I Officially Don’t Have Any Screws Loose!

This time last year, I was desperately fundraising in an attempt to get neurosurgery that all the NHS neurosurgeons had expressly told me not to get.
I was dealing with the most severe pain I had ever experienced on a daily basis, continuing to deteriorate neurologically loosing more and more function each day, and I was getting to the point where I was impersonating Dory from finding Nemo!

When I look back at where I was a year ago, it is with a combination of immense happiness to be in the position I am in now, yet with an overwhelming sense of sadness that I have watched so many years of my life tumble away with absolutely no appropriate medical care that should have been there to stop it happening.

I have avoided writing about my experience of going through surgery up until now, but with being a year post surgery, and some time to reflect, I feel like it would be a good time to start writing once again. Perhaps the next few posts may enable someone else fighting this beast to trust their intuition before it gets to the point where longterm damage is a real possibility.

A couple of weeks ago, I was officially discharged from my neurosurgeon. I had a scan completed, and this is showing a small section of bone grown around my fusion. I was also told that I officially have ‘no loose screws’ – something I need framed for the next doctor I meet who tries to tell me its all in my head!

I wish I could tell you I was jumping from the rooftops in joy – part of me is, but the other part of me is acutely aware of the overhanging neurological symptoms that are still part of my day to day life. I knew surgery would not fix everything, and I am so grateful to be in a position where things won’t deteriorate further, but had this surgery have been done sooner, I would not be in the position I am now in. I would not have had this much neurological damage to heal from.

Leading up to this follow-up I was terrified. I knew that things were much better than they had been, but I also knew that I still have so many symptoms that are still proving problematic. Before surgery, my surgeon had told me it could take up to a year for my brainstem to heal, so this elusive one year deadline felt terrifying when I knew that I still had so many issues leftover. My head was filled with worries of whether this would be as far as my recovery would get. Whether the damage was too far gone to recover from.

What he hadn’t told me at the time was that he hadn’t actually expected me to get any better at all – owing to the level of instability, and severity of the chiari, he had only expected to halt the progression of symptoms. I am very pleased to say he has managed to do much more than that.

Today’s life is very different to life before surgery. The improvements to my memory and ability to converse and communicate are overwhelmingly positive. I no longer feel dissociated on a constant basis, and it literally feels like the world is in colour again after years of it being in black and white. As soon as I woke up from that surgery, it felt like I had woken up for the first time in 5 years. I was no longer stuck in a fuzzy world where nothing felt real. I was present for the first time in so long that I have no real inkling of when that feeling first started. I am no longer dealing with periods of paralysis in my lower legs, and my swallowing has improved immensely. My levels of pain day-to-day are so much more manageable, and the periods of time in which I pass out purely because I sit up are thankfully largely gone (something that was happening too many times a day to count). I am gradually experiencing less sudden electric jolts through my body, less randomly thrown items across rooms, and far less vertigo upon moving my head. My migraines have gone from 2-3 a week to just 2-3 a month! So much has changed that I can’t even begin to write it all down.

However there are still many symptoms overhanging – and although some have started to improve, I don’t know how many will disappear completely. I still experience balance issues, loss of sensation and periods of ‘forgetting’ to breathe. I still have weakness down one side of my body (hemiparesis), and still experience some periods of my body jolting as if plugged into the mains. I still have issues with my grip, and my fine motor skills are clumsy and uncoordinated, and my memory is still sketchy to say the least. I am still experiencing bedwetting at random, and periods where I don’t even realise I have had an accident whilst wide awake until I touch my trousers and realise they’re wet (thankfully not too often) owing to loss of sensation that is still present. My core muscles are almost non-existent, and because it took 5 years to get treatment (for most of which I was unable to keep active), I have lost more strength than I could ever have imagined throughout my body. My vision is still variable – I still get times of double vision and times where it seems like someone has turned the lights off for no apparent reason, and my proprioceptive difficulties still leads me to walk into door frames and walls on a regular basis. These are just a handful of leftover symptoms – but there are many many more.

On top of the leftover issues, I am only now starting to realise the true extent of the trauma left over from not only undergoing such a massive surgery, but also from having to fight tooth and nail to get surgery that has allowed me to stay alive. Even I wasn’t aware of how dangerous my situation had become, and it was only after being shown a video of my skull literally sliding about freely upon my spine taken during surgery that I really began to understand. The physio, who had been briefed by the team, sat me down in the first appointment I had with her, and asked if I realised how dangerous that was. She told me I was extremely lucky not to have already died, or at best ended up completely paralysed needing a ventilator to survive.

For 5 long years, almost every neurologist and neurosurgeon I had seen on the NHS had told me that there was absolutely nothing wrong with me. That every symptom was psychosomatic, and that I was essentially just making it all up due to mental illness. Not once was a proper neurological assessment carried out – even though I had been referred due to vertigo, loss of sensation and hemiparesis. All of which are bog standard symptoms for a neurologist to look into and treat. Due to their insistence that nothing was wrong with me, my family spent years believing this, and were understandably reluctant to believe me over medical professionals. I knew something was majorly wrong. I know my mental health, and I knew that the two were not related, but I didn’t carry as much clout as a neurologist does in proving this. I spent 5 long years being told that ‘under no circumstance’ should I go ahead with this surgery, and that there were absolutely no medical grounds to do so – yet look where I am just 1 year post surgery.

