At this point there is disagreement over whether hypermobility syndrome – HMS – is simply a subclass of Ehlers-Danlos syndrome or a separate disorder. What is known, is that the problem of generalised hypermobility (where it affects all joints, and is not just an issue of shallow sockets) is a problem of collagen. While it is constantly invoked in skincare ads of doubtful veracity (this amazing serum will stimulate the production of collagen! Sure it will. Sure), it is actually a fairly important protein (edit: collagens are actually a whole class of diverse proteins, but for simplicity’s sake, I’m referring to it as the one item). Collagen is a major component of your connective tissues: skin, ligaments, tendons, and so on. It needs to be reasonably strong in order to keep bones and muscles attached where they should be, but it also needs to be reasonably flexible, in order to allow them to move.
In the case of hypermobility, there is a genetic flaw in the production of collagen (thanks, Mum). It is too flexible. For example:
I thought it was normal to be able to pull out stretchy skin (note: hyperelasticity is not a feature of hypermobility- generally it’s associated with other forms of EDS. However, people with EDS/hypermobility class or HMS will generally show more elasticity in the skin than most people).
I thought it was normal to be able to bend your wrist back past ninety degrees, or your knees and elbows back past zero (hyperextension).
I even thought it was awesome that I could bend over and put my hands flat on the floor. I thought it meant I had flexible hamstrings. Sadly, it just means I have a very flexible lower back, and that this stretch is just going to do me damage in the long run unless I consciously “hinge” from my hips (at which point I actually am stretching my hammies, and not my back).
…although I suspected that my very bendy feet had something to do with the mysterious plague of sprained ankles that has haunted me throughout my life.
I’m not that severe, to be honest. My joints shift and subluxate (partially dislocate) but don’t fully dislocate (with the exception of my kneecaps, which have wandered off far too many times for my comfort), and unlike some people with HMS, I’m not in constant, severe, chronic pain.
It’s still bad enough to cause me injuries. Hypermobile people tend to sprain things. They tend to experience overuse injuries. More importantly, it’s quite common for some very useful muscles to simply not activate the way they should, because they are not properly supported.
Knock-on effects due to leverage
It all comes down to angles.
If, as a small child figuring out how to walk, your joints are lax, then your legs are going to move at a different angle than they are “supposed” to. Instead of staying straight, your knees might fold inwards and forwards due to loose hips. This can in turn send your ankles outwards. What this means, O bendy small child, is that you aren’t actually using the same muscles that everyone else uses, even when you’re trying to do the same thing. Muscles activate in response to a particular angle of pressure.
An easy example is the upper arm. When you want to curl your arm up towards yourself, you activate your bicep. When you want to extend it, you activate your tricep. That’s a really obvious one, but when it comes to hips and back and shoulders, there are a whole bunch of medium sized muscles that kick in due to different angles of movement.
This means that the muscles that bendy child is supposed to be using don’t develop properly, or at least not to the same extent that they do in non-hypermobile people. We end up with sway backs, weak core muscles, and butt muscles that just don’t do what they’re meant to. I can activate some of these muscles, but it has required physiotherapy, clinical pilates, and sheer bloodymindedness to get them working, and I’m still not very good at it.
These are called “bad motor habits”, and they have long-term consequences.
Why does that matter, as long as you get from A to B?
Well, setting aside that one gets a certain amount of heckling when one spends one’s childhood and adolescence “walking funny”, it turns out that you end up putting pressure in places that weren’t meant to experience it. This leads to sore backs, shoulders, and joints in general; a tendency for joints to buckle unexpectedly; and an overwhelming tendency to fatigue.
I am not good at holding a position. Anyone who has sat next to me at a conference (or in a lecture, or on a plane…) is probably aware of this. I get comfortable. Then I shift. I cross my legs. I straighten them. I lean forward. I sit back. I turn to the side. And generally, I take a seat at the end of the row so I don’t bug people (ever since I realised that not everyone does this). Why is this to do with HMS, though? Couldn’t I just be fidgety?
When most people maintain a seated position, they have their ligaments holding their joints in place with very little effort (if any) on their part.
My stretchy ligaments aren’t going to hold jack shit in place, so I end up activating a whole bunch of small (underdeveloped) postural muscles to hold a seated position. These muscles get very tired, very quickly. It is very, very tiring for me to hold a position, so I am constantly shifting to work different muscle groups.
This is a special kind of hell on long-haul flights. Everyone else seems to be able to sit upright with their feet on the ground whereas I desperately want to lock my feet up against the back of the seat in front (something of a faux pas I understand if one is older than six years of age) because otherwise sitting up requires me to use core muscles that, in my case, suck and it is miserable and painful and exhausting. It is far more comfortable for me to just fall into my joints.
Not only that, but when you have HMS, you are always, always thinking about what your body is doing and how it is doing it (case in point: while typing that sentence, I just hooked my right ankle around my left shin to lock it in place). If you don’t think about it, even just in the back of your mind, that’s when ankles roll and muscles tear and you trip over your own goddamn feet (again). And that’s pretty tiring and distracting too.
