Tired
Everyone, ill or not, living with disabilities or not, sometimes gets tired with life, tired with the struggles this human existence and crazy society create and we must all face.
Of course, some fights are harder than others, and if we look around, we'll see someone who is worse off than we are. Small mercies do exist. But it's not always easy or possible to hold on to that fact and find solace in it.
Fears
In Pain, I talk about the many agonising medical conditions I have. Discovering how badly affected my body was by Ehlers-Danlos (EDS) was a shock to me.
It was hard to cope with and took time. I felt like my very identity had been forever changed, eroded, and I no longer knew who I was.
Now, nearly two decades after those disturbing discoveries, I can feel my body ageing and my severe Hypermobile Ehlers-Danlos Syndrome (HEDS) getting worse. What is the hardest part for me, though, is the psychology of this physical step down and how exhausting it is mentally.
As I told a fellow EDS sufferer today - I. Am. Tired.
It's natural to lose certain physicalities as we age, to struggle with things that were easy in our younger years.
But when you have a condition like EDS, it means the very proteins that our bodies are made of - skin, ligaments, tendons, muscles, blood vessels, nerve sheaths, internal organs - are not constructed properly.
Our collagen cells are malformed. They are weaker than they should be. Further complicating and worsening this is that the 'glue' between these collagen cells can also be effected if we have issues with the TNXB gene. This dictates how our bodies manufacture something called Tenascin X. Another protein, that holds our collagen proteins together.
If you don't make that properly in addition to making faulty collagen cells, you become vulnerable to sudden internal organ ruptures, and it increases the risk of your aorta popping.
If either of these things happen, you're dead. It's as simple as that.
Especially in a body that cannot heal itself, so preventative measures like surgery to 'net' these internal structure, provide a sort of scaffolding for them, is not an option.
Indeed, no surgery is a safe option.
As we age, all of our collagen production worsens. This is why we wrinkle, why we sag. With EDS, this is multiplied to the Nth degree and it hits our bodies harder than those with normal protein production. Also, as we age, things like certain Cancers may arrive and if they do, knowing you cannot cut them out of your body because that is more likely to cause death in the short-term than the Cancer itself, can be terrifying.
What do you do with that information?
We can try to not think about it, but we are not super-human. Yes, we are remarkable people who live and deal with the sort of pain, injury and issues you, hopefully, cannot imagine and will never experience.
Some of us come close to dying as we try to live in these malconstructed physical forms.
We experience daily injuries, from subluxations to full dislocations, superficial cuts and scratches that refuse to heal properly and leave permanent scarring. My body doesn't have to bleed to scar permanently. Just break the skin...
Sooner or later, however, no matter how positive a mindset we are determined to stay in, it's only natural to sometimes succumb to the fear that accompanies living with something like EDS.
Basic things like the ability to eat and digest food, drink and keep fluids in a stomach that wants to throw it up, standing, walking, even breathing can be problematic and cause a lot of problems and pain - and internal bleeding, as I experienced yesterday. Feeling your bones dislocate, pop out of place, having to relocate them, rotate the bones and force them back into the joint not only hurts, but is scary. The pain never stops. The injuries never stop and they never heal properly.
Facing up to this, in addition to the usual health issues and risks we all face as we age, is exhausting. Mentally, it is exhausting.
Trying to remain happy in your own head is exhausting mentally and emotionally. Especially when you're facing it all alone. One of the worst things a person could do with someone in my position is tell us to be positive, to try to ignore these feelings.
While maintaining a positive head space is vital in life, we
are
permitted to have down days, and acknowledging the more negative feelings is important. It has psychological value in terms of us getting it out of our heads and carrying on, and studies have found that expression can also help to decrease physical pain levels.
But how do we decrease the emotional
pain
of having our mobility, life choices and so many freedoms taken away by life-long and worsening illnesses? How many times do we voice our feelings, our fear, our mental exhaustion to another person before it
actually
helps allay them?
Is there a way of diminishing these fears when they are based on medical facts and feeling your own body deteriorating week by week?
I'll be fifty years-old in the blink of an eye, and although my menstrual cycle is still as it always was, Menopause will hit sooner rather than later - it has to. With it will come hormonal changes that will not aid me in any way, they will worsen my HEDS, worsen my bone health and this is frightening. Certain synthetic hormones are not particularly safe for me to take due to them increasing the risk of Breast Cancer, which runs in my family and has killed some of my female relatives. If it ever comes to me, I'll have to let it be. Can't cut it out.
That is a terrifying thought.
What, though, is worse than this terror?
Arguably, it's the frustration and the changing of your identity that comes with living in badly behaving bodies.
