EDS and ME - is there a connection?

Moderator note:
This thread has been split from
https://www.s4me.info/threads/feedback-from-stakeholder-engagement-workshop-for-the-nice-guidelines-on-me-jan-2018.1964/

I think I may be a knowledgeable specialist in this field since I did the initial study of mitral valve prolapse in hypermobility syndrome (now EDS3) with Rodney Grahame in around 1978. Rodney and I did a hypermobility clinic. Rodney became convinced that hypermobility caused pain. But since patients were only referred to the clinic if they had pain I could not see how we could deduce that. In forty years I have never seen any reliable evidence that EDS3 cases pain beyond dislocations in a very small subgroup.

So I can be a primary source for Rodney's belief. He has talked about nothing else much for twenty years. But in all the time I have seen him give presentations I have never seen a scrap of evidence.

There is no confusion about the difference between ME and EDS. They are completely different concepts. The vast majority of people with 'EDS3' are completely unaware of any problem. What needs clearing up is whether there is in fact any association with fatigue and pain at all. I don;t think Rodney can ever have had any information on this because all his observations were on people who had come to him because they had pain. He never saw the people with EDS3 without pain.

I really think this is the sort of stuff that should be kept away from the NICE guidelines. It really has no relevance to the management of ME and there is no science behind it.

 

Yes they are different.
Some patients are not having comorbidities like EDS addressed.
Where there is confusion it isn't originating from patients. Thanks for your reply re: Rodney Grahame it was illuminating.

 

I agree, it is originating from physicians who do not know anything much about heritable connective tissue disorders or biomechanics confusing the patients.

 

Jo, I suggest a conundrum here! NICE need to catch up with "patient experience" even if the "science" at present is not there. They might actually learn something and be able to effect some positive change.

People you have "close to you", I suggest may be part of the problem. They are establishment who probably haven't looked, therefore will not find. This will account for limited research papers though Julia Newton's work may help. ( though Julia admits that ME patients may not tolerate the standard tilt table test for PoTS). I recommend that you speak with the most excellent GP on my table (Group 6) Emma Reinhold about this issue -overlapping/comorbid conditions, such as POTS and EDS- which you admit you are unclear about.
Emma spoke very knowledgeably and powerfully about the PoTS - ME situation, and her personal experience and treatment at the hands of the medical profession (consultant level included). She stated, (and I hope she will not mind me quoting her here) -that she was embarrassed and ashamed of her profession on this issue.

She attended on behalf of POTs UK. http://www.potsuk.org/ She is on this forum ( S4ME) and has been tasked with writing guidance document on PoTS etc for GPs for the Royal College of General Practitioners.
https://www.researchgate.net/profile/Emma_Reinhold2
Emma Reinhold currently works at Royal College of General Practitioners. Emma does research in Psychosomatic Medicine, Rheumatology and General Practice. Their current project is 'Ehlers-Danlos and the Collagen Disorders.'
Current Institution
Royal College of General Practitioners | RCGP
London
Current position
Freelance GP



http://www.potsuk.org/gp_guide says;
WHY IS THIS IMPORTANT?

• Diagnosis of PoTS is commonly delayed by several years.
• PoTS causes considerable disability - patients can become wheelchair or bed bound. They are often unable to continue education or employment (25%)
• Treatments are available and 90% of patients will respond.
• Patients frequently receive psychiatric labels prior to correct diagnosis. Tools used to assess anxiety commonly rely on somatic anxiety symptoms (which may reflect high upright catecholamine levels). Using the Anxiety Sensitive Index (a cognitive-based measure of anxiety), PoTS patients tend to have lower anxiety levels than the general population.

 

Sorry @Suffolkres, but that is not the road to go down. We need evidence. We need NICE to stick to evidence - or they can carry on as they wish. I do not think there is any good evidence for EDSIII being a significant source of symptoms in patients with ME. It is not even clear to me that EDSIII is a useful category. EDS (all groups) that causes significant clinical problems is rare. I never head a single case present to me in my 40 years in medicine, although I was aware of a few cases seen by others or who passed through my clinic by chance. Or to put it another way I never had patients with pain that could not be explained by specific joint, muscle or nerve problems and that was more plausibly explained by connective tissue laxity.

Remember that I started my career working in an 'EDSIII' clinic. I have specialist experience in this condition. Even in that clinic I did not see any particular reason to put symptoms down to the joint laxity.

I know Emma through email correspondence. I understand she is interested in EDS but I am not aware that she has any new information.

