I found actually starting this post really hard, mainly because where on earth do I start? So I thought well maybe I should just take this list that I posted the other day, and work my way through that list in that order (https://myheds.wordpress.com/2016/01/02/the-parts-or-pieces-to-the-whole-that-make-up-eds/)
So the first thing on the list is Hyper-flexible joints… Joints which moved beyond the range of “normal”. Children sometimes refer to them as “double-jointed” (which is completely wrong, no double anything, no extra joints!), medically they are referred to as Hypermobile, or Hyperflexible. (note: it is possible to be very flexible or have some Hyperflexible , without having EDS.)
Here are some pics of Hyper flexible joints in my family:
Not everyone with EDS is Hyperflexible. Not all joints in an EDS or Hyperflexible person are Hyperflexible. Some joints that are Hyperflexible are not visibly so (bend a knee backwards and it obvious but not so for jaw or TMJ, neck and spine, hips etc).
Joint are Hyperflexible because we have tendons and ligaments which connect our muscles to our bones, and they kinda hold things all together and keep the joints stable. When they are too flexible, the joint bends further than it is designed to. Hyper flexible joints are usually not painful to start off with (hence kids who have them often show them off for “fun), but over time the joints become irreparably damaged. Cartilage wears out faster than usual, …. causing arthritis. Tendons themselves tear and heal with scar tissue, the scarring building up and seeing as scar tissue is made from connective tissue and with EDS the connective tissue is faulty… well, you can see that this all a recipe for disaster! The joints either stiffen up or become more and more lax, become more and more painful, and when they become worse, they start dislocating and subluxing (partial dislocation) which as you can imagine is also extremely painful! I’ll get into the complications and dislocations etc in another post, for now we are just doing the Hyperflexible thing.
Specifically for my family:
The photos above are from some of my family, but for the most part we never thought of ourselves as Hyperflexible. There has been no “double jointedness”, and nothing obvious. But then again we weren’t exactly looking!
Specifically for me:
My husband has always thought that some of the angles that I bend are … um … unusual and some of them freak him out, …. but in general I don’t look like a super bendy person! I paint a lot and when I get carried away and spend hours on end with a paint brush, the joints of my fingers bend right back… which is creepy of course, but it is also extremely painful, and scary! they don’t bend back on their own … AND, they ache for months, and when I knock them on things (which I do a lot) they hurt like hell and flip backwards when I pick up things, and so for decades I would strap them up with band aids, and curl them in to protect them…
Also I have always needed to put my feet up whenever possible (now I know why) and when I do so I could never work out why I needed to put a pillow under my knees otherwise they would soon hurt a LOT, and very much like the finger situation. I could also never work out why I can’t sleep on my back (why is REALLY annoying seeing as I have other issues which make side sleeping a no no … wait for the post on that) … and basically it boils down to the fact that on my back my knees take strain because gravity (which I have always said hates me!!) means that my knees sink down and turn slightly the wrong way around resulting again in the above issue! I need to keep my knees bent at least slightly, all the time, so that the tendons don’t stretch and ache and the joint over stretch the wrong way! And as you can imagine, having my knees REALLY bent as far as possible also stretches the ligaments the other way…
I have also learnt that the reason my family is not so bendy, and we have such strong muscles, is that our muscles are the kind that try to compensate for the hyperextension! Which means that they work HARD all the times except when we are asleep. So some people with EDS don’t have this (the majority) and so their bendiness and resulting joint damage is way worse than ours, but they don’t have the long term muscle fatigue and pain that slowly gets worse over the years 😦 … so it’s a bit of a trade off, and one that we don’t get to choose.
So the bottom line is that bendy is not a medical emergency, but what happens inside the joints is. We need to keep our muscles strong but also teach them to relax (in the pool is a great way), and braces for the joints help too. Compression stockings when they aren’t TOO bad helps, and being super careful when we get around, and not mention we have to avoid too much twisting and turning (which is harder to do than you think! … we spend almost all our mobile time doing it without thinking). One of my jobs on this list is to contact the Hand Specialist who will make some braces for my fingers and hands so that I can paint and work without my fingers bending backwards, and using my crutch when ever I am out the house keeps me walking straight, spreading the load, and most of all I need to wear comfortable shoes and stop the second my body says no more!