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Ehlers-Danlos Syndrome (EDS), Mast Cell Activation 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, MCD (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. 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!
Last updated September 12, 2023
News and Events
September is Pain and Chiari Malformation Awareness Month
As well as Suicide Prevention Month. Which sounds odd to me, since wouldn’t we want to prevent suicide every month? Thank my literal autistic brain for coughing that up. But seriously, I consider the work I do here to bring awareness to these far from rare conditions and many resulting issues and pain to be helping to waylay suicide for folks who have given up hope of being believed, otherwise. Or so I hope.
And, I consider suicide to be one of the leading causes of death in this community, from so much pain, invalidation, and medical gaslighting. Please stop calling EDS etc. rare, and start getting folks the help they need without so many obstacles. They are not rare, at all. Just rarely recognized and diagnosed.
If you are in the USA, you can call or text 988 for immediate help if you are feeling suicidal. Here is a list of suicide hotlines from around the world for other locations.
Chiari Malformation is another one of our much too common but also rarely recognized co-occurring conditions with EDS and HSD again, thanks to being considered rare. Well, it was one of the very first co-occurring conditions I learned about as a newly minted support group leader in 2012, so it can’t be that rare! It is also one of the hardest to recognize and diagnose properly too.
The most easy to recognize form, Chiari I, involves visibly dangling “cerebellar tonsils”, that is, bits of your hindbrain that hang down out the back of your skull, and may be getting impinged as they protrude out of the “foramen magnum“. Some doctors mistakenly believe that this is the only way you can have Chiari malformation. But this is not at all true, and why I’m trying to raise awareness about it this month.
It can lend to increased cerebral spinal fluid pressure, and various hard to measure and define neurological issues. Some symptoms include but are not limited to:
- Vision related problems (double vision, blurred vision, involuntary eye movements, excessive sensitivity to bright lights)
- Numbness
- Muscle weakness
- Stiffness or pain in the neck or the back of the head. The discomfort grows worse with coughing or sneezing
- Cranial nerve compression leading to apnea, and swallowing difficulties
- Diminished arm strength
- Diminished sensation in the arms and legs
- Issues with balance and coordination
It can be asymptomatic in some cases, where people, and they may never know they have Arnold Chiari Malformation. Others may experience lots of issues and pain but not show the most easily recognized signs, and thus go undiagnosed. There is some treatment for some cases, involving opening up the base of the skull a bit more, or sometimes cerebral spinal fusion, to help keep the head up off the neck. Not a casual undertaking at all, but I’ve lost track of the number of friends and people I’ve seen deal with this.
Pain is one of the most obvious and common symptoms and issues that comes with Hypermobility Spectrum Disorders and the forms of Ehlers-Danlos syndrome. It comes in so many forms and variations, it needs it’s own entire website to be properly represented. Many EDSers have fibromyalgia, and often get that diagnosed first or only, as doctors then just stop there, and write everythign else off to “fibro”. But this is a disservice.
I won’t say that all who have fibro have EDS or an HSD, but… I’ve yet to meet anyone who doesn’t have signs, personally. But, other pain is driven by weak or torn tendons, ligaments and muscles, IBS, mast cell activation (a biggy), subluxations and frank dislocations, headaches of all kinds, often multiple drivers simultaneously, and more. Again, too big to cover here.
And just to throw a wrench in the works? While some of us feel more pain than average for whatever reason (go me, sigh), some forms of EDS (like classical-like EDS, or clEDS) lend to lack of enervation of all the body parts, so some folks actually feel LESS pain than average, and injure more easily or worse without even realizing it for this reason. (I joke that I got some of my friend’s dendrites, and she can have them back!)
This is NOT the benefit you might think it is – they can and often do permanent damage or even die for this reason. And, as my friends with Familial Dysautonomia (Riley Day syndrome) can attest, you may not be able to even regulate your blood pressure or oxygen levels properly from this. Which can prove lethal if not properly monitored or treated. (Two fo my friends need 24×7 oxygen now, sigh.) I would love to lend them some of my dendrites so we could both just live in peace. Alas, no dice.
So, careful what you ask for, you may get it, but with more problems than you might think. To your health.
~~~~~~~~~~~~~~~~~~Left over from May in case it’s useful still for some doctors:
ETA May 17, 2023: Check out these two new papers intended to guide medical practitioners in recognizing, diagnosing and treating hypermobile patients with chronic pain:
Hope for Hypermobility: Part 1—An Integrative Approach to Treating Symptomatic Joint Hypermobility
Hope for Hypermobility: Part 2—An Integrative Approach to Treating Symptomatic Joint Hypermobility
Daylor, Victoria BFA; Gensemer, Cortney PhD; Norris, Russell A. PhD; Bluestein, Linda MD
They are only available free until June 15th, I believe, so grab them now! (You just have to pay after that.)
To your health. Jan.
***
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!
***
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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January 2023: I am now also on Mastodon via the Disabled.social server as @ohtwist since #Twexit began in earnest October 2022. (I remain on Twitter still too though.) Bear with me as I try to keep all these plates spinning while disabled and living alone in pain thanks. (It’s a lot! Including Facebook, too.)
Good News! As of late January 2022 followers will be pleased to learn that the EDS Toolkit for Doctors 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:
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 in 2020
COVID-19 emerged on the world stage in early 2020, and is continuing well into 2023 now as you all know. We’re just letting it rip now, in February 2023, much to our chagrin as often immunocompromised patients, vaccinated or not. After a minor summer dip, numbers are cratering back up as of September 2023 as school goes 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’m now planning to start 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.) Stay tuned. (It will be under my resources tab, once born.)
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 “infect” 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.
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!