M is for Mental Illness

Following on from yesterday, this one is another difficult topic.

“Mental illness in EDS is frequent, diverse, and strongly associated with pain” is literally the title of a paper I was reading a few weeks ago, and I am honestly not surprised.

Or, to put it another way “I’m not shocked that being in unpredictable, incurable pain is going to make someone a bit mental.”

As far as we can tell though, there’s even more to it. I’ve mentioned before that we’ve got slightly strange adrenal systems – For whatever reason (possibly because of all the pain, actually) we get spikes of adrenalin at the slightest provocation, which causes, amongst other things, sudden spikes of anxiety. And it doesn’t take all that much for a disposition towards anxiety to turn into generalised anxiety disorder, or OCD, or anxiety-and-depression, or bipolar, or any number of other things. Likewise, I saw an article (which I should have saved the link to, actually) which suggested that hypermobile brains and autistic brains shared a lot of basic similarities, and there’s a known statistical correlation between hypermobility and autism (though that’s a correlation, not anything more definite than that, and there’s correlations between all sorts of unrelated things).

 

It shouldn’t be a shock that our brains aren’t quite “normal” – There’s collagen in the brain, after all.

 

This, though should be balanced with what I always think is a fairly sensible and obvious statement, but at least a few medical professionals that I’ve known have completely misunderstood; Hypermobility is not the mental illness. Being in pain is not the mental illness. It is possible to be completely mad, and still be factually correct when you say “My leg hurts more than I can bear” or “The reason I don’t walk is because I can’t walk”.

Because some medical professionals still see someone that’s in crippling pain, that can’t manage their daily activities without serious fatigue and loss of function, and automatically decide that they’re just “functionally impaired”, as in that their mind has convinced them that they’re ill. Somatisation, so to speak. This is especially cruel with things like subluxations which resolve on their own, resulting in trips to A+E ending in “But I wasn’t fine when I got here” and assumptions that you’re either mad or drug-seeking.

 

Honestly, today will be a short entry because I am actually feeling pretty bloody mentally unwell anyway. Sleep time.

 

(Notable runners-up to be “M is for…”; Molluscoid pseudotumors, morphine, motion sickness, muscle relaxants)

L is for Loss

This one is a bit nebulous, but it’s important, or at least it feels important to me.

Getting a diagnosis of EDS-HM in adulthood, when up to that point you’d always been fit and active and driven, is a shock. Realising that the fast, often painful, decline you’ve been in for the previous months or years is not only “not just a run of bad luck” or “a couple of injuries” but permanent and basically irreversible is terrifying. The world shrinks – Both in your expectation of what you will be able to do in future, and what you do in the present. Suddenly, that career that was supposed to take you to the Antarctic, or the Olympics, or to Hollywood gives way to working out how you can fit the job that you have around the interminable hospital appointments. Or losing your job altogether, because the pain is just too much – I’m still not sure if quitting in defeat, or waiting until you’re sacked is worse.

This would be bad enough on its own, even if the same force that was making having a stable job so difficult didn’t extend to everything else in life – The same distracting pain and crippling fatigue that makes it difficult to get to work on time makes it difficult to get to the gym, or the shops, or to sit and sew or read or even watch telly.

And, well, all of this applies to the social life as well. It’s difficult to keep long, meaningful friendships when your own life is going sideways compared to the majority of the population, and it’s even harder when physically meeting people is even more difficult; Even if you’re not one of the many hypermobile people who use a power chair (great for improving access, as long as buildings are designed for it… but most buildings aren’t designed for it), going out and meeting people is exhausting. And, well, after the twentieth time that you’ve said “no” to meeting up, most friends just stop calling. Skype helps, likewise forums and social media, but it’s still not easy.

A very wise friend, not long after I was diagnosed, pointed out to me that basically, it would make sense to consider it a process of grieving a loss. Not losing someone else, but losing the life I’d been going to have; And as much as that wasn’t something tangible, we don’t criticise people who want to grieve a miscarriage, so why is it strange to grieve, on some level, the loss of your own life?