Patient experience is of no use here. A patient may have pain or fatigue and may have hypermobile joints but they have no way of knowing that one is due to the other. That can only be established by gathering epidemiological data and demonstrating the physiological mechanism.

If there are 120,000 PWME in the UK there are likely to be about 12,000 PWME who have lax joints, because about 10% of people have lax joints. Those people often get sent to hypermobility clinics run by doctors who believe that hypermobility is the cause or pain or fatigue so the story is perpetuated. Rodney Graham had a clinic full of such people. I was asked to take the clinic over when he retired because I had worked on hypermobility years before. I said no because I knew I had nothing useful to say to people with hypermobility. I was not prepared to pretend that I did.

 

What a bizarre site. They don't even know what POTS stands for ... the "O" is for "Orthostatic", and they shouldn't be omitting it.

Regardless, the problem in ME/CFS is usually a different form of orthostatic intolerance, not POTS. The science is there, and it doesn't support POTS or EDS being of particular significance other than a somewhat common co-morbidity.

 

I've seen quite a few people drop the 'orthostatic' from POTS. I think that's a decision rather than an error.

I agree with some of Edward's points on this, but I thought that there was now population based studies showing that EDS III/HMS/etc were associated with a greater risk of pain and disability?

This isn't an area I really keep up with. This abstract turned up on google but doesn't look that great: http://onlinelibrary.wiley.com/doi/...ionid=E0208F1CCC329124E2761FB0524809CE.f03t01

I get the impression that these diagnoses are becoming increasingly stigmatised amongst the medical profession though, and that there's a growing push for a 'biopsychosocial' approach to them. This is a bit of a shame, as I get the impression that an important reason why these diagnoses are valued by patients is that they let them escape the 'biopsychosocial' approach to ME/CFS.

 

Well done for due diligence, E12.

With Debbie Symmons in the author list I think we can be confident that the stats will be good. However, judging hypermobility and pain by questionnaire I would consider pretty useless. Hypermobility assessments are variable enough when done using a formal Beighton score by a professional.

I would expect a noticeable bias in self-reporting towards a spurious link to symptoms (i.e. people with pain will more often had it suggested to them that they are hypermobile). And 'widespread pain' is something I have never actually encountered in patients. People often have pains in several places. I have pain in six places, but I know why in all six cases. The only thing of relevance would be pains attributable to joint laxity and not to anything else, and this study is not going to tell us that.

Then if we look at the result it is of no clinical interest - or at least it is of interest as a negative. The difference is between eighteen and a half percent and sixteen percent. I very much doubt that would ever have been noticed if it were not that certain physicians wanted to make their name out of claiming it. In fact I am slightly surprised that it is not larger simply because the idea of an association is quite widespread in the population now. Two to five percent difference from subjective bias would be expected.

So to me this is a very nice demonstration that in all likelihood there is no association whatever between some special sort of general pain and 'EDSIII'. Of course the criteria for hypermobility in the study might have been too lax - with eighteen percent hypermobile. But there is no clear cut definition of EDSIII that makes biological sense.

I understand that but if they are bogus diagnoses that backfires. The attitude of physicians to 'EDSIII' is divided. There is a large body of GPs and rheumatologists and others (including Peter White) who seem to think this is a useful concept as a cause of pain. (Note that most of these like the biopsychosocial label.) Then there are physicians with a critical approach who, like me, think it is probably of no use to anyone. POTS is more complex but I think it is often a bogus label.

So the irony is that patients are hanging on to diagnoses given them by the BPS crew is part of a sort of mystical conceptual Irish stew. They are not used by people like myself who think that ME has a physical cause that we as yet have no understanding of.

 

Yeah - I was sure I'd seen a population based study which showed a much stronger association, but it was possible I'd just read something which cited the "after adjusting for age and sex, hypermobile participants were 40% more likely to report the most severe CWP [chronic widespread pain]". To me, it doesn't seem implausible that joint laxity could lead to additional strains on people's body which can lead them at greater risk of other problems, but there does seem to be a lack of good evidence on this stuff.

18.3% of participants being classified as hypermobile indicates that the 28% who returned their questionnaires are unlikely to be representative of the general population.

While people like Grahame can talk about a 'biopsychosocial' approach, it seems that this is different to the 'biopsychosocial' approach typically taken to ME/CFS. One just needs to compare the DWP information on EDS III/HMS provided by Grahame to that which they have for ME/CFS to see why an EDS III/HMS diagnosis could make life easier for patients. It's a shame that the politics around diagnosis and the management of many peoples health problems is such a mess that these sorts of political issues might be important.

