Tag Archives: hypermobility spectrum disorder

HSD v hEDS

I’ve been asked to write a post on the differences between Hypermobility Spectrum Disorder (HSD) and hypermobile Ehlers-Danlos Syndrome (hEDS) and how each of the conditions are diagnosed.

Before I start, I’ll just talk a little about the new and old terminology.

  • EDS III has been replaced by hEDS – both refer to hypermobile Ehlers-Danlos Syndrome.
  • Benign Joint Hypermobility Syndrome (JHS) has been replaced by Hypermobility Spectrum Disorder (HSD).

Historically there has been much confusion between general hypermobility (HSD) and Hypermobile EDS (hEDS) and in 2017 an international group of doctors and patient groups came together to devise new criteria to help differentiate between the two.  Having said that, it’s now thought they are basically the same disease just on a spectrum and it’s possible that although you may be diagnosed with HSD at one point in time, as you age new symptoms could present themselves and your diagnosis could change to hEDS.  This was the case for me.  I was initially diagnosed with Joint Hypermobility Syndrome (now called Hypermobility Spectrum Disorder) but this was later changed to hEDS as my symptoms increased and new symptoms appeared.

When women reach their 40s and start to enter peri-menopause symptoms definitely may change.  For example I used to be hugely hypermobile, scoring 8/9 on the Beighton Score, but now I’m 50 I’m so stiff I’d be lucky to score 3/9!  So the new criteria take this into account, with the question “can you or could you ever” do the splits for example.  And of course, symptoms like dental over-crowding are most relevant to children not adults so taking a good history is very important in the diagnostic process.  So what exactly are the new criteria?


HYPERMOBILE EHLERS-DANLOS SYNDROME (hEDS)

Patients need to have ALL 3 of the criteria below:

Criterion 1: Generalized Joint Hypermobility (GJH)

Tested for using the Beighton Score.
Children, younger adults and older adults are treated differently as hypermobility decreases with age:
Children need a score of 6 or more out of 9.
Adults need a score of 5 or more out of 9.
Adults over 50 need a score of 4 or more out of 9.

However, some people’s score may be affected by surgery or, for example, an unrelated disease (my Mum has Dupytrens Contracture and can’t even straighten her fingers let alone bend them backwards).  In these cases a 5 point questionnaire is used.

  • Can you now (or could you ever) place your hands flat on the floor without bending your knees?
  • Can you now (or could you ever) bend your thumb to touch your forearm?
  • As a child, did you amuse your friends by contorting your body into strange shapes or could you do the splits?
  • As a child or teenager, did your shoulder or kneecap dislocate on more than one occasion?
  • Do you consider yourself “double jointed”?

A “yes” answer to 2 or more questions suggests hypermobility.

My experience of the Beighton Score

If I had seen a Rheumatologist not really clued up on hEDS I would probably never have been diagnosed.  I have never been able to place my hands flat on the floor with straight legs, my thumbs do not bend inwards to my wrist and my knees don’t particularly bend backwards, yet I was given a score of 8/9!  The reason being I was clearly hypermobile in other joints.  My thumbs bend outwards by 45 degrees, not inwards.  My wrists are hypermobile.  I used to be able to do the splits.  I’ve been told my whole life by doctors that I am “double jointed”.  I have hypermobile ankles.   My knees roll inwards just not backwards!  I have hypermobile toes.  I was also 42 when diagnosed, so no spring chicken.  Taking all of these non-beighton scores, and my age, into account my Rheumy decided I was clearly hypermobile and she was correct to do so.  There are photos of my joint hypermobility on my blog here.

Criterion 2: Two or More Among the Following Features (A–C) MUST Be Present (for Example: A and B; A and C; B and C; A and B and C)

Feature A: systemic manifestations of a more generalized connective tissue disorder (a total of five must be present).