I feel so angry at the injustice of this, not just for me, but for everyone else I know in the EDS community who has fought or is still fighting to get help for these conditions. I am livid that, potentially, my residual symptoms will be permanent, and that this would have been preventable with prompt treatment. Even I can see logic in the argument that if every other part of my body can be hypermobile or prone to herniation, then the same could apply to my spine and brain, and that neither would be good news to a spinal cord sat in the middle of these two important structures. I am also frustrated with the inability to get adequate follow-up care on the NHS – had I have been in a car accident and caused neurological issues in that manner, I would be able to get rehab on the NHS to help with this, but because my instability was caused by my EDS rather than trauma, none of this is open to me. Not only this, but since surgery, every neurologist I have spoken too has since congratulated me on getting the surgery they told me I should never have gotten – talk about messing with someones head.

I still have a long path to recovery ahead of me, and as per usual with a bendy body – you fix one thing, and another falls apart. I am now in the process of getting my hips diagnosed and treated (waiting on an MRI and steroid injections into both), and help for my lower spine as I have herniating and degenerative discs that are now causing a problem further down. I now also have 3 herniating discs in my cervical spine and am waiting for another referral to neurology to look into what my neurosurgeon suspects is thoracic outlet syndrome (I am getting numbness in my arm any time I lift it above shoulder height).

I will write the process of surgery in a series of posts – but I wanted to bring awareness to the psychological impact of such systemic neglect of a patient. Since surgery, I have noticed an increase of my PTSD symptoms specifically around medicine, and am also struggling to keep on top of my depression. Both of these feel inextricably linked to the gaslighting received from those who should have had my best interests at heart over the last 5 years. This is not a negative post by any means, but I feel that it’s important to say that although I am physically much better than I could have imagined possible (BIG thank you to all of you who helped me get there), the trauma of the last 5 years has not disappeared alongside my physical improvements.

The temptation to sit here and not mention this when so many people contributed to my surgery is very strong – but I have always wanted to give a realistic view of things in the hope this will be helpful to others facing or working with people in similar positions. It’s ok to feel grateful, angry and grieve post surgery – or any other combination of feelings.

Life After Getting My Head Screwed On Properly!

Life After Getting My Head Screwed On Properly!

So, I have been very quiet on here for some time.
I finally had my surgery in Leeds in November just gone, and without doubt it is one of the best things that I have ever made the decision to do. It was however one very big procedure and it has taken me some time to lick my wounds, and get my head around the changes my body has undergone.

I don’t for a minute regret the decision – I feel and know that I am lucky to be alive, to be well (as much as I can be with EDS) and not to have already ended up paralysed or dead (something I wasn’t made aware of until after having surgery).

I will be writing a series of posts on the processes involved in having the surgery, but today I wanted to start with where I am now – and the other big decision that looms over my head.

I had always told myself that having a preventative double mastectomy (I carry a breast cancer gene mutation) would be pointless to even think about until I had my neurosurgery. There was no point going through the turmoil of chopping off two very important parts of my body until I knew I would be alive long enough for it to really make a difference. I spent going on three years fighting hard for neurosurgery, and during that period of time, BRCA2 slid to the back of my mind and got shut away in a box marked ‘saved for later’.

The odd thing about having the neurosurgery is that I no longer have an excuse to ignore the two rather large problems positioned at the top of my torso. The stress of 6 monthly testing (they found an area last time they wanted to keep an eye on) affects me particularly badly having witnessed a very close friend die of cancer, and the more poking and prodding that takes place, the more I feel its time to start thinking about surgery.

I would love anything other then surgery to chop my two girls off, but logically it makes sense to follow through – I have so many other health problems that adding cancer to the list just seems plain silly if I have a way to prevent it. After all – total strangers and so many caring friends and family have just contributed to a surgery that has literally saved my life – I feel that not having this surgery sort of throws that back in peoples faces.

I am by no way ready to follow through just yet, but I am in a place where I am starting to figure out my headspace and what I will need before surgery.
I am researching, talking to professionals, other BRCA carriers and generally perving at pictures of different reconstructions – its all boobs these days!

I know that to feel ok about surgery, I absolutely need to get ahead of my very distorted body image, and find a way to judge myself on the person inside my body, and not on the body itself. I need to connect to the person I locked away decades ago and allow her to be judged on her strengths, not her body, not how she achieves and certainly not her sexuality.
Quite how I do this, I have absolutely no idea – but I know full well that chopping off the only parts of me I have never hated will have a serious impact on my mental health if I don’t.

I also know I need to start talking about it. Start building a support system full of wonderful and supportive people who will stick by my side on days where I can’t pretend to be ok anymore. I need all the bad boob jokes, all the silly conversations and funny upsides to living with ‘imposter boobs’. I will need people prepared to hear me cry, and prepared for the very dark humour that so often follows.

It was so easy to lock the BRCA box and throw away the key – but I know that if I leave this until its too late, I won’t forgive myself.

