They way I explain EDS has changed the more I’ve learned about it. I started out thinking it’s “just double jointed but with subluxations for spice!”

When I talk about it now, I stress that it’s a “connective tissue disorder” and the most obvious external symptom is hyper-extending joints and subluxations, but that it’s actually a systemic problem. It affects every part of my body and very corner of my life!

Wikipedia says “Connective tissue is one of the four basic types of animal tissue, along with epithelial tissue, muscle tissue, and nervous tissue.” (https://en.wikipedia.org/wiki/Connective_tissue). Connective tissue is everywhere—it’s between other tissue types everywhere in the body, including the nervous system. It includes things like ligaments and tendons, but also the walls of vessels and organs. 

The Ehlers-Danlos Syndromes are a collection of 13 related connective tissue disorders, and each one affects a different aspect of connective tissue. They are genetic, so I’ve had it my whole life and there is no “cure”.  It isn’t really an “illness” (although I will use the term “chronic illness” because that captures the lived experience of the situation) but rather is just how my body is made. The genes encoding how to construct the connective tissue are faulty somehow and the collagen they make doesn’t perform the way it should. There is no direct treatment or way to fix this since I’m stuck with the genes I have (and collagen supplements don’t help).

Some types have extremely stretchy skin, while other types of EDS have serious issues with organ and vessel integrity (and organ rupture or vessel rupture can happen). I have the hypermobile type which is the most common of the 13 known types of EDS and (luckily for me) isn’t associated with the vessel and organ rupture (those happen in vascular EDS).

EDS affects joints, skin (stretchiness, bruising, scarring, and general fragility, including wound healing), heart valve prolapse, autonomic dysfunction (POTS/blood pooling, Reynaud’s, poor temperature regulation, altered sweating), GI problems (GERD, hiatal hernias, slow motility), and organ problems (prolapse or other sagging). The joint problems lead to muscle and fascia problems.

The history of EDS and HSD (hypermobility spectrum disorder) reveal that it’s “an area of active scientific research”, which is my way of saying “things keep changing and we have a lot of unknowns”. HSD used to be called BJHS: benign joint hypermobility syndrome (this is what I was initially diagnosed with in 2019). I know “benign” has a technical medical definition, but it’s a very frustrating (and patronizing) term to hear as a layperson. There is *nothing* benign about a life filled with hundreds of joint subluxations, intense fatigue, daily muscle spasms, dental nightmares, and prolapsed organs. I get that it’s not imminently fatal like vascular EDS, but it’s also not benign in the common meaning of the word (“gentle and Kindly”, “does not threaten health”, “having no significant effect”, “not dangerous or serious”, “not harmful”, “does not have harmful effects”). I get mad when I think about it because it’s already hard enough to get diagnosed, and that label feels like another layer of “this really isn’t a big deal, and we don’t know why you’re complaining about it”. 

So, they changed from BJHS to HSD, and EDS has gone from just a few types to now being 13 types. HSD and hEDS have an enormous amount of overlap, and many consider them to be the same thing. In 2017 they made the criteria for hEDS much more restrictive hoping that by catching only the most ‘serious’ cases they would be able to find the gene underlying hEDS. This is the diagnostic criteria as set out in 2017
https://ehlers-danlos.com/wp-content/uploads/hEDS-Dx-Criteria-checklist-1.pdf

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