  • Unusually soft or velvety skin.
  • Mild skin hyperextensibility.
  • Unexplained striae such as striae distensae or rubrae at the back, groins, thighs, breasts and/or abdomen in adolescents, men or prepubertal women without a history of significant gain or loss of body fat or weight
  • Bilateral piezogenic papules of the heel.
  • Recurrent or multiple abdominal hernia(s) (e.g., umbilical, inguinal, crural)
  • Atrophic scarring involving at least two sites and without the formation of truly papyraceous and/or hemosideric scars as seen in classical EDS.
  • Pelvic floor, rectal, and/or uterine prolapse in children, men or nulliparous women without a history of morbid obesity or other known predisposing medical condition
  • Dental crowding and high or narrow palate.
  • Arachnodactyly, as defined in one or more of the following: (i) positive wrist sign (Steinberg sign) on both sides; (ii) positive thumb sign (Walker sign) on both sides
  • Arm span‐to‐height ≥1.05
  • Mitral valve prolapse (MVP) mild or greater based on strict echocardiographic criteria.
  • Aortic root dilatation with Z‐score > +2

My experience of Feature A

  • I have been told my whole life that I have beautiful skin – I didn’t realize that other people didn’t have skin which felt velvety to the touch.
  • One of the reasons I was initially given a diagnosis of JHS and not hEDS was that my skin was only mildly stretchy, so it’s good to see that it’s now recognized that circus freaky stretchy skin is NOT needed for a diagnosis of hEDS.  There are photos of my stretchy skin on my blog here.
  • I have no striae (stretch marks to you and me!).
  • I have piezogenic papules on my heels – this is what they look like:
Photo of piezogenic papules

Piezogenic foot papules

  • I have never had a hernia.
  • I have atrophic scaring in two sites – a small scar on my forehead after a childhood fall and also on the scar from my spinal surgery.  This is what an atrophic scar looks like, it’s kind’ve creased!

Widened, atrophic, cigarette paper scar

  • I’ve never had a prolapse.
  • I had dental overcrowding as a teenager and had to have 4 of my molar teeth removed.
  • I do not have Arachnodactyly (which is long, slender fingers or toes).  In fact, just the opposite – I have short, fat, stubby fingers and toes!  Read more about the Steinburg and Walker Signs here (I have neither).
  • I have no idea if I have an armspan to height ratio of greater than 1.05 as I’ve never been tested but I doubt it – I’m 5ft 2″ and have diddly little arms.
  • I do not have mitral valve prolapse or aortic root dilation.  Both of these symptoms must be checked by having a heart scan.

So, I personally score 5 out of 12 which means I fulfill Criteria 2, Feature A.

Feature B: positive family history, with one or more first degree relatives independently meeting the current diagnostic criteria for hEDS.

I have a real issue with this being part of the criteria.  What if you’re adopted, your parents are dead or you’re an immigrant whose parents are in another country like Syria?  What if, like me, you are not in touch with your biological father?  As it happens, I’m fairly sure I inherited hEDs from my Mum however she’s 78 years old, very ill and not willing to go through the testing procedure so I can’t say for certain that she does have hEDS. 

I can’t prove that I fulfill Criteria 2, Feature B.

Feature C: musculoskeletal complications (must have at least one)

  • Musculoskeletal pain in two or more limbs, recurring daily for at least 3 months
  • Chronic, widespread pain for ≥3 months
  • Recurrent joint dislocations or frank joint instability, in the absence of trauma (a or b)
    • a. Three or more atraumatic dislocations in the same joint or two or more atraumatic dislocations in two different joints occurring at different times
    • b. Medical confirmation of joint instability at two or more sites not related to trauma

My experience of Feature C

  • I didn’t start partially dislocating (called subluxing) my joints until I was in my early forties and I’ve never thankfully had a full dislocation.
  • I’ve had chronic, widespread and musculoskeletal pain for decades.

I’m not sure if having subluxations meets the definition of ‘frank joint instability’ (though I suspect it does), but nevertheless I pass the other two symptoms and therefore fulfill Criteria 2, Feature C.

Conclusion

Based on the evidence of having 2 out of the 3 Features listed above, I pass the test for Criteria 2.

