Features

Pain

Pain is hard to quantify, isn't it?  Paramedics, nurses and doctors all ask 'What's your pain on a scale of one-to-ten, with ten being the worst pain you can imagine?'

I've never given a ten, because I've never had a limb amputated or suffered with countless other injuries I don't want  to imagine. 

Thresholds

That's how my pain quantification starts in that setting.  If paramedics are asking, my pain is usually at a nine to nine-point-five, which is more than enough.

Even this, though, doesn't begin to cover the level, depth or breadth of pain bodies with Ehlers-Danlos can be forced to sustain.  Or the ways it impacts upon your very being...

One thing a life with severe Ehlers-Danlos Syndrome (EDS) has taught me is how ironic a genetic condition it really is.  How it's packed full of give, give, give.

Just when you think your improperly-built body can't turn one more trick, you realise another problem, complication or issue exists.  For example, physically, we are some of the most fragile bodies you will ever - or never - meet. 

This delicacy, ironically, also makes us some of the toughest characters upon this planet. 

Pain.

It softens and hardens you all at the same time.

Because we know what suffering and fear truly feel like, our constant pain can make us have more empathy for the suffering of others.  Especially when so much is so needless.

And many of us with EDS do experience constant pain.  Hurt that only stops in our sleep, and even then often wakes us from our slumbers or finds its way into our dreams.  With this particular connective tissue disorder, there is no real relief.

Another cruelty is that, while we are trapped in bodies that cause constant physical torture and injury, this medical condition is resistant to pain medication and local anaesthetics.  So our pain cannot be easily, if at all, controlled and simple things like dental work, mole removal or a small wound stitched are utterly agonising. 

In my medical records, my pain is officially recorded as 'Intractable Pain'.  Intractable means hard to govern, control or manage.  That translates into me using Fentanyl, Oxycodone, Codeine and Paracetamol/Acetaminophen (US) at the same time, as a matter of course, and still experiencing pain levels that dominate, dictate and control my every choice and action in life - down to whether or not I can breathe normally or sit up at all.

Yes, my body must be treated like the easily-breakable creation it is, but psychologically, the amount of pain with which I live, the thresholds to which I must push to and operate at have left me with a mental strength that borders on coldness when necessary.

Some might argue that the chill is never needed when they have been the ones to receive it, however, I obviously disagree.  To be in receipt of it, you definitely deserve my icy...And you probably ate your way through my afore mentioned empathic side first.

The fact is, when your body is non-stop hurt and it is only ever getting worse, sometimes harsh decisions and choices must be made.  This includes the how and who of dealing with other humans.  Priorities suddenly become very basic when you're at the mercy of something you cannot change or beat.

Especially when that something has the power to keep you down after completely knocking you off your feet.

Worst case scenario? 

Tonsillar herniation, the

bottom bits of your brain falling through the hole in your skull where your

neck bones go. 

Comorbidities

Before going much further, the ways in which EDS causes pain need to be understood a little bit better.  It will help deepen your understanding and, hopefully, your  empathy for the Bendies like me and all the other humans who are suffering from hideously painful medical situations that you can't see and fortunately do not feel. 

I explain the underlying problem in EDS - our bodies do not make different collagens or the 'glue' supporting them properly - in 'Velvet'.  Around 30% of our bodies are made up of these proteins. 

They are in our bones, skin, muscles, tendons, ligaments, blood vessel walls, nervous systems and internal organs.

Our bodies did not and cannot make themselves correctly or strongly or as they should be.  Everybody's collagen quality deteriorates as we age.  It's a kind of degeneration we can ill afford, but still must face.

My type of EDS is the Hypermobile type.  The one we haven't yet found the gene for.  I'm super bendy.  Pathologically bendy.  There is not a joint in my body that stays in place correctly, which means constant daily subluxations and dislocations.  I don't even have to move for my spinal bones to be popping in and out of place.  My shoulders drop out of their sockets when I'm sitting still.  The bone in my left forearm comes out if it's kept still at a right angle and I have to rotate the bone to force it back in at the elbow - usually with a very audible, dry cracking.