And much like the other kind of grieving, it’s healthy to recognise it for what it is, and to set parameters on it. There’s no sense in hiding away from everything and everyone, forever, but on the other hand there’s definitely no harm in letting yourself admit that you’re upset, and giving yourself time to think through and come to terms with what it’s going to mean.

And be realistic – It may take a long time to work out what a sustainable level of activity is. At first you’ll either overshoot or undershoot; Knackering yourself every afternoon in an attempt at having something that looks like your “previous life”, or becoming terrified of the outdoors and shutting yourself away for fear of pain and fatigue and humiliation. Fear of the unknown is normal – But it’s possible to, after a while, make it all the “known”; So that you can distinguish between the pain that means “carry on” and the pain that means “stop now, this is dangerous”.

Plus, there are a lot of things which society thinks are “true” about becoming disabled which actually aren’t true at all, and it’s very easy to believe, in advance, that they’re all true and thus that life is going to be over. There are very few truthful narratives about disability in the mainstream media; Most of them focus on the idea of disability needing to be “overcome” in order to have a good life, rather than just worked around. Think of it as like living in a house with a mouse problem – You don’t need to get rid of the mice, you just need to stop them shitting in your food. Maybe this means buying some storage tins for the flour, and getting into a routine of throwing out uneaten food. It definitely doesn’t require that you move out, or devote a hundred percent of your time to eradicating every last mouse in the building.

But, until you’ve reached that point where you’re used to the mice, and can work around them with as little disruption as possible, it’s perfectly reasonable to feel a bit consumed by loss, and even beyond that point – When you find the metaphorical mouse shit in the biscuit tin, or when you get completely frustrated with always having to store the flour on a high shelf – there’s always the chance that it will creep up on you and ruin your day.

All I can say is, you’re not alone, and don’t feel that you can’t talk about it. Even if nothing else, my inbox is always open.

 

(Notable runners-up to be “L is for…”; Ligaments, lungs, Loeyz-Dietz Syndrome)

K is for Kyphoscoliosis

 

This one may be cheating, since it’s three or four things in one.

First, there’s the rare Kyphoscoliosis-type EDS (Also known as Type 6, where Classical EDS is Type 1 or 2 depending on degree of skin involvement, Hypermobility type is Type 3, Vascular is Type 4 and there is no Type 5. Dermatospraxis and Arthrochalasia types are 7c and 7a/7b respectively, and even rarer) – Less than 60 reported cases worldwide, and characterised by, surprisingly, kyphosis and scoliosis which get progressively more pronounced over time, blue scleras, and extreme muscle weakness.

Then there’s kyphosis – Also known as having a hunched back, or a dowger’s hump, or even just a slouch. People with it will get either told off for not standing up straight (as teenagers) or will be correctly identified as having an unusual outward-curvature of the spine, usually at the top, which makes the shoulders seem hunched and the head seem to be pushed forward. It can cause pain in the shoulders and, sometimes, lung problems.

Next is scoliosis, which is where the spine is unusually kinked sideways, making it asymmetrical or s-shaped. Amazingly, this is sometimes only discovered upon x-ray, even though it causes uneven muscle development up and down the spine and across the shoulders and hips, often resulting in pain or compression of the ribs or lungs. Much like kyphosis, it can be compensated for slightly by deliberate strengthening of certain muscle groups, and there are surgical solutions available, with varying degrees of success, but largely speaking it’s just something that “is” rather than “is a problem in its own right”.

Then there’s lordosis, the third of the trip of spine mis-shapes; Where the lower spine is kinked in towards the belly, also known as “swayback” or “ewe back”. It looks inoffensive, in fact, it mimics the lordosis posture which is a pretty common “come hither” in… basically all mammals – But it puts horrendous strain on the lower back, thighs, and pelvis. And, well, hurts.

