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Ehlers-Danlos Syndrome (EDS), Mast Cell Diseases (MCD) and Dysautonomia – Oh! That’s Why I‘m So Tired!!
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 intertwined systemic conditions I personally unofficially call the “Chronic Constellation” for lack of a better name.
Find Emergency Information and “Cliffs Notes” for Doctors Here <–
(Including a bonus dental freezing / numbing formula that’s tailored to EDS patients.)
Each syndrome alone is enough to take a person down at the knees, but combined as we so often find these, they can be downright show stopping, if not even lethal sometimes, sadly.
Oh, That’s Why I’m So Tired!!
Yet barely 10% or so who have this “trifecta” of a form of Hypermobility Spectrum Disorder or Ehlers-Danlos Syndrome, Mast Cell Disease *(any type) 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 over 25!)
Most are currently getting diagnosed with arthritis, Fibromyalgia or ME/CFS, depression and anxiety if anything at all. They may have all of these diagnoses, but the doctors stop short of seeing an underlying connective tissue disorder and hypermobility, so miss their EDS or HSD to be clear. This site is my attempt to help remedy that for everyone – both patients AND doctors!
Besides my standard pages in the menu above, you might like these Selected Posts and FAQs to start too. They feature my best, most popular posts that are not linked in the menu but are also helpful.
Hypermobility Spectrum Disorders and EDS aren’t rare, they’re rarely diagnosed!
* Editor’s note: I realize my site still refers to the outdated term “MCAD” in several places, including in my menu and links. I’m slowly working to update this, but it’s tricky- I’m back linked in several places, so a bunch of links will break once I change page names and URLs. Thanks for y our patience! MCD is now the proper umbrella term for all forms of Mast Cell Diseases, whether they involve (over) activation or not. Including MCAS, just one form.
Last updated July 4, 2024
News and Events
BREAKING NEWS! June 11, 2024: The Norris Lab just released the preprint of their genetic study which found a Kallikrein gene to lie behind some cases of hEDS…
Follow Chronic Pain Partners for more and to keep up with the latest EDS news and views.
July is Disability Pride Month
As well as, apparently, “Wrath Month“, pun intended. I only just saw someone tweeting about this for the first time this year, but apparently it’s been a thing in the Pride community since 2017. And is intended as a bad pun on the Seven Deadly Sins, as Wrath follows Pride. (So, Wrath month follows Pride Month right?) Heh. That said, I’m watching my elders in the disabled community speak of wrath over still having to fight for BASIC INCLUSION in everyday activities: work, school, entertainment, eating. Because the ADA absolutely is NOT enforced and in action everywhere. And, no, you cannot “just call the ADA”. There IS no ADA “to call”.
Like, literally. No one is coming for us. If you still have stairs and no lifts or ramps to your establishment, we can’t sue you or call anyone else. We just have to bypass you or miss out – again. And in an emergency? Most of you just leave us to rot or burn. This is not equality, at all. Not even close.
Further, as COVID19 is resurging (it never went away, it just subsided and got less deadly as vaccines rolled out and is on the rise again big time), many who are immunocompromised are STILL feeling quite overlooked and left out. Because, while you may have had a very mild course, or not gotten it, many of us have not fared so well. (Some have even died.)
It’s been proven that hypermobile folks with EDS are more prone to Long COVID. One of my best friends with clEDS actually developed not one but THREE autoimmune diseases after getting COVID19 (Celiac disease, RA and dermatomyositis, which remodeled the walls of her heart to be thinner leaving her on 24×7 oxygen, no joke). So, yeah, we’d like you to mask up to flatten the curve again please.
Another dear friend is fighting for her life with COVID triggered cancer – AGAIN. She has to literally beg for funding for she and her family to afford the monthly chemo infusions keeping her alive. It’s a curable cancer, but not if she doesn’t treat it. And, it’s preventable: if you stop spreading COVID!
Disabled lives matter. All of them. Not just those you can see or use to provide inspiration porn. But all those locked away in our beds and rooms trying to survive safely, while managing often complex conditions with very few resources. Please throw us a bone and show us you care by wearing a mask if you can, thanks. (We know, not all can.) And at the very least, not harassing or hassling us when we do.