 

I would like to discuss further your use of lax joints and repeated references to pain. Many people with EDSlll do not merely experience lax joints and pain - that is not the issue. In fact i do not have significant pain at all. The issue is that if it is a connective tissue disease then the problems patients face go way beyond pain and affect many areas of the functioning of the body. For me personally i have a whole host of issues, which i do not believe are attributable to M.E, but may well be to EDS of some kind (i still haven't seen the correct specialist, which is another story) eg: Mitral valve prolapse with regurgitation, double pelvic organ prolapse, dropped arches (which in turn have caused my knees to turn inwards, causing pain) coccydynia, which may be due to dislocating coccyx (surgeon couldn't be sure), MCAS (which appears to be much more common in people with connective tissue disease) and possible OI of some kind (cardiologist refuses to test)

New criteria developed recently for HEDS/EDSlll

https://ehlers-danlos.com/wp-content/uploads/hEDSvHSD.pdf

 

Yes, but it is not a connective tissue disease. It is not a specific disease, but just a collection people with loose joints. EDSIII is a re-naming of the hypermobility syndrome. Way back in 1978 Rodney Grahame and I looked to see if there was an increased incidence of mitral valve prolapse in the syndrome. We published a paper that said that there was but my own analysis of the data did not show this. I admit to feeling guilty that I did not query this with Dr Grahame at the time. Subsequent work showed no increase in incidence.

Since then the name EDSIII has become popular but when I look at definitions and criteria like the one you quote all I see is a vague compilation of things that Rodney Grahame thought might be associated. I am not aware that anyone has ever identified a single syndrome or gene defect here. IN fact the DWP account says that no single gene defect is found.

I am sorry to disappoint people but my honest opinion is that this 'syndrome' is all made up. Sure there are people with lax joints and some have pain and some have fatigue and some have prolapses and so on but nobody has ever found a connective tissue defect. And nobody has explained why tissue laxity should give fatigue. And it makes no particular sense for it to give pain unless there are injuries. Dropped arches are common to a high proportion of the population and as far as I know are not particularly associated with joint laxity. The foot is usually stiff and flat.

I ama bit doubtful there are any 'correct specialists' here. There are geneticists who deal with the rare forms of EDS that affect vascular tissues but I doubt they would pass much comment on 'EDSIII' where no genetics are known.

 

Yes, i am aware there are currently no known genetics for EDSlll. The guidelines I quoted are up to date clinical guidelines, presumably compiled by many Drs who specialise in this area? they are not made up by wishful; thinking patients.

In terms of 'correct specialists, what i am referring to is a specialist who will rule out other forms of EDS, preferably, where indicated with genetic testing. These kind of decisions, such as affect my fami;y members ( have children with similar issues), shouldn't be judged by a GP, but by a competent specialist. What i was referring to was that i had ha no additional work ups based on my history to exclude other forms of EDS. The main reason for this is my M.E diagnosis and the NICE guidelines which restrict GP's from further investigations specifically.

As you know Julia Newtons team find 30% of us misdiagnosed, with many other dx found. The NICE guidelines need to reflect this need for more rigorous work up of patients with exclusions and common co morbid diagnoses laid out. The situation we have instead is many patients spending a lot of time and money searching for themselves for clues and Drs that can help them. Many of us are misdiagnosed when we have things like EDS, Behcets. Lupus, OI, MS, Lyme disease, MCAS as the primary causes of our illness.

 

No, do not get me wrong, I have never suggested these guidelines are made up by wishful thinking patients. The problem is that they are made up by wishful thinking doctors.

Pretty much all guidelines in the UK I suspect are compiled By Rodney Grahame. If not they are almost certainly compiled by one of his assistants - Dr Alan Hakim or Dr Hannah Kaz-kaz. These are all delightful people I have known personally for years, but I have never known what the guideline information is actually based on. It looks to me very much like what one would dream up if one wanted to invent a syndrome called EDSIII. However much I am grateful to Rodney for having helped me greatly in my career I cannot honestly say that I have reason to think there is any more basis to it than that.

I doubt there is any need to rule out other forms of EDS because they are extremely rare and do not produce fatigue or any of the other symptoms of ME as far as I know. Most of them are obvious enough to be diagnosed early in life. I do not see why the ones that have cardiovascular risks are more likely someone with ME symptoms than anyone else. I agree with Julia Newton that many people diagnosed with ME may be misdiagnosed but I do not see EDS as likely to be relevant.

