hEDS v ME

There is a fascinating discussion taking place on Jen Brea’s facebook page about how many people with a confirmed diagnosis of hEDS (hypermobile Ehlers-Danlos Syndrome) also have a confirmed diagnosis of M.E. (aka ME/CFS) as defined by the International Consensus Criteria.  There is a poll and so far it stands at 91% of hEDS people who have also been diagnosed with M.E. (as per the ICC).  As one commentator rightly pointed out there is likely to be bias, as people with M.E. follow Jen and people without M.E. don’t, but even so it’s a high percentage.

Reading the comments, I was amazed to see that patients were confused about how you differentiate between M.E. and hEDS, and that some doctors had taken away their M.E. diagnosis when they were also diagnosed with hEDS.  WTF?!  While it’s true they do share some symptoms,  they also have very separate and distinct diagnostic criteria and as someone who has both it’s easy peasy lemon squeezy to differentiate between the two.  However, to avoid confusion I thought I’d look at the overlaps and the differences.  I will be using the 2017 International Nosology for hEDS and the International Consensus Criteria  for M.E.

OVERLAPS

When it came to looking at the common diagnostic criteria for hEDS and M.E., you will probably be as astonished as I was that they only share 1 common symptom:

  • Chronic pain

That is it!

However, when it comes to hEDS there are symptoms which are reportedly common in the patients population but which don’t form part of the diagnostic criteria because they haven’t been definitively linked to causation.  These should be taken into account when diagnosing hEDS in conjunction with the official diagnostic criteria.  More of these symptoms are shared with M.E. including:

  • Autonomic dysfunction
  • Dysautonomia/POTS/orthostatic intolerance
  • Gastrointestinal symptoms, eg nausea, IBS, pain, dysmotility (constipation/diarrhoea), early satiety (ie feeling full quickly)
  • Allergies/hypersensitivities to chemicals, odours, environmental triggers, foods (ie MCAS!)
  • Headaches/migraine disorder
  • Impaired proprioception (ie being clumsy)
  • Genitourinary issues (ie weeing a lot, pain on urination, painful periods/endometriosis)

 

DIFFERENCES

hEDs requires:

  • joint hypermobility

Plus 5 of the following:

  • 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

And/or:

  • A positive family history of EDS
  • Dislocations or frank joint instability

Plus:

  • Absence of unusual skin fragility
  • Exclusion of other heritable and acquired connective tissue disorder
  • Exclusion of other heritable and acquired connective tissue disorder and diseases which could account for the above symptoms.

None of the above form part of the diagnostic criteria for M.E.

M.E. requires

  • Post-exertional neuroimmune exhaustion characterised by:
    – Marked, rapid physical and/or cognitive fatigability in response to exertion which is debilitating and causes a relapse.
    – Postexertional exhaustion which may be delayed by hours or days.
    – A prolonged recovery period, typically 24 hours or more.  A relapse can last days, weeks or longer.
    – A substantial reduction in pre-illness activity level, by at least 50% (which tends to be forgotten and is why I query anyone having an M.E. diagnosis if they still work full-time).

Plus the following:

  • Neuro-cognitive impairments which become worse with activity.  These include:
    – confusion, disorientation, speech problem, new-onset dyslexia, cognitive overload.
    – short term memory loss.
  • Unrefreshing sleep & disturbed sleep patterns, eg. insomnia or hypersomnia (not due to pain or other symptoms).
  • Neurosensory and motor disturbances.  These include:
    – inability to focus vision/abnormal accommodation response of the pupils.
    – sensitivity to noise, light, vibration, touch.
    – muscle weakness, twitching, ataxia, abnormal gait/positive Romberg sign.
  • Chronic flu-like symptoms which worsen with activity, eg. sore throat, tender/swollen lymph glands, fevers/chills.  Also:
    – susceptibility to viral infections with prolonged recovery periods.
  • Energy transportation impairments, such as:
    – air hunger.
    – loss of thermostatic stability.

None of the above form part of the diagnostic criteria for hEDS.