So my second boob MRI is this Thursday, and I am intending to ask to talk things through with a surgeon in the near future. I want to know what reconstructions they would be prepared to offer an overweight bendy body like mine. I know I want a flap reconstruction (using my own tissue taken from other parts of the body) – but I don’t yet know if its something they will consider for me. I know the recovery is much harder, but I also know they will feel more like my own, with the added perk of perhaps changing with weight fluctuations like my own little ninjas do.

With so much to think about, I will leave you with my latest BRCA2 artwork… I will be back very soon with more on the rest of my body and surgery!

Update after my visit to Dr Henderson

Hello my lovelies,

It has been a while since I posted, but that doesn’t mean nothing has happened in my little bubble. As some of you will know I had one big adventure flying over the the USA to visit a neurosurgeon for a second (or 5th) opinion on my somewhat wobbly head.

Not in a million years did I think I would be flying for 8 hours to see a doctor, but I am pleased that I did. It has been so easy to fall into the trap of ignoring something that didn’t feel right. I have had so many appointments with UK based neurologists who have dismissed any issues present on my scans, and point blank refused to do a neurological exam – even with numbness, balance issues and visual changes. It left me feeling unsure of myself, and even though I could tell that something wasn’t right, I didn’t have the confidence to look into it further.

Having had 3 people tell me there were these issues present, and 3 doctors state that there were no issues on my scans, it felt like I was loosing the will to find an answer. How so many different views could come back from 1 set of scan images is beyond me, but I couldn’t keep going as I was.

So, a little over 2 weeks ago, my dad and I hopped on a plane all the way to Washington DC. It was my first time flying on a long haul flight, and I really had no idea what to expect. Armed with a bag full of pillows, my trusty neck brace and some extra pain relief, I am pleased to be able to say that the flights were tolerable, and although fatigue and pain levels have predictably skyrocketed since getting back, I am pleased that we made the trip.

We saw Dr Henderson who is so much friendlier the anyone I have seen here. The appointment went as I expected; an hours discussion which included a history of my symptoms, viewing the scan images and a full neurological exam (the thing that my UK doctors have refused to do). The neurological exam highlighted a lot of issues and these were definitely more pronounced then they had been during my visit to Dr Gilet a year and a half ago. I have very little sensitivity to pain, no gag reflex, loss of balance on closing my eyes and when walking heel to toe, and abnormal eye movement when looking to the right amongst other things. This shouldn’t have been a surprise to me, but I hadn’t quite realised how much worse things are now to where they were 2 years ago.

He ordered a rotational CT scan to measure for Atlanto Axial Instability which was present, and suggested that I would need both a fusion and decompression to stabilise things and give room for the CSF to flow adequately as my MRI showed the Chiari was causing ‘significant blockage to CSF flow’ – something which I was sure was present, but the UK neurologists swore blindly was not showing on my scans.

This is now the 4th Doctor to state that these issues are present, and I finally feel reassured enough to trust that 4 unrelated doctors wouldn’t be making this up. The UK for whatever reason is unwilling to help me with either surgery or symptom management (I am just being handed from pillar to post by doctors who either say they can’t operate or that there is nothing wrong in the first place), and I am getting to the stage where I am done with fighting the NHS.

So I have finally started to come to terms with the reality of the situation. If I want this fixed, then I will have to travel for surgery and begin fundraising in order to be able to do so. It is a scary amount of money that is needed, and the number is far outweighing anything my family could help me with. My only option will be to make a very public attempt to raise funds, which as an introvert, is about the worst thing I could ever think of doing!!!

I have a couple of decisions to make first about where I will go for surgery, and how long the fusion will be, but once that’s decided and I have an idea of the price, it will be time to start raising money to get myself better.

I really hope this will get me into a place where I am functioning again, and can get into work once more.

As always, I will keep you all in the loop, so watch this space!

When Your Home Feels Like a Prison

Most people would give anything for some time at home away from work. When I was working, sick days felt like a holiday – I truly had no idea how anyone could find it boring. That was until all of my days turned into sick days.

Now days I spent the majority of my time in one of two places – my bedroom or the living room. I spent literally most of my times staring at the same 4 walls, and it feels like torture. I have watched everything I could think of watching on TV, I have listened to all of my audiobooks multiple times and I don’t have the energy to do much else.

Everything that I used to do is now too difficult to manage. I am living with the feeling of someone smashing an axe into the back of my head whenever I am upright for more then 10 minutes, I have 3-4 migraines a week and all of the other wonderful pain and feelings that EDS brings. All of these symptoms are made a million times worse when I can no longer leave the house enough to break up my day, or do any of the hobbies that kept me afloat. I now spend hours in the day asleep because I am in too much pain and too exhausted to do otherwise.

On the rare occasion I get out of the house, it is like a weight gets lifted off my shoulders. I can breath again, and life doesn’t seem so dark. Yet inevitably I know that there will be a payoff for pushing myself, and know full well that that payoff means I will spend the next 3-4 days imprisoned at home once again.