 

Just to add in relation to the remark:
'People you have "close to you", I suggest may be part of the problem. They are establishment who probably haven't looked, therefore will not find.'

The point is that these people close to me are the very people who have told patients that their fatigue and pain are due to EDSIII. If they haven't looked then clearly there is no basis for this advice. I agree they haven't looked, and that is why I think the whole story is speculation.

 

mr edwards
you may need to refresh your evidence and knowledge from 1978 there has been a lot of work done on the eds spectrum and nosology had been created in 2017

so a lot of what you are declaring here mr edwards is completely outdated and even the term eds 3 is now not correct term to use.

it is hughly important that we rule out other connective tissue disorders as from memory they were the largest group of misdiagnosed pwme in her chart.

pots is very common in pwme. from 17 years as a patient and doing both the poor mans stand test and those who like myself had full check up with pots specialists.

it is much higher than you think both the pots in pwme.

we MUST concentrate on getting correct specialists in the clinics. so we can really make sure al other dx are ruled out. this is vital. it will help research as we will be excluding the chronic fatigue patients who actually have other often serious illnesses which some can be fatal and also treated.

i cannot state this enough.

mr edwards please would you consider reading this paper and connecting with the eds groups who have put a lot of work into this.

http://onlinelibrary.wiley.com/doi/10.1002/ajmg.c.31552/abstract

the pots which i knew i had 10 years ago took me that amount of time to get the official diagnosis form a pots specialist. i did the poor mans test myself.

drs refused to believe my home heart blood pressure monitor said it was faulty. three new machines later still same results.

we need pots and dysautomia specialists and testing available to patients along with rhumy drs in the clinics to pick up on differential dx.

why has julia newton found connective tissue disorders so many in the misdiagnosis paper? it is the largest group that are misdiagnosed in the paper.

also heart and sleep disorders.

 

This post seemed to have disappeared, sorry if duplicate.

I am working from 2017 guidelines. The only thing new I have seen is the paper Esther12 found which suggests that if there is any link between hypermobility and pain it is pretty slight.

I am happy if EDSIII has been abandoned but what is there instead?

I could not open the link you cited. Do you have the PubMed citation?

What CTD did Newton find? I cannot find a paper on misdiagnosis on PubMed.

 

pretty ill here mr edwards but you can research on the eds groups and find the links to the research and lots of info.

ps if you are working from those guidelines you need to look at the way the hypermobility and eds3 have been discussed and defined. it is stated in those guidelines.

joints out of place do tend to cause pain though. check out rugby games.

there is research as i said on julia newtons paper. maybe you could contact her directly.

she is working closely with the heart and pots. or contact pots uk or stars charity who deal with pots and other areas that pwme have problems with.

i have been better and able to stand with off label drug and can stand better.

though few head injuries later. so this is very serious and needs investigation. head injuries can lead to death quite easily.

pots drs in uk listed on the pots uk website and stars are very good charity. check them out

 

As far as I can see Dr Newton's only reference to connective tissue disease under misdiagnosis is where it says 'connective tissue disease/autoimmune disorder' which is using connective tissue disease in the unrelated sense of rheumatoid arthritis or lupus. I don't see any reference to EDS?

I had a look at the other pages but I remain very unclear why EDS has anything to do with ME/CFS. Maybe we should try to set up a population based study. Maybe the Biobank cohort would help but I think it would need blinded assessment of evidence for hypermobility.

 

I do think that there are problems with weak evidence around a lot of the claims of EDS III, and also the danger of people fitting their own observations to fit with a preconceived story, but there have been quite a few instances of CFS patients finding that they fit within this EDS III pattern of hypermobility, PoTS, marfanoid habitus, mitral valve prolapse... it does seem that these things could be related. I take Edward's point that clinicians working in this area can end up with a misleading impression if they happen to be referred patients who just happen to share these features, and that the evidence we have for this as a meaningful syndrome is still less than clear, I think that there's still a good change that there is something there.

I was thinking about how difficult it would be to get clear results on this from a population based study on this, without it being really expensive, seeing as there are undoubtedly many people who are hypermobile, but do not have EDS III/HMS/etc.

 

erm...so lay people can't decide it's possible they have something because they aren't specialists and specialists can't because they are misleading themselves into believing a condition exists, because they mainly see people who meet the criteria for diagnosis?

Is it just me........