Zugzwang

So, a few days ago I posted this deliberately-upsetting video to YouTube;

 

 

Which quickly got found by a big Dr Blog, cranquis.tumblr.com, ran by an American medic called Dr Cranquis (It’s very good, he’s very funny and obviously a very good doctor too).

 

And this in his response made me jump out of my weird, stretchy skin;

 

“When EDS is taught, the emphasis usually is about the plasticity of the patient’s connective tissue, the genetic transmission, the “double-jointedness”, and the risk for blood vessel ruptures. There’s always at least one old-timey picture of a “circus Plastic Man” stretching his neck skin way out, for some reason. But while the “easy joint dislocations” are mentioned, I don’t recall anyone emphasizing the (obvious, in hindsight) risk this would create for excruciating pain.”

 

Because this exactly reflects the complaint that I keep making; Medical teaching programmes and textbooks, when they go into EDS at all, always emphasise the visually-dramatic. They go for the most obvious even-my-dog-could-tell-you-that’s-luxated luxations, the stretchiest stretchy skin, the most pronounced micrognathia, enlarged orbits, and visible veins and the most extreme weirdly-proportioned habitus. And they take the photographs in such a way that they show up the criteria as much as is possible, foreshortening and back-lighting and putting in perfect profile, whilst twisting the limbs and fingers around into the most extreme examples of the Beighton warp.

 

So doctors learn by seeing the most blatant, and not the most common, cases. They don’t see the tall girl with long fingers who’s always clutching her back in pain but insists that it’s just overtraining. They don’t see the boy with huge blue eyes, whose hips click a bit when he walks but in ten years’ time he’ll be in a wheelchair. They don’t see the rugby player that keeps “popping” their shoulders whenever they stand up from the scrum, that lives in a constant cloud of Deep Heat and freeze-spray. They definitely don’t see the forty-year-old with visceroptosis and a uterine prolapse, but otherwise no symptoms other than sore hands that they put down to old age. They see the depressed teenager who can’t eat, who claims to feel full after two bites of a pasty, and has a drawerful of laxatives, and they insist that it’s an eating disorder even when he cries over how thin he’s getting. They see the toddler with fist-sized bruises, and they call social services, and refuse to believe that they really do just keep falling over and that the parents are just as worried as the doctor. They see the pain episodes, like the one above, and say that it’s drug-seeking, psychosomatic or exaggerated, because EDS is just that thing that makes you a circus freak, isn’t it?

 

We need some kind of campaign that says that the reason that a lot of people don’t see zebras is because we look and sound basically like stripey horses.

My Brain On Drugs

I’ve written more than a few times about the long-term use of drugs, and pain, and family and friends’ attitudes to medication and the shame that always seems to come with being dependent, and Bruce Alexander’s Rat Park Experiments, as compared to how GPs seem to think about addiction, and even about overdose, but I’ve recently realised that most of these are buried at the bottom of posts about my own situation, rather than standing on their own.

So here’s some kind of masterpost of my thoughts on drugs. I might have directed you to this because you asked a flamingly obvious question, or because you were generically ignorant, or because you were spreading hateful misinformation. Please, read on and be enlightened.

 

GPs and medication

For some reason, some GPs seem to think that they are are be-all and end-all of medical knowledge. Even when a consultant, or a surgeon, or a physiotherapist, or a headpoker has told them otherwise, they will always insist on sticking their neb in and fiddling with a patient’s prescriptions. They pad their ignorance of the case-in-hand by talking about side effects, the dangers of addiction, and just “not being comfortable…” with particular courses of action. Even though, usually, all of these things have been talked through between the patient and the specialist, and the prescription suggested is the one with the best chance of causing the most improvement with the least unacceptable side-effects. If they are somehow persuaded to just issue the prescription, they’ll try to reduce the dose, or reduce the number of tablets given, or insist on reviewing it themselves every month, in spite of having no useful input since they never take useful notes on the patient and never trust what the patient is telling them about their condition.