I desperately want to be working, to socialise and to keep up with the hobbies I love. I want to be the person I was 10 years ago. I want the highlight of my day to be getting out and doing something, not a carer turning up for 10 minutes to do the things I am unable to do.

I keep on thinking that if I just push myself a little harder, or ignore how my body feels that I will be able force my body back into normality, but sadly it just doesn’t work. God knows I have tried. Each time I push, my body pushes back harder, and once again I am stuck lying down trying to get rid of the severe pain in my head and back.

I long for a normal persons sick day. This isn’t a holiday. This is hell.

At Least I get a Soda-Stream…

Yesterday I had an appointment with the gastro team.

I am no stranger to problems in this area, suffering from dysmotility, GERD, a sliding hiatus hernia and difficulty swallowing. Over recent years this has progressed and I now take anti sickness tablets daily to stop me vomiting, and am finding  food is getting stuck much more to the back of my throat when I try to eat or drink.

What I found peculiar about yesterdays appointment was just how matter of fact life changing diagnoses have become for me. I went in smiling, and the doctor was very friendly, we had a bit of a laugh and a joke, but in amongst it all the main message was ‘if its what I think it is, there is nothing we can do for you’. That basically translates too ‘sorry, good luck and goodbye’.

What is my reaction to this you may ask? Was I crying and unbearably distressed by this news? Of course not; I shrugged my shoulders, and carried on making sarcastic jokes. I find it so surreal that a serious diagnosis with such little option for treatment is just one more word added to the pile. I don’t think I even realised what it really meant: that I will likely continue to get worse, and that if I’m already needing a soft food diet (and struggling with that) what in earth happens when I get to the point where I can’t swallow anything at all?

I react this way because EDS gives me little choice. I have so many diagnoses of problems that can’t be fixed. Problems I will live with for the rest of my life in every system of my body. Not once have I reacted as I probably should have done because crying about it seems like a huge huge waste of my very limited energy supply. Each diagnosis leaves me a little more numb to my emotions, and a little more distant to those around me.

I haven’t mentioned these issues to friends or family – I haven’t called anyone to tell them, and I feel like I cant talk about them because these illnesses are so hard to understand. I don’t know that anyone could realise the implications on my everyday life without sadly being in this position them selves. EDS makes you a very lonely person.

I wish that I could react like you may do the first time you get diagnosed with a life changing illness. I wish that I wasn’t so used to hearing ‘theres nothing we can do’.

He told me there are ways to cope, and mentioned fizzy drinks as helpful (something I realised long ago as it forces food down my oesophagus which won’t budge by itself) – finally a medical excuse for my Diet Coke addiction! He even suggested that I should buy a Sodastream to help.

So here I am with a body refusing to work, struggling to eat and swallow without choking; but at least I get a Sodastream.



Lets Talk Medical PTSD

Part of the reason I have been so quiet lately is that I am so exhausted of the fight that goes hand in hand with trying to source the appropriate care in this country. I have spoken to many others in the same position, and I know for a fact that I am far from the only one to feel like giving up – so here you go, lets talk.

I have suffered from plain old PTSD for over 12 years. I am familiar with the panic attacks, the dissociation and the avoidance. I have made my peace with it for the most part – I know that the dissociation, however impractical, is there to protect me when things get too overwhelming, and for the most part I am now in control of my triggers. Of course I would rather not have it at all, but compared to a few years ago, the effects on my day-to-day life are minimal. I mention this because it’s from direct experience that I can say that those same feelings and reactions are now linked with a new trigger for me – that trigger is medicine.

Something as small as going to the GP now sends me into overdrive. I am so fearful that I will get the same old reactions that are so common for patients with EDS. The doubt, the way you are looked at like some sort of mental case and the arrogance that comes with talking over a patient and simply refusing to listen. When I go to the doctor, I know it is a million times more likely that I will be told that non of these conditions exist, or that they are far too rare for someone like me to have then for a doctor to actually accept that I am ill. They will sit and talk over my head to the other person I have with me rather then make eye contact and discuss things with the 30 year old woman in front of them – and this is far more damaging then I can express. I am made to feel sub-human. I am made to feel stupid, and attention seeking and small. Worse still I am now so disheartened that I spent over a year sitting with new and worsening symptoms without seeking further help.

This systemic neglect cuts deep into the minds of someone with a ‘rare’ or chronic illness. To belittle a patient to the extent that they can no longer seek medical care seems outrageous, but it appears to be a very common occurrence. I know so many others who now avoid medical intervention completely. We simply don’t have the energy to keep fighting any more.

Recently I had to change GP surgery. It took 4 years of neglected referrals and wasted appointments to push me to a point where I had no choice, but what surprised me in this was that my reluctance to change was simple: there was no guarantee any doctor would be any better, and I couldn’t face the idea of going through everything again, getting my hopes up, and then being shot down once more. The more that this happens, the worse it feels. I may have been strong once upon a time, but these days I have no energy left to withstand another onslaught.