My ankles are as mobile and reliable as a newborn infant's and my kneecaps come out every few steps.  My jaw comes out every time I yawn, brush my teeth, kiss, give head or chew.  My femurs slide out of my hips at will.   None of my thumb joints would know how to stay in place if they watched a lecture on it, my wrists and remarkably weak, my fingers pop out down to their very tips.

My skull slips about on the top of my neck and when that isn't sitting properly, it stops everything.  No sitting, no standing, no moving - unless it's for the basics, like drinking or peeing.  Everything, including washing and eating, has to wait.

For however long it takes. 

All of this hurts.  All the time. 

All the time.

It started with my knees when I was eleven or twelve, with the onset of puberty.  It's been a downhill ride ever since, with my body's ability to hold itself together decreasing more and more with each year that has drifted by. 

With each year that I have lost or that was taken from me.

As severe as my EDS is, my current state is not a natural progression.  I can't yet share all the details here for various reasons, but that should change within a few months.

For now, just the subluxation and dislocation situation should paint a pretty vivid part of the full picture of my pain. Because, EDS truly is just one piece in this incredibly fucked-up jigsaw I sit within.  Where dodgy genes go, comorbidities usually follow, and Ehlers-Danlos has more than it's share of associated conditions. I'll try to make my personal rundown brief.  Are you sitting comfortably?  Then I shall begin.

Dural Ectasia - sort of sounds like a band from the Eighties, but it's actually related to Spina Bifida, and is a neural tube defect.  This means the membrane around the bottom of my spinal cord in my central nervous system did not form properly in the womb.  It's baggy and presses on the nerves around it, filling with too much Cerebrospinal Fluid (CSF) and dropping the pressure of the Brain Juice in my head.  External symptoms of DE are 'coffee stain' marks on my lower back that look like birthmarks and a small patch of what looks a bit like pubic hair on the top of one of my buttocks.  I remove my little tell-tail...

Tarlov's Cysts   - they're actually more aneurysms than cysts and it's likely that they also formed while I was in the womb.  Again it's an issue with the membrane wrapped around my nerves, but this time, its at the root of the nerves as they come out of my spinal cord.  They also fill with and drop the pressure of Brain Juice.  They squash surrounding nerves. They're a massive bulk for my tiny body, collectively measuring around 15cmx11cm.  They can erode the bones in the bottom of your spine (the sacral region). 

They can interfere with digestive function, cause numbness in the legs, cause very strong spinal pain and sometimes light my uterus up as if I am giving birth again, with just gas and air.

CSF Leaks   - these don't often happen to me, thankfully.  When they do, I usually get CSF or Brain Juice leaking out of my nose.  You can tell if it's Cerebrospinal Fluid because the proteins dry on tissue in a target pattern.  There is no certain answer as to why these happen in my case, so suffice it to say it's probably something to do with weak membranes having a little tear somewhere near my brain.

Brain Juice - if there's too much Cerebrospinal Fluid (CSF)at the base of your spine and central nervous system, it generally means there's not enough at the top. CSF runs around and through our spinal cords and brains, acting not only as a kind of buffer, but also as a cleaner, helping to remove junk from our system. When Brain Juice pressure drops in your skull, so does your brain. Worst case scenario?  Tonsillar herniation, where the bottom bits of your brain fall through the hole in your skull where your neck bones go. 

Best case?  You have to stay totally flat and still, just grit your teeth and bare the pain, try to sleep through it, until the problem resolves and your brain is floating more freely again.

Starting to see what I mean about being mentally tough?

Degenerative Disc Disease

Does it hurt?

Like Hell sometimes, stopping me being able to even move my

left leg, but it is what it is

and it won't kill me.

Encore

Wait!  There's more! Just above all that neural tube, brain juice-sucking junk I have degenerative Disc Disease, with numerous herniated or absent discs. 

I think the discs in my neck have gone too, but due to the ongoing COVID situation, I haven't been able to get a spinal MRI scan to confirm this. Plenty of people know the pain of DDD. 

Mine was found in my early-thirties.  Started in my twenties.  To be honest, it's the least of my concerns and ailments, most days.  Does it hurt?  Like Hell sometimes, stopping me being able to even move my left leg, but it is what it is and it won't kill me.

