Happy Rare Diseases Day!

So, today being Rare Diseases Day, the day for sharing awareness of rare diseases, I thought I’d share some awareness about mine.

All right, what’ve you got?

I have Ehlers-Danlos-HM, also called Joint Hypermobility Syndrome (JHM or JHMS), Ehlers-Danlos Type 3 (EDS-3), Benign Joint Hypermobility (BHS, BHM, BJHM, or BJHS), Chernogubov’s Syndrome, or (memorably) “That pigfucking hyaenabitch from hell”.

What’s that like then?

The headline is right there in the name; HM; Hypermobility. Most of my joints started life being much more bendy than the average person, due to a defecit in my production of some types of collagen. Effectively, where a non-hypermobile person’s ligaments and tendons are steel cable, mine are bungee elastic.

Over the years, repeated injury to the joints can stiffen them up – This has happened noticeably in my lumbar spine, meaning that I can’t even touch my knees with my legs straight, never mind my toes. My hips, elbows, wrists, knees, ankles, digits and cervical spine are still much more bendy than average.

Most of these joints, and many things that the average person wouldn’t think of as a joint (Tempromandibular. Sternothorassic. Sternoclavicular. Intra-pelvis. Coccyx. Carpus.) dislocate. Frequently, and without warning. This is officially called “With or without trauma” – As in, a dislocated shoulder might come from being wrenched or struck, it might happen because I was lying on it wrong, or it might happen because the stars aligned wrong. The stars tend to align wrong dozens of times a day, as I documented earlier.

This also causes pain. Lots of pain. Every joint, every muscle.

After this, there’s the guts; For whatever reason, largely to do with incredibly stretchy collagen being badly calibrated with the stretch receptors in the stomach and other things, I don’t eat very normally. I can feel ravenously hungry, minutes after eating, or I can be about to faint with hunger and not realise that I need to eat. I do literally faint with hunger sometimes, but next to that – Sometimes, no matter how hungry I am, I just can’t eat. This is related to gastropareisis – Sometimes, my guts just don’t move properly; peristalsis stops or slows down to the point that it doesn’t do its job. Closely related to this is the constipation – My colon will sometimes stretch, rather than passing stool, so I get fairly regular and painful impactions. I also get horrifically travel-sick.

After that is the stress; for unknown reasons, zebras tend to process adrenaline unusually. So we can end up very easily stresse and anxious, prone to anxiety attacks, and also prone to depression.

Then there’s the migraines; For whatever reason, EDS-HM is comorbid with migraines. I get them, messily, with the sensory disturbance and photophobia called “aura”. Sometimes I end up aphasic (And can usually communicate in German, French or Japanese again before I can communicate in English, due to the interesting way that the brain works).

Then there’s the bruising; Due to the same terrible collagen being present in the walls of the blood vessels, they burst under the slightest pressure.

Then the scarring; The same bad collagen in the skin makes any damage to the skin heal badly, resulting in papery-looking scars and wide, silver, deeply recessed striae. My skin in-general is translucent all over, and you can see the blood vessels basically everywhere.

Then there’s the fatigue; It’s just exhausting. I get tired incredibly easily, both physically and mentally. Sometimes I just can’t deal with people at all, sometimes I can’t get out of bed.

Then, possibly worst of all, there’s the drug resistance; Lidocaine and related -caine anaesthetics don’t work at all, so any surgery that could be done under a local anaesthetic needs a general one. Due to being unable to explain this to a dentist as a child (And due to having the dental overcrowding typical of EDS) I ended up having six teeth removed with no anaesthetic.

So, what’s the cure?

No, there isn’t one.

Palliative care is as follows;

-Physiotherapy. With exercise. Keep fit, keep a low bodyweight.

-Painkillers. Opiates, and lots of them.

-Laxatives, to keep the gut mobile, with the possibility of tube feeding if matters get worse.

-Surgery, to tighten the ligaments and remove scarring from around the joints. Also the possibility of hip and knee replacements.

How does EDS being rare affect you?

At this point it would be traditional to say “I’m the only person I know that’s got it, or even heard of it, so I feel tremendously lonely.”

Actually, my best friend has it (And we didn’t meet via a support group) and a slack handful of my online friends have it (Also not met through support groups), so I know plenty of people who share the horror and the gallows humour of having ELS (that’s “Exploding Limb Syndrome”).

The big problem is when dealing with new medical professionals – Most of them won’t have seen very many EDS patients in their careers, and won’t know what it is at first. Many of them will underestimate quite how bad it is, since the on-the-box description of “Is very bendy” doesn’t sound very bad.

Finally, EDS being rare means that there aren’t many treatment centres for it in the UK. I was sent to London, which isn’t a trivial trip. When I get inpatient care, it’ll be at the only dedicated inpatient centre for EDS-HM in the country. There is also a complete lack of funding for reasearch, meaning that there’s not a massive amount of hope going around.

And, obviously, laypeople have literally never heard of it. The situation is improving (We have a soap character with EDS now, played by a genuine bendy!) but it’s still more likely to  elicit an “Eh?” than an “Oh, my cousin has that!”

Anything else?

Erm… Not really. Oh, colloquially we’re all “bendies”, which also includes the Marfan’s and Osteogenesis Imperfecta communities (Other things which cause hypermobility), and due to being a rare disease, we also get “zebras” (which fits nicely with the highlighter striae, at least in my case).

Why zebras?