Criterion 3: All the Following Prerequisites MUST Be Met

  • Absence of unusual skin fragility, which should prompt consideration of other types of EDS
  • Exclusion of other heritable and acquired connective tissue disorders, including autoimmune rheumatologic conditions. In patients with an acquired connective tissue disorder (e.g., lupus, rheumatoid arthritis, etc.), additional diagnosis of hEDS requires meeting both Features A and B of Criterion 2. Feature C of Criterion 2 (chronic pain and/or instability) cannot be counted towards a diagnosis of hEDS in this situation.
  • Exclusion of alternative diagnoses that may also include joint hypermobility by means of hypotonia and/or connective tissue laxity. Alternative diagnoses and diagnostic categories include, but are not limited to, neuromuscular disorders (e.g., myopathic EDS, Bethlem myopathy), other HCTD (e.g., other types of EDS, Loeys–Dietz syndrome, Marfan syndrome), and skeletal dysplasias (e.g., OI). Exclusion of these considerations may be based upon history, physical examination, and/or molecular genetic testing, as indicated.

My experience of Criteria 3

You rely heavily on your Consultant to fulfill Criteria 3, so you can only hope you see someone knowledge in hEDS.  I do have fragile skin which tears and bruises easily, but this is part of having hEDS – the trick is in knowing if this is abnormally fragile or not and I’m not sure where the cut-off point is.  In the UK, suspected hEDs patients aren’t routinely offered gene testing which I personally think is a big mistake.  There are times I feel I have cross-over symptoms with Classical EDS (cEDS) and the only way to rule this out is by gene testing.

Excluding other connective tissue disorders is also not as easy as it sounds.  There is no conclusive test for Lupus, for example, and I’ve never been screened for it to my knowledge.   Even diagnosing arthritis isn’t straight forward, as I know from first hand experience with my best friend who had been ill for 2 years before finally being diagnosed with a rare form called Palindromic Rheumatism (an autoimmune arthritis) which many Doctors have never heard of let alone look for.  I peronally have had general rheumatism excluded but little else.

Again, you’re reliant on your Consultant being extremely thorough and knowledgeable in rare diseases such as Bethlem Myopathy and OI and as we all know this is usually not the case.

I therefore have no clue if I pass Criteria 3 or not as none of these other diseases have ever been mentioned to me and I’ve never been offered gene testing for other types of EDS.

Other considerations

Under the General Comments section below Criterion 3 of the new diagnostic criteria, it is stated that other symptoms should be looked for when diagnosing hEDS, though they don’t officially form part of the criteria because they haven’t been studied enough yet.  They include things like POTS, gastro-intestinal disorders, dysautonomia and chronic fatigue.


HYPERMOBILITY SPECTRUM DISORDER (HSD)

So what happens if you don’t fulfill the diagnostic criteria for hEDS but are clearly hypermobile and having widespread pain and possibly other issues?  You are now classed as being on the hypermobility spectrum and there is now a new classification called Hypermobility Spectrum Disorder (HSD) which has replaced the old Benign Joint Hypermobilty Syndrome (JHS).

There are now different categories of joint hypermobility:-
Localized (L-HSD), in which the hypermobility is confined to less than 5 joints
Generalized (G-HSD), in which the hypermobility is seen in more than 5 joints
Peripheral (P-HSD), where the hypermobility is confined to the hands and feet
Historical (H-HSD), usually when the patient is older and no longer scores highly on Beighton.

Hypermobility in and of itself is fairly common, particularly in children, and can often be symptom-less and of no consequence.  However, a diagnosis of HSD should be considered if other symptoms are present but the diagnostic criteria for Ehlers-Danlos Syndrome isn’t met.  These symptoms include:

  • Trauma.
  • Chronic Pain.
  • Poor proprioception (ie being clumsy).
  • Other musculoskeletal traits, eg. flat feet, mild scoliosis, kyphosis or lordosis (ie upward or inner curve of the spine).
  • Other issues, such as POTS, gastrointestinal problems, anxiety, bladder dysfunction.

MY CONCLUSION

Since the introduction of the new criteria in 2017 the criteria for hEDS has become more strict so it’s harder to obtain a hEDs diagnosis, but the new HSD incorporates everyone with hypermobility, which wasn’t the case before.

