their pathology that does include connective tissue problems. And I think there is a substantial group of people with a range of symptoms including some who could be diagnosed with ME/CFS, where the flexibility is probably irrelevant.

So, I don't think someone who believes they have hEDS is stupid. They may in fact even be essentially correct. Certainly the families with multi-generational connective tissue issues have a strong reason to think they have a genetic pathology underlying their symptoms.

But I think the category of hEDS is being used to label people with a whole range of issues and to suggest that the same sort of pathology accounts for all of their symptoms. One hypermobiliy clinic webpage I saw actually says

These conditions often can masquerade as other conditions and can be difficult to accurately diagnose. The catchphrase, "If you can't connect the issues, think connective tissue" very much applies to these syndromes.

The label also leads people to assume things about their risks for future health events and the treatments they need that probably have little evidence to support them.