Finally after 4 more referrals being refused (all whilst being told they had been done repeatedly) through 3 GPs at that surgery, I made a move. These referrals were important ones. One was for breast screening that should have been made 6 months before my 30th – and seeing as dad has had related cancer twice, and mum is now going though treatment for breast cancer, I really don’t want to ignore the 85% lifetime chance that I carry with the BRCA2 gene mutation I have. Another was for a scan of my spine after one leg went completely numb for 6 months for no apparent reason. I have also had several occasions of short term numbness/paralysis of both legs (thankfully only lasting a couple of hours each time) and to date, although the GPs suggested a scan of my spine, no referral has ever been made. I now have limited feeling in one leg and it is comparably very weak to the leg on my other side. The third referral was to the CMHT after my depression got unmanageable over the summer – the GP openly admitted I had scared her with how low I got this time round, yet although she repeatedly told me that this referral was in the pipeline, again it was never made. Finally, I was told that the horrific vertigo like dizziness I get, and the issues with loss of sensation I get are things that she would not treat unless I was under the care of a neurologist. She suggested a referral to Neurology was needed, and supposedly this had been made back in September. Sadly yet again, this was never made, and worse still I had been strung along for months awaiting for referrals to come through that never would. Each of these referrals were suggested by the GP. I did not push for them, I didn’t ask for them. So why lie about doing them?!

Each and every time issues like this happen, a little more of my resolve dies alongside it. It is almost impossible to keep on pushing when every appointment is at best a waste of time, and at worst a personal attack.

I am exhausted of fighting, of proving, and of undergoing frankly unpleasant medical tests only for the results to be dismissed once conditions are picked up on. I am tired of trying to fight for the care that people with more common conditions get given automatically. I am just so tired.

Flipping back PTSD, it truly surprises me just how many PTSD symptoms are now cropping up whenever I need to make contact with a doctor. My first appointment with the new GP felt horrific. I had been dissociating all day, and the nearer It got to my appointment time the more I started to panic. My heart was racing, my breathing shallowed, I was physically visibly shaking, and by the time I had to go I was terrified. “What if no one believes me? What if he makes me feel like I’m attention seeking? What if, what if, what if.” I found it extremely hard to talk to him, and although I brought a stack of hospital letters with me (as proof I wasn’t making things up), I found it very difficult to know what to say. Although he was surprisingly nice about things, I came away shaking at home for a further 2 hours after that appointment.

So far, he hasn’t been as bad as my last GP, but there is so much trust gone that I am still finding it extremely difficult to build up enough energy/courage to make further appointments. So much has been neglected in the past that I feel terrified that continued appointments will only make him think I am making the whole thing up. I am living with numerous symptoms left unmanaged, pain that is uncontrolled and fatigue worse than its ever been. I have ongoing issues with depression that frankly I have avoided bringing to medical attention due to the persistent idea that its the mental ill health that causes symptoms rather then the reality which is that you get depressed because your symptoms have dismantled your life one ability at a time. Of course you would be depressed if your body is so unpredictable. I have gone from being able to dance for 16 hours a week alongside a full time degree to barely managing 2 hours sat up before I am exhausted in just 5 years. I am wobbly, I am in pain, I am unable to manage basic self care without assistance. Who wouldn’t be depressed?! Yet I can’t discuss this with medical professionals for fear that they automatically then write off all other symptoms as psychosomatic.

It shouldn’t be like this. We shouldn’t be terrified of making a doctors appointment or attending a hospital appointment. We shouldn’t be scared of being honest about how low we feel due to the situation we find ourselves in. Non of this should be happening to any of us. EDS is not a mental illness, but managing the neglect we are faced with often causes either depression, anxiety or PTSD.

For those on the outside looking in, it is a frustrating situation to observe. I know that for those 4 years that I didn’t have the guts to face my fears and move frustrated many around me. I couldn’t explain to people why I couldn’t move surgery, or why I was ignoring symptoms. If you are a friend or loved one of another Zebra, please understand that this is not a sign of someone not helping them selves. Be there for them, talk to them and most importantly listen. Support could make all the difference in weather someone feels strong enough to face another battle – we can’t always do this alone. For any stripy people out there, you are not alone – we may not always be vocal about it, but you are certainly not on your own. Fear is a nasty business, and I hope that one day this situation changes. In the mean time I will be continuing my push for help, and perhaps one day, going to the GP will feel just like it should for anyone.

I’ve been believed! It only took a year…

Last week I had an appointment that has completely thrown me off my balance. Not because it was a bad one, and not because the doctor was bad – it has thrown me off precisely because he is the exact opposite. I have no idea how to react to it.

Since having my upright MRI a year ago, I have come up against the most horrific consultants within my local hospital. I have spent a year being told that my scans show absolutely nothing, that I have no neurological symptoms whatsoever (tho had he of carried out an exam, he would have discovered many) and that I have no idea what I’m talking about. They have been quick to judge me as stupid and gullible, and even quicker to suggest that everything is mental health related. This toxic way of being has leaked out into other areas, and it has gotten to the point where I have repeatedly chosen to avoid appointments rather then put myself through another round of humiliation.