Well, they tell first-year medics; “If you hear hoofbeats, think horses, not zebras.” Meaning, obviously “Always pick the simplest and most common explanation” – It’s Occam’s razor. Sore joints in a fit 20-year-old is probably overtraining. Constipation is probably bad diet. Migraines are probably just migraines. Fatigue and depression are overwork. It’s only when you put them all together, with the stranger, more unusual symptoms that you get the whole picture.

We are the zebras. We are here.

Being disabled is a bit horrible, really

Basically, I want to talk about internalised disablism, since it’s a thing I get a lot.

I’m also going to start this post with a very wry, very self aware “I can’t be disablist! Some of my best friends are disabled!”

It’s reached the point where, in some circles, complaining at all about being disabled is called internalised disablism; This line of logic suggests that the only reason that someone would want to climb a mountain or finish reading a long series of novels or go for a month without self-harming is because they, at heart, hate themself and have aligned their life goals with a society which views the achievements of disabled people as being either less important, or only important if they can be compared to the achievements of non-disabled people (Note – I’m saying disabled and non-disabled here because I want to include only people that’re both able-bodied and non-mentally-ill in the category of non-disabled, It’s just easier that way.) One of the things that’s routinely cited as an example of internalised disablism is not wholly embracing the Social Model of Disability.

Quick primer; The Social model says that a disabled person is not disadvantaged or suffering because of their body or mind, they are suffering or disadvantaged because of society’s attitude to their body or mind, and that the primary thing that we should do to prevent disabled people from suffering is to alter society to fit better. The Medical model says that the disabled person is suffering because of their body or mind, and that we should take steps to bring their body and mind into line with being ablebodied and sane/neurotypical (Note – I have used neurotypical here to include that Autists and Aspergics are broadly included in the medical model. I’m not on the Spectrum, so I shan’t presume to talk about how the Social and Medical models apply to Autusts or Aspergics. Anyone on the Spectrum who wants to make an addendum to the post or help me work out which resources are good and which are bad, feel free).


Note, in saying that I believe that the Medical model is valid, I’m not saying that society is doing its best. Society needs to get its fucking act together and make accessibility a priority, both in the physical sense of “If it’s a modern building, carve it up until you can get a wheelchair through it”, and the social sense of understanding that “we put in a wheelchair ramp” isn’t enough to make it accessible – There’s plenty of walking wounded, and it’s difficult to find places to socialise where Deaf or Blind people are automatically included, there’s often nowhere to let someone recover if they’re coming around from an injury or a seizure (Memorably, last year at the opera I ended up hiding out in the cellar stairs behind the bar, since there was nowhere else that I could stretch out my sorely-put-upon spine without being jostle or disturbed).


Anyway, I sometimes wonder if the people making these arguments have never done something purely for the pleasure of doing it – If they haven’t considered that maybe the view from the top of Blencathra is beautiful, or that stories are fascinating, or that not having bleeding infection risks on your body is better than having them.

When I say “I would like to have a sub-60 second 100m again”, I’m not saying “Disabled people are worse than non-disabled people” I’m saying “I enjoy the sensation of going fast in water, and it is natural to want to measure progress quantitatively”.


In a couple of discussions with friends, I’ve found that there seems to be a bit of a dividing line between who wholeheartedly disregard the Medical, and people who have problems with the Social (And, indeed, finding someone who is completely one or the other is rare anyway). The dividing line seems to be what I’d describe as “endemic suffering”. If you have one leg, but are sitting in a ski-lift with a cup of hot chocolate and an attractive Bavarian next to you, the only thing that will disable you is if there’s no way to get down without using two feet. If you’re sitting in that ski-lift, but instead of your leg being missing, it’s dangling a beartrap, you’re going to be disabled by the pain. Likewise, if you can’t even contemplate the lovely Bavarian calligrapher because your vision is slowly filling up with swarms of illusory but terrifying hornets, you’re going to be disabled by your own mind.


The weird middle-ground that I’ve found is physical fatigue; Some people with it prefer the Social model (“We shouldn’t lionise sportspeople so much, it perpetuates the disablism that anyone who doesn’t do physical exercise is somehow a bit morally suspect”) and some prefer the Medical (“Are there any stimulants that I can take so that I can at least get as far as the gym? I want to look like Stuart Broad.”) Indeed, the middle-ground in that seems to fall on how much the person in question enjoys or values sport for themselves, or what their primary experiences with sportspeople have been – If you were bullied by the entire rugby team, or went to a hockey-centric school whilst you were an avid bookworm, you’re likely to have a less positive view of sports in-general than you would if your experiences with sportspeople en masse have been positive or neutral.


(The first time that I was accused of being disablist towards myself was about sports, actually. And came from another disabled person, so it had plenty of weight. My love of the Paralympics and my belief that sport in schools, or more broadly the opportunity to exercise in school, was a good thing, was held up as being part of the culture that oppresses the disabled. I was told that, in participating in the glorification of competitive impulses and the perpetuation of the idea that sporting achievement was a good thing, I was adding to the harassment and othering suffered by disabled children. I was also reminded that the Paralympics sets impossible standards for disabled people to live up to, and that seeing one disabled person excel in their chosen discipline was tantamount to treating all disabled people as performing monkeys. So that, basically, all disabled people should opt out of practising any kind of sport, because it was perpetuating disablism to do so. It’s kind of stuck with me. Firstly because this was someone telling me that my only healthy-ish coping mechanism, hard exercise, was an outright evil however I practised it, and secondly because I couldn’t think of anything more disablist than saying “Sport is for the ableds”. On, and thirdly because the person telling me this had waited until I was exhausted and miserable and trapped on a long plane journey and in no mood to argue back to tell me. I understood the reasoning, and it was based in experience, and the person with the opinion was perfectly entitled to hold it, but it still made me feel like a terrible human being who didn’t deserve to live.)