Somewhere in the mix of those herniated discs is a permanent spinal fracture. With no idea how I fractured my spine, because I spent nearly ten years stuck in bed or stuck on a sofa, for the first year after injuring myself, I assumed the pain I was feeling was just another disc or two that had 'gone'.  Hindsight told me that I was wrong, and the intense agony shooting up through my core each time I bent this way or sneezed or coughed or lay on my left side was, in fact, this fracture. 

It's pretty much settled now, I'd say, insofar as a fracture ever 'settles'. 

My brain didn't recognise the bone as broken and thus never tried to heal it. Today, I guess my brain just doesn't know if a pain is from that or the other problems in my back.

Moving up to the top of my spine, I have two types of curvature between my shoulder blades, and I try hard to stay as straight as I can.   Above that is my neck, which is stupidly unstable.  I should probably be wearing a neck collar for at least a significant part of my time and when doing certain activities (like travelling by road).  I don't because I'm yet to find one that doesn't increase The Ow.

Were I to be properly examined and investigated, there's a high likelihood I would be diagnosed with CranioCervical Instability (CCI), where the neck and head are not probably attached.  This has been discussed with experts in several chats.  I'm not pushing for 100% confirmation because I can't have the treatment. 

The treatment involves inserting titanium rods into your body and screwing your skull on to your spine (fusing it).  Nobody in the UK will perform the surgery on an EDS body because of it's failure rate due to our poor healing.

This means it also involves needing at least £250,000 to pay for the procedure to be carried out in Spain.  More if we go to the US.  Double that if we need to return to Spain for another operation as is often the case.

My spine has already proven it won't heal its own bones.  Drilling and screwing into them is simply not an option I can consider. 

On top of all this structural stuff are two neurological conditions called Dysautonomia and POTS  (Postural Orthostatic Tachycardia Syndrome). 

POTS is a kind of Dysautonomia, but is not in and of itself Dysautonomia. 

Clear as mud? 

Good.

Remember these two names, because they will become big names in terms of Long COVID.

Dysautonomia is a catch-all for conditions where the autonomic nervous system (ANS) is not functioning properly.  Your ANS controls all of your unconscious bodily operations, including digestion, heart beat and heart rate, blood pressure, pupil dilation, salivation, swallowing and a lot more besides. It's often called the 'Flight Or Fight System' because it also controls your stress responses. 

In a nutshell, the POTS means when I stand or sit up, my ANS doesn't respond appropriately, my blood pressure drops and I don't get enough blood, or therefore oxygen, to my heart or brain.  To compensate, my brain raises my heart rate to sometimes dangerous levels and if that doesn't work, it will drop my body, making me pass out so that blood and that precious oxygen it carries does reach my brain.

For me, Dysautonomia also means that when I am able to eat a decent meal (I rely on a prescribed liquid feed to stop me starving to death), having a full belly often makes me crash too, because my ANS puts too much blood into digesting the food, once again leaving my brain needing some help.  I call this Food Drunk, because my speech becomes slurred, I become giggly and very dizzy.  My heart races and sleep happens soon afterwards and is not really a choice. 

The pièce de résistance and final part of this painful picture is something called Osteitis Pubis  (OP).  This is the part I can't yet publish in it's entirety.  What I can disclose now is that when I was 31 years-old, an ankle dislocation led me to fall and injure my pelvis. After a week stuck in bed in agony, I implemented my Seven-Day Rule and went to the local A&E department.  A catalogue of medical errors and neglect followed, and a few bedbound months later, I was finally diagnosed with OP. 

The '-itis' bit in the name translates to swelling or swollen.  Arthritis, for example means 'Arth-', joints, '-itis', swelling - swelling or inflammation of the joints. We love a good medical label, don't we?

My injury means the pubis symphysis, the big joint that holds the front of your pelvis together, keeps swelling.  There is not a lot of space in this body part and when the inflammation starts or gets bad, it stops life.

The pain spreads down both legs, which I must tie together at the knees because my legs must not move apart even a millimetre. The flesh in those limbs must be remain as relaxed as possible and the pain pulses down to my very toes, burning my soles.   

My second child's life stolen.

My motherhood with my first so limited and ragged. 

My liberty to choose my

path in life gone, as I turn

around and realise what

was left behind me... 

It creeps up through my abdomen, which also must stay soft and untensed. It hurts to breathe at this point, each breath as shallow as I can possibly make it, as few as possible, as slowly as possible. 