Worse still, without the backing of an NHS doctor to confirm my conditions, I have come up against family and friends who refuse to believe its possible for this type of treatment to take place. I have been constantly questioned about how I know the radiologist or surgeon in Barcelona are legitimate. “How do you know they aren’t just trying to make money from you?”, “How do you know you really have these problems?”, “How do you know you’re really going to get worse?”, and “I can’t believe that there’s no one in the UK doing this surgery, you just don’t know what your talking about” have all been repeatedly thrown at me during conversations like some sort of twisted broken record.

There is nothing worse than having to prove that you are ill when you feel this horrific every day.

However, last week everything changed, and I won’t lie – I have no idea how to respond to it. I have spent so long fighting to be listened to and believed that I never considered how it would feel when that happened.

I had an appointment with a neurosurgeon down at Kings College Hospital last week. Its an appointment I had repeatedly rearranged because I couldn’t cope with another dismissal, but come the 3rd appointment I really couldn’t get out of going any longer. I reluctantly packed an over night bag, and took my PA down to my parents for the night. The feeling of dread I had was worse than it has been for any exam.

We arrived at my appointment, and as I sat in the waiting room of the neurology clinic I really was questioning how I had gotten there. How was I sat in a room full of such obviously ill patients? How was I sitting there and recognising the types of movements, twitches and weakness as things that my body has also started doing? Is that really the way that these neurological issues progress? If so I don’t want to stick around for the ending.

We eventually got called in and to my surprise the consultant was not only happy that I had had an upright MRI, but also agreed with everything on my report. He went through it pointing things out, and after doing a proper neurological exam, he also found that I have hemiparesis on my right side, sensation changes, and balance problems – just as the consultant in Barcelona told me. He also checked my eyes, and the sight in my right eye is significantly worse then my left – as this is on the same side as the weakness in my body, he rang me to ask if I could talk to my GP about having it evaluated by ophthalmology at my local hospital as a ‘base line’ to keep track of future changes.

He took time to explain what the normal procedure would be for someone without EDS – usually this would be treated with surgery that removes a chunk of the skull to allow extra room. He also told me that due to my EDS and instability surgery would be much more complicated. For this reason he is referring me to the skull clinic at Kings, and also for the skull clinic MDT meeting to get ‘as many heads on this as possible’.

I am utterly shocked that all it has taken is a 2 hour train journey for my scans to suddenly show just what I already knew – that this is real. It seems like some sort of sick magic trick – now you see it, now you don’t.

Along with the shock came the initial relief. I hadn’t had to argue, I hadn’t had to prove myself. I was relieved that I was believed, and for a few days those feelings stuck, but with being believed comes the knowledge that I really do have something serious going on. These conditions are not something that I will just get over, nor will they go away without treatment. I will likely continue to worsen over time, and the end result of that is a very scary place indeed. Considering how much worse I am now then just a couple of years ago, its not exactly a slow process.

So now from being believed comes a ton of emotions that I really didn’t bargain for. I spent so long convincing myself that maybe I was wrong, and so much time telling myself it wasn’t really that serious. I have spent a year trying to tell myself it hasn’t existed, so now I am hit with all of the scary emotions I should have been able to have when I found out what my scans showed in the first place. I should have been allowed to be scared, upset and angry a year ago, but instead I had to fight continuously to be believed. I wouldn’t wish this treatment on my worst enemy.

I am happy that its now being counted as an actual medical condition on my records, and happy that I have at least 1 doctor on my side, but the reality is still that they are unlikely to be able to treat me surgically in this country. We still have no one that has done these operations on EDS patients in the UK, so I can’t help feeling like no matter how understanding this consultant is, I am still just as stuck as I have been.

The only way out would be too fundraise to go abroad, but I cannot face the idea of becoming a sob-story or being known only for my illnesses. I can’t face the idea of begging people to help me when there are so many others who are equally needy but who have families or partners that need them to be around. I really don’t know whether to laugh or cry. How on earth did my body so spectacularly go wrong?!


Being the EDS patient

If someone were to ask me about the hardest part of living with EDS, I can answer the question without even thinking. It isn’t, as you may expect, living with joints that pop in an out multiple times a day. It isn’t the fatigue, the constant and unending fight you need just to get through the day. It isn’t even living with chronic pain. Hands down I can truly say that the absolute worst thing about living with EDS is the sheer level of neglect you experience from 99% of the doctors you see.

It is exhausting beyond belief to continually need to advocate just to get basic care, referrals and medication. I thought that things felt bad enough before being diagnosed with Chiari and AAI, but really that was easy in comparison to getting the right help now.

This week I discovered that the 4 referrals my GP was meant to do have not been done. This in itself would be far less frustrating if she were honest and upfront about not doing them, but instead I have spent the last few months trying to find out when the referrals would arrive. Each appointment she looked me straight in the eye and told me they had been done. Finally a couple of days back, I asked my carer to find out from the reception staff where the referrals were up to. It turns out she hasn’t made any of them.

I could understand it if it were for less serious issues – maybe I could get my head around it if it were to a dietician or physio even. Yet one of these was for an MRI of my spine after my leg went numb for 6 weeks (and still has altered sensation and weakness over 2 months on). Another was to see a neurologist after my dizziness and issues with, balance and weakness have gotten worse. Both of these were referrals she told me I needed herself as she isn’t comfortable treating the symptoms without the referrals being made.

