What’s it like to have open heart surgery? Part II

It’s been five months since my surgery, and I am still – touch wood – alive and healing. Actually, I’m feeling great. Please keep that in mind while you’re reading this!

I may have been putting this post off. When I started this series of blogs, my intention was to write a plain description of the experience to give prospective patients a clear idea of what they were going in for. But that approach comes with a lot of responsibility. The absolute last thing I want is for someone to read about my experience and think “No thanks, not today, I’ll pass if it’s all the same to you”. I don’t want to put anyone off, but I also don’t want to pretend it’s a doddle.

It shouldn’t be a shock that living with heart disease is all kinds of annoyance and misery, but I think it does need to be reiterated from time to time. As the staff nurse who greeted me as I walked into the ward said, “This is going to be rough.”

My surgery date was April Fool’s Day because of course it bloody was.

I took my big black bag with me. I wore a plain hoodie and some jeans, and took out all my piercings except my nose stud. Amazingly, they let me keep that in for the entire experience. I signed a form stating exactly what I had with me and what was being left in the hospital safe. My surgery was set for 8am the next morning as that was the slot least likely to be cancelled. Unfortunately, that meant my family were sent away and I was left alone with my thoughts all night.

2BD84076-344C-4426-9CBF-5C5CA63B6622My window was on the ground floor, looking out onto Papworth’s green surroundings. A little out of sight was the famous duck pond. I saw a heron fly over, seeking fish. There was a lock on the window. I looked at that lock all evening. Before bed, I was told to shower and wash my hair with antibacterial gel.

At 10pm one of the surgeons came to see me and discuss valve options. My surgery was planned as valve-sparing, meaning I’d have my own aortic valve reattached when my new prosthetic root was in place, but because that isn’t always possible I needed to choose between a mechanical valve (meaning lifelong blood thinners to stop it rejecting) or a pig valve which could wear out within fifteen years. He explained the various pros and cons of both options, and I made a decision and signed for it. Then he outlined the really fun statistics – mortality and whatnot. With my aorta measuring 4.5cm, it was statistically more dangerous for me to be walking around than lying on an operating table. Average mortality is really, really low for aortic root replacements, even with Marfan syndrome taken into account, and the figures are actually even lower with my surgeon, Mr Nashef. He’s kind of a rockstar.

Nevertheless, it’s quite a thing to sign your name under that statistic, sitting there in your glittery slippers.

No, I didn’t sleep a single solitary wink. It wasn’t so much anxiety as my heart monitor shouting every few minutes to tell the nurse I was having ectopic beats. I told myself I’d have plenty of time to sleep the next day and settled down listening to Rammstein all night. Fight music for punching the Grim Reaper in the eye socket. Before the nurse came to take me away at 8am, I had to repeat the shower ritual and get into a hospital gown and my fancy slippers.

I was wheeled to theatre. I could have walked, but they just default to wheelchairs for some reason. I’m the kind of prisoner who jokes on the way to the gallows. Somehow the nurse and I were talking about crossword puzzles. Surprisingly, I was then held in a queue, parked up with a row of old men in identical gowns. There was one young girl, bald from chemo, but she was sent to the other side of the room. No one spoke. Staff whirled around. It was cold. I kept telling myself it would be over soon. There’s really nothing else you can tell yourself at that point.

My team introduced themselves. It takes a lot of people to replace one aortic root. I met so many people, I hardly took it in. When my two anaesthetists asked if I had any questions, and because my brain is the way it is, I blurted out something about the possibility of waking up in the middle of it all. And yes! That can happen if you have really lousy anaesthetists. Now you know. Hooray for me for asking that unbelievably stupid question at that precise moment.

I was wheeled into a small, chilly antechamber with a bed and several nurses, as well as my (not lousy) anaesthetists. Here I would be given a sedative through a cannula, followed by my general anaesthetic. I think they also attached me to some monitors in here, though my memories drift into vapour at this point. I can remember holding a nurse’s hand and saying “Just feel free to mess me up” as the drugs took hold. They do the job – you feel drunk and accepting, and full of big hilarious love. I’m pretty sure I told everyone in that room they were perfect angels. I wasn’t aware of them inserting the larger cannula that goes into the underside of the wrist, and for that I’m grateful.
wfms-usability-ehr-041_0I was in theatre for seven and a half hours.

I don’t remember waking up in Critical Care. I’m told one of my aunts was there to witness me first opening my eyes, and I gestured at the ventilator tube down my throat. I was worried about that part, waking up with the tube, but I don’t remember it being uncomfortable. My aunt thinks I was gesturing apologetically, as if to say “Can’t speak”. I certainly wasn’t panicking, but the nurses are prepared for that eventuality and just sedate you again if you’re a flailer. It’s rare, though. They have to leave the tube in until you can prove you can breathe without it, and though I don’t remember it being removed I know the bottom of my lungs were some of the last parts of me to wake up, so breathing is a lot like when you’re wearing a corset – upper lungs only.

When you come round, you’re on a lot of medication. It’s not like waking up from a small procedure. My family tell me I looked very peaceful and amazingly healthy, and if it weren’t for the enormous bag of spare blood and all the wires, I’d look like anyone else having a pleasant nap. From my perspective, I felt strangely immobile. I knew I couldn’t move, and though it wasn’t panic I was feeling, it was very unfamiliar. I was alive and relieved but I’d been airlifted into some new reality and yesterday was a million miles away. Slowly, very slowly, as I slid in and out of sleep, I became aware of all the things attached to me. I had tubes coming out of just about everywhere, but at that stage no pain. Weirdness, but no pain.