 

All, effectively, caused by the collagen in the connective tissue in the spine being too spongy and stretchy, and thus the bones forming slightly “squished” in the first place, and then the whole spine being supported basically by a combination of muscular tension and gravity. This is pretty much also why so many of us have sciatic nerve impingements, herniated or thinned discs, or just generic “back trouble” that follows us around like, well, a backbone.

Broadly speaking, this is why a lot of us don’t do very well in ordinary chairs – even allegedly “ergonomic” ones. No matter how tall or short we are, the lumbar support will be in the wrong place, the headrest will be at the wrong angle, and bits of either vertebrae or pelvis or scapula will dig into the upright of the chair. You can pretty much spot a zebra household by the amount of cushions, well-used chaise longues, unused dining chairs and foot-marks on settees. Most of us either squat, kneel up, sit asymmetrically, or sit with our feet up, if we’re forced to sit upright at all – Otherwise, we recline, lie flat, or  (if we’ve got the leg strength) just stand up and pace around.

It’s bad enough when the limbs aren’t cooperating, but when even the torso is out to get you, it starts to get harder and harder to make the body do anything constructive.

(Notable runners-up to be “K is for…”; Kyphosis, keratin, ketamine)

J is for Joints

Relatedly! I found out today that http://www.autisticzebra.wordpress.com is doing an EDS A to Z as well, so go and have a look!

 

I’ve mentioned before that not all joints are created equal, and that not all joints are even things that a healthy person would think of as a joint. And, as is traditional in hypermobility syndrome, there are a dozen fascinating ways that each one can cause different kinds of problems.

 

Hinge joints are probably the simplest and easiest-to-understand joints in the body, as well as being pretty common. Three in each finger, three in each toe, elbows, and slightly complicated examples in the knees and ankles. In each case, there is one degree of freedom – The joint can fold open or closed, like a door, and like a door, there is supposed to be a point at which it won’t open any further. In hypermobility, that point is much further than is usual. Six of the points on the ten-point Beighton criteria relate to hyperextensions of hinge joints – Bending the little finger back at 90 degrees past “flat”, and hyperextending the elbows and knees past straight – and thus it’s fair to say that hyperextending hinge joints is an important clinical feature of EDS-hypermobility in otherwise uninjured patients (It’s important to note, however, that in older patients or patients who’ve repeatedly injured their hinge joints, they might no longer be able to hyperextend them due to a build-up of scar tissue or a shortening and tightening of the muscles and connective tissues around them). Luxations of the hinge joints tend to involve a slipping-sideways, in any other axis but the one that they’re supposed to move in – Especially notable in the fingers and toes, which can slide off and past their correct orientations due to the surfaces of the bones being so flat and smooth and relying so much on the joint capsule to make them articulate. In the elbow, the olecranon (the hook of bone which locks the elbow in place when it’s straight) prevents luxations when the elbow is extended, but not when it’s bent, and the knee (Being a complicated hinge with four bones articulating at it) often ends up with the patella luxating, even if the rest of the joint is still basically intact. Despite the ankle being technically a hinge, in hypermobility is has so many degrees of freedom that it almost moves more like a wrist.

 

Wrists, and the joints between the carpus and tarsus and their phalanges, are condyloid joints – Elliptical sockets with elliptical “balls” which allow movements from side-to-side and up-and-down but without allowing rotation. Confusingly, the wrist itself is actually two of these joints (The distal and proximal radioulnar joints) and also two pivot joints, which do allow rotation. These joints partially luxate by sliding around within the sockets, and fully luxate when the ball comes completely out of the socket. In the case of the wrists, the sockets themselves can become deformed from being move beyond their natural range (since the multiple bones making up each one move apart from each other) causing a luxation. Even before getting to the point of luxation, the hypermobile person has more movement on each of these articulations than a non-hypermobile person.