Another was for breast screening which I should now be having on a yearly basis. This should have been made way back in September, and it really is the one referral I can fathom a refusal too. Surely if they know the risk of cancer is high, and I have tested positive for the gene mutation, there is absolutely no reason for it to be refused.

I am so frustrated with it all. I really did think that with so many serious issues beginning to happen, that maybe the care would be slightly more forthcoming, but instead it is worse than ever. I spend my appointments being talked down to and treated like some sort of hypochondriac or gullible young girl. The entire thing feels like some sort of sexist power play. It is patronising, dehumanising and quite frankly it is simply wrong. I can’t help feeling that if I was male, 5 years older and had a beard, I would be treated very differently indeed. Maybe then doctors would do what they promise, talk to me directly and be straight up and honest about their opinions.

So what effects does this have on a person? Sadly the effects go far beyond what you may first imagine.

Having had years of my symptoms being ignored or belittled, I have for the most part, given up on seeking medical care. I only go to the GP when something major happens, and not once did it cross my mind to ‘bother’ the doctors in an emergency room after the feeling in my leg disappeared. I didn’t see my GP for 3 days until I was absolutely sure that it wasn’t just something that had just popped out of place or something that would sort out on its own. I refused to go because I was scared. I was petrified of being laughed out of the waiting room, or that someone would try and suggest that it’s all psychosomatic, because most of the time they are exactly the reactions that we get. This continual drip feeding of negativity is unbelievably damaging, and if you don’t struggle with confidence, depression and anxiety before hand, you certainly will by the end of it.

You are left constantly second guessing yourself. I must analyse myself better then any doctor ever could every time I consider asking for help. I spend so much time hearing that my symptoms cant be real, that I have a genuine fear that maybe they are right. Maybe I’m just too lazy to walk without walking aids, maybe I secretly just don’t want to get better. Perhaps my brain trying to escape my head is simply some sick fantasy I’ve dreamt up out of a need for attention. Even though I have symptoms, test results and scans coming out of my ears, I now question myself every single time I need to ask for help.

EDS already leaves you unable to have the life you should be having, but on top of that comes the alienation that seeps out into your personal life as a direct result of lacking supportive medical care. The more I struggled to get treatment or help, the more family failed to believe me when I said that something was wrong. Everyone trusts a doctor will be right, so what hope do you have of convincing those around you that you are genuinely ill when your doctor prefers to chalk everything up to feeling a bit down? I spent years having to fight for care by myself due to incorrect beliefs that it was all ‘in my head’, and this was painfully lonely. I would give anything to be able to be genuine and honest about how I am feeling, but I have now learned it is much safer to keep my medical issues to myself rather then risk dismissal from those that should be some of my biggest support.

With the Chiari and AAI, the effects are amplified exponentially. Your symptoms are rare,  unexpected and odd. They are not what a typical doctor comes across. Not only that, but in the UK no one has an interest in fitting the puzzle together for EDS patients. Instead you are left to flounder alone. There is no expert to ask, there is no guarantee that anyone you see will treat you fairly or with dignity.

The panic I now get before appointments is not unlike the panic I experience from PTSD, because I know that if I see the wrong doctor, or see the right doctor but on a bad day, I could potentially land myself with a diagnosis of mental illness rather then any help for the symptoms I so desperately need relief for. I can not explain to you just how exhausting, tiring and frustrating it is to know that there is treatment out there that’s successful in not only halting the progression of symptoms, but very often in reversing a lot of them. It is out there, but unless you are lucky enough to be born in the right country, you need to somehow find an unbelievable amount of cash in order to access it. Not only that, but this country is full of doctors who refuse to even accept that these problems a) really exist for EDS patients (claiming that the term hyper-mobility instead of instability should be used in EDS patients purely because we obviously have superpowers that mean damaging our spinal cord or brainstem isn’t in any way dangerous!). or b) take note of numerous case studies and scientific data coming out from specialist neurosurgeons abroad who have been doing these operations with great success for many years.

It is all so unethical and unjust. It is wrong. I am a capable woman who lives with chronic illness. I am not attention seeking, and I am not making everything up for fun. I am simply asking to be treated like any other patient – with fairness, dignity and respect.


The Disabled Student Experience

It has been too long since I last wrote, but I guess everything got on top of me more then I would like to admit.

I will go over my trip to Barcelona in another post, but at the moment it is too much to think about, so instead I’m going to talk about the other changes in my life.

As often happens when I get severely depressed (we are talking 6 weeks of crying/sleeping/insomnia/dissociation/suicidal thoughts and isolation which landed me on a second antidepressant and a very concerned GP) I have put some changes in place. I couldn’t live the way I have been living. Life felt aimless. I couldn’t work, wasn’t at university, and I was filling my time with pointless activities which (although creative) couldn’t give me any sense of achievement or purpose. Life became nothing more then watching my body disintegrate and pretending not to notice whilst being petrified behind closed doors. I couldn’t cope with another year like the last 3, especially with my latest health updates.