Well, this has been quite a segue, but that brings us quite neatly back to the point; At that point, I felt like I was a Bad Cripple, and that’s one of the two axes on which I feel internal disablism.


The Bad Cripple Factors


-“It’s disablist, on TV, that when a disabled character dreams they’re usually not disabled.”

Well, it’s a cheap plot device, but I always feel guilty now that when I dream, I’m not in a wheelchair and wracked with pain, I’m usually walking comfortably through a huge city with watercourses everywhere that I can dive into and swim without fear or fatigue.


-“It’s disablist to present a mobility exoskeleton as a desirable thing, walking isn’t the be-all and end-all”

I would be first in the queue for an exoskeleton. I would cut my own legs off with a rusty hacksaw if it meant I could have a robot body that was better than my own.


-“It’s disablist to present the non-mentally-ill perspective as the objective/correct one, and the mentally ill as the subjective/wrong one”

And yet, if I think that someone has said something incredibly offensive to me, I’ll always ask a more sane friend “Did he mean what I think he meant?” and the answer will often be “No, but I can see why your depression might tell you that he did”. I feel terrible that I sometimes need a reality-anchor in the form of my friends, but without it I would probably have moved to Slovakia and bought a ferret farm in a fit of mania by now. Or, more seriously, I would have cut ties with everyone I know, defaulted on my mortgage, and fled because I thought that everyone was plotting against me. Or I’d have killed myself, when faced with an insoluble problem like how to say that I didn’t want to go to a meeting.


-“It’s disablist to want to not be disabled”

And lo, I can feel bad for saying things like “I wish I wasn’t in pain” or “I wish I could climb that” or “I wish I could finish reading this book without needing the longer paragraphs read to me”. Being in pain is bad. Being unable to walk or swim or climb as well as I want to is bad – And that’s not unique to my disability, I’m pretty sure that everyone with a competitive streak wishes they were stronger and faster, and everyone with an appreciation for the countryside wishes they could spend more time comfortably out in it. And being in pain, jesus christ, being in pain is bad. Waking up, every day, knowing that I have to make the choice between too sore to move or too stoned to think? That’s bad.


-“Keeping fit has no bearing on disability, you can be a couch potato or a world-class athlete and still end up disabled”

And yet, the only thing that mitigates the symptoms of EDS is regular, muscle-building, weight-reducing (when people have weight to lose) exercise. A lower body mass puts less strain on the already-knackered joints, and greater muscle mass allows finer control of the limbs, and lessens the likelihood of becoming so exhausted from simple activities that injuries happen. It doesn’t apply to all disabilities – probably not even to most – but it applies to mine.


And then we combine those with the other side of internal disablism; The I Am Not Worthy.


The I Am Not Worthy Factors


-“Why would anyone want to spend time with me? I have nothing interesting to say.”

-“Why would anyone ever find me attractive? I’m underweight, I smell of opium and ketones and I bathe twice a month.”

-Why would anyone ever want to share a meal with me? I pick at the food then usually get stomach cramps and give up.”

-“Why would anyone want to go on day trips with me? I always hurt myself and we have to go home early.”

-“Why would anyone want to be my partner? Their lives would be so much easier with someone normal.”

-“Why would anyone want to fuck me? It’d be so much easier with someone less frail.”

-“Why would anyone want to live with me? I create endless mess and fill the house with mobility kit and drugs.”

-“Why would anyone want to be my friend? I have crises over nothing and alternate between misery, mania, and suicidality.”

-“Why would anyone pick me for anything, over literally any other human being? I’m not as good as other people.”


To which there is just one answer;


-“And yet, they do.”


…I’m working on it. I don’t think that I can be the only person who feels like this; Like I’m not good enough at being proud to be disabled to fit in with other disabled people, but like I’m not good enough at being a normal, functioning member of society to fit in with the abled people.


But mostly, probably to the chagrin of campaigners for wholehearted uptake of the Social model, I’m working on it by picking my battles. I complain incessantly to First Buses, since their drivers (Other than a few, and whenever I see them on the bus I am cheered) as a whole are careless and disablist and don’t care if they accidentally fling a passenger down the length of the bus, refuse to help with wheelchair access when the wheelchair space is blocked (And don’t start me off on the recent legal action which determined that wheelchair users have no right to use the wheelchair space), and don’t wait at stops for long enough for passengers with mobility difficulties to exit the bus. I speak up to defend the disabled in conversation, and am frankly ashamed of how many of my friends still casually use invocations of mental illness as a shorthand for dangerous/ignorant/unpredictable, and how many of them really like the idea of adding “And then they were crippled/insane” as things to make a character more Dark And Edgy. I write to local businesses to get them to improve their accessibility, and they often do. As a balance to this, I try my hardest to keep fit, since I prefer to be able to walk and keep my mind my own. I don’t expect people to take my hallucinations seriously, or to listen to me when I’m obviously talking nonsense. I don’t denigrate the worth of activities which I can’t do.


There’s probably something much more intelligent and much less longwinded to say about this. Maybe later.

Never look back.

Am I odd, in that reminiscing about the past at all makes me violently depressed?

I went and had a look through my old blogs tonight (2002-03, 2005-2009, 2010-2011 and 2014-) and, even though none of them have particularly depressing content (Other than, well, seeing that yes, I really have been very depressed, very insomniac, and in constant pain for my whole adult life, and that so many of the lovely people with bright futures in the early entries are now dead or vanished), it made me cry uncontrollably.

