Hypermobility, Episode 2: The flesh machine

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.


Why I love the gym since this often confuses people… (Hypermobility, episode 1)

My love of the gym is weird to many. Here is my explanation (no, it’s not just endorphins)

When I was a kid, it didn’t take me too long to work out that most of the other kids were a bit different from me. It wasn’t the reading or the red hair or the good marks that really set me apart, at least not as far as I was concerned. It was the fact that other kids appeared to be able to do magical, superhero things with their bodies.

My first ever P.E. report (tender age of five) read, “Kate is unco-ordinated.” This meant nothing to me at the time. Looking back, it’s just a concise summary of how I felt.

I felt like, when all the other kids were receiving normal human kid-bodies that leapt and ran like fleet-footed bipedal gazelles and tumbled and swung like unusually tall spider monkeys, I had somehow been given a weird, lumpy, alien flesh-machine. Where others raced and flew, I bounced and flopped. I was out of breath. I was chubby. I couldn’t cross the monkey bars. Nothing seemed to quite work the way it should. There was nothing in particular wrong with me (this is true. I have always been able-bodied and am in no way trying to claim a disability here. This is just the story of how I live in my body). It all just felt wrong.

This didn’t stop me doing some things. I rode my bike (slowly). I swam (badly). I jumped rope (differently from how other people do it, which is relevant to this story – I never actually jumped, I just kind of stepped quickly and kicked my legs back). I liked stretching and doing forward somersaults on the big gym mats. These were my limits, though.

Some of the lack of co-ordination was down to undiagnosed short-sightedness. After I got laser eye surgery a few years back, it turns out that I can (at least sometimes) catch things thrown to me, rather than simply squeaking, ducking and trying to blindly bat them away from my head. That helped.

I felt like I could never quite explain why some things were so hard. I knew I was unfit, but that wasn’t the whole story. Things hurt. Joints hurt. Not in an arthritic way, not in a broken-bone way, and not in a “your muscles are just weak and will get stronger as you go” way.

Several years ago, I was having knee problems. My doctor explained that my patella was, and I quote: “rather like the surface of the moon.” Great. I’d started going to gym and doing high impact exercise, and this is what my freaking body does to me; it breaks its goddamn knees. Nice work, cartilage. Thanks a bunch.

A few years later, in the hope of running without knee pain, I started running barefoot, and discovered that I quite enjoyed it, but ultimately this lead to other problems.

Enter the physio and the podiatrist, and my current epiphany, which explains everything right down to why I never jumped rope the same way the other kids did.

The flailing. The bouncing. The hurting. The lack of control and co-ordination. It all comes down to range of motion. Since my joints are too mobile, I can’t control where they go – or rather, I can, but only if I am doing things very slowly and concentrating rather fiercely. This explains why I can’t run (at present) but why I can, in fact, swim without too much trouble (the water pressure contains the excess motion and it’s easier for me to be in charge of where all my limbs go). Swimming is glorious because it is, for me, almost pure cardio – the muscles work and build but they are not frantically working to keep my hips and ankles and knees where they should be. I’ll concede that it does get a bit trickier with a tank, 10+kgs of dive weights, and a current, and that there’s a reason I went with split fins for scuba diving.

My friend Nadia convinced me to try gym classes and I finally plucked up the courage to walk into a BodyPump class (this is back before the your-knee-is-full-of-craters conversation). If it sounds strange that one needs courage, most of my previous experiences of exercise with other people around tended to result in at least embarrassment on my part, if not downright humiliation.

I was a convert.

People look at me strangely for treating a trip to the gym as a reward for a job well done, and that’s fine. Not everyone likes gym. Weight training is gym training, essentially, and I will always love gym because of weight training (also stretching, but that’s another story).

When I train with weights, I am isolating muscle groups. I am working on one thing at a time. I am planting my feet or seated on a machine and I only have to worry about one set of joints. Usually the exercise is done in such a way that it braces joints anyway, although bench press gives me trouble because of my wrists (they don’t hurt, I’m just constantly rotating them to make sure they don’t tip back too far, because they really want to). Tricep kickbacks. Bicep curls.

One thing at a goddamn time, and it actually works and I can actually feel it. It feels right. It feels how I imagine most other people’s bodies feel most of the time. It doesn’t bounce and flop and hurt.