 

Ball-and socket joints allow the greatest degree of freedom, and are best typified by the hip itself, where the femoral head sits in the acetabulum, locked in by a sturdy joint capsule which isn’t all that sturdy in a hypermobile person. These joints rotate freely on all axes (so can pivot and hinge in any direction) and, unfortunately, in hypermobility they also stretch – The joint capsule stretches, letting the bones pull apart from each other and causing luxations.

 

It’s not just the stretchy collagen though, which causes hypermobility – In many cases, especially in the other hypermobility syndromes like Marfan’s or Osteogenesis Imperfecta, the joints themselves are an unusual shape – The classic one being that the sockets of ball-and-socket joints are too shallow, meaning that less force is needed to make them luxate, or the contact surfaces of condyloid or pivot joints aren’t quite matched to each other, making them slippery. Another problem, when repeated luxations have happened, is the formation of a false joint – This basically means that instead of there being one stable “correct” configuration for the joint, a second “socket” has been worn into the bone by the pressure from repeated luxations, meaning that the joint can be stable, but in the wrong place, which might reduce function or cause pain.

Detecting all these problems in the joints really needs long-term, serious observation, not just single x-rays every time the hypermobile person presents at A+E with an acute injury. Joints are, generally, fairly amorphous, imprecise things, and the difference between “in the right place” and “so far wrong that the limb doesn’t move” can be a few millimetres, depending on the joint, and without a “before” picture, the “after” picture often looks quite complete and correct – Much like finding a Neolithic arrow-head and surmising that they never used shafts for their arrows, because you’d never seen one. Unfortunately, long-term serious observation costs money, and requires medics at all levels to just believe the patient, which seems to be par for the course for high-ranking specialists, but completely beyond the wit of the average A+E locum.

And this is why we’re having to try to increase awareness, amongst other things.

(Notable runners-up to be “J is for…”; Jellyfish, jejunum)

I is for Injuries

One of the big problems of hypermobile EDS is the pain. It’s not hyperbolic to say that, in general, we’re always in pain. From waking up with a sore back from having slept the wrong way up (every way is the wrong way up), to the last crack of a shoulder as you brush your teeth at night, something is going to hurt.

At this point, many people will say “Ah, chronic pain, we understand that”, but they’d be wrong. Chronic pain, technically, is pain which has long outlasted the actual damage to the tissue around it – It’s what’s called neuropathic, meaning that the problem is in the nerves themselves, not in the things they’re connected to – The equivalent of a “check engine” light which is always on, regardless of the status of the engine. In the case of EDS, the check engine light is on, because there is something wrong with the engine.

Painfully bending a joint far beyond its capacity to naturally move is an injury – Think of the number of TV shows where someone is tortured by having their fingers bent backwards. Dislocating or subluxating a joint is an injury – Think of footballers and rugby players being stretchered off the pitch with their shoulder or their kneecap immobilised and iced up. Compressed or herniated discs in the spine are an injury. Prolapsed internal organs are an injury. Bruises and strains and hairline fractures are an injury.

Effectively, the problem in hypermobile EDS is that just getting out of bed can cause the kind of injuries that you’d expect to see in a rugby scrum, a bareknuckle boxing match, and a car crash, combined.

And that’s where the problem of pain relief comes in. In most cases, in long-term pain, doctors will put the patient on a GABA-analoque or amytriptaline or similar; Something that will treat neuropathic pain. This is, going back to the car metaphor, something that will fix the check engine light and let it switch on and off normally again. What’s needed in hypermobility, though, is something that will fix the problem in the engine, and something else that will cut the wires to the light altogether, at least for a little while at a time. The first is an anti-inflammatory; An NSAID or (in acute problems) a steroid which reduces the swelling around the joints, which are no doubt going to be at least a little swollen, even if not visibly so, from repeated injuries, and which incidentally takes down bruising as well. The second is, invariably, an opioid, which makes some doctors nervous, and some just thankful that they can offer something that works.