Seriously, I should just never look back. I hate my life now, I hated it then as well, and yet I can’t help but look back and think that it has just got quietly worse.

A dance with the devil

I don’t know a single zebra who hasn’t at some point been told something like “I hate it when you take your painkillers”, by someone that they love and value. Best friend, partner, family member; The kind of person whose opinion matters, and where you might want to do anything to make them think better of you.

“I hate it when you take your painkillers”

Let me count the ways that that sentence is fucked up.

“I hate it when you take your painkillers” translates pretty neatly to “I prefer it when you’re in pain”. Regardless of if they’re trying to say “I prefer it when you’re in pain to when you’re socially inappropriate” or “I prefer it when you’re in pain to when you’re sleepy” or “I prefer it when you’re in pain to when you need someone else to drive and cook for you.” it, at its heart, is “I prefer it when you’re in pain.”

The most insidious justification of it that I’ve heard has always involved children, or pets (Disclaimer; I don’t have children, but I do have a dog) and it’s generally put as “But you can’t look after Isertana/Fido when you’re on painkillers!” It’s then followed up with “You’re irresponsible” or “You’re a bad Mum/Dad/dog owner for taking drugs in front of your child/pet”. And at this point, people tend to look smug – It’s a big social win, after all. Who wants a drug user, no, better than that; Who wants an opiate addict in a position where they’re responsible for the welfare of another living creature?

The picture paints itself; Mum laid on the sofa, surrounded by empty pill packets and half-demolished takeaway cartons, whilst toddler Isertana sits in their underwear, eating stale bread and margarine. Bloke staring at the television, numb, whilst Fido pisses on the carpet for the nineteenth time, thin and flea-ridden. Hogarth would weep with joy.

But let’s look at this in a much more realistic way. Here’s our two people, minus their pain relief;

Mum sits on the sofa, wracked with pain. Little Isertana watches her curling up and screaming over and over again, and wonders why she can’t help her poor Mum. Mum, for her part, is trying everything that she can to stop the pain – Yoga, acupuncture, better food. All of those cost money, and take time. Isertana’s clarinet lessons have to go first – They’re expensive and they clash with Mum’s reiki. Dinner becomes a massive trial, as the whole family switches to macro-probiotic-super-green-organic-gluten-free, and Izzie wonders why she’s being punished with horrible food. In addition to this, Mum snaps and shouts at her over the tiniest things, because she’s in so much pain.

Dog-owner Bloke lies in bed all day, unable to take his dog for a walk. He can’t even get downstairs to put food in the bowl, since every time he tries his knees explode with pain. He gets more short-tempered and stops talking to Dog, other than to tell it to get down off the furniture and to shout at it until it stops crying for food and attention. Dog pisses on the floor anyway, as Bloke can’t get to the door to let it out.

Now, let’s give them back their painkillers;

Mum sits on the sofa whilst Isertana plays, and they chat and play games. Sometimes, Izzie goes and brings her Mum a cup of orange juice, because she likes to help, and her Mum thanks her for it. When Mum is in pain, she takes her medicine, and goes a bit sleepy and easily distracted, but she still likes to sit with Isertana and they watch cartoons together. Sometimes they work together and cook cakes and make big meals, even if mostly they just eat instant, but once a week they order in a Chinese takeaway and it’s a real treat. Both are happier.

Bloke takes his painkillers, and at least makes it downstairs to feed Dog and let him out into the garden. He’s also in a better mood due to not being in pain, so although his dog doesn’t get as many walks as it could, it gets plenty of affection. Since he’s not distracted by the pain, he books a dog walker for a few days a week to make sure that Dog gets exercise. Both are happier.

So, why do people keep saying “I hate it when you take your painkillers”?

My theory is because they can’t imagine the alternative. They assume that (zebra)-(pain relief)=(functional person). Rather than (zebra)-(pain relief)=(person in incredible pain).

They see their partner and think that without the morphine their partner would be more lively, more focused, more of a “responsible adult”. They don’t realise that, usually, without the morphine, their partner would be just as lethargic and scatterbrained, only instead of being just lethargic and scatterbrained, they’d be lethargic and scatterbrained and suffering tremendously.

This is a difficult notion to get into people’s skulls, for a couple of reasons;

1) Society tends to parse “Very stoned” as being a desirable state to be in. Thus, partners will often assume that you just want to be stoned rather than sober.

– This one is difficult. There’s no denying that the feeling of being on morphine can be pleasant; In the right circumstances it’s soothing and restful, it comes with a tremendous sense of peace and affection to those around you, and ‘codeine skin’ (that feeling of having incredibly sensitive, velvety, tingly skin all over) is distractingly lovely.

Note, however, that I say “In the right circumstances”. As an alternative to being in horrific pain it is nearly always desirable. At the end of a long day, when all you have to do is lie around with good company and gently drift off to sleep whilst having inane conversations, it’s pleasant.

In the wrong circumstances (like at work, or on the bus, or at the shops), it’s horrible. That sense of tremendous emotional connectedness translates into painful, terrifying vulnerability. The peace and relaxation becomes exhaustion and grogginess and confusion. The all-over tingling becomes itching and irritation with every buckle, zip and strap.

I’ve said before that it’s like being drunk – Imagine if every morning you were forced to neck half a bottle of vodka, and then sent out into the world. You’d quickly stop feeling as if being drunk was in any way a desireable thing.

2) Society at-large is very suspicious of opiate use, and we have no cultural way of drawing the distinction between “drug dependent” and “drug addict”.