I cannot move.  I cannot move my head, my arms, my legs. I have been minutes away from death. I have pissed in pots next to my bed, put pillows to still me either side of my head, staring at the ceiling day after day, week after week, month after month, year.  After.  Year.

Until I turned around and a decade had gone. 

My second child's life stolen.  My motherhood with my first so limited and ragged.  My body now dependent on addictive opiates and narcotics that do not even control or some days touch my pain.  My freedoms and liberty to choose my own path in life gone.  My career in tatters around my tip-toeing feet as I now face fifty, turn around and realise what was left behind me... 

 Prospects

The current condition of my body is  not a natural progression of EDS.  I will repeat this.  Medical neglect and mistakes put me here.  But, my pain was controlling my life choices for the future by the age of 12 and the time came to start thinking of my higher education and career. 

It would be another 12 years and a  huge fight to secure a diagnosis. 

Going into television production had been a real possibility, with one university offering me an unconditional place on one of their degree courses.  It was what I longed to do, hopefully leading me to a career in editing videos for the music industry. I had been filming with HTV as one of their presenters for several years, had also been a camera operator on corporate programmes and had been paid for voiceover work. 

Ehlers-Danlos, though, already had a tight grip on my physical well-being and a career in production was out of the question before I had even finished my two-year Media diploma.

Remembering my love for writing, I ended up attending the UK's first ever School For Journalism and obtained an Honours degree in Journalism, Film and Broadcasting.

At the time of application, I had no idea what a prestigious and well-respected institution it was. 

Far from the focus that I was told others thought I had, my educational path was based on nothing more than medical restriction, a title and coincidence. After graduating, I moved to Israel for just over a year, wrote a book for a publisher there, and some corporate documents that helped a start-up secure a couple of million dollars' worth of investment. 

When I returned to the UK, it only took a couple of months to get my first 'career job' as the Editorial Assistant for a new internet company called Oodlebee. 

This was back in the day when, to get a website online, you needed £150,000 in old money each month (that's about £257,510 today).

Oodlebee could have been the Amazon of its day, predating the expansion of the huge online retailer.  A beautifully crafted site, half of it was an online shop with all sorts of products, the other half was a lifestyle magazine with editorial promoting the products we were going to sell. 

Sadly, this was the era of the so-called Dot Com Bubble bursting, and our investor decided to stop funding the company, leaving us with four hours' notice of the fact we no longer had jobs.  I moved to another internet company called BigBlueSpot. 

BigBlueSpot was aimed at university students.  It was a huge website with forums, magazine content about music, gigs, films, competitions and giveaways.  We were one of the first UK sites to feature video footage (film trailers) and we were one of the three big sites of its kind in the country at the time. 

We gave computers to students all over the UK on the condition they all used us for their email addresses.  Initially on a three month contract as the Marketing Assistant, when the two Editors left, I took over both their roles and my salary rose by £3000 overnight (it should have been much more, but I was happy enough).

It was a great company to be part of and our name was known within Manchester - when we wanted a night out in the city centre, the words 'We're BigBlueSpot,' meant we jumped bar and club queues while others had to still wait on the wet city pavements.  I got gig tickets for reviews (colleagues begged for the Dido tickets), and I admit to us sometimes being a bit naughty if we were sent prizes that we wanted. 

The boss had a draw full of Weed, we smoked cigarettes and drank at our desks on Friday afternoons and we worked and partied equally hard.

Once again, though, investor confidence fell and lay-offs started happening, with my team of remote writers being the first to go.  So, not only was I doing the work of two Editors, but I had was suddenly the only writer making content for this huge beast. 

A couple of months later, I was told my job was also gone.  It was a  mistake for the company. 

The work I had been doing was good.  Great. 

I introduced a new general news section to widen the appeal of the site and we saw a monthly increase of 20,000 unique users coming to read the new material.  I was the only qualified writer they had left, and making me redundant when their income relied upon written words...It was never going to work for them.

My medical team gave

me a 50% chance of

surviving the birth

because of Ehlers-Danlos.

Having faced the threat

of dying during childbirth,

I was not going to miss a

minute of motherhood.

