What is EDS?

EDS stands for Ehlers-Danlos Syndrome. In technical speak it’s a genetic defect of the Type II Collagen. There are different ‘types’ of EDS, broken down into categories of symptoms and doctors/geneticists lump you into the ‘type’ that lines up with the majority or the severest of your symptoms. This can lead to more confusion, however, because most people with EDS have cross over symptoms of the other ‘types’.

So, in non-technical speak- what is EDS? It’s something different for each person that has it, to the degree they have it and even to the degree that each of the individual symptoms of it manifests. To understand EDS you have to understand that Collagen is ta substance in the body that occurs naturally. It’s responsible for making things stretchy, by things I actually mean EVERYTHING. Think for a moment on everything in your body that stretches. The most obvious are tendons/ligaments that allow your joints to move. With out stretching your arms wouldn’t bend, nor your knees. That is obvious enough, right? Okay, now spread your mind out further and try thinking of other things that stretch. Collagen is a major ingredient in the cosmetic industry because collagen is what makes your skin stretchy. As you age you loose your collagen and wrinkles form. The often over looked stretchy things are blood vessels, eyes and internal organs. Think of how a doctor measures your blood pressure, it is by the expansion and contraction rate of the blood vessels as they carry the blood in pulses away from and back to your heart. Without collagen those vessels would not expand and contract.

Now, the basics aside, people with EDS actually have too much collagen. Things are too stretchy. Probably the most dramatic of the types involves the skin and leads to the person being able to pull their skin away from their underlying structures. It has been featured on television shows and medical mysteries series. It’s very easy to see and understand that there is something obviously not ‘normal’ going on. In the other cases of EDS, it’s not nearly so easy or clear. The vascular type mainly affects the blood vessels and these people are very prone bleeding, scarring and sudden death due to aneurysms. This type killed my father when he was only 29, although it was not discovered until my own diagnosis that this was the case.

In hypermobility type, which is the ‘type’ that I fall into, the joints are all very loose and dislocate or sublex (partially dislocate) very easily. This type is often seen in circus shows with contortionists who bend in ways that make a normal person cringe. Beyond the obvious joints that are affected, knees, elbows, etc, there are the ones that people do not even consider to be joints. These include the small bones in your feet and hands or your ribs. A bear hug can literally cause me hours of agony by displacing ribs. I personally dislocate just about everything to some degree, my most stable joints are actually my elbows, which is opposite of the ‘standard’ hypermobility case.

As I have aged my joints have become more unstable, and bouts of extreme stress seem to accelerate jumps in the progression. I have had difficulty all my life, but because EDS was considered to be so “rare” it was never even discussed. I do recall one doctor saying I was far to hypermobile when I was very young. I was having difficulty in gym class and my gym teacher gave me an ‘unsatisfactory’ mark, when asked to explain why, he said it was because he’d never seen a skinnier lazy kid. Every time I was asked to do something that involved running, I would run a bit and fall down. His assessment was that I was falling down to get out of the running. The doctor’s assessment was I was hypermobile and having trouble with actually physically running. The problem followed me through my entire school career.

About 10 years ago I underwent a situation of extreme stress that seemed to set in motion a rapid progression of some symptoms. My joints became more unstable, my skin thinner and easier to scar and tear, bruising came with slight touches. It has progressed in fits and starts since then, with my hip becoming the largest ‘problem’. After two years of trying to figure out why surgeries didn’t help with my knee hyperextensions and why my hip would just not stay in place I ended up at the Mayo Clinic. There was diagnosed in the Genetics Dept has having EDS, hypermobility type. It opened up a whole new world. I learned that the things I had been experiencing were common problems with EDS’ers and I began to feel less and less like I was totally alone with this. The problem is that EDS is still considered to be a ‘rare’ defect so it’s not taught in medical schools outside of a short paragraph in a book. Trying to find a doctor that even knows what EDS is, much less what to do and not to do with it/for it is a real challenge.