– In the absence of pain, I wouldn’t take opiates. I don’t think I’ve ever taken them other than to either dull pain that I was feeling, or in anticipation of pain that was about to happen. This, of course, means that I take between 20 and 120mg a day, every day.

To the layman, someone who takes 120mg of laudanum a day is an addict. We don’t really have a word for “Person with chronic pain who requires regular pain relief”. It’s similar, almost, to the attitude that some people take to antidepressants – That anyone who is taking them is somehow weak, or an addict, or otherwise morally dubious. It’s still bollocks. Nobody (or not many people) take that kind of attitude to people on long-term statins, or insulin, or antiretrovirals.

Thus, to many people, watching their best friend or their partner turn into the morphine queen feels like watching their partner turn into a drug addict, society’s second most hated bogeyman (after paedophiles). And thus they want their partner to stop taking the evil bad drugs.

3) Many people see looking after yourself, and putting your own needs first, regardless of what they are, as selfish.

– They are generally hypocritical in this. I have found that most people who say “I hate it when you take painkillers” hate it because they want you to be able to do something for them, whether that thing is look after another person, clean the house, go to work, perform sexually, or listen more carefully to whatever they’re saying. My only counter to this is to point out that, in the absence of the painkillers, you’re not going to be better, you’re just going to be in more pain.

And finally

4) People who don’t suffer from long-term pain don’t viscerally know what it’s like.

– When they say “Oh, don’t take your painkillers”, they imagine that it’s like having a headache, or a twisted ankle, or a gravel burn. They imagine that it’s like the kind of pain where, if you just leave it for a minute, it’ll go away. They possibly imagine that it’s like having pulled a muscle or cricked your neck – that it’s bad, but that you can work around it.

It’s not. When you’ve been in pain all the time, you’re already at “I’m working around it” levels of function. The pain that means that you neeed to take more pain relief on top of that is called “breakthrough pain” and it is exactly what it sounds like – It’s a pain that’s so bad that it shatters through any pain relief that you’re already on like a bull ripping through a paper bag. Suddenly, there is all the pain, and you’re feeling it all at once, and it can be anything from “Completely ruins your concentration” to “Curled up, screaming, begging for death”.

I volunteer a solution – Anyone who says “I hate it when you take your painkillers” has to promise to swear off pain relief entirely. And then has to wear shoes full of broken glass for a year. And then they might get the idea.

Short of actually hurting your partner/friend then refusing to give them pain relief, though, how do you get them to understand?

Well, it probably all depends on the person. Someone who dislikes it when you take painkillers because they’re frightened that you’ll become an addict will need a very different tactic to someone who dislikes it when you take painkillers because they find it embarrassing when you’re confused in public.

The common steps I’ve found are basically as follows;

1) Remind them that your painkiller use isn’t about them. It’s your experience, your pain, your choice of which is the lesser of two evils. If they push, find out what it is about your painkiller use bothers them, and gently remind them that without them, you won’t be “better”, you won’t be more attentive or more responsible, you’ll just be in pain.

2) Remind them of the clinical guidelines for the use of whatever painkiller you’re on. If you can demonstrate that what you’re taking is safe, that might help.

3) Point them at the Rat Park experiments. I know I bang on about them far too much but they’re just that important.

4) Involve them in some kind of ritual around your painkiller use sometimes. Not every time, obviously, but a compromise like “When I’ve taken my morphine and can’t have a real conversation, we can settle in with popcorn and watch those trashy old films you like”. Demonstrating over and over the difference betwen a morphine night (Relaxed, quiet, low-energy) and a pain-but-no-relief night (Crying, tension, possibility of a hospital visit, conversation always centred on pain) is a good way to get someone that you’re close to to be firmly on the side of morphine.

5) Reassure them that you want to be “you” as much as they want you to be you. I don’t know why so many people can’t see how upsetting and painful it is to be forced into a stupor against your will at unpredictable times. Especially when the alternative is blinding pain. There’s no win there.

I don’t know, there’s probably more, but that’s the stuff that comes to mind. Empathy is the key thing, really. And, well, good luck all of you.


Today was… Surprisingly not that bad.

Made it on-time to my appointment, only to have the reception staff tell Dr G that I’d gone for a coffee and delay my appointment by an hour – I was sitting reading my book the whole time, in the corner of the waiting room. He was incredibly apologetic when I got to the consultatiion, despite it not being his fault.

We talked for a while about my options, and elected to try fitting it there-and-then, no anaesthetic, no sedative, no waiting. He was irritated that my GP had suggested he could have me sedated there, but was happy to defer to me on the non-use of lidocaine (As was his repeated refrain; “It’s your body, you’re the expert, I just have to listen because you’ll tell me what the right course of action is.”) and was also happy to sign off on a general anaesthetic if I couldn’t get it fitted there and then.

He left the room, I stripped below the waist as-instructed, was handed a stress-ball shaped like an ambulance by the nurse, sat down on the table and promptly burst into tears. The nurse, talking me out of crying and gingerly patting me on the foot suggested that I might be better off with the general anaesthetic, and also probably a large cup of tea.

I promptly made things worse by assenting, standing up to get my clothes back on, and dislocating a hip, flinging myself face-first onto the floor and smashing my nose on the corner of the desk in the process. By the time I was back in my trousers my head had stopped spinning and there was a cup of black tea in my hand, so something must have gone right.

Dr G returned, booked me in for a general anaesthetic at some point “As soon as possible”, whilst also brushing away my apologies with more “No, really, I wouldn’t want to risk hurting you, we did the general anaesthetic last time and it all went really well so we can do that again.” and then sent me over to pre-operative assessment. Pre-op had a slot at 13.20, so I spent the next two hours reading and drinking coffee in one of the big airporty-looking lobbies that StJ is so good at, then went back up at 13.00 and was seen by 14.00. I’d have a letter within ten days, and would hopefully be re-coiled by the end of March.