This current situation though...I'm not sure
how to work through
this one.
Once again,
my future has changed.
Past, Present, Future
I first discovered EDS in 1995. Just turned 21, I was new to my degree and had access to the internet in the university's libraries. I decided to search the symptoms I'd been experiencing since I was 11 years-old - increasing pain, being the main problem.
The first EDS indicator was the fact I'd been born prematurely. Not much, just a few of weeks, but an indicator nonetheless.
The second sign was how damn hypermobile I was, with my ankles, elbows and knees being the most obviously bendy part of my little body. Falls were regular due to my ankles coming out of joint and dropping me like a concrete balloon.
Indeed, when I was 29, my ankles would change and then nearly end my life...
The third pointer was that a wound reopened and stared bleeding again once the stitches were removed when I was about three-years-old. At the time, my mother thought the medic removing the sutures had recut my flesh and made me bleed again. They had not.
My faulty collagen just couldn't hold itself together and, ideally, the stitches would have been left in twice as long as normal to help my proteins heal. I still have that scar - slap bang in the middle of my forehead - and the way it looks has never bothered me.
Looking back, my inability to sleep all night as a very young child, the issues I had with certain foods, the reoccurring 'stomach bugs'...they all point to EDS and Dysautonomia. We just didn't have the facilities to research and learn back then, like I did once I became an undergraduate.
I sat in the university library and typed in my symptoms, hoping to find some pertinent information that would make sense of my growing pain and problems - and I did.
I found the abstract of a study about how local anaesthetics were less effective to useless in the bodies of people with something called Ehlers-Danlos. I read the listed symptoms, feeling a growing excitement and certainty in my gut...
This! This is what I have!
Taking note of the author's name, I promised myself then that I would find him, somehow see him and get this mysterious condition diagnosed. It took four years for me to finally meet with the doctor in question, Professor Rodney Grahame, in a London clinic, and after examining and measuring me, not only did he confirm my self-diagnosis of Ehlers-Danlos Syndrome, he told me I was 'one of the most severe and hypermobile cases' he'd ever seen. That didn't compute. It's only now, nearly thirty years later, I understand what he means.
Don't get me wrong, compared to some Bendies, I have it easy. I don't have Gastroparesis (stomach paralysis), though I did have a number of years where eating and drinking became pretty impossible and doctors wanted me to have a feeding tube. I refused. I'm still prescribed a liquid feed. I still vomit pretty often. I eat what I can, but there are times I have to avoid eating because the tubes after my stomach don't move properly, and there is only so much food you can push into a system that doesn't move properly before there are problems - including internal bleeds. Bleeds I haven't discussed with any medic, because what they can they do?
Nothing,
My pain started in my knees when I hit twelve, I had to give up all sports because I couldn't walk afterwards and the pain spread from there. By the time I was fourteen, my jaw was hurting and locking, sometimes for hours. My neck had started hurting and making strange clicking sounds. Then my lower spine joined in. The the rest of it. Headaches were common, as were Migraines. By the time I was sixteen years-old, I hadn't had a day without pain for five years. I'd been told it was 'Growing Pains'.
Whatever that means.
I'd had hospital appointment after hospital appointment. Tried one type of physio after another, all of which just made things worse, and that I would later come to learn were actually damaging for a body with bad EDS like mine. Some of my friends were really supportive, but some youngsters were unkind. I looked 'normal', therefore I was faking. I was accused of faking it all many times, by kids in school and medics.
By the time I hit my twenties, I could have days stuck in bed in utter spinal agony, a close friend helping to turn me over because I couldn't do it on my own. The summer I'd spent working in factories before university had permanently damaged my wrists and hands. Writing became an issue, as did dressing myself, feeding myself, using my hands at all...This new problem was discovered during my first set of exams when I had to write three essays in three hours and could only write one before I couldn't move my hand at all. From there forward, all my exams were done in a room on my own on a laptop.
I couldn't walk very far, I couldn't really run anywhere, using my hands was problematic, standing was an issue...Things were just getting worse. Having exhausted all physio therapy options, I just got on with it. Doctors prescribed me pain meds that didn't seem to do much but that were better than nothing, and I just went through life dealing with increasing pain - until I couldn't...
When I was twenty-nine, I decided to make my son a sand pit that could also be used as a patio. It involved digging out a big rectangular patch of earth. I'd nearly finished this, when my left ankle dislocated and I fell, doing the splits box-ways and injuring my Pubis Symphysis, the joint a the front of your pelvis where it meets in the middle. I was told - incorrectly - that 'your pelvis will be fixed' within a couple of months. That it would fix itself. I can't go into more details here for a few reasons, but I can tell you that that doctor was wrong.