The decision was made for the company to keep all three Marketing staff and sack the only writer.  The boss asked me to teach the Marketing Manager how to write in one afternoon.

My jaw hit the floor.  I'd studied for five years and had written since the age of six - things that are impossible to replicate within another in one afternoon.  It was slap in the face, an obvious minimisation and disrespect for my skills and training, and a lack of understanding for how important original content was. While I moved on to my next job, BigBlueSpot lost users, finally lost funding and closed.

It was all a little incestuous, with the original Oodlebee team getting each other jobs in new companies every time one went down.  In BigBlueSpot, three of the seven staff members had all worked together at Oodlebee.  After BigBlueSpot, my Editor from Oodlebee got me a job at his new company, eMedia. By the time I left that company, five of us were working there.  Some would call it nepotism, but we were all good at our roles and had the sort of internet-building experience that not everybody had.

Millions

eMedia was part of a gargantuan project with a budget of £25 million ( £52,901,547 in today's cash).  We were building a giant travel website, while cruise ships, airliners and shop fronts were being rebranded to match the online presence. 

The creatives were in a studio at the top of a building in the centre of Manchester, and around 100 testers sat two floors down constantly running the website we were building to find all the faults and flaws before launch.

My role?

To check and prepare the words we were paying a London-based company £10,000 a month for (£18,000 today).  It was meant to be quick, easy and only last a month or two.

A year later, I was still in the seat because the content we were paying for was so bad. 

In one meeting, I negotiated £40,000  (£78,000 at today's prices) off the yearly contract price.  Secretly pregnant by this time, I'd had to leave the meeting to throw-up because of Morning-Noon-And-Night Sickness, speaking through my nausea with nerves so strong, the tip of the rolled up spreadsheet I'd made quivering as I trembled.

Success was mine, though!  More success than I initially realised.

As we all paused at the door on the way out, the boss of the London company asked me, 'Are you a travel writer?'

'I am now,' I replied. 

He handed me his card, telling me, 'If you ever want a job, just give me a call and I'll have one for you.'

My Editor, Sub-Editor and the Content Manager standing behind me were all as stunned as I was, and the word obviously got back to the Big Cheese at eMedia, because within a week or two I was offered a permanent job setting up a new department. 

Rather than pay a company for words covering hundreds of international holiday destinations, they wanted me to establish a team of in-house writers and co-ordinate their global travels, ensuring we absolutely had all our bases covered. 

Another pay rise was offered, and at the age of just 26 I'd seen my salary rise by £13,000 in one year, to what would be just over £42,000 a year with today's inflation.

I turned it down. 

Newly pregnant, I knew I would have to raise my child alone, and with my EDS getting worse, single-parenthood, degenerative illness and a fifty to sixty hour week just wasn't possible for me.  I didn't want my child being brought up by nursery staff.  It was the most important role I was ever going to have, and I wanted to raise my offspring. 

After my son's birth, I left my professional life in Manchester and moved back to rural Wales where my child could play in streams and mud, away from all the superficiality and concrete of city life.

My amazing medical team gave me a 50% chance of surviving the birth because of Ehlers-Danlos, before Pre-Eclampsia kicked in and further increased risks. 

I was admitted to hospital for the last six weeks of gestation, though my specialist had told me had he had his way, I would have been in hospital for the full nine months. 

Having faced the threat of dying during childbirth, I was not going to miss a minute of motherhood.

Loss

I took to motherhood easily and didn't miss my city life or burgeoning career. By the time I was 31, my son was an older toddler, I was working again as a freelance writer and  engaged to a man I'd known and been involved with on-and-of since the age of 18. 

Wales was receiving plenty of cash from Europe then, and a scheme existed to support my self-employment. 

I was the first internet company to take part in the scheme and the first single-parent in Wales to secure full childcare costs, a precedent that all other lone-parents would benefit from.  Although my pain was always present and I felt my EDS getting worse, it did not control my life. 

Little did I know that everything was about to come crashing down and change forever.

In early 2006, I ended our engagement by taking a hammer to the ring I was given.  Two of its diamonds must still sit between the patio slabs in my old garden. 

Sounds harsh, maybe, but believe me when I say it was warranted. 