We’re tired. And getting more tired as the planet heats up, and our resources dwindle. So, I think Wrath Month fits with Disability Pride Month now, too.
ETA June 26, 2024: I just finally cooked up my “How to Diagnose Mast Cell Diseases” page after only ten years, smile! Finally! Behold, my bouncing baby MCD diagnosis page. (We even hatched a whole new diagnosis since I first started – HaTS. See the page for more on that.)
ETA May 18, 2024: This study freshly published by the Norris Lab at MUSC (first shared on socials May 17, 2024) helps to highlight how poorly the 2017 hEDS diagnostic criteria describe the hEDS cohort. But, I still humbly submit that EVERYONE (including the Norris Lab and TEDS) are missing the elephant in the room: you aren’t seeing many boys and men in the studies because the criteria are based on the phenotype of a thin white woman. And social pressure keeps boys and men away from the doctors, who also probably dismiss them just as much. And let’s not forget non-binary folks too.
So, gee, no surprise, you mostly get women in your studies… (much like how the diagnostic criteria for autism are based on white boys in Germany 70 years ago right? And miss girls and non-binary folks too.)
~~~~Breaking news from March / April 2024:
(In a surprise to almost no one…) People with joint hypermobility are 30% more likely to suffer from long COVID, according to a new study by Brighton and Sussex Medical School (BSMS) and King’s College London and published in BMJ Public Health. In addition, this is likely related to higher levels of fatigue in this group.
Ron Davis and family (Whitney and Janet Dafoe) have finally published some findings showing a connection between the immune system and MECFS in a hypermobile patient with some potential for possible treatment here. (April 2024.) Here is some interesting discussion of this paper by the MECFS community.
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I sometimes feel like I’m just collecting diagnoses on this site. But I truly couldn’t make this stuff up if I wasn’t witness to them all in massive online groups (60,000 in one, almost 100K in another now, no joke, and plenty on Twitter) and in real life every day.
That’s why I called the whole kielbasa (collection) The Chronic Constellation here back in 2016, for lack of a better name. I “see” more patients than most doctors do, just socially, but with the advantage of more time and less pressure so we can share more than is often allowed in the doctor’s office. That’s why I’m sharing so much here – to give all you doctors a leg up!
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I use the space above on my home page to highlight some of our more common comorbidities – aka co-occurring conditions every month. We have many, so you will see many through the year!
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Good News! As of January 2022 followers will be pleased to learn that the EDS Toolkit for Doctors (aka the “GP Toolkit”) formerly hosted by the Royal College of General Practitioners in London since 2018 has indeed been saved and is now hosted by Ehlers-Danlos UK here:
https://gptoolkit.ehlers-danlos.org <—NB that’s “dot org” at the end, not “dot com”, which would bring up TEDS, an entirely different organization.
“Development of this toolkit in 2018 was led by Dr Emma Reinhold, with contributions from pharmacist Lisa Jamieson MSc, Prof. Lesley Kavi, Dr Hanadi Kazkaz, Dr Alan Hakim, Nikki Paiba, Dr Gemma Pearce, Dr Philip Bull and Jan Groh. The toolkit was hosted by the RCGP between May 2018 and November 2021.”
EDS In the News:
‘Despite appearances, I finally realise I am not able-bodied’: novelist Daisy Lafarge on her hypermobility disorder – Daisy Lararge in The Guardian May 12, 2024
Calhoun couple fights to bring son home after they say his medical diagnosis led to abuse charges via Atlanta News First March 30, 2024 – Follow Chronic Pain Partners for more about the upcoming movie ‘Complicated’ being released later this year will be addressing this very topic. (Not necessarily this family.)
Woman accused of faking symptoms of debilitating illness dies aged 33 via Independent UK September 5, 2023 (How a young Auckland support group leader was gaslit about her very serious EDS and POTS symptoms and ultimately died much too young.)