It is now recognised that issues such as POTS, Mast Cell Activation Disorder, Chronic Fatigue, Gastrointestinal disorders and Dysautonomia are seen more often in the hEDS and HSD population and being diagnosed with these should point a Doctor in the direction of looking for connective tissue disorders, but not enough is known about them yet to include them in the actual diagnostic criteria, which is a shame because we all know how prevalent they are!  However, papers on all these symptoms are included as footnotes in the new criteria and can be found here.

This review has only focused on Hypermobile EDS as that is the type I have – different criteria apply for other forms of EDS, such as Classical and Vascular.


Further information

You can read all about the new criteria for hEDS here.

The criteria for HSD is here.

An excellent toolkit for GPs can be found here and is actually easier to understand than the official EDS information listed above!

 

 

 

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Aging, hEDS & Invisibility

I’ve rambled on a bit in this post, so you might need a brew and half an hour to spare 😉

As I’ve mentioned before on my blog it’s disappointing (I’m being polite) that there is virtually no information on the effect of aging on the hEDS population, despite the fact we’re all going to get old.  Quite why the over 50s are ignored when it comes to the management and progression of the disease is baffling because if we knew why some people’s illness progressed well while other’s progressed badly we’d know how effective management in younger adults actually was, but it still seems to be something none-one wants to explore.

According to the 2017 guidelines (known as nosology – why can’t plain english be used instead of medical jargon, the EDS charities need to improve on that) there are 3 phases to hEDS:

  • the hypermobility stage
  • the pain stage
  • the stiffness stage

though as far as I could determine this information is based on a pilot study of only 21 people, so why it made it into the new guidelines and is now relied upon by clinicians as fact is a bit of a  mystery.

Although I’m in agreement that, for most people, there are stages to having hEDS the 3 stages listed above don’t really tally with my illness experience.  In particular there needs to be a ‘menopause’ stage because there is overwhelming anecdotal evidence that many women don’t even know they have hEDS until they hit their 40s and their hormones begin to wane (I appreciate that men get hEDS too and their illness experience will be totally different).

For me, I had few symptoms of hEDS until I reached my late thirties.  Note I said “few” and not “none”.  I’ve had gut symptoms my entire life and I started having back pain at the age of 11 which resulted in surgery when I was 16, though it sadly didn’t cure the pain which has been ongoing ever since.  But on the whole, I was in the hypermobile stage until I was around 36.

For me the pain stage didn’t follow the hypermobile stage.  Nope, I skipped that and went straight to the stiffness stage.  Around my late thirties I noticed that my ankles were stiff in the mornings, to the point where I could barely walk to the loo.  Thankfully after an hour or so they loosened up but this was the first indicator that there was something wrong (I had no clue I had EDS at the time).  My back also stiffened up and I went from someone who could do backflips despite the ever present pain to someone who couldn’t bend to put her socks on virtually overnight.

At around the same time I was emerging from my 10 bedridden years with M.E. and starting to be more active.  It was then I noticed chronic, widespread pain.  It wasn’t joint pain, more an overall muscle pain which felt like my ligaments were tearing at even the smallest of daily tasks.  My fingers, wrists and elbows in particular burned constantly.

By the time I was 41 my feet and knees had joined in the pain party.  My neck was so stiff I couldn’t turn it to reverse the car and the range of movement in my hips had reduced down to 30% on the right side and 10% on the left.  When I suddenly starting sub-luxing my shoulder and jaw in my sleep I knew something other than M.E. was going on and fought for my EDS diagnosis.

Despite all the stiffness and pain, however, I remained hypermobile in my peripheral joints.  I can still touch my nose with my tongue, do the reverse Nemaska, bend my fingers to 90 degrees and do all the other party tricks I always have.  My hips, neck and back are a different kettle of fish, though, and the range of movement in my spine diminished rapidly the second I hit 40.

For me, the stiffness and pain stages happened simultaneously and I’m as certain as I can be that they arrived because I was entering peri-menopause.  There is an urgent need to study women with hEDS in their 40s because there are hormone drugs already on the market which might help stave off the deterioration which seems to happen when women reach peri-menopause.  The physio given to younger women simply doesn’t work when you’re middle aged and I find it infuriating that all the management advice is aimed at children and younger adults.  My muscles and ligaments have changed beyond all recognition as I’ve aged and there is a dire need to look at why and how we can best manage EDS in the middle aged and elderly.