Alone again, I focused on my motherhood and continuing my work.  Then, one afternoon, as I was digging a hole to turn into a sunken sandpit for my son, my left ankle dislocated and I fell, resulting in the pelvic injury (Osteitis Pubis) I explained earlier. 

As previously stated, I cannot give details here and now, but the catalogue of medical neglect and law-breaks that would follow over the course of years caused a devastating cascade to happen within my body, leaving me either bed or sofa bound for the next ten years. 

Now in 2023, it is 17 years since that fall and the losses that stem for it are, perhaps, more emotionally painful than the physical suffering I experience every day.

My time was not all wasted. 

For two years I worked for EDSUK, researching, writing and working on their magazine from my bed, my desktop's monitor on a little table made for me.  Indeed, I told this charity to change their name to EDSUK, based in my prediction that a global network of EDS organisations would evolve within the following years, which is exactly what happened.  This new name I gave to them would convey a more professional and stronger position in an international context.

They had previously been called the EDS Support Group, which I felt demeaned and under represented their full potential as a charity. 

Sadly, this potential is still yet to be realised, as nothing of any tangible value has been achieved that helps EDS patients in the UK.  Hopefully, this will one day change.  I gave up this job after two years as it became too stressful to deal with the bitching, backstabbing and awkwardness the trustees kept bringing to me. However, the role did provide me with two of the proudest moments of my life as a writer...

...At one conference, the woman who set up the charity thanked me for what I turned their magazine into, saying it what what she had always envisioned for it, but had never been able to create due to them not having a journalist on staff. 

At that same conference, an internationally respected EDS specialist who is one of the founders of modern EDS knowledge and medicine, Professor Rodney Grahame, complimented me on a contentions piece I had written about cardiac issues in EDS and why people with the Hypermobile type should receive regular aortic scans to monitor their aorta to help guard against that popping and killing them. 

As mentioned elsewhere on the site, this was a direct challenge to the established idea that internal ruptures do not happen in HEDS.  They have and they do.  To be congratulated by Prof. Grahame who thought I was a trained medical journalist, was an amazing thing to happen and further grew my confidence in my ability to research and understand complex medical ideas.

I have since acted as Editor on one more edition of EDSUK's magazine, but having received not even one word of thanks for my efforts, I will never help this, what I see as useless, charity ever again.  I certainly will never donate to it, either.

What's the point in telling you all of this?   

I can't possibly describe to you the physical pain levels I have lived with and experienced. 

My hope is that by detailing my history a little, you will better grasp how much EDS and unacceptable medical attitudes and behaviours have taken from me - and continue to take from others too.

I am far from the only one.

I was officially deemed unemployable by my Neurologist by the time I was 35. 

If employers had been willing to pay people with disabilities to work remotely, and were willing to give us some wriggle room to work around our medical conditions, life would be very different for many of us.

Sadly, despite the pandemic  proving that remote work is entirely possible, the levels of ableism in Britain are still disgustingly high - and nowhere near improving.

So, I have been stuck on Welfare Benefits since 2006, with no prospects of regaining a salaried career and increasing my income.  No pension.  No savings.  No funeral plan,  No life insurance.

As I sit here writing this, I have not had any heating on for 48 hours, I am wearing three layers of clothes including a pair of ski salopettes and, unable to eat any solid food for the last 60 hours, it's a struggle to keep warm. 

This does not help my EDS meat.  It makes my pains worse as my already struggling muscles become cold and further stiffen.

If I were able to earn the equivalent of £42,000 a year aged 26, it's pretty safe to say that had the NHS treated me as they are legally bound to do, I would not be here, 22 years later, sitting in the cold worrying about being to able to afford heating.

Money gives us freedoms.  It gives us the chance to choose where we live, if we drive or not, what foods we can eat, what social life we can have and what opportunities we can give to our children. 

Those freedoms and choices were taken from me. The motherhood I had envisioned for myself was also destroyed, and this is, perhaps, the most painful part of all.

Even more difficult than remembering the night the pain nearly killed me and I was minutes away from death.  The idea that pain can kill a person was a mystery to me.  How does that happen?  I found out after around four years stuck in bed.

Dealing with ongoing pain requires mental strength.  You must keep mental control.  If you lose that, I found out the hard way, you can lose your life.  Let me explain.

'So this is how pain kills. 

At least I know now...'

