After receiving a comment from a fellow blogger (find them here) they suggested I should explain more about #EhlersDanlosSyndrome and honestly I should.
What is Eds?
So Ehlers-Danlos syndromes (EDS) is a group of 13 individual genetic conditions, all of which affect the body’s connective tissue.This Connective tissue lies between other tissues and organs like a glue, keeping these separate whilst connecting them, this glue is tasked with holding everything in place and providing support.
In EDS, there is a gene mutation (like an X Man, the nerd in me loves this!!) that causes a certain kind of connective tissue, what kind will depend on the type of EDS (which type out of 13) (Hypermobile Eds, Hypermobility spectrum disorders, Classical Eds, Vascular Eds. They all fit under these umbrella labels)
This gene mutation is usually a form of collagen, this causes it to be fragile and stretchy. This stretchiness can sometimes be seen in the skin of someone with EDS (NOT ME) My skin has never stretched like that, but I scar and bruise incredibly easy. Individuals with the condition may also be able to extend their joints further than is usual (This is ME) this is known as being hypermobile, bendy or double-jointed. As collagen is present throughout the body, Everyone’s bodies, people with EDS tend to experience a broad range of symptoms, most of them less visible than the skin and joint differences. These are complex syndromes affecting many systems of the body at once, this makes it hard to connect and diagnose, despite this EDS is often an invisible disability.
Symptoms commonly include, but are not limited to, long-term pain, chronic fatigue, dizziness, palpitations and digestive disorders. Such problems and their severity vary considerably from person to person, even in the same type of EDS and within the same family.
Now it’s important to remember my #EDS isn’t the same as another #EDS as we are all individuals, and with that said it’s also why it’s incredibly hard to diagnose. We have a collection of symptoms, that are easily explained away and not connected.
I mean who thinks, velvet soft skin is connected to a thundering heartbeat because you stood up? So they came up with a series of movements to test for it.
The old way to classify Ehlers Danlos Syndrome was to use the Beighton Scale. The Beighton score is a set of manoeuvres used to judge if there is evidence of joint hypermobility. There are 9 points, and a score of 4 or more is considered a sign of there likely being generalised joint hypermobility present.
Now at the time of diagnosis in my early 30s I scored 8 of 9. Now there’s a more comprehensive way of scoring, found here Now I’m older and a lot more stiffer, so flexible I am not anymore. Therefore I would fail this tests above, but that doesn’t mean I don’t have it.
But these new ways of scoring takes that into account. So if you could touch the floor with flat hands without bending legs, bit no longer can you still score a point.
This helps the reclassification of certain symptoms, so I was diagnosed with EDS type 3 which is the Hypermobility type. Now it’s known as hEds Hypermobility Ehlers Danlos.
How does it affect you?
How does it affect me? Good question that I’m not entirely sure of myself. I know I feel pain all the time, from holding my own skeleton together and in the correct position. It’s so easy for my shoulders and hands to go beyond their natural range. Now I personally find this extra lax during my menstrual cycle, it’s like every joint I have comes out of joint, super super easy. It’s due to hormones creating the environment for this to happen around the cycle.
But as I said earlier it affects how I heal, so bones take longer to knit together, but likewise I’ll produce more scar tissue inside healing sites. Scars are often keloid or stretched and wrinkly. I bruise from being barely touched at times.
My three biggest ways I’m personally affected is the early onset of Osteoarthritis, my hip was grade 4, my mobility is awful, but I use to do gymnastics and horse ride. Now I can barely walk unaided. My hands are stiff and painful as they too have Osteoarthritis too.
My teeth are weak and crumbling, and dental visits are scary. For many years I have cried and complained that the anaesthetic doesn’t work, but no one listened to me, and then a few years ago they found it was an EDS symptom!! I have dentist now that knows about this and adjusts accordingly.
Finally how does it affect me, in a major way it screws up painrelief, I’m in pain but the meds don’t work. So fentanyl strongest drug out there? Nah doesn’t work, worst way to find out was when I had my first big op, it was a triple pelvic osteotomies and valgus femur lengthening. I was given Epidural (this does NOT work either) and Fentanyl, worst week of my life. But some gps out there, think you are attention seeking or drug dependant. You need these meds to function, to complete normal daily living, not to get high!
Since my first surgery, we’ve adjusted it to spinal, and morphine pump that I control. Now surgeries haven’t been as bad since thankfully, as I’ve had another 9. I find natural morphine better for me than synthetic versions of it that don’t work.
Now I’ve prattled on long enough, I best let you go. Hopefully by now you’ve learnt a bit more about Ehlers Danlos Syndrome. I’m still learning myself.