I am now 50 and in the late stages of peri-menopause.  When I’m resting my pain levels are fine, apart from my back which hurts no matter what I do, but the second I start to do anything the burning and stinging in my muscles starts, consequently I can’t continue activity anywhere near as long as I could a decade ago as the pain simply becomes too much.  My stiffness has also ramped up a notch or ten.  Far from being confined to certain joints it’s now widespread and there are days when I can barely move first thing in the morning.  My body doesn’t loosen up anywhere near as quickly either and the stiffness lingers throughout the day.  I have to sit to put on socks and shoes, can barely move my head left or right, can’t chop vegetables, struggle to put on jumpers and walking the dog is like climbing the north face of the Eiger in terms of strain and effort.  I “uuuumpf” when I get up out of the chair and can crouch down to pick something up off the floor but can’t get back up again.  I literally have the body of an 80 year old.  Scratch that – my Dad is more supple than me and he is 80!

The only other information I could find about the phases of hEDS came from the UK’s Royal College of Physicians and states ” The final phase is the emergence of psychosocial sequelae such as anxiety or depression, obesity (often associated with comfort eating), work incapacity, isolation and despair. At this stage, there is often a downward spiral of loss of mobility, self-efficacy and self-esteem as the quality of life diminishes” though there is no citation or link to the research on which this statement is based.  Where is the hard data which shows that as we Zebras get older we all become depressed, obese and live in despair, because if that information isn’t available the Royal College should be ashamed of themselves for putting this crap out there.  For a start menopausal women tend to gain weight even among the healthy population (as do men over 50), so unless they can demonstrate via well-controlled clinical studies that weight gain is solely down to the progression of hEDS it is irrelevant, and for a second thing women tend to gain self-esteem as they age not lose it, it’s one of the few perks of getting old!  My body may have fallen apart during my 40s but I’m happier than I have ever been and have more zest for life, not less.  I’m so appalled at the statements made in this piece of mis-information I can barely bring myself to include it, but if this is what our health professionals are reading and basing their views on we need to know about it.  It’s a shame really because the article is otherwise sound, if now a little dated.

Going slightly off topic, the UK hypermobility charities are currently fundraising to research the effect of diet on the symptoms of hEDS and hypermobility spectrum disorders and I admit I am not happy.  We don’t even know the basics of these conditions, so to look at food intake as a means of treatment feels like we’re putting the cart before the horse.  There is no epidemiological information on connective tissue disorders (CTDs), ie how common they truly are in the general population, the ratio of the sexes, at what age diagnosis is obtained or anything about outcome.  We have no clue about progression, or how far the family link extends.  We don’t even test adequately – all people with suspected connective tissue disease should be gene tested, not least to rule out other types of CTD or Marfans’s Syndrome, and this data put in a bank for study – how else are we ever going to find the gene responsible for hEDS?  It’s this basic kind of information which forms the building blocks for research into any disease and data researchers often need when studying illness.  No food is ever going to cure my spinal stenosis or my dental overcrowding, my hernia or my genetic predisposition to Osteoporosis.   It also doesn’t help us understand why many people with hEDS also have mast cell disease, POTS or M.E., or why puberty and the menopause affects sufferers so profoundly, information much more vital and fundamental to our knowledge of EDS in my very humble, but hugely invested, opinion.  For the estimated 10% of us Zebras with MCAS, and who are usually on a restricted diet, altering the foods we eat may be impossible in any event.  Yes we need to look at treatment but only once we have a proper grasp of the disease, its incidence and progression.  Which isn’t as hard to do as you’d think.