And then on the way home the bus driver threw me down the stairs, then got snappy about it and insisted that there was no complaints procedure. So I’m going to have to have another fucking fight with the bus company.


Feeling really abandoned. Had four people lined up who’d all said they could help me out with tomorrow, and all four have cancelled. I’m not surprised – this literally always happens, I have only two or three times in my life had someone come with me to hospital when I’ve needed help – but it’s really unpleasant.

So I have two options;

1) Don’t have the procedure done. Spend another few months with no contracteptive.

2) Have procedure done with no sedation or pain relief. Cause damage to self that will take weeks to heal.

The fact that there should have been Humane Option 3) (Sedated at clinic, fitted with coil, supervised for rest of day by friend) is just rubbing it in that either I’m the unluckiest person on earth, or I’m just not worth worrying about.

(For clarity, before anyone tells me to man up – Yes, I know that coils can usually be fitted without any kind of sedative or anaesthesia, and this is pretty common on the NHS. I have a connective tissue disorder which means that any small tear in my tissues can quickly become a major problem. I also have uncontrollable muscle spasms which have, in the past, caused a speculum to break and leave a four-inch-long cut internally. My pelvic floor is abnormally intractable. My cervix is literally around a 110-degree corner. I have PTSD related to sexual assault with an item, meaning that people rummaging around my cunt occassionally makes me disassociate and become violent. I’m not just being a whiny bitch, these are genuine problems, acknowledged as such by my doctor, and that’s why I’m going to the specialist clinic in the first place.)


Inspider by Dr Inky’s recent post I thought I’d talk a bit about sports and how sports and disability can go together really, really well.

Firstly – I’m not saying “Oh, all disabled people love sports and exercise” because that’s patently bullshit; You can’t say “All disabled people [verb phrase]” any more than you can say “All [non-self-selecting-group] [verb phrase]”, but here’s a couple of reasons why you might find more disabled people at your local sports centre than you might expect (if you assume disabled people to be unathletic) and thus why, yes, parking in the disabled bay outside the sports centre makes you a tosser.

1 – Exercise is fun

Not specific to the disabled, but exercise is fun. Plenty of people who don’t exercise much say that they would, if only they had the time. Disproportionate numbers of disabled people are out of work, whether because they’re signed off sick, or just because employers discriminate against them. So, we often have time to take up some kind of sport, or stick to a training regime, or start a class or similar.

2 – Exercise is social, and usually social in an easy way

Going to the gym is simple. You get to one building, usually wheelchair-accessible, and you can move around inside that building. There’s often somewhere to eat, there’s people to chat to, and you can arrange to meet people or not meet people as it suits you. There’s also staff on-hand so if anything does go wrong, you can be scooped up and put back together or sent home in an ambulance as-needed. Compared to a night out drinking, it’s easier to get some human contact without horribly injuring yourself or being stranded waiting for a taxi at three in the morning by doing your socialising at a sports centre.

3 – Exercise at a sports centre is more accessible than any other kind of exercise, usually

Think about how much exercise an ablebodied person gets, just by walking. Taking the stairs, going back and forth to the kitchen, walking between bus stops, ferrying things around at work. Now, imagine that instead of these things being something that you do without thinking, that they’re immeasurably painful and make you want to cry, and that at the absolute worst, doing them could result in you needing a hospital trip and surgery. For a lot of disabled people, it’s a matter of either planning in exercise at the gym, or getting no exercise at all.

4 – Exercise is often good for the soul, so to speak

On one hand, there’s nothing worse than someone saying “If you had any self-respect, you’d get fit” but on the other hand, the sense of ownership of your own body that’s engendered by getting really caught up in a sport is a delight. Having a condition where so much of what your body does is out of your control, being able to guarantee a couple of hours a week of testing it in controllable ways and making measurable progress is wonderful.

5 – Exercise is sometimes the only cure

We have physiotherapists, or we have instructions from them, which often say something like “Train until you need to be poured into a bucket and carried home, then poured into a hot bath and poached back into shape”. Plenty of GPs will prescribe exercise as well, and give us cheap and/or free memberships to the local facilities (For example – I have a card which lets me swim or gym at any session for £1.60-£2.20, as opposed to the more usual £3-£5, and which gives me access to a number of completely free sessions as well, which are usually at odd times of day but are still available at any council-owned facility in the county) because it seems to help a lot of people. In the case of EDS, the only “cure” is to be fit; I’ve been told by enough doctors that I need to be the fittest person I know to have the best chance of a reduction in pain and increase in function, and it seems to be true. So you’ll get plenty of people who treat the sports centre almost as a place of worship – Make sacrifices to the bitch-goddess, and be rewarded.

But, as the Senator’s post points out – Most people don’t make the link between disability and sports-centre-use. The idea of facilities for the disabled, at a sports centre, seems like a joke to a lot of people (as in “As useless as a wheelchair lift at the climbing wall”) but really make a lot of sense. Every four years we hold the Paralympics – Where do you think the athletes train for the other 1,200 days? And that’s just the people who get to international levels in their sports; Trust me, ablebodied reader, if I was to cut off both of your legs now, you would not be winning the 100m T43 sprint in Rio. Disabled athletes aren’t just “Good for cripples”, they’re just plain good. (Interestingly, no matter how severe the actual symptoms, EDS swimmers aren’t allowed to swim in any paraswimming category. This annoys me, as although I can’t compete with ablebodied swimmers any more, I still want to play. Any other bendies fancy a race?)