 

Addiction

Addiction is pretty much always presented as a) likely and b) the worst possible thing that can happen to a long-term opiate or benzodiazepene user. The truth is that it’s been pretty conclusively proven, in multiple studies, that substance-mediated addiction doesn’t really exist. So a patient taking morphine for pain, even for years and years on end, won’t become addicted. They might become tolerant, and need a higher dose to achieve the same effect, and they might suffer physical withdrawal effects when they initially stop taking it, but they will not become addicts.

I maintain that addiction is still only a problem if it puts the user in danger. If the drug supply comes from a reputable source, and the dosage is kept low enough to not cause physiological problems, and the user’s normal functioning isn’t impaired, who cares if they motivate themselves to go to work in the mornings with the knowledge that they can get high when they get home?

 

Tolerance

It’s a popular myth – That someone starting out on a small dose of opiate painkillers will have to take larger and larger doses over time to get the same effect. It’s just, fortunately, not true. There’ll often be a large increase in the first year, whilst the doctor and patient work out together what the right dose is for the patient to get all of their symptoms under control, and indeed what the right type of pain relief is (Lots of PRN? Slow release? Patches? Combinations? Multiple families of drugs or just one? How do they conform to their routine, do they need something simple to remember, or can they handle a dozen things with a dozen different dosing parameters?) but developing such a tolerance that a drug just won’t work is rare.

 

Balance

It’s sometimes hard to work out what’s a detrimental side-effect of a condition, and what’s a detrimental side-effect of medication. It’s personal to the person with the condition, especially in what someone will tolerate – Some people would rather feel slightly queasy all the time because of a drug that disagrees with them, whereas others would rather put up with the original condition. The most famous of these dilemmas is probably with SSRIs, when used to treat “simple” depression – Some people prefer to deal with the depression on their own or with talking therapies alone, rather than to have their sex drive and performance ruined by SSRIs, which is one of the more common side-effects. And the same applies to painkillers, and even with PRN painkillers, from dose to dose; On Monday, Jim might prefer to be in more pain, but more clear-headed, whereas on Saturday he might give himself a “day off” and take enough to no longer be in pain, even if it’s enough to make him floppy all over and to want to tell his friends about a lovely imaginary cow called Grenache.

 

The Fabled Morphine High

It goes away. Someone on slow-release morphine won’t really be “always a little bit stoned”, they’ll just not be in pain. For the first week or two, they might feel a bit weird, but that’ll probably be the all of it. Someone taking their prescribed dose of PRN morphine, after a few times, won’t feel a high from it, they’ll again just suddenly be in less pain. Occasionally, the appropriate amount of morphine for a situation will still get you high, even after years of use. It’s not unpleasant, it’s no more dangerous than the amount of pain that it’s masking, and frankly it’s often difficult to untangle it from the pain-relieving effect. Various opiates and opioids have different effects – Some are extremely soporific, some are extremely efficient at only effecting the nociceptors, some have a gentle antianxiolytic effect, some just make the pain feel less urgent, as if the mind is floating away from the body. They’re all components of how they relieve pain.

 

Willpower

There’s no grand moral imperative to not take drugs. That’s not a natural law, it’s some kind of conservative anxiety about other people getting an easy ride, or a puritan anxiety that people might be enjoying themselves, or possibly an anti-technological anxiety that drugs are science and science is bad. Sometimes, it’s a bigger act of willpower to say “No, I’m going to stop doing the [fun or necessary thing] and lie down and take my medication and rest instead. Even though I don’t want to,” than to try to continue as is nothing was wrong. Society hammers into us from an early age that we have responsibilities which must be discharged before we can rest, and some of those make sense – You have to get the bairns home from school, or turn off the open gas flame on the hob, because those are things which will be unsafe if not attended to – but some of them are not sensible; There is no reason why you must vacuum the living room carpet or go out to a dinner party if you’re exhausted and in pain and just want to lie down. They’re not as fundamental on Maslow’s Hierarchy as not being in pain. And for that matter, there is nothing wrong with delegating. Sometimes it’s fine to say “No, you can make the tea, I need to take a load of pills and lie down in a dark corner for a while.”