I remember back in the late 1990s when very little was known about M.E. and severely affected  patients were completely ignored, Action for M.E. (Britain’s 2nd largest M.E. charity) sent out a survey with its regular magazine to all of its members and received approx 2,300 replies.  The results led to a report called ‘Severely Neglected: M.E. in the UK’ which was the largest ever survey done on the disease.  It turned much of the information ‘out there’ on its head, made a massive impact on our understanding of the prevalence and impact of severe M.E. and became hugely influential when NHS Clinics to treat patients were eventually established.  Over the last two decades, the ME Association have also regularly sent out questionnaires with their quarterly magazine on a wide range of topics, and run a monthly quick survey on their website for anyone with the illness to fill in not just members, all of which have helped our understanding of M.E.   Surveys aren’t difficult to do, although collating the data is time consuming, and I really don’t see why the EDS charities don’t do something similar – at least then we’d know some basic information, such as the percentage of men v women, how many people are receiving no treatment, what treatments people had found effective, progression and if ethnicity is a factor (which might help in the quest for the hEDS gene).

It appears to me, and obviously this is only a personal opinion and observation, that the EDS world shies away from asking the hard questions and I’m not quite sure why that is.  It’s great that children are being diagnosed with hEDS sooner, but appalling that older people are still being completely ignored despite the fact we are often at the more severe end of the spectrum as our bodies age and naturally deteriorate.

I’m fed up of being ignored.  I was ignored as a severely affected M.E. patients for years, as the emphasis was put on those at the mild end of the spectrum because they were well enough to campaign and be more vocal.  I’m totally ignored as an MCAS patient, receiving no monitoring or treatment whatsoever.  As an older person with hEDS I’m also ignored, for reasons I can’t quite work out.  And the fact I live in the north of England (by which I don’t mean Manchester, there is life beyond!)  with any of these diseases means I might as well not exist.  I can’t name one single EDS event which has taken place north of Birmingham and anyone living in northern Ireland or Scotland must feel completely invisible, a situation which is simply unacceptable.

 

 

That darned F word

Arrrrgghhhhhhh!!!!!  I’m glad I’ve got that out of my system because the current trend of confusing “chronic fatigue” with M.E. (I’m not using the term ME/CFS because I don’t have CFS) makes me want to scream.

A man wrote a post on the M.E. Association’s Facebook page this week saying that for the last month he’d been getting up at 7am, going to the gym to lift weights, having a steam session, then going back in the gym to lift more weights and do some cardio and was feeling much better.  There was understandably outrage in the comments section.  For a start most people with M.E. struggle to surface out of bed before 10am and any kind of extreme heat, like you’d find in a sauna, would floor them.  Our muscles do not function correctly and lifting my toothbrush can be beyond me some days – lifting weights on a regular basis would be absolutely and utterly out of the question.   Whoever diagnosed this person with M.E. needs to be sacked.

Ehlers-Danlos syndrome can cause significant fatigue, but that doesn’t mean you also have M.E.  That’s because M.E. has nothing to do with being tired, and lots to do with being ill, following trivial activity.  And by trivial I don’t mean a session at the physio (I wish).  I mean having a shower or getting dressed.

When you get the flu (the proper flu, not man-flu or a cold) you lie in bed with every muscle aching and feeling like cement.  Your brain pounds with cotton-wool fog, light and sounds are too bright and too loud, your throat is swollen and sore and walking 10 steps to the bathroom is like tackling the north face of Everest, after which you collapse back into bed feeling like you’ve been battered by a hammer-wielding maniac.  That is what M.E. feels like………..every second, of every day, of every week, of every month, of every year.  If you can call it ‘fatigue’ at all it’s an immune-induced ‘fatigue’ that is crushing and stops you doing the normal tasks of daily living every day.  It is not tiredness after activity, it’s feeling flu-like with associated all-emcompassing heavy exhaustion beyond most people’s understanding of ‘tired’.  And, more importantly, it is not relieved by rest.  So, after I’ve done something I shouldn’t – like a little bit of gardening – I wake the next day feeling poleaxed.  I spend the day resting in a foggy, poisoned stupor and wake the next day even more poisoned, exhausted, achy and foggy than I did the day before.  Each person with M.E. is different, but for me my symptoms peak 48 hours after the activity and finally abate after about 4 days (by which time I’ve done some other task of daily living and the cycle starts all over again).  And many forget that mental over-activity brings on the same symptoms.  If I’m on the computer for too long I start to get a sore throat, feel dizzy, nauseous and generally feel fluey and unwell.