You’re never left alone at this early stage. I had a nurse called Rose – I think? – sitting right by my face all night, mopping my brow and comforting me. I was whispering all sorts of hoarse nonsense to her, and she just smiled and agreed with me. Another nurse was able to tell me that the girl with the bald head had got through her surgery too. I asked him to tell her I was proud of her (there’s that big hilarious love again).

I was able to move my arms a little, but Rose brushed my teeth for me and kept my glasses clean, giving me small sips of water. I was sweating like a pig. Your body is cooled during the surgery, so when you wake up your ability to gauge temperature is completely out of whack for days or weeks. I could only speak in whispers. Every physical act was a monumental task, but at least I was in bed with nurses doing everything for me…

Hollow laughter.

Once upon a time, heart patients were kept in bed for weeks. Those days are long gone. Never mind that your feet are made of concrete and the floor is so far away you can barely dream of it, on day one you’re expected to get out of bed and into a chair. Lying still is an invitation to pneumonia and suffering is the path to enlightenment or something. It was morning, Rose had finished her shift and I had a new nurse who, honestly, I didn’t take to. While Rose had held my hand and dabbed my tears, this new nurse had had it up to here with my shit and was getting me out of that bed even if it killed me, which it quite possibly would.

After open heart surgery, your sternum is held together with internal cable ties. It is possible to break or dislodge them, so you’re forbidden to use your arms to take your own weight. Enter… The Teddy.

American hospitals give you a cute cuddly heart, but here in the NHS it’s a towel wrapped in a sheet held together with surgical tape, and yes, that’s blood you can see. I now have a Pavlovian response to the word ‘teddy’. It’s a sickening helpless fear, and I’ll probably never leave it behind. When you hear “Okay, hold onto your teddy” you know you’re about to be marched up Everest without an oxygen tank, and nothing you can do or say will get you out of it.

So there I was, clutching Teddy to my chest. The idea was I’d slowly roll onto my side and heave myself into a sitting position using the power of positive thinking. This was when I discovered my leg was all cut up. My left thigh and ankle were bandaged and stiff as a board, and I had no idea why. Wires, drains, catheters, a temporary pacemaker, something coming out of my neck, cannulas, compression socks… wriggling myself onto the edge of the bed was all about avoiding tugging on any of these things and not using my arms. Worse still, when you’re 6’3″, no piece of furniture on God’s Earth is made for your body. The chair was about ten inches too low for my body’s natural levers, so getting me on my feet and down into it was so much more terrible than it needed to be. I cannot tell you how exhausting it was. I’m getting stressed just thinking about it.

And then you’re expected to sit there. Sitting is tiring. Sitting is simply awful. I threw up. They offered me ice cream. I threw up again.

Sitting time was over, now I had to stand again. Without using my arms. From a chair so low it might as well have been a child’s.

“I can’t I can’t I can’t-”
“You can.”
“I can’t I can’t I can’t-”
“You can.”
“Don’t drop me.”
“We won’t drop you.”

Yeah, they dropped me.

I had a nurse either side of me, but I still managed to go straight back down into the chair with all the finesse of a grand piano jettisoned from a Chinook, and the whole of the Book of Revelation flashed before my eyes.

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One for the ‘Gram. I’d gained a stone in water overnight. Pictured: teddy and hateful ice cream.

I stayed on Critical Care for two days. In that time, I managed to eat a teaspoon of ice cream and as many vials of liquid morphine as they would give me. Unfortunately, morphine makes me sick, so that necessitated anti-emetic drugs on top of everything. I was now in pain, yes, but it was controllable, and I was still getting intravenous dopamine, which probably helped keep me doped up enough not to care as much as I might.

Mr Nashef, my surgeon, came to visit me. I was so pleased to see his smiling face, mainly because cheerful surgeons don’t give bad news. My aortic root had been replaced with a synthetic tube as planned, and I’d been able to keep my own valve, which he’d tightened up because it was a tiny bit leaky. The only snag was that one of my coronary arteries had started to dissect and couldn’t be reattached, so I had an unscheduled bypass – known as a CABG – which explained my wounded leg. A vein had been taken from my thigh because when they tried to take one from my ankle it was no good. I’d chosen to have surgery at the right time, Mr Nashef told me. My tissue was like paper, and I wasn’t far off ‘problems’, meaning… you know.

I instantly forgot most of this conversation because I was on drugs. The merciful thing about this part is that you’ll forget most of it.

Anyway, that’s enough fond reminiscence for one day. To be continued.

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What’s it like to have open heart surgery?

I haven’t blogged much lately, have I? For a change, I have a solid excuse…

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I had open heart surgery. Planned, elective, but still an enormous ordeal. I’m almost eight weeks post-surgery right now, and I’ve only just summoned the energy to write anything about it beyond the odd tweet.

Before I decided to get on the waiting list, I naturally went looking for people’s experiences. And though I found a few exceptionally lovely bloggers who held my hand and kept me going, there wasn’t that much out there from a young woman’s perspective. And sure, it’s a heart, we’ve all got hearts, but when you’re facing something so massive, so life-changing, it helps to be able to see other people like yourself who’ve been through the same and come out fighting.