 

Other People

It is not up to anyone but you how you take your medication. Anyone saying “Just take less of it! I hate it when you’re on morphine!” is saying “I prefer it when you’re in pain, because you’re more useful to me like that!” Work out what it is that they want – Whether that be housework, a share of your wages, sexual performance, more attention or any of the other billion things that one human can want from another – and make it abundantly clear to them that, without your painkillers, you won’t be able to give them more of what they want, you’ll just be disappointing them whilst in pain, rather than disappointing them whilst in relative comfort.

 

Bodily Harm

Being in pain, long term, causes damage. Pain causes spasms, which can rip muscles out of insertions and snap bones (An orthopaedic surgeon once memorably told me about a muscle spasm snapping someone’s femur. It may have been a diseased femur, but it was a femur. Think how thick a femur is.) even before you come down to the way that a muscle spasm will make you drop whatever you’re holding, make your paintbrush wobble across the page, or make you slam the throttle of your bike so hard that you fly forwards at a hundred miles an hour. Pain causes hypertension and an increased heart rate, which can lead to heart attacks and all sorts of other things. Pain causes lack of appetite, resulting in long-term weight loss and often tooth decay from never chewing, so never producing sufficient saliva. Pain causes anxiety and depression and frankly can just make you into a horrible person to be around. This is not to mention the number of people who kill themselves every year due to being in untreated, long-term pain.

Long-term opiate use is safe and effective. The constipation is treatable, easily and safely. The chance of liver or kidney trouble, if taken within prescribed amounts, is infinitessimally small and can be tested for long before it becomes a problem, if there are any warning signs.

 

This post will get longer over time. Thank you for reading.

Ask Lots Of Questions, Don’t Eat The Bullshit

I’ve commented before that most zebras seem to be somewhere near the middle of this Venn diagram, and that I think I’ve only ever met a couple of zebras who weren’t on it at all, especially if I broaden “scientist” to include all kinds of research-and-stats-based jobs;

zebradiagram

(Go ahead, zebras, put yourselves in the diagram. I’ll be amused.)

As much as it could really be that there’s a genetic disorder that strikes only bisexual autistic physicists who cam in thier spare time to fund their research and are writing a novel about the whole experience, I think it’s much more likely that it’s more to do with the bias on who gets diagnosed;

All of the above states predispose the zeb to either asking questions, or to having a healthy disregard for social expectations.

The scientist zeb has a background in assembling data and testing theories, so is more likely to work out that all the Just-So stories add up to one overarching condition, and the medic zeb might even have heard of EDS in the first place.

The autistic zeb is more likely to spot the pattern of symptoms in the first place and to insist on taking them seriously, plus probably already has some knowledge of the inner-workings of the medical system, so will know where to stick the claw to get the treatment or diagnosis that’s needed.

The sex-working zeb has already got a foothold in the door of ignoring social mores and pursuing something which society tells them not to, so is more likely to keep asking the doctor the questions that need asking. And they’ve probably got plenty of people-skills, to make sure that they get answers.

The artist or writer zeb is probably better at explaining their symptoms to a doctor than the average ineloquent sod, and the zeb that works with their hands (including the musicians) is more likely to spot the early signs as their work starts to get sloppy and their wrists start to hurt, prompting them to seek diagnosis earlier than someone who barely picks up a pencil.

Finally, the LGBT zeb has already probably found one community that they weren’t born into (see any number of articles on the LGBT community, which is inherited by finding, vs most communities which are inherited by birth or proximity) so will be quicker to find the disabled community and to integrate into it, therein to find other zebs and put a name to their own condition.

(I’d be remiss at this point if I didn’t also posit that, due to the idea of zebra-senpai and just how friendship groups spread, I’d be surprised if there isn’t a self-selecting bias there based on the kinds of people that I and my firends are friends with anyway.)

So here’s a brief “What to expect if you’re expecting to be diagnosed with Ehlers-Danlos Hypermobility or Joint Hypermobility Syndrome”.

