And maybe that’s why you’re tired too… join me on a journey of discovery about one of the most poorly recognized and unbelievably tangled health webs you can find… involving not just one, but several intertwinedsystemic conditions I personally unofficially call the “Chronic Constellation” for lack of a better name that are each alone enough to take a person down at the knees, but combined, can be downright show stopping, if not even lethal sometimes, sadly.
[Editor’s note 4/17/19: Unsure why my banner up top has suddenly gone dark. Looking into this.]
Yet barely 10% or so who have this “trifecta” of a form of EDS (or an HSD since 2017), MCAD and Dysautonomia (usually POTS) plus many variations and additional issues including autism and weak immune systems get properly diagnosed, and it takes 10 years on average to get diagnosed with a form of Ehlers-Danlos Syndrome. (It took me 25!)
Most are currently getting diagnosed with Fibromyalgia or ME/CFS, if anything at all. This site is my attempt to help remedy that for everyone – both patients AND doctors!
A majority of autistics I follow online would like to ditch the puzzle piece(s) and stop “lighting it up blue for autism awareness” this month. (Google the hashtags #RedInstead and #ToneItDownTaupe for some alternative ideas.)
They feel strongly this stands for efforts to erase, eradicate and even prevent their existence. And you can kind of see their point: what if people set about trying to prevent your existence?
They humbly request we say “nothing about us without us”, and I must say I agree now. I’ve been infantilized and erased and ignored enough myself, despite not being diagnosed that I can relate a bit now. And I’m highly verbal.
(Hint: consider all behavior as happening for a reason. Then ask why. Is it too loud or hot? Or maybe they have a migraine? Imagine if you had a migraine but couldn’t speak and tell anyone easily!! How might y ou behave or act to release the stress and pain? While some autistics have learning disabilities, not all do, and some have amazing hidden troves of knowledge that may really surprise you. And not just about their main interests.)
(Please don’t confuse many of the common comorbidities and emerging issues that arise with the fundamental autistic neurotype. These other things may warrant medical intervention, yes. But not autism itself.)
Can parents use more support so they don’t feel so eager to erase autism? Absolutely. Especially those with non-verbal kids who they are struggling to communicate with. But that is not the same as saying your child should not have been born, or needs “fixing” so as to think like you or anyone else.
I hope you’ll join me in going against the tide and slowly but steadily recognizing and rooting this ableism out for everyone’s benefit.
January 21, 2019 Prior NEWS From The Ehlers-Danlos Society:
They will be launching the debut of a new “Project EDS ECHO” in April 2019, a platform intended for ALL doctors and medical practitioners to use to collaborate and “cuss and discuss” (my words) EDS and the HSDs in the clinical setting. I.e, present cases, share tips and tricks AS PROVIDERS. So this is not for patients, sorry (unless you also happen to be a provider as I know some are). But this should go a long way to helping ease the burden on patients to bring their clinicians up to speed. It’s a two-way platform, not meant to just be “presentation only”, but foster true two-way dialog among all who attend each week. It will be free to doctors, and CMEs will be available. Learn more here! And share with your doctors!
Your ongoing support…
On a different note (March 2018), I’m very happy to present a way for those of you who are willing and able to support me financially to do so via my Patreon page now! And I can really use your support! I’m having to step down from my part-time job after all due to another flare recently, and additional trouble reacting to a neighbor’s vapors in my apartment. (Just missed work again today because of it, boo.) You can start as low as just $1/month! That’s less than a cup of coffee almost everywhere these days…
All of my current content will still be available for free, and all of my blog posts will ultimately be free, but they may be available first to my paying supporters, then later to non-paying viewers after a suitable interval.
I care too much about getting the word out/supporting this community to put it behind a paywall! Especially since many of you are disabled to varying degrees as well. But in case you’re not, or you’re a doctor or other medical practitioner who’s benefiting from my site, I truly welcome your support here now TY!
Regardless, I have put in hundreds of hours on this site, and on Facebook and Twitter, working to bring you all the latest, greatest and most reliable information in the field at all times. This has been all out of my own very meager pocket on disability. Which now only pays my rent, so… I could really use your help staying fed and watered too!
Checks may be sent to Jan Groh, PO Box 86824, Portland, OR 97286 too.