In short – No, we do not “get used to it”. There is no talking therapy, no mindfulness meditation, no regular routine of baths and walks and stretches that will make a sudden injury not hurt. As much as it’s possible to tune out an injury that you’ve had for a few days, it would take superhuman willpower to tune out a serious injury as it happens, and in the minutes or hours straight after it. Try slamming your finger in a door, or something similarly painful-but-not-that-damaging, and try to not feel it – Not to be able to tolerate the pain, but to not feel it at all. Now, imagine that happened to you every half an hour or so, every day, with no respite, not even when you were asleep, moving from place to place, so you couldn’t even just rest a single limb or know in-advance to avoid a certain movement. It’s been said that hypermobility is sometimes a bit like being followed around by someone who hates you, and keeps hitting you with a cricket bat. It’s pretty much accurate.

It’s exhausting, and constant, and that it’s so little understood even by people who know “technically” what it’s like is bloody criminal. A doctor I know admitted; “In training, we get shown old-timey photos of circus performers with stretchy skin. We get told about the luxations, but… For some reason, it never occurred to me that they hurt. Which is obvious, really. Just nobody ever mentions it.”

 

And that needs to change.

 

(Notable runners-up to be “I is for…”; Invisible illness, indigestion, ignominy, insomnia,  irritable bowels, itching)

H is for Heat

As with everything else in zebra country, our reaction to and relationship with heat is a paradox.

On one hand, we need to be kept warm; Since we can’t really rely on the structure of the joint itself to hold any position, we’re held up entirely by muscular tension, so being cold and thus more tense causes problems; Ranging from just stiffness and exhaustion, all the way through to dislocations and spasms. A good rule of thumb is “Even if you don’t feel cold, if you don’t feel hot, you’re too cold.

Another reason to keep your average zebra warm at all times is that most of us have some degree of Raynaud’s. Raynaud’s sign (Or Raynaud’s syndrome, or primary or secondary Raynaud’s) is where in cold weather, or when under stress, the blood vessels in the extremities (Hands, feet, ears, nose, genitals, and in severe cases scalp, calves, forearms and breast) constrict, reducing blood flow even more than is normal for in cold weather, resulting in an array of unhealthy colours (Ranging from white and grey through to dark red, purple or blue), loss of feeling and function in the affected parts, and then absolute howling pain upon sensation and movement returning later. As you can probably imagine, trying to reduce a luxation that was cause by the spasms caused by the cold with a hand that is basically just a lump of insensate gristle is a lot like trying to fold an origami crane, out of gold leaf, whilst wearing boxing gloves and someone is standing behind you with an electric cattle prod to zap you every time you make a mistake.

So, even if we’re enjoying the cold, even if it feels refreshing and pleasant to be outdoors on a crisp winter’s evening, we’re probably going to suffer for it the next day.

Plus, heat is a really useful painkiller – even on a hot day, applying a hot water bottle, or a rice pack (A pillowcase full of rice, heated up for about 1 minute per 300g of rice in the microwave, is a shockingly good emergency heat source) to a sore joint or a set of cramped muscles can be a lifesaver. Not to mention the sauna or the steam room, both of which are invaluable weapons in the fight against deconditioning – Swim hard, sauna, three-minute cooldown with drinking water, then stamina swim, then sauna or steam, then a shower, then home to sleep it off.

“So” I hear you thinking “It’s simple then. Treat your zebra like a tropical plant, and they’ll be fine.”

Nope. Once again, it’s a paradox.

Too much heat – Even being slightly too warm for slightly too long – is a disaster. Low blood pressure and a fast heart rate means that overheating, and especially overheating and dehydrating, results in fainting, confusion, fatigue for hours or days afterwards and general failure. Personally, once I get above about 23 degrees, I need to spend most of the day lying down, drinking something with ice in it, or I’ll end up falling through the day like a drunk, slurring my words, not really managing joined-up-thinking, and having to sit down (or curl up on the floor) every few minutes to rest and stop the world from spinning. Apparently, the answer is to eat more salt – In my case, keeping a couple of packets of salt from the canteen in my wallet – but even that doesn’t help much, and of course, overheating completely kills the appetite. We sweat too much, or too little, and either way, heat isn’t good for us at all. Not to mention that in many cases, the heat, and bright sunlight, and the dehydration that comes with that can cause a full-blown migraine.