I would just love it if there was more awareness of disability sport as being a thing that people can participate in, rather than being just an inspirational macguffin that gets shown off twice a decade. Not just one or two Paralympians doing a lecture circuit (Although that would be amazing in its own right, and I would love to hear more) but more acknowledgment that we do sports in general; More depictions of disabled people doing sports, so that clubs are prepared when they get members with impairments. More people talking about the differences between going to disability-specific club, and being a disabled person training with ablebodied athletes. Likewise, more knowledge and facilities for sports played mostly by disabled people (From boccia and walking football to the terrifying sledge hockey), and more acknowledgment that they aren’t only played by disabled people; Plenty of people will play wheelchair rugby, even if they’re not even a part-time wheelchair user, usually to keep a wheelchair-using friend company, but still. And just ingeneral, an acknowledgment that, yes, someone can be elegant on horseback, or a brilliant archer, or unbeatable over a 200m sprint… But still need someone to tie their shoelaces or take them to hospital appointments.

In the social justice sphere, there’s been a couple of “body positivity” posts which seem to pit “super fit” and “in a wheelchair” as being opposite ends of a scale (Paired images, fat next to thin, tall next to short… you get the idea). And they really aren’t opposites, they’re two completely different axes. An office drone who lives on Greggs pasties and hates going outdoors will be less fit than a wheelchair user that’s really into rowing and weightlifting and has a freezer full of steamed chicken and broccoli for immediate post-workout protein. I reckon that we’ll have full equality when the world at large recognises that the disabled can be annoying gym-rats, just as much as the rest of the world.

I realise this was mostly just a ramble, but it’ll all get re-done into a real post one day. Right now, it’s just thoughts.

Weigh-ins and how hard society fucks us up.

Before going into rheumatology, I had the usual blood-pressure check (124/90, high because I was in so much pain by this point already) heart rate (85bmp, again, pain) and weigh-in.

I spluttered when the nurse told me my weight, and she immediately tried to console me; “Oh, that’s not that heavy, I mean, you are very tall!”.

I did the maths in my head. “That’s just under nine stone!”

She loked at me blankly, then smiled.

“See? Not bad.”

I twitched. “I’m five foot nine. I’m supposed to be about ten stone. I’ve lost a stone, without noticing it. And that was my weight in  a leather jacket and assault boots.

“Ooh well lucky you then, even lighter! You can knock off a couple of pounds for them as well!”

On reflection, this may have been when my uncontrollable eye-twitching started. How have we reached a point wherein everyone is supposed to want to lose weight, even if they’re waking up ravenous in a haze of ketones every morning, and haven’t had a full meal in days? How is it that, even though if the nurse had really looked at me, it’s obvious that I’m underweight and suffering because of it, her automatic thought was that I must think I’m too fat and to try to console me that I can always be thinner?

Below the cut is a, probably fairly disturbing actually, photograph of me as I’ve been all morning – Naked but for my boxers, basically immobile. [spoilered for people who just wanted the commentary, and not my self-indulgent whine].

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Strange fortune

I’d gone to Rheumatology at StJ yesterday, to see Dr D, which was productive;

-He’s going to send me for a hip and shoulder MRI

-He’s going to point me towards a constulation with a hip surgeon* and a shoulder surgeon

-He’s going to chase Stanmore for me (Hooray!)

-He’s going to get my GP to put my Diazepam on repeat

-He’s going to look into different brands of pain-relief patch, just in case

-He’s going to ask C1, the head physio, to give me a list of textbooks and things so that I can be my own physio from now on

-He’s going to give Physio G a bollocking for being an inattentive, ignorant shite (even the Student Nurse that was in with Dr D gasped at the utter twattishness of saying to a hypermobile patient “Oh, no, there’s no such thing as a hip sublux. A Student!)

-He’s writing me an Official Letter to say “PLEASE for the love of all that’s good in the world stop trying to give him lidocaine!”

-He’s going to send me to another psychology service, to see if they’re more appropriate in helping me deal with my combined mental (The mixture between my EDS-linked stress, depression and anxiety, and my suicidal thoughts and bipolarity).

On the way into his appointment, my back and left shoulder went into spasm, and I ended up taking a lot of morphine (20mg) on the way in. Setting himself apart as a REALLY good doctor, he took the time to let me get settled (Perched on the balls of my feet on top of a chair, shoulders hanging forward like a chimp, as is my wont when I’ve fucked up my back and collar) and went immediately for both reassuring back-pat (At the prescribed 0.03m/s, circular, centred on the point of pain) and for genuine investigation of the worst of the shoulders – Feeling around the acromiclavicular and sternoclavicular junctions, measuring left against right, sticking a finger (carefully!) into the glenoid process and investigating under the scapula and around the bits of the spine (as usual, just between the lumbar and thorassic vertebrae, where I’m now sure there’s a problem) that hurt. He let the morphine do its work before trying to have any sort of sensible conversation, and he promised to send a letter covering the appointment, since he knew that it was both important to me, and that it was likely to be something I’d forget due to all the pain. He also pointed out that, even if I wasn’t actively noticing it, all the muscles around my shoulder and my back were in spasm, and that that was probably a bad sign.

On the way out, my back started getting worse. I had to drop to a squat three or four times in the corridor down to the main entrance (I may or may not have mentioned this before, but one of the great joys of StJ is that the main entrance hall looks like the departure lounge of an airport, and I’m really fond of it. Plenty of warm, accessible places to sit, a coffee shop and vending machines, and accessible toilets. Also always supervised.) and can vaguely remember wobbling down the central staircase since the lifts were still broken.