If you Google ‘cardiac patient’, you’ll see plenty of this guy:

Reducing-body-temperature-saves-brain-function-in-heart-attack-patientsI’m 32 and I don’t own a pair of overalls. So I wanted to write a detailed guide from my perspective. I can’t promise it will be interesting to anyone with a healthy heart, but the one thing I wanted most of all before my surgery was someone to talk me through the nitty gritty so I’d go in feeling as informed as possible. Not everyone feels that way. One lady I met in the pre-admission clinic didn’t even want to know the name of her procedure, and that’s absolutely fine. Give this a miss if that sounds like you.

Obviously, this is my experience, not yours. I was told about countless things I needed to prepare myself for, and then they never happened. So just keep that in mind.

Regulars will know I have a connective tissue disorder called Marfan Syndrome. Put simply, Marfans means the tissue holding your body together is too elastic to do the job effectively. For Marfs, the whole body is delicate, but the heart most of all. People with Marfans are at high risk for aneurysms, and since I was a teenager I’ve been monitored with an annual MRI as my aortic root (the big part at the top of the heart) has been slowly getting larger and weaker. By age 31, my aortic root measured 4.5cm, double the healthy average. This is the measurement where surgeons recommend Marfan patients think about elective aortic root replacement.

I knew it was coming, but it was still a sickening surprise when my cardiologist referred me to a surgeon for ‘a chat’. It was even more surprising when a nurse took blood for a transfusion, measured me for DVT stockings, and handed me a consent form.

By the time the nurse was finished with me, I was dizzy with anxiety. With the consent form in my hand, I was ushered in to see my surgeon. It was all going so fast. I’d always told myself the surgery was years off. I had no symptoms. I was pretty fit for someone with my condition. I had DMs and a leather jacket, I didn’t need any bloody DVT stockings! Except I did. I actually did.

Mister Nashef is literally the poster boy for the hospital, incidentally.

Mister Nashef is literally the poster boy for the hospital, incidentally.

My surgeon was Samer Nashef at Royal Papworth. I liked him straight away. I’ve never felt comfortable with doctors who are evasive or try to make things fluffy for fear of overwhelming you, and Mr Nashef was willing to take me through everything in a matter-of-fact way that kept me calm and well-informed. I needed the David Procedure, which you can read about here. My heart would be ‘switched off’ and the enlarged section of my aorta would be removed and replaced with a synthetic tube. It would take around five hours, with thirteen weeks of recovery. “It’s huge,” the surgeon said. But I had youth on my side, and the best team in the country.

The choice was presented to me. I could carry on living my life, put off the surgery for a few years, but there would come a point where the risk of waiting outweighed the risk of the procedure, and that point would likely come soon. Some patients are lucky, some are not.

I went home to think about it.

*

From that day on, everything was about surgery. I couldn’t focus on anything else. Every ache and pain had me fighting back panic. I’d look at my chest in the mirror and envision the gnarliest possible scar. Worse, I had no instincts. I went back and forth on all the options, spoke to fellow patients, friends, family, read all I could on the subject, but still it seemed unreal. If the surgeon had simply said “Now is the time” it would all be so much less agonising. If I felt ill, even. In the end, I phoned Mr Nashef’s secretary partly to put an end to the tension. The waiting list was sixteen weeks, but at least I was on it.

Here follows a musical interlude lasting nine. whole. months.

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At the six month mark I got the call to come into Papworth for a pre-admission clinic. There I was weighed, measured for bloody DVT stockings again, had blood taken, MRSA swabs, stood for a chest x-ray, and spoke to my surgical team including the anaesthetist about what to expect. This took up most of a day, so when it’s your turn be sure to take snacks and a book. You’ll be presented with tubes of antiseptic gel to squeeze into your nostrils twice daily, and an equally appetising bodywash for the night before your procedure. You’ll be given a lot of paperwork, too, so it helps to have a folder to keep it all safe. Some of these papers are guides for family, including vital rules about visiting. The importance of Sniffling Cousin Jimmy staying away before and after surgery cannot be overstated. For two weeks before surgery, you can’t take supplements, and that includes vitamin C. Anyone with a cold needs booting out of the nearest window.

There’s a hell of a lot to take in at this stage, and even more to plan for. Prepare thyself. You’ll need to put a lot of thought into the reality of your recovery and spend a fair bit of money to make it as easy on yourself as possible. Remember, you’ll be working with a broken sternum. For a long time, you’ll be so tired even drinking a cup of tea will send you to sleep. How will you feed yourself? How will you wash? What will happen when your enormous husky wants a cuddle?

I needed, at the very least…

A tall, high-backed chair that could be moved from room to room
A recliner chair
A foam wedge for sleeping sitting up
Loads of button-up shirts, pyjamas and dresses
A single bed downstairs near the bathroom
A post-surgery bra
Cushions to protect my sternum when walking or using seatbelts
A travel bag for hospital with everything I needed, but nothing I’d mind losing
And the small matter of someone to look after me 24-hours a day for untold weeks

It turned out I had plenty of time to arrange all these things, because my surgery date was cancelled twice. Can’t be helped, but ugh… it was harsh. The first time, I was all set to go into theatre when two emergencies were blue-lighted in and I had to peel out of my paper knickers and go home. What do I do with myself now, I wondered? Getting back in the car that night was overwhelmingly strange after almost a year of revving myself up for the biggest physical challenge of my life. So I ordered a pizza and let my brain just shut down.

