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.
And as I’ve said many times before and elsewhere on this blog, I do not think the collection of all forms of the Ehlers-Danlos syndromes (EDS) is at all rare, any more. Even if you subtracted the most common “garden” variety, hypermobile EDS, aka hEDS (and the newly defined Hypermobility Spectrum Disorders since 2017), for which we have no single genetic marker. (Unlike all the rare types, which do all have genetic markers now.) I still don’t think the remaining collection of forms of EDS is all that rare. Just rarely diagnosed. Truly.
Seriously. The more I serve the houseless in my local area (Portland, Oregon), the more I see signs of EDS and autism and ADHD out there. But folks are just too busy trying to just survive outside to be able to catch their breath, and recognize a systemic condition or three. (Though they quickly do any time I nutshell it for them.) And because the vast majority do street drugs to cope with their pain of all kinds (mental and physical and emotional), their doctors “treat ’em and street ’em” as fast as possible. Truly. (This explains one of my prior doctors’ behavior I now realize. She thought I was drug seeking and she treated me like absolute shit. Needless to say I switched doctors.)
Again, most get diagnosed with just depression, anxiety and the still poorly defined “fibromyalgia”, if anything. Or for one of our very many annoying and outright painful co-occurring conditions.
So few doctors connect the dots, and see the whole person in front of them, and recognize a more systemic issue going on. I don’t know why this is so hard. I saw it immediately once I learned about EDS in 2011. Boom. The penny dropped, and I had answers for my whole entire life experience. (Or most of it- MCAS, dysautonomia, autism and ADHD entered the chat a bit later as most of you know.) I could then see it easily in many friends and others. And still do.
But most deny it, or have never heard of it, or don’t want to. Like most of our doctors, they focus in on one or two issues, but fail to pull back and see the bigger systemic picture. Or, wrongly ascribe all their issues to any co-occurring autoimmune diseases if they have any. (And most do. It’s common in trauma victims.)
(From the website above: “Median Arcuate Ligament Syndrome (MALS) is a congenital anatomical anomaly that occurs in 15-34% of the population. It is caused by the median arcuate ligament compressing the celiac artery and the nerves of the celiac plexus, causing symptoms such as pain after eating, nausea, exercise intolerance, weight loss, and more.“)
And many who have Marfan Syndrome are misdiagnosed with EDS and vice versa. It is another connective tissue disorder that involves faulty fibrillin, not collagen. But results in very similar issues to EDS. Be sure to do proper differential diagnosis. (Tell the two apart, and diagnose Marfan if needed.)
Some common tell tale signs include: dislocated eye lenses (or lenses that move a lot), and congenital heart defects and aortic enlargement and aneurysms.
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!
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:
“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.”
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:
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.
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.)
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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Want to help keep this site as up to date as possible, and keep me off the streets? (Not joking – my rent is over 3/4 of my SSD income now, eek.)
I can really use your support here for as little as $2/month on Patreon
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 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. 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 recognizedHypermobility 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!