  • You’ll have been to your GP complaining of pain, lots of pain, or will have turned up in A+E with a mysterious dislocation. Either way, (In the case of the A+E scenario, after having reduced the dislocation and sent you home with painkillers) your GP will want to take a lot of bloods to rule out autoimmune conditions (Sarcoidosis, rheumatoid arthritis) and infections. The bloods will have come back “all normal”, so you’ll be sitting in the GP’s office, munching co-codamol by the handful, and they’ll prescribe you an anti-inflammatory (Usually an NSAID), a painkiller to take when the pain gets too much (Usually an opiate, probably either more paracetamol-codeine, codeine itself, or dihydrocodeine), will forward you to rheumatology, and will put you on the list for physio. They’ll send you home.
  • The painkillers may or may not work, and you might have to go back a few times, often over the course of more than a year, to get settled onto a regimen that suits you. Talk to your doctor – Try different combinations of every-day (Prophylactic) and on-demand (PRN) medications. Try different families of anti-inflammatories, try steroid injections, try topical creams. Try opiates as slow-release pills, as patches, as injectables, as syrups, as sub-lingual melts. Try higher doses of weaker opioids, and lower doses of stronger ones.
  • The rheumatologist will ask more questions – This is where you’ll be most likely to do the Beighton Warp and get asked about things on the Villefranche list; How do you sleep, how’s your eyesight, do you get migraines, how are your bowels, do you bruise easily. They might want to do a fairly close examination – I was down to my pants and having the skin over my hips stretched, my legs moved around at odd angles, and my back and shoulders really flexed and put through their paces. Thankfully, most rheumatologists are used to patients in pain, so they’ll do this in a warm room, with a comfortable table, and will be amenable to stopping and taking breaks.
  • At this point, you might get a diagnosis, or you might get sent on to a specialist hypermobility service. The big point of the hypermobility service is to confirm that it is hypermobility syndrome, and to get access to more specialist treatment options if they exist in your area (Specialist physio, for starters) and to suggest referral to services like Stanmore’s hypermobility unit.
  • You might be referred to the appliance or occupational therapy departments who’ll fit you up with splints or braces as-needed, will install grab-rails in your house, and will in incredibly rare cases possibly fund you for a wheelchair. I have never known ANYONE get the NHS to get them a wheelchair though, so if you need one, and can afford it, just buy it. It’ll save you grief in the long-term.
  • You’ll also be getting physio, on-and-off, to deal with the pain and the losses of function. This is where the problems often are. The rheumatologist and the hypermobility specialist will say “Physio, once a month, for the rest of your life, to keep your exercises up-to-date and monitor your progress, and probably spot any minor problems before they become major ones.” The local physio department will say “Six weeks of physio, then, sorry, you can’t just keep coming back, we need to treat people who will get better.” then discharge you.

This brings me to the biggest point about getting a diagnosis and treatment;

  • It’s only the people at the bottom of the pile who will be unpleasant. Your GP will call you a faker and try to convince you that you have a personality disorder, NHS Direct will be convinced that it’s not a dislocated shoulder, your local walk-in service nurse will tell you to go away and be in pain somewhere else – But rheumatology and the specialist hypermobility service will have nothing but sympathy for you. thye’ll listen, they’ll believe you on the worst and weirdest of your symptoms, and they’ll often apologise for the behaviour of their underlings. If you can get past the GP, and have a rheumatologist of your own, you’ll do fine.

This, incidentally, all explains why most zebras who know that they’re ebras tend to be highly-motivated and highly-educated; They’ve got to get themselves diagnosed with a condition that doesn’t present in bloodwork, has symptoms ranging from “My knees hurt” all the way up to “I need to be fed intravenously”, and which in all likelihood their GP has never heard of beyond a brief lecture at the start of first year saying “You’re never going to see a patient with any of these conditions…”.

I firmly believe that the NHS needs to have a complete restructure of how it treats patients with rare conditions.

So, what do we do, as zebras and friends of zebras? Look back up at the title.

Go to appointments with a notebook and a pencil. It makes doctors nervous and commensurately less likely to fob you off with “go away”. Plenty of “Can you just repeat that, so I can write it down?” “Right, so if I phone [service] and say you sent me…” “Why is that an appropriate treatment? Why does [problem] not worry you? WIll it get better on its own? Why not treat the symptom whilst I have it then?” And write down everything.