Depression is a major consideration when you’re going into a battle like open heart surgery. When you can’t make plans, can’t enjoy the present, or control your own future, you can’t help but go a little mad. I went numb for two weeks. I couldn’t read books or weather trivial daily annoyances. I’d wake up shouting. When my next date came a couple of months later, I promptly contracted a wisdom tooth infection. The mouth is an infection superhighway to the heart, so my surgical team told me to sit tight and get well. It was bad luck, putting it mildly. I felt like The Girl Who Cried Surgery.

My third date was April Fool’s Day. Ha. Ha! I’d believe it when I woke up on a ventilator. So when I rocked up to Papworth with my bag and my paperwork, part of me was coolly convinced I was about to go home again.

To be continued. Because I’m knackered.

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“For children… perhaps.”

This post is brought to you by a cocktail of painkillers, hypnotics, and anxiolytics, administered by a lovely NHS anaesthetist whose name I can’t remember – only the sight of her leaning over me with a smile: “You’re going to forget my name.”

Just some routine Marfans stuff. But two days later, I’m still swimming through dreams. I like to think the lingering hypnotics are giving me the authentic Rossetti experience, minus the raccoon hiding in the wardrobe. And the genius.

Hanging onto the tail of this wooziness, I want to talk about childhood, but also about Jan Švankmajer’s gorgeously surreal 1988 film, Alice: “A film for children… perhaps.”

alice

Years ago, a counsellor complimented my socks. They were pale blue and white striped, up to the knee, and they were perfect, she said, “because you’re Alice in Wonderland.”

This probably wasn’t wise of her.

Like a lot of only children travelling with military parents, I was prone to dissociation. That’s the psychology term for intense daydreaming. And I mean intense – to the point that reality was almost entirely blocked out for the majority of every waking day. Mostly, it was paradise, but there were snags. Teachers found they had to full-on yell to catch my attention. I once kicked a cannonball-sized hole through a porter cabin wall without noticing.

Dissociation is a creative coping mechanism for when life is unstable or lonely. It’s very much like the intravenous sedative experience – a protective measure that picks you up and whisks you away. Eventually, children who habitually dissociate grow up to remember more about their dream worlds than the reality of the past. Some of them become writers. Ahem.

Onto Alice. As a chronically fantastical child, I was rankled by the Disney version of Alice In Wonderland. It was all so very pastel, so very clean. Nothing pristine, I knew, could ever be magical. Childhood is frightening, and nonsensical, and inappropriately hilarious – much like the original Wonderland tales.

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Children inhabit another planet. Lewis Carol knew that.

So on the other hand, you have Jan Švankmajer’s Alice. A film for children… but probably not for parents. The White Rabbit is taxidermy, leaking sawdust. Socks have teeth and the jam is full of drawing pins. Wonderland itself is a wreck of broken china and splinters, pickled specimens, potted tongue (prone to slithering), and other Victorian relics. Alice – the only living person in the film – has a way of turning into a decidedly spooky china doll and back again, tearing off the chrysalis of painted skin and making a run for it. Like the doll, her facial expression never changes. She never smiles or frowns. Those are things you do for others, and this Alice is content to be alone with her imagination.

Drowsy and doped as I am, it strikes me how strangely authentic Švankmajer’s vision of childhood imagination was.

In 1989, the year after Švankmajer released Alice, I was three. The Navy posted Dad to Scotland, and we ended up living on a dismal estate near a submarine base. Someone had spray-painted a peace symbol on the garden fence for our arrival. I wasn’t ready for school, and we never stayed anywhere long enough to make friends. I think I tried, on a few sparse occasions, but it was so much effort for so little return when inside was infinitely better than outside.

Perhaps it was the jerky stop-motion animation, or the twisted quality – lurking on the indistinct border between dreams and nightmares – but watching Švankmajer’s Alice for the first time a few weeks ago, I began to spontaneously remember scraps of day-to-day surrealism from that time in Scotland.

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Someone – I forget who – told me bubbles were living things, and that when you popped them, they died.

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There was a nearby play park where a boy knocked himself out trying to make the swings turn full circle. I saw his body in a red tracksuit, face down on the ground. ‘Cracked his head open’, I thought for years, was literal. Bash the hinge hard enough and the skull pops open like a spring-loaded box.

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That play park was an island of concrete amongst hillocks of unkempt greenery. One day, some other children took my doll, so I wandered off to where you could see the Forth Rail Bridge, muddy-bloody coloured, in the distance. Over a hill, I found the skull of a ram, picked clean. The coiled horns looked like the fossils in my dinosaur books. I can’t recall the doll.

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From the spare bedroom, if the light was good (by Scottish standards), you could see dark cylinders moving slowly through the cold seawater. I’ve never grown out of the fear of submarines, but the house held hidden dangers of its own. I was playing in the garden. Making a pile of gravel. The house had been arsoned before we moved in, so the gutters full of gravel also contained all the glass from the shattered windows. Not that I noticed. I was grabbing handfuls, piling it up, until I registered I was bleeding from dozens of tiny cuts. I took myself up to my parents’ bedroom and triumphantly held out my hands to them. It didn’t hurt.