Doctors missed my debilitating condition for 20 years – I diagnosed myself after watching a TikTok May 25, 2023 Metro News UK
Folate-Dependent Hypermobility: Researchers at Tulane’s EDS Clinic Look Into New Possible Mechanism For Hypermobile EDS May 2023 via EDS Awareness (a more sober take than put out by PNN last month)
‘Written off’ – call for more help to get millions of long-term sick back into employment (featuring an English woman with EDS) via Sky News March 2023
Ramon S. Barthelemy talks about coming out “again” with his hEDS via the Salt Lake Tribune, March 2023
Billie Eilish: Singer says ‘I feel like my body was gaslighting me for years’ January 4, 2023 via Sky News
CNN once again highlights Ehlers-Danlos in an article from a writer whose daughter has EDS asking: “Why isn’t the medical profession paying more attention?” December 2022
Jameela Jamil Shared She Has Ehlers-Danlos Syndrome. Here Are The Symptoms Of The Rare Condition via Buzz Feed news December 2022
Revenge of the gaslit patients: Now, as scientists, they’re tackling Ehlers-Danlos syndromes Dec 12, 2022 by Isabella Cueto via STAT News
EDS Awareness aka Chronic Pain Partners featured Guinness World Record holding para climber Anoushé Housain in their December 2022 Newsletter.
The Ehlers-Danlos syndromes, Fibro, MCAS and dysautonomia are finally recognized to possibly be behind or go with some cases of ME/CFS in this article in The Atlantic about Long COVID September 2022 (Ed Yong)
Marvel’s New Spiderverse Hero Shows the Struggle of a Real Disease – September 2022 via Screenrant (Follow Sunspider’s creator who has EDS on Twitter at @MizTeeFranklin.)
Two medical doctors share What a lifetime of gaslighting by other doctors feels like via KevinMD June 2022
Halsey reveals new health diagnoses after hospital stints: ‘I’m still looking for answers’ – Hannah Yasharoff via USA Today May 10, 2022
Phoenix’s Fox 10 covers EDS patient and research scientist Dr. Cortney Gensemer’s story at MUSC April 2022
In which the Washington Post gets EDS mostly right for a change in this nice writeup on fellow patient Dr. Zingman’s journey December 2021
As well as The Guardian here, in February 2022
Via CNN travel edition January 2022: Another patient uncovered her EDS and MCAS after a spinal fluid leak derailed her life in 2017
The Emergence of COVID-19 Worldwide since 2020
COVID-19 emerged on the world stage in early 2020, and is continuing well into 2024 now as you probably know. We’re just letting it rip now, since February 2023, much to our chagrin as often immunocompromised patients, vaccinated or not. After a minor summer dip in 2023, numbers shot back up as of September 2023 as school went back in session in the northern hemisphere at least.
The actual death rate may be down now thanks to vaccinations, but… many of us are still having to lead isolated lives still shielding due to our fragile health and primary immunodeficiencies many have. We humbly ask you to wear a mask if you can in any crowded public spaces for our sakes, thank you. (They do help.)
Here is what The Ehlers-Danlos Society advised in 2020 for what it’s worth. (Mostly WHO guidelines without specificity to EDS.)
A couple of other articles of interest to our community in particular:
- Covid-19 hyperinflammation and post-Covid-19 illness may be rooted in mast cell activation syndrome 2020 Afrin, Weinstock, Molderings
- COVID-19, Mast Cells, Cytokine Storm, Psychological Stress, and Neuroinflammation – Kempuraj, Selvakumar, Ahmed et al July 2020
- Orthostatic Intolerance after COVID-19 Infection: Is Disturbed Microcirculation of the Vasa Vasorum of Capacitance Vessels the Primary Defect? December 2022 Wirth and Lohn
January 2023: I’ve hatched a COVID Resources page as well now, as the EDS/HSD community seems to be disproportionately impacted by Long COVID, with significant overlap with the ME/CFS community. (Who are finding increasing overlap with the world of connective tissue disorders, hypermobile or not.)