I resigned myself to the fact I was going to die in that bed, next to the room

where my very young son was still asleep.

After about four years of hideous pelvic pain and being mainly bedbound by it, I just couldn't cope with the pain anymore.  It had been hurting to breathe for weeks, legs tied together as I lay flat on my back just trying to get through the agony. 

In the early hours of one morning, I lost mental control, and my brain took over.  It started shutting down my peripheral circulation. 

With a lack of oxygen in my tissues, my hands went into spasm, my fingers tense, straight and pulled together in contorted claws. I could feel a numbness spreading up from my feet, moving up my shins to my knees, the same in my arms. 

It.

Was.

Terrifying.

Fortunately, I had given a neighbour and friend a key to my house and managed to call her.  She awoke, picked up her phone and could hear only my desperate gasps for air.  She rushed over, expecting to find me sitting on the sofa downstairs. 

Instead, she was met with complete darkness, my rasping breaths guiding her up to my bedroom.  She entered and I held my hands aloft for her to see.  A look of horror filled her face and she immediately called for an ambulance.  My body was in shock and trying to keep my internal organs alive by shutting down blood flow to my limbs. 

While it was easily one of the most frightening experiences of my life to date, internally I felt a strange sense of calmness, as if I were almost an observer and not going through it myself.  Time seemed to slow down inside my mind, and I recall thinking, 'So this is how pain kills you.  At least I know now...'

I resigned myself to the fact I was going to die in that bed, next to the room where my very young son was still (hopefully) asleep.

I don't know how long it took the paramedics to arrive, and they immediately cannulated me to pump Morphine into my blood to try and stop the process my brain had begun.  A second dose was administered when the first was not enough, and they stopped in my street because I started experiencing sensations in my chest that could have signalled that my breathing was about to cease.

All I could think of was how my little child had probably woken up to blue lights flashing through his bedroom curtains, listening to the sounds of his mother struggling to breathe and the tones of the male paramedics humming through the wall and reaching his ears.  My biggest fear was the worry of what impact this would all have on his psyche.

I spent a useless month in hospital where more laws were broken and mistakes made.  When I returned home, my sheets were still stained with my blood from where the paramedic rushed to get the IV needle in.  IV needles scar me, leaving a countable record of traumas and near misses upon my skin.

My son had to move to a new school yet again and live with his grandmother until I would eventually find a new house closer to her so he could come back and live with me.  His life changed forever too, and suddenly he was without his mother and without all the friends he had made at that tender age.

Among all this, despite being told I would never conceive again, and despite sex not often happening (obviously), I did conceive.  Both the father and I wanted that baby with every fibre of our beings.  It was impossible, though.  And after a two hour meeting with the four doctors who got me safely through my first pregnancy in Manchester, it was decided with complete certainty that this pregnancy would kill both the baby I was carrying and myself.

When my pelvis is bad, I still cannot sit.  It was worse back then.  You cannot lie flat for the entirety of a pregnancy. 

As the baby grows, if you are horizontal, the weight of the baby presses on major blood vessels, cuts off circulation and you both die.  As my son's only present biological parent, and with him having inherited EDS, I had to live for him.

And so I had to have an abortion I did not want to have. Had I not already had a child, I would have taken my chances and continued with the pregnancy, dying with my unborn child if that is how it was to be. 

Again, none of this is a natural progression of my EDS.  It is the direct result of medical neglect, mistakes and medical law-breaking. 

One ankle dislocation would lead to the loss of my career and the freedoms a salary gives, the personal satisfaction working brings and any prospect of career progression.  It changed my son's childhood and gave him a life neither of us wanted for him. 

He grew up with fear and uncertainty, sometimes in borderline poverty and a mother who could not do what most mothers can.  The social impact upon him was wide and deep - never mind the emotional toll it must have taken upon his young mind.

For years, I discovered, he blamed himself, once asking me if I regretted giving birth to him. 

He believed that it was all his fault - had I not had him, I would not have tried to build a sandpit, I would not have fallen and the rest would not have happened.

Doing my best to wipe those questions and the guilt from his young mind, I was horrified he felt that way.  It's just another example of the emotional impact of pain and medical misbehaviour.

Comforting him wasn't always easy - I couldn't even pick him up or cuddle him in the ways I used to.

