Now I’ve had a few days for the new EDS criteria to settle in, I thought I’d clarify a few of my thoughts and why I had such a negative reaction.
My biggest gripe is that the new criteria for hEDS focuses solely on skin and joint issues. EDS is a disorder of collagen. Collagen makes up our entire body, from veins to bowel, eyes to hair. To say there is not enough evidence for issues like gut symptoms or POTS to be included in the diagnostic criteria blows my mind. I don’t know one single EDS patients who does not have some form of gut issue and I know people who are tube fed due solely to their EDS.
In the webinar, the speakers are at pains to point out that systemic symptoms are well covered in the paper (now online here). The paper which, by the way, is 245 pages long. The paper does state that systemic symptoms should be taken into account when diagnosing hEDS ” the presence of such systemic manifestations may prompt consideration of hEDS in the differential diagnosis” in which case they should have been included in the diagnostic criteria. The speakers say that the whole point of revising the EDS criteria was to make it simpler and for any doctor to be able to diagnose the condition. Which time-strapped NHS GP or general physician is going to wade through a complex 245 paper when they have no particular interest in EDS (which is now going to be considered so rare they might only see a couple of cases in their entire career)? Most Rheumatologist in the UK still know zero about EDS and they’re supposed to be the “specialists”!
The speakers say that they might produce a simple app in which you can tick some boxes to see if you have hEDS. Will this app take you to the multiple sections on all the co-morbid symptoms? No, of course it won’t. The co-morbid symptoms will simply be ignored, making life much………oh so much……..harder for those with the condition.
My second gripe is not updating the Beighton score. Being able to bend thumb to wrist appears 3 times in the new criteria (in both bits of Criteria 1 and also in Criteria 2). My thumbs are seriously hypermobile, but bend the other way for heaven’s sake! I can’t bend them to my wrist, but they bend outwards by 90 degrees (and are so painful they need splints due to a lifetime of over-extension). But the lay-doctor will ask me to touch my thumb to my wrist, see I can’t do it and fail me. Only the well-informed doctor will look at the fact my thumbs are hypermobile, they just bend t’other way (I have personal experience of health professions sticking rigidly to the Beighton score and not accepting my joint hypermobility because it didn’t fit ‘exactly’). If these new criteria are supposed to make it easier for any Tom, Dick or Harry doctor to diagnose hEDS they fail spectacularly.
My third gripe is the 12 point list in Criteria 2. I have atrophic scaring following my spinal surgery, but that’s not enough to fulfill point 6 in the list. I apparently have to have two atrophic scars. Sadly I became allergic to anaesthetic due to my MCAS (as a result of having EDS FFS!) so can have no further surgery as I might die. So if you’ve never had an operation, or fallen and taken the skin off your knees, how do you prove atrophic scaring? I’ve had a hiatus hernia for years due to my EDS which has been so painful it’s actually made me contemplate suicide, but this doesn’t fit for point 5 which relates to acute hernias. Point 9 refers to being able to place thumb to wrist, but as mentioned above I’d fail this despite having hugely hypermobile thumbs. I also take issue with point 7, which discusses uterine prolapse but only in “nulliparous” (ie child-less) women. 75% of the female population have children for god’s sake, so even if your womb is hanging out your vagina you wouldn’t pass this because ten years ago you’d given birth. Jaysus!!!!!
I did learn something about myself though, and it’s that I have Piezogenic papules of the feet. No Doctor has ever looked for these and it’s almost impossible to look for yourself, so I got my Mum to check and yes I do have the white fat nodules when standing.
If I saw a Rheumatologist who specializes in hEDS I’d probably comply with 7 out of the 12 points and pass Criteria 2. However, non-specialst Doctors would fail me because, as with the Beighton Score, they’d be too rigid in their assessment.
My fourth gripe also covers Criteria 2 and is about proving family history. What if you’re adopted? What if, like me, you are no longer in contact with your paternal family? What if, like me, your parent is ill and can’t (or doesn’t want to) pursue a diagnosis? What if your parents have died? What if you are an only child and have no siblings? This must be the worst section of the entire paper and is hugely discriminatory to anyone but the bloody Waltons Family!
My fifth gripe is about Criteria 3 and having other conditions excluded. Good luck with that on the NHS! I had to travel 100 miles and pay privately for a 15 minute appointment with a Rheumatologist who’d even heard of hEDS (her NHS waiting list was closed to new patients as she had so many people wanting to see her) just to get a diagnosis. On which planet is an NHS patient going to get to see a handful of specialists in their field (haematologists, dermatologists, geneticists, neurologists) to rule out every other cause of their symptoms?! No offence, but you can tell the American influence on the writing of the paper from this Criteria. You want genetic testing over there you find a doctor, pays your money and gets it done. The UK just doesn’t work like that, and what about patients in Africa or India where it’s not even an option?
The more I think about the new hEDS criteria the more angry and upset and frustrated I become. Thank God I already have a diagnosis and according to the webinar speakers there is no need for this to be re-evaluated, but for all those out there still searching these new criteria have muddied the already dirty waters. What was the reasoning for making it harder to gain a hEDS diagnosis (which the speakers at the webinar clearly state was the goal?). I would guess that many people with hEDS are now going to be placed in the Hypermobility Spectrum Disorder (HSD) category. The reasons for separating hEDS from HSD is stated as being that they require different management strategies and it will help further research. But if people with actual hEDS are not being diagnosed and placed in the HSD category it means they won’t get the treatment they actually need and the research will be skewed. I’m not sure how that will further the cause of either condition.
I sound like a proper negative Nelly on the new criteria, but my view is skewed by over 2 decades of seeing the same fight for good diagnostic criteria in the M.E. world. We eagerly awaited each new criteria (and I think there have been 7 so far in the past 22 years) only to be gutted that they were still basically not fit for purpose and didn’t separate CFS from M.E. which, like EDS and HSD, meant that patients weren’t adequately treated and any research wasn’t worth the paper it was written on. I am heartened, however, that the new EDS diagnostic criteria are stated as being “provisional” and can only hope that the Beighton score is revised as a matter of urgency.