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A fisherman on a jetty shouted at me for dropping a stone in the sea and disturbing the fish.

“I want to cut him in half,” I said, and I can still clearly see the satisfying image in my mind, of a man sliced in two like a pink salmon.

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Sugar, spice, and murder fantasies. There’s a whimsical grotesquery to childhood I think we’re programmed to erase. Perhaps it’s only free to come out when we’ve been dosed up by friendly anaesthetists without names.

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#HospitalGlam

Hello again. Remember me?

I haven’t blogged properly for a while, partly because I’m wrapped up in The Mighty Healer, and partly because the boyfriend and I have both been varying shades of ill, ill, ill, for several months. Nothing sucks up time like hospital visits and bedrest.

Here in the UK, the general election is about to topple onto our heads. That means blanket coverage of men in bad suits shouting at each other about the NHS, not because they care that it’s circling the drain, but because winning debates is more fun than losing them. After a while, all the chatter ceases to sound like real life and enters Pink Floyd video territory, particularly when Cameron gets a bit sweaty and you sit there hoping his rubber mask will slither off to reveal the circuitry within.

So I want to share something fun.

Karolyn Gehrig is a queer disabled artist from Los Angeles. Like me, she has a connective tissue disorder and spends a lot of time traipsing in and out of hospital. To pick herself up, Karolyn started taking selfies in a medical setting, tagging them #HospitalGlam.

They’re gorgeous.

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“I started posting #hospitalglam because it was frustrating to me that every time I got sicker I’d disappear from my commitments and then feel shy about explaining where I’d been when I knew there was absolutely nothing wrong with pursuing treatment for my disabling chronic illnesses.”

The idea struck a chord. With the success of the #HospitalGlam hashtag on Twitter, Karolyn set up a Tumblr for fellow patients to submit their own selfies, giving them something to do in those doldrum waiting rooms, and winning back a bit of self-esteem at the same time. Any gender, any illness, artistic or silly, made-up or fed up.

One nice effect of #HospitalGlam is finding like-minded souls.

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Here’s Heather of Mortuary Report. She’s a funeral director and all-round fascinating human. You should read her blog on reclaiming sexiness and personal empowerment whilst chronically ill, but here’s a chunk that stuck out for me:

“There are few places where one feels less empowered and beautiful than in a medical setting, and it’s not just because of that awful beige color the walls in doctor’s offices are always painted. Most humans are fortunate to only associate doctors with tedious time spent in waiting rooms and an annual check up. For those of us who are chronically ill, however, the experience of spending as much time as we do in medical settings becomes a spin-cycle of misery, depression, and – often – questioning our own sanity. […] Being disabled in an able-bodied society quickly became a source of shame and frustration.”

Being chronically ill isn’t as glamorous as the brochure made it out to be. The invisibility Karolyn talked about – self-inflicted or otherwise – has an accumulative effect on the psyche. That’s on top of the media’s relentless commodification of the body, where presentability is bound up in youth, health, and unattainable conventional beauty. That’s why it’s so important to see images of people with chronic illnesses and disabilities where a) they’re in control, and b) pity porn, or the godawful ‘inspiration’, doesn’t come into it.

Model Melanie Gaydos will be familiar from Rammstein and Die Antwoord videos. Here, she talks about growing up with Ectodermal Dysplasia and bringing her individuality to the modelling world.

“When I go on a photo shoot, if there’s other industry models there, they normally don’t really know what to make of me. And they’re usually like, ‘What the fuck is this?’ […] I love modelling. A time for me to be completely open. I guess it’s kind of like a therapeutic process for me.”

#HospitalGlam is a weapon for those of us with chronic illnesses. With it, we reassert our individuality, regain our playfulness, and have a ‘frivolous’ place to escape to when all else might seem out of control. It is therapeutic. And it’s fun. And all the while people ask what the fuck, we know it’s needed.

Our Lady of the Cathode Rays. Thine be the free hospital mochas.

See you on the hashtag.

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Rare Disease Day, the DWP, & ghosts of Girl Guides past

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Today is Rare Disease Day, an international day of awareness-raising activities under the slogan “Join Together for Better Care”. Yesterday, the House of Commons held a debate on the effects of welfare cuts on the sick and disabled. As you can see, it was teeming with people who care.

While the debate dribbled on, I read a discussion on one of the Marfan Syndrome social forums. A man was expecting the birth of his son, and wanted to know how he could bring the boy up to see Marfans in a positive – or at least neutral – light. A Marf parent has a 50% chance of passing the mutated gene onto their child. If his son isn’t born with the syndrome, he will still experience the fallout of strangers’ reactions to his father. Which, let me tell you from experience, will not be the stuff gleaming Liberal utopian dreams are made on.

This had me thinking. If I could go back in time to offer my child-self advice on living with disability, what would I say?

Anyone with a rare disease or disorder will be well-acquainted with the word ‘awkward’. It usually arrives wearing a friendly mask: mild teasing when you explain you can’t help your colleague move their heavy desk, or the tutting of your fellow Girl Guides when you faint on the way to the world’s oldest toy shop and have to lie panting on the Oxford Circus floor tiles, watching commuters’ feet hurrying by.