The next thing I’m really clear on is feeling a bit of spite towards the obviously-healthy paramedic who nipped into the disabled toilets ahead of me, then curling up on the bench outside the toilet, in pain, then handing off my medic alert bracelet and EDS card to someone who worked for the ambulance service, then being in a transport chair, then being almost flung out of the transport chair when it went over a bump, then a lot of screaming as my back arched to the point that my ribs at the back slipped under my pelvis, then a lot more screaming, then being in a cubicle on a bed in A+E, hearing someone say “He’s stopped screaming, but he’s biting his wrist really hard and I think I can see blood” then “We’re going to give you some morphine…” and having a syringe stuck between my teeth. (A further 10mg). I later found out that between the howling and sobbing, I was muttering “I just want to go home, please, let me go home” which apparently influenced my treatment.

At some point, I managed to re-open my eyes for more than a couple of seconds, and found myself talking to a rather sweet young doctor T, with blond eyelashes, who batted away my apologies, fitted a cannula to my right hand  (And he was right, he WAS the bloke to go to when the patient had odd veins – He listened to which one I told him was the good vein, used a very tiny needle, and investigated with the needle tip under the skin before going for the venipuncture, to ensure that the vein had run out of escape-room and that he wouldn’t just tear it open, like so many people did) with two ports – One for more morphine, one for dexamethasone.

It was probably about half an hour later that the nurses came and flushed it – cold up to the elbow, perfect – then admitted the morphine (Stopped at 4mg, due to a local skin reaction) then more water, then dexamethasone (No idea how much), then found me a cold flannel for my arm to stop the itching, since my veins in that arm were now black and raised up tight against the blotchy red skin with inflammation.

More time passed. I slept, I think, and the pain started to subside from “screaming whenever I stopped sobbing and biting myself” to “unthinkable” to eventually a dull ache through my whole body. The doctor came back, and volunteered that my best chance of saving the day without needing any worse intervention was to hurry home and take as much morphine as I could hold once I got there, and maybe a diazepam or five, then sleep it off.

One of the nurses removed my cannula, and I apologised for having been snitty and short-tempered, which she accepted with an “In that position, I would probably have been just as snappy” and I was allowed to go home. I shuffled out through the front doors, poured myself into a taxi, and broke down onto the settee in the living room. For the next five hours I drifted between sleep and dreaming, half-watching TV and feeling my left eye start to tic violently.

The tic continued, and by evening it had been joined by a horrendous ache in my sartorius in both thighs – that was an ache I would have expected in the morning, perhaps, after the previous night’s long swim (A performance in which my lanemate was a little dismayed that the Go-Faster-Juice I was liberally swigging as I churned through a 200×4 IM and 2km freestyle was morphine sulphate, not some kind of strong stimulant) but now it was happening and it really, really hurt.

Leery about taking more morphine (With the morning’s slow release and the fill-up when I got home, I was pushing 100mg) I fell asleep at about 2am, and slept like the proverbial brick.

Today, I’m sore, and my eye is ticking, but I feel remarkably good considering the previous day. Here’s to the dexamethasone doing me some good, and once again, hurrah for compassionate doctors who know that sometimes their job is just to alleviate suffering.

Also today, my letter arrived telling me that I have an MRI booked for the 1st of March (Yes, the Sunday) at 1.20pm, at CA X-ray department. With instructions to take out all my jewellery and bring a CD – I’ll probably go for Reise Reise or Rosenrot. Or both. This of course reminds me that I have no idea where any of my CDs are.

Next post is going to be a little pictographic coda to this one, about a single encounter with a nurse which was the only bad encounter all day, and it wasn’t really her fault so much as Society’s fault.

*”Who’s the coolest person in the hospital? The hip surgeon! What about when they’re on holiday? Then it’s the hip replacement!”

Pain upon pain

I’ve mentioned a couple of times that I sometimes self-harm, largely as instant pain relief (This is why I want to get some faster-acting painkillers, so that I don’t end up resorting to the scalpel), and yesterday’s complete shoulder-girdle pop (Acromiclavicular, sternoclavicular, glenohumeral, scapulothoracic… argh) was enough to induce an episode. Unfortunately, it was enough to induce an episode even after filling up on morphine. The result being a lot of very deep cuts that I didn’t really feel very much of, and needing to limp over to the pharmacy (On wrecked hips, making matters worse) with my cane in my off-hand (due to my left shoulder being in a million pieces, and my left forearm leaving a trail of blood almost all the way from my door to the shop) to get bandages and antiseptic.

Arriving at the pharmacy, though, I obviously couldn’t just get the bandages and leave, because I was bleeding all over their floor and obviously shaken up and with one hand almost completely grey and an immobile shoulder. I was sat down in the consulting room, wiped off with alcohol, asked politely if I was on any psychiatric medications, then allowed to bandage myself up and go home.

The irony being that by the time I’d wrapped up, the painkilling effect of the damage had worn off, and I was just as knackered and sore as I would have been otherwise – In addition to the pain of having to go out and buy all the sodding antiseptic.

Ah well, rheumatology tomorrow. In preparation for that, I rang Stanmore today to see if they had my referral, from either UCLH or from my GP, and they’ve not got me at all. So it’s honestly starting to look like all the London stuff was a waste of time and effort on my part, sine nothing has been done about it. And I still have to fight tomorrow to get imaging on my shoulders and hip. That’s the imaging that I was first told that I needed in July and November, respectively.

The wheels of bureaucracy do grind slow.