Update March 2018 – The non-specialist LAY versions of those 18 crunchy papers above are HERE now too for the win! Big thanks to The Ehlers-Danlos Society for writing these up for the rest of us who don’t speak medicalese, or whose brains are just too tired to parse it. I think even some doctors will appreciate them too! And…
Update May 2018 –The Royal College of General Practitioners in London, England just successfully rolled out a brand new, brilliant “EDS Toolkit“ for their GPs to learn and study from! And I got to help draft it at the invitation of the primary author, Dr. Emma Reinhold, a GP with hEDS and MCAS herself I met on Twitter along with some other much esteemed company. I’m very pleased to have played even a small part in “midwifing” this project along. Please share it far and wide with any and all doctors you can! It is freely available to anyone and everyone on the web, not just the NHS.
The International 2017 EDS Classification via The Ehlers-Danlos Society
You might wish to refer to this FAQ document previously shared by The Ehlers-Danlos Society for clarification on the new criteria and terminology update as well. We will now be referring to EDS in the plural as the “Ehlers-Danlos syndromes” with an “s” on the end. (Or trying.)
As well as diagnosing an entirely new category of severalHypermobility Spectrum Disordersfor those who look a lot like they have EDS, suffer much the same, but don’t meet the criteria for any more specific diagnoses involving hypermobility of varying degrees yet.
I.e, they are subclinical for hEDS as I was for my first 44 years, and may now be technically again since I stiffened so much with age and arthritis. Kind of like how Pluto was re-classified to a dwarf planet 10 years ago right? It never changed a spot.
These forms of HSD also all supplant and replace the former diagnoses of Hypermobility Syndrome, Joint Hypermobility Syndrome, and Benign Joint Hypermobility Syndrome to my understanding. I like to think of the HSDs as the less visible bulk of patients “below the water line” as shown here:
The criteria for what has been called hypermobile EDS or hEDS were just tightened, so I will no longer refer to this form as common anymore accordingly. I actually no longer meet the new criteria since it requires a much higher Beighton 9 pt score than before. I now fit one of the forms of the new catch-all bin of the Hypermobility Spectrum Disorders better even though I was extremely bendy as a child, but…
NB you do NOT need to “lose” your diagnosis of hEDS until/unless someone requires you to, such as to meet the criteria for a research study. Further, you will still have a diagnosis that represents a hypermobile disorder, but just with a different name. It is hoped this will lead to speedier presumptive care and management rather than just leaving patients suffering because they did not meet the new higher bar of hEDS diagnosis.
I, Jan, unscientifically personally now feel very strongly that the newly recognizedHypermobility Spectrum Disorders as such are NOT rare at all, but also just rarely diagnosed – and not just because they are newly introduced. But because they’ve always been dismissed as “normal” or early aging, or “just depression”, fibromyalgia, chronic fatigue or hypochondria under any name/classification scheme when they should not be!
It is my deepest wish that one day, this disease cluster will be as well known as multiple sclerosis, and much more quickly recognized. Everyone knows someone with a hypermobility spectrum disorder, if not even EDS. I will continue to post and tweet to that end, while I finish writing my book. Join me.
Older news of note
EDS, MCAD with high tryptase (aka familial tryptasemia) and POTS linked by NIH October 17, 2016 – two articles delineating the results of Dr. Josh Milner’s study of comorbid MCAD and HEDS (plus dysautonomia) seen in a statistically significant number of patients at the NIH came out this week:
Yes, I was part of this study. No idea if I had elevated tryptase or not in the blood I sent, but I highly doubt it. So the $64K Q for me is: do I have this mutation? I also don’t know – yet. (Update April 2018: Just learned there’s now a commercial DNA test available to check for it, link above.) That said, I’m already managing the conditions (plural) pretty well already, so I’ve got that bit handled, which is my main goal. (Everyone else can split hairs and fight over how to classify and recognize and validate us while I continue rebuilding myself TYVM.) Still, nice to see the fruits of his labor help move the ball a little bit down the field. And maybe make it easy to diagnose another small subset of the hEDS population with familial “tryptasemia” as he calls it. See his earlier recent update videos on my resources page.
Please note, the vast majority of Hypermobile type EDS cases even with MCAD of any flavor (masto or MCAS), POTS or other dysautonomia, or otherwise, still remain to be explained. Again, this only explains a small subset. Intro to my blog and me for new visitors…
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