That injury cost me a decade in bed, my career, my motherhood with my then toddler son, my mobility, any choice regarding where I could live - and the life of my second unborn child.
It devastated me physically and mentally.
But Ehlers-Danlos was far from done with me, and the pelvic injury would also lead to me discovering I had other medical conditions, which was something I had not considered at the time.
MRI scans showed that I was born with something related to Spina Bifida. Called Dural Ectasia, it means the membrane that wraps around the very bottom of my spinal cord wasn't formed properly. It's made of proteins, right?
And EDS means my body doesn't make proteins properly.
The membrane is baggy. It balloons out, fills with too much Cerebrospinal Fluid (CFS), and that not only caused issues with the surrounding nerves at the bottom of my spine, but it also drops the CFS pressure in an around my brain and spinal cord. It hurts.
Just above the Dural Ectasia, are a group of huge cysts (we're talking 15cm x 11cm at the last scan over fifteen years ago).
Called Tarlov Cysts or Perineural Cysts, this is where the membranes around the roots of my nerves as they leave my spinal cord were not formed well either. They too are full of CFS and also help drop the pressure of fluid in and around my brain and spinal cord, as well as impinging on the nerves in that area. They can have some nasty impacts - mainly pain in my head.
Let me tell you this - when the pressure of your CFS drops, the pain in the your skull can be immeasurably bad. It's life-stopping. Your brain only had to drop the tiniest amount in your skull, put pressure on your brain stem, and it's game over until it decides to top the tank up again. You can't even sit upright, let alone do anything. You just have to lie flat and still and focus on breathing and getting through the next minute, hour, day, night...
Scans on my pelvic injury would also reveal that I have a permanent spinal fracture (though I have no idea how I fractured it) and Degenerative Disc Disease, with some discs having herniated and impinging on nerves. I'm sure plenty of you know the pain this can cause. I have less feeling in my left leg than my right.
I've had times I've dragged my leg around rather than be able to move it. I now think the discs in my neck have also herniated, but I'm yet to have that looked at because I'm focusing on other things - like the seizures I started having four years ago.
The seizures that have, once again, made me feel like the bottom of my world has fallen away, and are having horrible impacts on my brain. I've worked time and again to accept, process and deal with the various diagnoses I have been given. This current situation with the seizures, though...I'm not sure how to work through this one. Once again, my future has changed.
I don't even know if I have one.
Not pushing through the pain can feel like failure.
It can make your body
feel like an enemy.
There is only so much physical pain we can take.
Losing Myself
I've always been independent, stubborn from a very young age. When my mother would try to put me in the car to take us somewhere I didn't want to go, I would place a foot either side of the door, lock my little hyperextensible knees and scream, making it impossible for her to place me in the back seat.
Looking back at that now, I don't think it was a tantrum, I think it was Autism showing up (and being missed). EDS and Autism are known to be connected somehow - so many of us Bendies also have Autism, it is beyond coincidence and a statistical link exists.
Personally, I think that altered connectivity in our brains due to altered proteins has to be part, at least, of the reason so many of us are impacted by Autism.
I never really noticed it in my teens, twenties and thirties - but I've been noticing it heavily in the last few years. I mask well. I'm 'high-functioning'. While most people in my life may not have noticed it historically speaking, I think more of them are noticing it now, mainly through my absence in their lives.
By the way I have, for the last few years, outwardly shutdown more and more, increasingly disengaged from people and just gone into my own head and stayed there.
Life has, undoubtedly, added to this vanishing. My body has a lot to do with it too. While it deteriorates, challenges me not just physically but also mentally, I feel myself disappearing within it. I always knew EDS got worse. I just didn't quite appreciate how much worse.
There are things I could do a year ago that I just cannot do now. I can no longer push through the level of pain caused by those things. My house needs a good clean and redecorating. Outside is a mess.
I cannot sort it all out. I just can't.
I can't hurt that much any more. Even if I could physically climb ladders, Epilepsy means I can't in case I have a seizure and fall.
The frustration caused by looking at the things I cannot physically cope with now is beyond frustrating. It hurts emotionally.
It feels like you're failing - even when you know just remaining in a body like mine is some sort of success. Not being able to push through the pain can in and of itself feel like failure.
It can make your body feel like an enemy. There is only so much physical pain we can take.
Me?
I'm close to my limit.
Had I been someone's cat or beloved pet dog, they would have been forced to put me to sleep years ago. It's been a long road. The lack of medical care available to me where I live is another problem that allows things to get worse. Not that there is a cure for Ehlers-Danlos. Not that there are any really effective therapies when your case is 'severe'.