Indeed, evidence is mounting that we are disproportionately affected by this virus as I feared. (March 2024)
Please take care not to “whale watch”, that is, go leaping from one highly touted solution to another. “Oh look, a solution over here!” “Oh hey, there’s another one over there! Ooh!” leading to “wildly careening about the boat” so to speak. For COVID or any other condition.
We are all desperate for answers and “cures” but this is how snake-oil salesman make their livings. Pause, and gather data. And consult YOUR doctors and practitioners first. And listen to your own bodies. Always. There are no panaceas, sorry.
And, as of 8/11/20, per this article, it appears your home made cotton masks DO help some, so keep wearing them if you have nothing else. It’s helping.
ETA 1/3/23: I now highly recommend a KN95 mask if possible though for better security against Omicron, XBB.1.5 et al. But, any mask will still help reduce your viral load, truly. So whatever you have will still help.
Ongoing news
Good news, Project EDS ECHO for doctors was rolled out in May 2019, and is officially underway. This should really help to update more doctors with the latest information and insight into the forms of Ehlers-Danlos syndrome and Hypermobility Spectrum Disorders, so patients don’t have to do all the heavy lifting anymore! It’s found here: http://ehlers-danlos.com/echo .
So far over 1000 doctors have signed on and earned CME’s for participating, thank you! (As of 2022.) The Ehlers-Danlos Society hopes to reach as many as possible every year. (New “sessions” roll out periodically.) Help us reach that goal by sharing it with all your doctors! (Along with the EDS Toolkit for doctors freely available here too.)
EDS in Celebrities
Yes, I know that Sia, Jameela Jamil, Halsey and Lena Dunham all have EDS, among others. Apparently including the late Elvis Presley per this post in 2021. I’ve long suspected he and the late Prince, David Bowie, Marylin Monroe and Michael Jackson myself. (I think MJ was confirmed somewhere?) ETA: Billie Eilish spoke up too via Sky News in January 2023.
Chronic Pain Partners (aka EDS Awareness) started a slowly growing list of Popular People with Ehlers-Danlos Syndrome in 2023 they periodically update. Keep an eye on that page for more over time. (It is not comprehensive to be clear, just some of the higher profile folks we know of who are active on social media.)
Hypermobility lends to great vocal ranges and agility, dancing ability, and… chronic pain after all. I wouldn’t be surprised if we continue to slowly uncover more. (Or they uncover themselves, smile. I’m also suspecting Lady Gaga and Celine Dion now too.) And I mean outside of all the acrobats who appear annually on all of the talent shows, smile.
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The International 2017 EDS Classification via The Ehlers-Danlos Society
For those unaware, this is the FIRST update since the Villefranche nosology was presented in 1997 (over 20 years before!), and this also supplants and replaces the Brighton (with an “r”, not “e”) Diagnostic Criteria for Joint Hypermobility Syndrome which some also used to diagnose hypermobile type EDS also. So there will at least be less confusion between Brighton and Beighton going forward, even if many of us no longer count as bendy!
UPDATE 4/10/18: There’s a new 14th type out now too. No doubt more will continue being uncovered with time. (July 2023: we are still eagerly awaiting the SNP news from the Norris Lab, among others.)
You might wish to refer to this FAQ document previously shared by The Ehlers-Danlos Society for clarification on the new 2017 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 several Hypermobility Spectrum Disorders for 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. We just changed our minds about it.
These forms of HSD also all supplant and replace the former diagnoses of Hypermobility Syndrome, Joint Hypermobility Syndrome, and Benign Joint Hypermobility Syndrome according to TEDS. I like to think of the HSDs as the less visible bulk of patients “below the water line” as shown here:
I, Jan, unscientifically personally now feel very strongly that the newly recognized Hypermobility Spectrum Disorders as such are NOT rare at all, but also just rarely diagnosed – and not just because they are newly introduced and poorly know about yet. 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!
We are the frequent flyers in all medical offices, after all!
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 also EDS. I will continue to post and tweet to that end, while I finish writing my book. Join me.
I can use your financial support at any level to help keep this blog online here thank you!