“Hamleys is going to close soon,” someone whined in my ear. “Just stand up.”

Disability is just as much a social challenge as it is physical. You learn you are not simply inconveniencing people – you are the inconvenience.

I grew up before the Internet was permanently glued to the palms of our hands. When I eventually got a modem and an hour each weekend to play on it, it didn’t occur to me to look for advocacy groups, support services, or other people like me. My fear was that I would type ‘Marfans’ into the search field and find nothing but derision and cluelessness, as I had in real life. “If it was really that bad,” my PE teacher said when I tried to explain that trampoline + weak connective tissue = disaster, “I would have heard of it”. Then she made me get on the damn trampoline.

13-year-old Verity internalised the message that disability equals inferiority.

It took me years to gather the courage to seek out other Marfs online. When I did, I realised how valuable support groups are for those of us with rare disorders, and not merely in a fluffy, join-hands-and-sway-to-the-folk-guitar way.

“If you ever get a fuzzy black curtain descending over one side of your vision, drop what you’re doing and run to A&E – your retinas are detaching “ Good to know, yes? Or: “Get a medical alert bracelet. If you’re on the floor with a collapsed lung, you won’t be able to talk.”

Nobody had told me. I was so used to explaining the little I knew about my condition to my own doctors, to being the star attraction for medical students, to fielding gigantic questions from people in power such as the DWP employee who asked, “So, can you just, like, explain to me what this thing is?“. I had been trained to believe that even if quietly soldiering on put me in danger, at least it meant I wasn’t getting in anyone’s way.

The social model of disability says that disability is caused by the way society is organised, rather than by a person’s impairment or difference. When barriers are removed, disabled people can be independent and equal in society, with choice and control over their own lives. – Scope.

Napoleon is a hardworking taxpayer and has no time for your nonsense.

Napoleon is a hardworking taxpayer and has no time for your nonsense.

Able-bodied or otherwise, we are brought up knowing the archetypes: The Inspiring Cripple, The Grateful Invalid. Strivers and Skivers. The poisonous leftovers of the Victorian Deserving/Undeserving dichotomy. I have said before that to discuss these issues one has to cloak oneself in charm and detachment, because if there’s anything those in favour of welfare cuts can’t stand, it’s the bolshie sick. Perhaps it reminds them of the uncomfortable old adage that a society is only as civilised as its treatment of the vulnerable.

In the UK, it’s a lousy time to be disabled. Vital services are being cut back, benefits are taken away, and women in comas are told they are fit for work. Quota systems deliberately work against us, disturbing documents are leaked, fees stand in the way of employment tribunals, and only a handful of MPs grudgingly turn up to welfare debates. You feel ashamed to seek help; you come to expect disdain. The social model of disability thrives on the active disempowerment of those already at a disadvantage. Watch it at work.

So, on Rare Disease Day, when I like to take stock of my network of support, and the possibilities of the future, I find myself looking at my country and not feeling terribly wanted. Nevertheless, I still have sufficient self-esteem to know that if I could go back and tell my child-self anything, it would be that disability does not equal inferiority. It doesn’t, despite the strenuous efforts of those more fortunate than you: people who may be elected officials, or wearing ATOS lanyards, or just little girls impatient to get to the toy shop before it closes.

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Marfan Syndrome and The Internship: Not a big-man’s disease

There’s been a ripple through the Marfan community this week. Some American comedians I’ve vaguely heard of (my world is muffled by nineteenth century poet’s letters to their dentists) have taken it upon themselves to mention Marfan syndrome in their latest film, The Internship.

Instead of using their immense media power to spread lifesaving information to the suspected thousands of people who don’t know they have Marfans, Vince Vaughn and Will Ferrell decided it would be easier to throw this out there, apropos of nothing:

Will Ferrell: not actually a doctor.

Will Ferrell: not actually qualified to take your ECG.

“C’mon, Marfan syndrome. You know, Marfan. Big man’s-disease. Giant killer.”

It’s crass, unfunny, and worst of all, untrue.

The National Marfan Foundation have released a statement urging the film’s producers to use this opportunity to spread the word about this often fatal disorder, and also pointing out the obvious: it was a stupid thing to say. Reactions from individual Marfs have ranged from “I’ve heard worse” to this, which reads like a punch in the gut:

A Virginia man, who lost his two-year-old son to Marfan syndrome in 2011, wrote that he was “extremely upset with the lack of taste, concern and respect concerning this disorder.”

The blogger Maya, also known as MarfMom, is, as always, joyful and positive, and has written about the film. She rightly thinks we ought to use this opportunity to educate, because the danger of the line is that it spreads misinformation. Half of people with Marfans don’t know they’ve got it. They go without medication, take part in dangerous activities, and may not find out until they’re in the back of an ambulance. Accurate information in the public eye is vital.

‘Big man’s disease’? Marfan syndrome affects men and women equally. ‘Giant killer’? Not all Marfs are exceptionally tall, and even so, they tend to be thin or unmuscular. In fact, if I had to pick a mythical creature to represent Marfans, it would be the willowy elves from Lord of The Rings. They’re long, they’re languid, and they’re sick to the back teeth of orcs crawling out of the woodwork. And what is this ableist obsession with fantasy monikers anyway? Giants, dwarves, monsters. Anything but human.