I can control my range of motion when I do weight training.

When I do other things that move too fast – aerobics classes, for example – I have to keep too many things in line at once. The hips will shift or the knees or the ankles or the wrists – something will go. While I am trying to keep those where they are supposed to be, that will overload the supporting muscles, so meanwhile the other muscles will try to compensate and get overloaded themselves, and it becomes a vortex of hypermobile disaster. I can do combat classes and step classes and so on, but I do them in a slow, low impact style because otherwise I’ll probably do my ankle.

The feeling of being in charge of how my body is moving has been so foreign to me, so unachievable, that doing a set of weights feels like I’m triumphantly flipping off the universe. I’m not amazing at weights. My technique is not perfect. All the same, when I do a clean-and-press, I might as well be dancing Swan Lake for how good I feel.

Learning how to run barefoot was like that, but multipled by hundreds, thousands, because I could never ever ever run before that. Running barefoot gave me more control over how my feet landed. Running in shoes makes me feel like I have weights glued to the end of my ankles. Remember how my ankles are hypermobile, and it’s hard enough to control where they go? Adding stiff, unbending weights to them multiplies the problem.

But running barefoot, unfortunately, means that, even though I am finally in charge of my feet to a marvellous and empowering degree, I am less in charge of my legs (at least, the right leg, which is the really stupid one). Not being in charge of my legs means I end up overloading my feet anyway (but at least not my knees. For once) (note: when I was starting out very slowly and working up to 5k barefoot, my legs actually survived the experience very well – but I did have to concentrate fiercely to keep my right leg landing straight on. Recently I started running again after a break and overdid it and now I have zero power to keep my leg straight).

At the moment the compromise is to have a very light, flexible shoe with orthotics in it. In combination with my physio strengthening exercises, it’s actually working quite well – the other day I completely forgot to change to my light shoes for the treadmill work and managed a good run without even noticing (very slight shin pain, so I’ve taken a few days off from running and will get back to it tomorrow).

In recent times I’ve investigated hypermobility more closely. It very much appears that I may have Ehlers-Danlo Syndrome / Hyper mobility type (or hypermobility syndrome, or joint hypermobility – names and classifications vary). I have the stretchy skin, the myopia (at least prior to the laser eye surgery), the sensitisation, the painful gut involvement, the (juvenile) asthma, the poor proprioception (exacerbated in the presence of progesterone. Yay?).

I also score 7/9 on the Beighton test – the only thing I can’t do is bend my thumbs back onto my arms, and apparently if you score 4 or more that fulfills a major criteria. The minor criteria include dislocations and subluxations (hell yes), myopia (yes), stretchy skin (yes, although I only worked this out recently when I realised that not everyone can pinch the skin on the back of their hand that way) and a bunch of other things.

In terms of classic hypermobility, it all does explain why I can’t do shoulder presses (my ligaments hurt) and even when I do the shoulders never get stronger, why I developed RSI from pipetting (Gilson pipettes – heavy spring action; I’m fine with Eppendorf and more lightweight and ergonomic pipettes) when no-one else seems to do so, and why stretching feels so amazing at the time and later on starts to hurt in a suspicious and sneaky way. It explains why I fell over at least once every day in primary school, why I could never cross the monkey bars, why it took me two years to learn to ride my bike without training wheels, why I can’t do cartwheels but I can put my hands flat on the ground without bending my legs, why I walk funny

I’ll be writing more on this as I am currently fascinated and it falls into my category of biomedical obsessions (with an intriguing genetic slant). The down side is that, since it is genetic, there’s no cure or treatment (so I’m stuck with the occasional agonising gut cramps that make me pass out but hey, codeine still works). The up side is that, if I know what’s going on, I have a much better chance of avoiding injury. I’ve already worked out some alternative weight training that will build muscles without overextending around the joint (i.e., no more shoulder presses or tricep presses. Assisted chin-ups and tricep pull-overs or kickbacks are much better).

I will also be visiting the local clinical pilates business and seeing if they can help me. Watch this space.

[This was originally posted on my private blog a few months ago, and the last few paragraphs were added in response to some reading I’ve been doing over the last few days. I feel as though I may have opened a biomechanical can of worms, here. – KN]