While my son was young and while he still lived at home, I pushed through the pain for him. I wasn't always able to, and after going into shock, nearly dying from the pain one night and ending up in a useless hospital for six weeks as they spoke of putting me in a care home rather than getting me to EDS specialists, he couldn't live with me.
That broke me all over again.
Made me feel like I had failed as a mother.
Eventually, I managed to find another house nearer to the new school he ended up in so that he wouldn't have to keep changing schools and, hopefully, we would rebuild some security, which we did. Not that the pain went away. The struggle still pushed me to my very limit psychologically.
Be not mistaken - the only reason I am still here is him.
Having lost someone I was very close to to suicide, I know the absolute ruin it leave behinds in the heads and hearts of those who love you. How could I put my son through that?
That is a question I find myself debating more and more as the days go by and my body continues to deteriorate and torture and terrify me. It is an internal discussion I find myself having increasingly often recently, in the advent of the seizures I started having in 2020. While it isn't fair to those you leave behind, is it fair to us to continue suffering and being scared just for the sake of others? That question, this inner debate, is something I can't reconcile yet.
Having had Clinical Depression in the past, I honestly do not believe I have it now. I think this internal debate is born purely of being tired of the pain.
Tired of the fear.
Tired of the degeneration and ever-increasing suffering. I still see joys in life.
There are still so many things I want to do - I just can't do them...
Having recently found out that the seizures I've been having are only half the story and the situation is far worse and more dangerous than I initially thought, I find my world bottomless again.
Find myself losing my sense of identity.
Find myself wondering if I can mentally rebuild myself this time.
Find myself wondering if it's even worth
trying.
It was the bottom of
my world dropping
out yet again!
I'm scared to fall asleep
in case it happens
again and nobody is around to make sure
I can breathe.
Limits
I said at the start that this isn't a competition as to who has it worse. I knew my life is easier compared to many, but it's been a lot harder than a lot of people will comprehend.
This now, apparently, seems to be manifesting in my brain, with one stressful situation finally tipping it over the edge.
Speaking with the Epilepsy Service last week, we discussed when my first seizure happened, looking for a possible trigger.
There are links between EDS and Epilepsy, with the number of us Bendies with Epilepsy being beyond coincidence and statistically significant (meaning there IS a link, we just don't know what it is yet).
A recent brain scan has shown an issue suggesting my brain is struggling and somewhat damaged.
The implications of the findings are such that I only hope it is Epilepsy triggered by trauma further to the predisposition EDS can cause, rather than it be related to certain ongoing condition that could basically obliterate me inside this head.
I've already lost my senses of taste and smell.
In recent months, my memories have been disappearing, my ability to recall more recent things growing ever more a struggle.
The very idea that I have dangerous seizures in my sleep is terrifying. I was completely unaware of them. I had been told about them by the man I write about in 'Filthy', but I didn't remember him telling me - until the second time. To him, it wasn't new news. To me?
It was the bottom of my world dropping out yet again! It was brand new news. I spent days trying to not cry and failing. I am scared to fall asleep in case it happens again and nobody, including myself, is around to help protect me, to reposition me to make sure I can breathe.
Above, I mentioned the suicide of someone I was close to.
A conversation with the Epilepsy Service concluded that this may have been the final trauma that pushed my brain to its limit and allowed the seizures to start.
Ironically, the man who died is the one person who would really listen to the most scary parts of my life. Who would call me the second he heard that something else had happened or I was struggling with my pain.
He would sit for hours on the end of the telephone letting me speak and cry if I needed to, always responding with plenty of words that helped me get through it all. I thought that the PTSD from his death and what came afterwards was the worst it could get. I was obviously wrong. He was the first, and often only, person I would completely open up to.
He always knew what to say and how to say it to me.
Don't get me wrong - it was a two-way street. He had many daemons and I too would spend hours listening and talking him through his worst moments. We were a very emotional thing. We held hands and each other in our sleep. I would punch a toddler in the face for one more cuddle with him today...
While I do have people who would listen to me now, I feel like he was the one person who knew how to respond to me. Is that an Autism thing? I do not know. I do know, though, that I am tired of not having that. Of no longer having this connection with him.
I am tired of the guilt and the regrets, the 'If Only'.
If only I had sent one more message, made one more call...
It circulates in my head day after day after day, such can be with way with PTSD curdling your brain. On bad days, like the ones of recent weeks, I want to talk him even more. It's something I haven't quite managed to rebuild myself from. It's still as fresh as the day I found out his body had finally been found. But, knowing how his death has felt, has kept me here so far.