I get tired when studying from having to shift positions over a textbook. I get tired when writing in a notebook from having to constantly change the angle of the book against my arm.
Why yes, HMS is linked to chronic fatigue syndrome. When sitting can make you ludicrously tired, fatigue is just a given. I actually find that a good gym workout is, in many ways, less fatiguing than sitting up on the couch. It’s still tiring – obviously I’m deliberately working to develop certain muscle groups – but the machines support all the flaily, falling-over bits of me while I work the target group.
I repeat: up until recently I thought all this shit was normal. It’s thought that between 5 and 10% of the population are generally hypermobile but don’t necessarily experience all these symptoms: you can be generally hypermobile without experiencing HMS.
There are other symptoms that are even less fun than being somewhat bendy and spraining the odd ankle (edit: I have a sprained ankle at the moment. The synergy, it burns).
Collagen, unfortunately, is all through your body. This means that HMS and/or Ehlers-Danlo are systemic conditions – having excessively elastic connective tissues leads to problems in your gut (weak intestines which in my case result in the occasional genuinely agonising gut cramp), problems with airways (tendency to asthma), problems with blood vessels (low blood pressure, orthostatic issues as in a tendency to pass out), problems with vision (yes, there’s collagen in your eyes; HMS is linked to myopia a.k.a. shortsightedness) and problems with various components of your autonomic nervous system – that is, the bits of your nervous system that are supposed to do things without having to be told. Some people can have seizures. Some people have temperature anomalies (hot head, cold feet, etc.).
What is common to most people with some form of this condition is chronic pain. I’m not sure how I got lucky on that one. I don’t have chronic pain (or if I do, it’s so mild that I’m obviously disregarding it).
At the moment, I have a sore left ankle bone on the inside, a weird stabbing pain in my right toe for no reason I can think of, shoulder pain and back pain. None of these pains are serious enough to even need ibuprofen, let alone anything stronger. For many who have HMS, however, the pain appears to have no cause and is a function of an over-sensitised nervous system.
So what does this all mean?
The problems that I have because of this condition are problems that I’ve had all my life. Although I’ve been tired enough to miss out on things from time to time, I don’t have chronic fatigue, and although I tend to be a bit achey and sore, I don’t have chronic pain. The only reason this is an epiphany is because it explains so many weird and odd things about my body in a rather elegant way.
But… HMS has implications for pregnancy and childbirth. The collagen problems are exacerbated by exposure to progesterone, leading to pelvic girdle pain, even looser joints than normal, and all sorts of problems in childbirth. You have a much higher chance of experiencing permanent damage throughout the process. Husband and I have been considering adding to the population some time over the next year or so, and in that light, suddenly a caesarean is starting to sound pretty good (because who wants their pubic symphisis to permanently separate? Freaking no-one, that’s who).
Also, the agonising gut pains… they’re pretty bad. To give you an idea of the pain, I was checked out for gallstones, kidney stones and appendicitis (none of which I appear to have). My vision goes grey. I’ve actually passed out from it. It’s like being stabbed (I imagine). It doesn’t happen often, but when it does, Jesus fucking Christ, it hurts.
Can any of this be fixed?
Well… not really. It’s genetic. It can get better, though, through rigorous exercise designed to strengthen the muscles supporting the loose joints and to stabilise and activate those neglected core muscles (at least one of which, apparently, everyone else activates automatically without even thinking about it, whereas I was staring at an ultrasound, randomly flexing, trying to figure out where the damn thing was). Apparently strengthening those deep core muscles will actually help with the gut pain. That’s promising.
One thing to remember is that, if you are hypermobile and show a bunch of these systems, exercise is not entirely optional (if you want to stay mobile and active and free of back pain and so on for an extended period).
When I went to the physio as a precursor to undertaking clinical pilates, I filled out a new customer form asking me what goals I hoped to achieve.
I kind of stared at it for a while. I knew I needed to do pilates if I was going to fix the problems with my core muscles, but that was part of a general desire for health and fitness.
In the end I wrote: “1. Stabilise core muscles. 2. Reduce pain and injury risk. 3. Improve proprioception.”
In the actual appointment, of course, the physiotherapist administered the Beighton test. When asked to bend forward and put my hands as close to the floor as they could go, I asked, “Really? As far as I can go?” because hey, it’s nice to be good at something, even if the thing you’re good at it is ultimately pretty bad for you.
About eight months on, and the core work has actually, genuinely helped. I can hold a ninety second plank. I can stand and walk for longer without getting lower back pain. My form with free weights is improved. I still can’t sit still in one position for very long, but I don’t think that’s ever really going to change – my joints are what they are.
Unfortunately, nothing can really be done about the gut pain that strikes like a vindictive ninja a few times a year. One takes a couple of very strong painkillers and tries to pass out until the pain goes away.
So, while HMS/EDS has an extremely varied presentation, this is what it means for me.