On balance – Cold-weather-problems are unpleasant, but at least they can be managed with a bit of rest, painkillers, and warm clothes. Hot-weather-problems, that result in days of fatigue, sickness, confusion and a horrible sense of helplessness, are a thousand times worse.

And this is why I often wonder about moving to Tristan da Cunha, where it’s a balmy 16 degrees, with low cloud cover, all year long.

 

(Notable runners-up to be “H is for…”; Hot water bottles, hands, heart murmurs, headaches, hyper- and hypopigmented scars, hyperactivity, hydration)

G is for Gastroparesis

I feel like I’m stretching a bit to talk about gastroparesis, since I only have a fairly mild case of it, but it’s an important part of the hypermobility syndrome, and I’d feel like I’d missed something out in not writing something about it.

Gastroparesis, broadly speaking, is paralysis or partial paralysis of the stomach – Either the whole stomach, a part of the stomach, all of the time or some of the time, so it can range from quite manageable, via eating small, frequent meals, through to needing surgical intervention in the form of placing a tube either through the nose and into the stomach or through the abdominal wall and into the jejunum, to deliver pre-digested food into the lower parts of the digestive tract in liquid form.

In person, gastroparesis causes all kinds of symptoms – The most common one is a constant, vague nausea, followed by feeling bloated (often with physical distension of the abdomen, to an extent), never really feeling hungry, or always feeling full even if you’re paradoxically still hungry, heartburn, acid reflux, palpitations, all-over aches, fatigue, and just generally a feeling of absolute malaise. And that’s before it gets serious – When it’s serious, the symptoms trend more towards things like bezoars (which cause intestinal blockage) and severe malnutrition. Basically, if food is sitting in the gut and not being digested, it’s going to cause problems. In biological terms, it’s sometimes best to think about the digestive tract as being “outside” the body – a human is basically a complicated torus, after all, with the mouth and the anus being the apertures into the “tube” through the middle, and as such the immune system doesn’t quite function the same inside the hollows of it – There’s no blood in there (In a healthy case) so immune mediation doesn’t work as well as in the rest of the body, so bacterial overgrowth on old food can cause bacterial infections really quite quickly in the whole length of the gut, especially if there’s an under-production of hydrochloric acid in the stomach, which is also a regular consequence of gastroparesis. As such, it’s one of those problems that compounds itself even beyond its original set of issues.

Many of us self-treat for gastroparesis at first – Switching to softer foods, or ones with less complex nutrients in them which need less breaking down, or supplementing with protein shakes or baby food. Gastroparesis itself is usually diagnosed via a gastric emptying test, in which it’s measured how fast a standardised foodstuff exits the stomach after it’s been ingested. Severity of actual paralysis, and severity of symptoms, aren’t actually correlated as directly as one might expect; It’s possible to have quite severe gastroparesis that you’ve barely noticed, and very mild gastroparesis that makes eating a vomity nightmare.

The biggest complication of gastroparesis, other than of course that it’s a problem in itself, is weight loss – Loss of appetite and inefficient digestion means taking on less nutrients, which in turn leads to fatigue, and then on to all the other problems of weight loss (or, indeed, the inability to put weight on in the first place). Ironically, the weight loss might be hard to spot at first, due to the stomach distension from carrying undigested food around in it – Again, thinking of the body as a complicated torus, the contents of the gut isn’t really part of the body’s “weight” in useful terms. So, at least at first, the complaint to the doctor of “I have no appetite and I’m losing weight” might be met with a shrug and a “Well, you look fine to me”.

As ever, perseverance is the answer, along with making sure that you know more about your own condition than any doctor is ever likely to learn in a hundred years.

 

(Notable runners-up to be “G is for…”; Grief, genetics, gastroenterology)