In losing him I learned how much suicide impacts upon those who love you, those you leave behind. Now I'm learning the hard way that it can also literally damage your brain. While I sit here typing this and trying to deal with this latest medical situation. while I feel like I am literally losing myself inside my own head, I am also having to cope with the worsening of my EDS and, let me tell you this - that's not getting any easier.
I can't keep being a 'safe place' for
all these people to keep talking at me, while
their ears remain closed.
Isolation
At first, my isolation was caused purely by my pelvic injury and being stuck unable to move much for ten years.
Most adults build a social circle around colleagues, but when your body makes you unemployable, you're not around people, you're stuck in your house alone.
Multiply that by a failing and worsening body, I have found myself isolating from people I used to be close to. Where one or two are concerned, this is for their benefit.
They don't need to be around all this. They're better off without it - without me.
That's what my struggling brain says, logical or not.
I cut out tens of people from my life for other reasons too, including me just not feeling like they understood me. Feeling like I spend far more time listening to them than they ever do to me, and deciding I just can't do that any more. I can't keep being a 'safe place' for all these people to keep talking at me, while their ears remain closed.
If you're the person others rely on to cope with life, who do you talk to when you're the one needing the scaffolding?
My mother trauma dumping on me when I was a child, coming to me, putting her arms around me to cry, telling me 'You're my best friend' when I was still a child trying to cope with her and my father probably programmed me to soak up other people's troubles.
In my first year of university, friends would often come to tell me their woes and secrets, with a few of them telling me my little room in halls was like a confessional box.
Conversely, I never really got to open up to them.
At a very young age, I learned to keep my our mouth shut because you were not going to be well received if you didn't. Maybe that's why I am so outspoken and gobby now? Not that I really talk to anyone about my deepest thoughts and feelings.
Those very few people closest to me now probably learn things from my writing here that they didn't know, even if they've known me for nearly thirty years.
I've been too hurt, too disappointed, by too many people. My traumatised brain decided shutting down and staying away from people is the safest option. If not the most psychologically healthy approach.
Maybe it's simply that I don't know how to put everything in to words well enough to sate the emotions inside me? Maybe it is that I have been too let down in the past to keep trusting and trying to connect with others on a deeper level?
But, I know my body also has a huge part in all this isolation. If breathing hurts, talking and tying and sitting and anything else you can list also hurts. You have to prioritise yourself. You have to not reply to people because you're just trying to get through this latest big flare-up. Not everyone grasps that, and some get shitty with you. They take it personally, which only makes my physical pain worse.
They think you look good, or you look okay, while inside you're in agony every. Damn. Minute. Of every. Damn. Day.
And when it comes to EDS, the pain may not even be the worst bit.
One thing I am struggling with more every day is the structural integrity of my permanently fractured and herniated spine.
For decades I have had to manipulate my vertebrae, click them 'back in' because it is constantly subluxing and moving out of place. Now, however, it is reaching new levels. I only have to breathe in normally and where my ribs connect to my spine is clicking. I can be sitting still and move my head a fraction of a centimetre and it is popping in and out.
I can cough and my skull will suddenly shift, click and pop back onto the top vertebra upon which it is meant to sit. The noise is quite impressive in its way.
The sensation can be terrifying and causes pain. It goes through me. It's a horrible and increasing worsening physical issue.
The treatment would be to bolt my skull onto my neck, then bolt my neck and entire spine together to help if stay in alignment.
But, as I said, I don't heal well enough for any surgery to be safe. Neither do have the at least £250,000 I'd need just for the first run at it all.
I can look at my skin and see intrinsic weakness in it far beyond the scope of normal ageing. I have days where I have to stop eating because I've started bleeding internally, the blood working its way out of my body in one way or another, and I don't know what to do with any of this.
I don't know how to not now be scared by the worsening I feel, the way my spine wouldn't know how to stay in place if that were what it was designed to do. I don't know how to process the fear, the loneliness, the disconnection.
I don't know how to how to make any of this better in my own head - and that is what I have always done...
I keep saying my world feels like the bottom has fallen out of it - yet again - and this time, I don't know how to rebuild even a thin floor upon which to try to stand.
I'm stuck between being scared to go to sleep in case another seizure happens and suffocates me, and some nights hoping to not wake up in the morning and have to feel all the physical pain and the fear it causes all over again.
I'm scared of what these new anti-seizure medications will do to my brain, how they will impact upon my ability to think, to be myself inside my own mind - which I feel like I am now losing.
And, beyond a physical
level, I'm exhausted.
I'm sore, I'm scared,
and I am tired.