Provide moral support to friends – If they want you in their appointments, go with them. Doctors are less likely to be patronising and aggressive when there’s a witness, especially if the witness looks concerned; “But [friend] is in pain, and [drug] is helping them. I was really worried about them. Why can’t they keep taking [drug]?” “I can confirm that it really is weeks on-end, I’ve been with them the whole time” and possibly the most cutting and guilt-inducing “Thank you for looking after my friend”.

Arm yourself, and get used to quoting chapter and verse – Memorise the exact details of both seminal and recent papers on EDS, on management of long-term conditions, on drug use and responses to other therapies. Learn what interacts with what, what’s controversial or not supported by enough evidence. Don’t just be a parrot either – You need to understand what you’re citing. if you don’t understand, don’t go out on a limb – You’ll sound more intelligent and confident if you stick to what you do know well, rather than if you try to bluff knowing more about a topic than you do.

Independently check everything that you’re told. X is better for you than Y? Go home and find the study that says so, or says otherwise. Z will go away on its own in a week? Find out from other people with the same problem. Just question everything, and let it be known that you’ll question everything, and eventually doctors will stop trying to fob you off.

But the short version is very definitely just “Ask lots of questions, don’t eat the bullshit.”

Wearing Silver

It’s overdose awareness day, so I thought I’d blog.

Overdose is one of those words that is freighted with an awful number of connotations, and those connotations are basically all awful.

“Benny died of an overdose.”

And the wheels start turning in the minds of the ignorant:

What did he overdose on?

Well, if it was prescribed by a doctor, he was either too ignorant to take it correctly, or he was suicidal. If it was something he’d been taking for years, he must have taken too much because a “normal dose” wasn’t giving him the right effect, and he was turning into an addict. If it was an accidental overdose, the doctor shouldn’t have prescribed him something so dangerous. If it was a deliberate overdose, someone (anyone!) should have been watching him more closely. Drugs like that shouldn’t be prescribed, ever.

Well, if it was over-the-counter, it shouldn’t have been available in the first place. What kind of world do we live in where things that can kill people are available without a prescription?

Well, if it was an illegal drug, he was either a terrible criminal or a poor fool who was preyed on by a terrible criminal. If he was an addict, this was always coming and it was his own fault. If he had just taken it for the first time at a party, his friends are all evil and irresponsible and should be pilloried forever, with no acknowledgment that they’re in mourning too.

Was it a deliberate overdose?

If it was accidental, the stuff – whatever it is -shouldn’t be available anywhere in the world ever, and posession of it should be the worst sort of crime, punishable by life imprisonment.

If it was deliberate, then the usual tropes about suicide come out. Selfish, or melodramatic, or uncaring.

And the question that everyone seems to want to ask, in the worst part of their psyches;

What did it look like? How did it feel?

Because enough of us have heard of Nembutal, or or just quietly turning up the IV morphine, or rock stars being found with the needle still in their arm, or horror stories about paracetamol or cocaine, that people have a prurient interest in these things.

No. there is only one kind of question that you ask;

“Are you all right? Is the family all right?”

I don’t have much to say on the emotive front. I’ve lost more than one person to overdose – One good friend, as a teenager, who was taken by a fatal episode of mental illness. One older relative, who knew that there was no light at the end of the pain tunnel, and made an exit soon after saying her goodbyes. I’ve attempted it myself, and learnt the indignity of stomach pumping, short-term dialysis, liver function tests, and not quite being able to look your friends in the eye.

So here’s the practical front;

What to do in the event of suspecting an overdose

Call 999 – An ambulance should arrive within 30 minutes, as an overdose is one of the highest categories of urgency.

Start CPR if the person isn’t breathing, continue until ambulance arrives.

If they are breathing, place in the recovery position.

Do NOT induce vomiting, but if they do vomit, encourage it (Sit them upright, lean them forwards, let them gargle with water in between heaves, without swallowing).

Do not let them eat or drink.

Gather up the packages of whatever you suspect that someone has overdosed on. If there are remaining samples of the drug, give them to the paramedics as well.

Try to ascertain if the person has taken any other drugs, including prescription medication, or alcohol, or food or drink.