Thranduil thinks your attitude stinks.

Elfking Thranduil thinks your attitude stinks.

Handy rule of thumb: If you don’t have a disability, don’t make jokes about it.

I’m going to have that printed on little glitzy flashcards to make it all the more memorable, because although it’s basic human decency, some people still struggle with it.

No.

No.

All the Marfs I know make light of our health among ourselves, our friends, and families. Laughter is useful, particularly when – and this actually happened to me – a saleswoman earnestly informs you your incurable illness will clear up if you just drink enough aloe vera juice.

But if you don’t inhabit that sphere, you can’t assume what people’s thresholds are. You can’t assume what people are just about managing to cope with, what their history is, who they’ve lost.

Another thing Vaughn and Ferrell might be unaware of is that people with a long-term health conditions are strongly encouraged to keep their feelings to themselves.  Classic derailing: “It’s only a joke, don’t take it so personally, no-one will take you seriously when you’re angry”. If you’re going to speak out about ableism you must cloak yourself in charm and detachment, as if this wasn’t your everyday life being discussed. Heaven forbid you become one of those frightening party-pooper militants who lurk in drains with Pennywise the clown.

Yes, it’s just a weak joke in a film unlikely to go down in cinematic history. But there will be kids who’ll suffer in school because of this. There will be adults who’ve lost a child or a parent or a friend who will have to smile politely at jokes like this from colleagues, strangers, and even friends who, because they’ve seen it in a mainstream film, think it’s harmless behaviour.

It isn’t.

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When I am queen, I will burn down the castle.

They say that one passion leads to another. Long before I discovered Dante Gabriel Rossetti, I lived in Gormenghast.

Gormenghast

Between school bullies, kidney infections, and the oncoming Iraq war (which, I’d somehow convinced myself, was my fault), the year 2000 was a dismal time to be fourteen. But when the BBC released a four-part adaptation of Gormenghast in time for the Millennium, something shifted. From my hospital bed, I imagined the mauve peaks and crumbling spires of the castle on the horizon. I stopped doing my homework. Mervyn Peake’s Machiavellian fantasy was a safe place to escape to.

I never grew out of it. At my first University graduation, seeing the professors traipsing down the aisles in their gowns and mortar boards, I whispered excitedly to the boy next to me: “This is just like Gormenghast!” He had no idea what I was on about.

Years pass. We grow up, our tastes evolve. I fell out with high fantasy, fell into the nineteenth century. But, over a decade after my first encounter with Gormenghast,  thumbing through my paperback trilogy, something sounded familiar…

Fuchsia-and Steerpike

“A girl of about fifteen with long, rather wild black hair. She was gauche in movement and, in a sense, ugly of face, but with how small a twist might she not suddenly have become beautiful. Her sullen mouth was full and rich – her eyes smouldered. A yellow scarf hung loosely around her neck. Her shapeless dress was a flaming red. For all the straightness of her back she walked with a slouch.”

Oh, hello, Jane Morris.

Jane Morris

For a fourteen-year-old reader, Lady Fuchsia Groan is an easy character to relate and aspire to. Living in isolation where ‘the halls, towers, the rooms of Gormenghast were of another planet’, her response to most things is to run away to her dark attic of storybooks and paintings. She is a petulant child playing Ophelia and Juliet, dying to fall headlong into a world of chivalric romance and adventure.

Fuchsia – in Peake’s own illustrations and his text – has unmistakable similarities to Rossetti’s Jane. Like La Pia, Fuchsia glowers with the lethargic energy of someone who wants to be somewhere else but isn’t sure where. Her unkempt hair and pronounced features give her the ‘unpretty’ Pre-Raphaelite beauty the Victorians were so bothered by. Jane was considered unfortunately unattractive by many. Fuchsia, too.

lapiafuchsiared

There are Pre-Raphaelite echoes in every corner of Gormenghast. Maybe it’s the meeting of the Gothic and the Chivalric, the tragic and the absurd, or Peake’s own network of literary sources including Lewis Carol and The Brothers Grimm. Peake’s childhood in China and later studies at the Royal Academy gave his work a sense of ancientness and the exotic that reminds me of Holman Hunt’s picking and choosing of historical and cultural details. You can see it in The Hall of The Bright Carvings and the almost Tibetan descriptions of the endless corridors and slanting roofs of the castle.

mervynpeakeuptree

Mervyn, acting casual

As a war artist in the 1940s, Peake saw terrible scenes of human cruelty in the rubble of the bombsites and the concentration camps. Perhaps it was only natural to head for the dusty safety of the past.

The BBC adaptation – which I realise is not to every Peake-purist’s taste – is funhouse mirror Pre-Raphaelitism. Nature is vast and unfathomable. Steerpike wheedles his way into Fuchsia’s favour by claiming to be “like the knights of old, your ladyship” only to find he can’t possibly live up to Fuchsia’s fantasies. In John Constable’s later stage show, Fuchsia is even given red hair. (Actual audience comment: “This is horrible. They said it was fantasy. It’s nothing like Harry Potter at all.”)

Fuchsia and Jane

The BBC costumes are luxuriant. Fuchsia starts off as a teenager in a loose red velvet dress embroidered with stars. As she gets older and sadder, her outfits become heavier, more stiffly structured, until she is dragged down into the foaming floodwaters like Ophelia, leaving flowers in her wake.