Let’s also not forget that hEDS is a genetic condition present from birth.  ME, on the other hand, is an aquired disease usually following an acute viral infection.

Conclusion

When you look at the situation in black and white I’m amazed there is so much confusion in respect to telling M.E and hEDS apart, because they only share 1 diagnostic criteria, chronic pain.   While there are several symptoms like POTS, allergies and gastro-intestinal symptoms which may appear in both diseases, when combined with the official diagnostic criteria it’s very easy to see how distinct and separate the illnesses are.

As someone who has both diseases, plus MCAS, endometriosis, severe migraine disorder and dysautonomia, I find it fairly easy to tell which symptom is coming from which disease.  I get chronic fatigue from my hEDS, but it’s nothing like the flu-like malaise and drained, sickened, unable-to-move-without-feeling-like-I’m-dying  collapse I get with M.E.  The pain from my Endo is nothing like the pain from my hEDS, which is nothing like my migraine pain, which is nothing like the tooth-ache all over gnawing pain of my M.E.

Having said all that, my gastro-intestinal symptoms could be due to anything (migraine makes me nauseous, pain makes me nauseous, MCAS makes me nauseous, orthostatic intolerance makes me nauseous, exhaustion makes me nauseous) so there are definitely overlaps, but nothing which muddies the diagnostic waters.

That’s all I can manage to write for now.  I went on a photography day out yesterday and, despite sleeping 7 straight hours, today I feel like a rag doll – my eyes are down by my knees, I have a sore throat, my dylexia is off the charts, I feel sick, I’m hot, I’m very pale, I’m a bit dizzy and disorientated, I have a headache, I’m ridiculous sleepy, but most importantly my brain function is dreadful.  All this is the classic, delayed PEM of M.E.  I also have quite bad leg, neck and back pain due to hEDS, but it’s my ME symptoms that are keeping me in my pyjamas for the entire day (and probably all of tomorrow too, without the energy for a shower despite the fact I know I smell!).

 

 

2 thoughts on “hEDS v ME

  1. Livvy Woodburn

    Dear Jak, I understand your point & think it’s fab you’ve gone to the trouble to compare & contrast these conditions especially re diagnostic criteria. The thing that bugs me most about eds is that the complications from it can cause a whole host of symptoms that are not part of the eds itself.. but yet there is still little understanding of the connections to these complications or acknowledgment of the suffering.. so for example I have confirmed hEDS diagnosis from geneticist but I also have many M.E. symptoms you describe and I find I can’t do what I see many edsers doing but I also have PoTS & mcas and confirmed cci & aai by neurosurgeon & scans.. so know ones knows how much of the symptoms of the complications of eds are causing the M.E. type symptoms? My PoTS improved massively with mcas management and also with severe restricted pacing when to sit up in bed and when to use a commode or bedpan.. by eds improves with specialist adapted eds physio but always my neuro symptoms get worse from swallowing, voice control, ability to hold head up without subluxation, left sided weakness, terrible head pain, inability to make decisions or put sentence together, poor memory function generally and weaker grip, neuro pain and spastic muscles, breathing slowed and hard to breath, doubled vision and muscle pain – all always better on rest and worse on moving especially in connection with arm movements and neck movements but generally any stretch.. so making physio impossible.. is this cci on its own or is me muddled into it? The specialist I saw believed that our brainstorms being slowly compressed caused Autonomic, digestive, immune and cns damage slowly over time due to our defective connective tissue..

    Liked by 1 person

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    1. Jak Post author

      Totally get your frustration Livvy.

      If you had a viral onset of your ME-type symptoms, and have delayed post-exertional malaise you need to be evaluated for ME. If neither of these apply maybe, for you, your symptoms are hEDS/MCAS/instability related.

      Many of my ME friends don’t have hEDS so it can’t be hEDS causing ME symptoms in them.

      My money is on mast cells being behind ME. And of course MCAS is more common in hEDS due to the fact mast cells live in connective tissue, which is why more people with hEDS have MCAS. So, for me, mast cells are the common denominator, not connctive tissue, but time will tell x

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