Talk to the person and try to keep them calm.

A key thing to remember, if you suspect that someone has overdosed, is that the first stages of a drug overdose often look nothing like the media portrayal of such – The person won’t simply fall to the ground, sweating, shaking and vomiting up brightly-coloured capsules by the dozen. Every chemical has its own syndrome, broadly speaking, and each syndrome will present differently depending on how the drug was administered (IV looks very different to something which has been swallowed or smoked). “A Small Dose Of Toxicology” (Steven G Gilbert) has any number of case studies, so I won’t write them all down, but here’s what a morphine (or a lot of other opiates or opioids) can look like in overdose;

What a morphine overdose looks like

-Extreme tiredness and faintness

-Dizziness

-Drooping eyelids

-Pinprick pupils, and light sensitivity

-Itching skin (usually)

-Slow pulse

-Shallow, slow breathing

-Nausea and probably vomiting

-Thirst

-Flushed skin

-Feelings of overheating

-Unconciousness

…And all of those can signify anything from “Has taken a bit above the required dose, will feel a bit grim tomorrow” up to “Get to the hospital, now”, depending on the person involved.

As for “Why on earth are these dangerous drugs even prescribed?” I’ll leave you with a bit of Paracelsus;

“Alle Dinge sind Gift und nichts ist ohne Gift; allein die Dosis macht, dass ein Ding kein Gift ist.”

All things are poison, and nothing is free of being poison; It is only by regulating the dose that we can prevent a poison from acting.

Paracetamol is toxic. Alcohol is toxic. Peppermint is toxic.

As long as we try to artificially teach people that there are some drugs which will kill them instantly, and other drugs which are one hundred percent safe all the time, we will fail people. We will fail the people who are in pain, but avoid opiates because “Opiates will kill them” and we fail the people who end up with their livers destroyed by having one too many paracetamol for their bad head.

As long as we try to teach people that simply touching a drug like heroin or meth is enough to taint them forever, we will fail the people who end up overdosing because they feel that their life will never be anything but their addiction. We will fail the people who do overdose, then refuse treatment and end up further harmed because they don’t want the stigma of their doctors or friends knowing what they took.

As long as we teach that dying of an overdose is something that only happens to the careless, the criminal and the inherently tragic, we do a disservice to all of the people who have been touched by overdose – Whether deliberate or accidental, fatal or survived, as the person who took the overdose or as someeone who loves someone who has overdosed.

We need to put our house in order about drugs, and start teaching based in evidence.

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.

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.

Swings and Roundabouts in the Rat Park

Yesterday night was a long, long night. Got to sleep around 4am, woke up at 5.30 after a nightmare that was violent enough that I fully dislocated my shoulder in it, leaving me basically trapped in bed, then dozed on and off unti leaving the house at 7.30 to get to my 8am GP appointment with Dr Hpm. I had five questions, and these are they, as I asked them, with the answers;

1) Can I get half of my Laxido swapped for glycerin suppositories?

a) Yes, of course. Here, have a non-repeat prescription for twelve infant-sized 1g glycerin suppositories, which the pharmacist advises “Oh, just take five at once”.

2) Can I have my NSAID changed to a stronger one?

a) Yes, of course, have some diclofenac. I somehow manage to leave without a diclofenac prescription though.

3) I’ve not slept normally since March, it’s making me exhausted and feel like shit, and I’ve been taking far more diphenydramine than I really want to. Can I have some help?

a) No, because you might end up addicted to sleeping pills. Have you tried a hot milky drink? (Literally. He actually invoked the legendary ‘hot milky drink’. He may as well have said ‘How about turning off all the screens and sitting quietly for an hour before bedtime?’ This ignores the fact that I no longer have a ‘bedtime’, and that I wake up screaming at least twice a night. He said that sleeping pills were only very rarely given out, nowadays, and that they were worse than heroin and stopped working after a week (All verifiably false, by the way, I know plenty of people who take zopiclone regularly and have done for years, and it still work.)

4) Can I have more diazepam, the stuff that everyone has been happily prescribing me on an as-needed basis for most of this year, and that has been the only thing to reliably stop the muscle spasms?

a) No, because it’s addictive. We don’t want you to end up with an addiction problem on top of your existing problem.