The costume department referred to some of the same sources the Pre-Raphaelites did – Velázquez and Botticelli – resulting in voluminous layers of fabric and detail (even hazelnuts as buttons!) like a mad dressing-up session in a museum vault. Actress Neve McIntosh would gain two inches in height after taking off Fuchsia’s weighty gowns. Rossetti, with his reams of fabric cluttering up the house, would have loved it.

Lady Gertrude Groan
Excuse the poor quality photograph, but wouldn’t Rossetti have made a great job of this still from the film as a painting? Minus the prosthetic chin.

I wonder if I would have reacted so strongly to the Pre-Raphaelites had I not experienced Gormenghast so young. One good thing leads on to another. What’s next?


Buy The Gormenghast Trilogythe BBC miniseries on DVD, or the fantastic soundtrack by Sir John Tavener who, coincidentally, has Marfans.

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Do you know a tall, skinny person?

Does this tall, skinny person have long fingers and toes?

Do they need glasses? Have stretchmarks? Feel the cold? Tend to faint?

Can they do this?

marfhands1

Or, worse yet, this?

marfhands2

If so, there’s a chance they could have Marfan Syndrome. Show them this website. It only takes a moment, it won’t offend them, and it could save their life.

#MarfanAwarenessMonth

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Slap a catalogue number on me, Henry.

Is there anything better than perusing glossy photographs of artificial limbs and trepanned skulls over one’s breakfast? I think you’ll find there isn’t.

This week, I won a copy of the Wellcome Collection’s brand new ‘Guide For The Incurably Curious’.

Wellcome is my favourite museum. If you have the slightest interest in wunderkammer, it’s a playground. The perfect balance of the medical, the historical, the scientific and the artistic, lovingly founded by Victorian philanthropist Henry Wellcome. Look upon his facial hair and tremble.

I’ve spent birthdays there, handling live leeches and drinking gin. I’ve seen Mexican miracle paintings there, verdigris mediaeval skeletons, and glass acorns for warding off lightning strikes. Once, an attendant saw how excited my friends and I were and fetched us goodie bags complete with wearable cardboard moustaches.

Science museums can feel unfriendly to artsy types, but at the Wellcome, the two disciplines interact. Upstairs, in the cool white Medicine Now room, slides of organs are displayed alongside barmy art (there’s a giant purple jellybaby as a metaphor for human cloning). Downstairs, in the darker, more anthropological Medicine Man room, you’ll find a wall of antique forceps and some beautifully detailed glass eyes which could easily be items of jewellery or sculpture. Plus, there’s a Bosch painting, and everyone loves a good Bosch.

But I think what I love the most about the Wellcome Collection is that, in a manner of speaking, I’m in it.

I have Marfan syndrome. The National Marfan Foundation explains:

Marfan syndrome is a disorder of the connective tissue.

Connective tissue holds all parts of the body together and helps control how the body grows.  Because connective tissue is found throughout the body, Marfan syndrome features can occur in many different parts of the body.

Marfan syndrome features are most often found in the heart, blood vessels, bones, joints, and eyes. Sometimes the lungs and skin are also affected.  Marfan syndrome does not affect intelligence.

Specifically, Marfans is caused by a kink in the fifteenth chromosome. So imagine the surreal excitement I felt when I turned a corner in the Wellcome Collection and came across this:

There it is. The Human Genome Project, chapter 15, subheading ‘Verity’s Wonky Genes’. I took it from the shelf with both hands. Buried amongst the reams and reams of baffling code inside was the string of glyphs that spelled out Marfan Syndrome.

Only one in five-thousand people have Marfans. The syndrome will generally make you around six feet tall and willowy in build, with exceptionally long, spidery fingers and toes. You may have a curvature of the spine or an uneven ribcage, and you can probably bend your thumbs into strange angles. Abraham Lincoln probably had it, as did Jonathan Larson, Joey Ramone, and, I strongly suspect, Lux Interior of The Cramps.

Marfans can affect you in all sorts of strange, annoying, sometimes life-threatening ways. Individual Marfs differ. As for me, I’m well looked-after by good doctors. I pace myself, I watch my diet and try not to be a stubborn ass when it comes to clinging to the barrier at Morrissey concerts or vigorous charity shopping the weekend after minor heart surgery. (Although holding hands with Morrissey and acquiring an antique nursing chair for £10 were worth the resulting drama).

One of the things about having an unusual health problem is that you can end up feeling alienated. That’s why I love the Wellcome Collection. Things that could be clinical or morbid, like Jennifer Sutton viewing her old heart after her successful transplant, are greeted with curiosity and joy.

It’s an ambition of mine to get Marfans into the Wellcome more prominently. Short of standing in the entrance hall with a sign on me, I don’t know how to raise awareness. I’m not quite ready to donate my hands. But it’s a syndrome that really lends itself to art. Maybe I can use my nonexistent artistic ability to chop up my MRIs in a nice lightbox, or draw an Edward Gorey-esque bunch of spidery fingers. Or, better still, persuade someone  who actually knows what they’re doing to put Marfans in front of the lens, like Alexa Wright’s ‘After Image’ series.

Where’s an artist when you need one?

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