The NHS's chosen actions not only destroyed my life and changed my son's forever, but they also stole the chance of a life from my second child before they were even born.  I still see them on the screen their tiny heart beating, as my womb was scanned in preparation for the abortion I did not want. I can still see the beautiful golden bubble they were in slipping out of my body into a cardboard bedpan inside a toilet bowl. 

I can still see their budding facial features and their miniscule limbs as I stood alone looking down at them in that bedpan, seeing their form start to change and disintegrate before my very eyes in what seemed like far too short a time.

How do I quantify this sort of emotional pain?

How do I make you feel and understand the colossal losses?

How do I make you understand the ongoing grief that still sometimes floors me and brings me to my knees?

The physical pain is bad enough, but the emotional trauma and aspect are, arguably, worse.

Some would tell me that it's all in the past now, it is all over and it's time to simply let go of it all and move on. But it isn't all in the past.  It is still very much in the present and it will govern my terrifying future.

Fifty is but a few years away.  Menopause is coming.  With it will come hormonal changes that only worsen my collagen and TNXB production.  My body will continue to fall apart, continue to increase in subluxations and dislocations, nerve damage and the pain will only ever keep increasing.

My already poor healing will continue to worsen, ruling out all and any life-saving surgeries I may need in my older years. Breast Cancer, for example, has a genetic history in my family, having killed two close female relatives and arrived in another.  Should it also hit me, there will be no surgery because my body cannot correctly heal itself and Chemo is known to be troublesome for fragile EDS veins...This one thought alone is frightening.

I still spend weeks stuck in bed, debilitated by various pains, and my pelvis still very much controls my life choices. While I can usually control thoughts of the past, there are times I cannot, and all the losses tear me to pieces.  My entire Thirties were wasted when they didn't need to be. Not if the NHS had acted ethically and legally. Knowing that, alone, can be indescribably hard to cope with.

It.  Didn't.  Need.  To.  Be.  This.  Way.

The truth is, mentally

we are stronger and more compassionate than most of undisabled society, who are

so selfish and

ethically weak, they

are happy to risk

our lives...

With nearly 30 years of in-depth medical research under my belt, I know how this goes.  I know how this goes. I feel it every time a bone comes out of joint for the first time.  I feel it as my skull and neck joint becomes more and more unstable and it becomes more painful to even move my head. 

I feel it when my shin bones come out at the knee, when my femur tries to do the same thing.  I feel it each day I am unable to eat, each time I vomit the food I tried to digest 24 hours ago.

I have helped other Bendies. I have even saved a couple of lives. 

But I cannot change or save my own. 

I have given up on the idea of finding a romantic partner because how could it ever be fair to put my worsening illnesses and disabilities into their life, no matter how much some nights I just want to be held beneath the sheets?

There are nights I lie in bed trying to sleep, feeling and hearing my heart bouncing and skipping inside me, terrified I am not going to wake up in the morning. 

Terrified that I will and the day will just be full of pain so strong, that taking all my medications seem like the only plausible future, the only way out of this suffering.  

There are days I sit with the memories of all that I have lost and I can barely contain the grief, the frustration, the anger and sorrow.  Unable to work these energies out of myself with exercise only makes carrying the emotional pain even harder.

There are times this emotional pain, the want for my Lost Little One, the desire to go for a walk, to work, to love are overwhelming.

My future is one of increasing pain, debilitation and fear. The Disability Benefits I depend on now will be taken away from me in my sixties and I have no idea how I will survive then. 

I am trapped in an ableist society who, at the start of the pandemic, rather than happily protect the most vulnerable, cried out online 'Keep them inside!  Only they die!'.  A direct statement from a sick, discriminatory society stating they see no value my life because of my genetic conditions. My death is acceptable because I have Ehlers-Danlos Syndrome. People With Disabilities are deemed weak by an ableist society. 

The truth is, mentally we are stronger and more compassionate than most of undisabled society, who are so selfish and intellectually and ethically weak, they are happy to risk our lives so they can show their noses, mouths and chins during an ongoing pandemic, while health systems across the world collapse.

We have already faced more than most of you can possibly imagine, even if we don't 'look disabled', and it all causes so much emotional pain. 

And that is often worse

than the physical agony.

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