4b) Can I have a non-addictive antispasmodic then? I do need something to stop the painful spasms which can cut through heavy doses of morphine and turn my hands blue.

a) No, have you tried putting a heat pack on them? Or an ice pack? Two pounds of frozen peas, that’s the best thing.

5) Physio told me that my GP was the one who needed to sort out the investigations into my hip and give me the steroid injections. Will you?

a) No, ask Rheumatology.

In fact “Phone them up and ask yourself” was the answer to all my questions about other departments and trusts – Rheumatology, Stanmore (With the addition of “Say you’ll take a cancellation, that you’ll get down there at a minute’s notice”), CBT for Pain. Everything.

He was also very, very concerned about the fact that I used morphine as a painkiller. Worried that I was addicted. And lo, has kept me on half rations for a second month, meaning that I’m going to spend another month in pain. If you give me 60 tablets to last 30 days, and tell me to take 1-2 every 12 hours, I’m going to run out by about week three. Or I’m going to go back to rationing out my medications, and spending most of my time in a medium amount of pain. And sleep less.

But now, really, I want to talk about addiction.

The title of this post for once actually has meaning, rather than just being a random thought that’s popped into my head whilst loading the page. The Rat Park experiments were a series of experiments (no shock there) done on rats (surprise!) by Bruce K. Alexander that involved altering various features of their environments and seeing how willing they were to take morphine in various situations. The (simple) version of the outcome of the experiments was that there’s very little basis to assume that a drug-induced adiction theory is correct.

So, what’s drug-induced addiction?

Basically, there’s two ideas about how people become addicted to substances; Drug-induced addiction is that the very nature of the drug makes people addicted to it (This giving rise to myths like “If you take crack cocaine once, you’ll always need more, because you’ll be addicted”) and the other is that it’s the social factors around drug-taking that make addiction possible in the first place (Alexander’s words were “Severely distressed animals, like severely distressed people, will relieve their distress pharmacologically, if they can”). Effectively, if (as a human) your association with morphine is that it’s the thing that you take for fun and to get high, you’re likely to get addicted. If your association with morphine is that you take it to relieve pain, even though you’ll incidentally get high, you’ll possibly end up with a dependency, but you won’t end up addicted.

If you’re interested, look up Bruce K. Alexander’s papers on addiction, he obviously talks about it in much greater depth than I can here, and with much better clarity.

I am dependent on morphine. I sometimes take it at levels which could be dangerous for someone who is morphine-naive, and usually take it at levels which would make someone morphine-naive very lightheaded and drowsy. This is because I have a tolerance for it, which is another side effect of regular use. My need for morphine is because I have regular, acute, musculoskeletal pain, radiating from constant fresh injuries, for which I must take a strong painkiller. The strong painkiller which works best for me is morphine. Thus, I am dependent on morphine. If I was no longer in pain, I would no longer take it.

You probably know what addiction feels like – Most people have felt headachey and faint after missing their morning coffee, or short-tempered and generally terrible when craving a cigarette. And with that feeling of “urgh” comes a craving for more – More caffeine, more nicotine. If I go for a week without morphine, I don’t feel terrible, and more importantly I don’t crave more to take the withdrawal effects away. For the first day, I get a slight headache and feel stiff, bruised, sleepy and emotionally fragile, but my drug of choice to fix that isn’t “more morphine”, it’s a paracetamol, and lots of fruit juice, and maybe a nice heavy breakfast. If I was addicted, my first instinct on feeling that distinctive bruising around my eyelids wouldn’t be “Oh, time for a big cup of tea and a lazy day” it would be “Take the morphine.”

I’m dependent, I’m not addicted. And I know that having a life without hypermobility related pain isn’t possible, but that the best chance I have for getting somewhere close to that is a mixture of exercises and medication. And the medication makes it easier to do the exercise. Grim as it sounds, even if I was addicted – If I motivated myself to go out and do things with the promise of morphine when I got home – that would still be better than spending my entire life in pain and miserable, for the sake of not offending the puritan sensibilities of a doctor.