The cardinal feature of M.E., I am sick of saying, is “post-exertional MALAISE”.  I’m not even sure ‘fatigue’ is mentioned in most of the diagnostic criteria.

The problem arose when M.E. was re-branded as Chronic Fatigue Syndrome.  This became confused with Chronic Fatigue which is a symptom suffered by millions of people with a hundred different diseases from insomnia to cancer, and the general population on a Friday afternoon!  IMHO, ‘hypermobility’, ‘hypermobility spectrum disorder’ and ‘Ehlers-Danlos Syndrome’ suffer in a similar way.  People with generalized hypermobility query whether they have HSD or EDS, people with HSD query whether they have EDS (some do, just like some people with CFS have M.E., but then equally some don’t) and EDS becomes a diagnosis that few qualify for even if they have it, just like true M.E.  It’s all such a bloody mess.

“Who cares?” I hear some of you say, “it doesn’t matter what you’re diagnosed with as long as you are diagnosed and get treatment”.  But actually, it matters a great deal and here is why:

  • If you are diagnosed with M.E. when your fatigue is down to another reason, such as Lyme or Lupus, you won’t receive the treatment you need for the actual disease you have.  There is no treatment for M.E., so other than being offered some CBT to adjust to living with a chronic illness you will be left to rot.  That is not good if you have fatigue which could be treated.
  • If, like me, you are unlucky enough to have both M.E and hEDS you sadly will not be able to tolerate the physiotherapy exercises needed to treat your hEDS.  If I hadn’t had my M.E. diagnosis I would have pushed myself to do the hydro and physio recommended for my hEDS and made myself sick as a dog – possibly even causing a permanent relapse from which I never recovered.  If, however, you have simple ‘chronic fatigue’ doing the recommended physio for hEDS might help – at the very least it wouldn’t lead to a deterioration in the condition.  Being correctly diagnosed is vitally important.
  • Any research carried out on an incorrectly diagnosed population is pointless.  If research on M.E. includes people who actually have simple ‘chronic fatigue’ and they get better by lifting weights, then all people with M.E. will be deemed to be ‘curable’ by lifting weights, when actually lifting weights would be hugely damaging.  Similarly with hEDS, if under the new criteria someone like me is classed as having HSD when I actually do have hEDS I will skew any research into HSD because I don’t in fact have it.  And research into hEDS will only include the most severely affected which skews research into hEDS too.  I didn’t start dislocating my joints until I was in my forties, so I wouldn’t have qualified for a hEDS diagnosis when I was younger despite having non-joint-related symptoms from childhood.

There is an alarming trend in the M.E. world for researchers to be studying ‘fatigue’ and energy production when the fatigue of M.E. is a by-product of immune dysfunction, just like it is when you get the flu.  The fatigue experienced when you have flu is just a symptom of the disease, not the cause.  It all feels like a huge waste of time and resources and will lead to yet more blind alleys (remember I’ve been following research into M.E. for 20 years now).

The bottom line is we urgently and desperately need a test to distinguish M.E. from CFS and chronic fatigue, just as we urgently and desperately need a test to distinguish hEDs from HSD and generalized hypermobility.  I know clinicians have tried their best to differentiate these diseases using symptoms as a guide but it doesn’t work, particularly in hEDS where symptoms can appear or increase as you age.

And speaking of age, there has not been one single study to my knowledge on older people with M.E. or older people with hEDS.  Research always tends to focus on children and young adults for reasons I can’t work out.  We desperately need studies on the effects of long-term M.E. on the over 50s, and we absolutely need this age group studied in hEDS as it is not uncommon for symptoms to suddenly appear or increase when you hit forty particularly in women. Even a simple survey on the over 40s undertaken by the EDS charities would be better than nothing.

Whichever bright spark decided 20 years ago to rebrand M.E. as CFS has a lot to answer for and if I could get my hands on them I wouldn’t be responsible for my actions.  And in 20 years time I may feel the same way about the current clinicians who rebranded hEDS and HSD!