So three weeks before term was meant to start I bit the bullet and applied to do a part time second undergraduate degree in psychology. I could have done a conversion course as a masters, but realistically I have no way of knowing if I can cope with studying, and lets face it – the longer I can stretch out my education, the less time I will have feeling like I have nothing to aim for.

So far it has been tough. I didn’t realise how much my body had deconditioned – 3 or so years with little activity, sitting on the sofa really has lost me a lot of strength and my fatigue is much worse then it used to be. I am also struggling a lot with pain levels, and thanks to my somewhat wobbly head, I am getting migraines flaring for the few days post uni which is leaving little time to study. Socially it has been terrifying (I am not good at people, let alone groups of them whilst I’m sat in a wheelchair looking so obviously different), but other then the odd issue, so far that has actually been a huge positive. Its the first time I’ve really met new people in years, and thankfully a lot of them are just as quirky and chatty as I am. I am struggling a lot with my PTSD flairing up. Dissociation has become somewhat of a norm again, but the university is supportive, and no one is batting an eyelid at the silly putty coming out in lectures. All in all, as hard as Im finding it, I am also relieved that I can hold my head up high (not literally of course) and say that I am doing something with my life.

Leaving music behind has been surprisingly difficult. I didn’t stop playing because I wanted to. I stopped playing because my joints were causing me too much pain, my fatigue was unmanageable and I was in no fit state to force my body to carry on. I think its the lack of choice that makes it so upsetting. Music up until now has been my life. Since the age of 3 I played almost every day right up until my late 20’s, and without it I am not really sure who I will become. Maybe one day, if my body miraculously gets more manageable I can come back to it, but until then, it seems that life has taken me off on another path.

So – lets talk about life as a disabled student.

Turning up to university is scary – but add that to turning up on wheels, and we are talking some major anxiety. What if people are nasty? What if people think I’m attention seeking? What if nobody talks to you? I had all of these what ifs and more, and fully freaked out for the entire time before I started. On the first couple of days it was awkward. I took my PA along to support me, and yes people didn’t really approach me to start up conversations. I found that I had to initiate conversation, but once I did and people started to get to know me, all of the what ifs have turned out to be false.

Universities are incredibly supportive most of the time. So far I have had excellent support from student services, and a personal tutor who goes above and beyond intruding to get everything sorted and comfortable. All of the tutors I have come into contact with have been brilliant, and no one has made me feel out of place. I know that I will have periods of time where I struggle more, but I feel like the university are prepared to help me with that, and I know that should I need to take extra time or time out from the course, the support is there to do this. This is where university is so different from work. I know that I can pace my learning according to my needs. I can stretch it out, take weeks, months or even a year off and still come back to it when I am well enough to do so. In work I would loose my job long before I got to that point as I am simply not able to be reliable enough. This is a middle ground between being off sick and working. Doing part time study is pushing me to the limits of what I can physically manage, but there is so much flexibility that the only pressure I feel is from me.

Mobility wise I knew that if I wanted to be able to study, something had to give. I had a manual wheelchair from wheelchair services, and my cardiologist was fully encouraging me to use it as part of pacing myself properly. I knew that to get around campus on my feet would have meant I’d have dropped out (or onto the floor) within a week or two of starting, so a wheelchair was the only option. This has been a hard pill to swallow. I have been so determined to resist looking like a disabled person (ironic considering I have gotten used to walking around with a rollater called Dorris). I didn’t want my disability/illness to be the first think people notice about me, but now it is unavoidable. The benefits tho far outweigh the loss of pride, and I know that being able to come in on my dizziest day or a day when my leg is completely numb is worth much more then my somewhat peculiar vanity.

This has been one of the biggest challenges I have done. Reading with a combination of dyslexia, brain fog, dissociation and vision changes certainly make for an interesting afternoon, but I am loving every moment of it. I feel lucky to have been able to give it a go, and relieved that I have a reason to get up in the morning.

Changes are always scary, and especially so for those of us with chronic illnesses, but I am a firm believer that it is worth every moment of fear to be able to try something you had thought was impossible to achieve.

I will update you on the health side of things as soon as I have gotten my head together again!

Taking the Next Step

Finding out about my instability and chiari malformation has been quite an emotional rollercoaster. I feel so many different emotions day to day that I really can’t begin to pinpoint where my head is. I cannot match up my body to the body that has an extremely serious condition, nor can I link my symptoms up to the term ‘brainstem compression’. I know full well that I am gradually getting worse, but my head can’t accept that my condition is so serious.

I have mentioned the headaches that prompted me to get the scan, but what I haven’t mentioned are the numerous other symptoms that I live with which could be caused by these conditions. My balance is bad, and over the last few years it has gotten much worse. I often feel like everything is moving around me, and quite regularly fall over just because I shut my eyes whilst standing up! I am constantly dizzy, but this is compounded by worsening coordination and clumsiness. My swallowing difficulties have deteriorated. What first started as the odd sensation of things going down too slowly has led onto constant slow motility, being unable to clear my throat when I swallow, regular choking due to inhaling bits of my dinner and regularly attempting to swallow only to realise that nothing is happening in my throat at all – this is especially unpleasant with a mouth full of tablets that you can’t get down!!! I vomit far too often, and my nausea now requires anti sickness tablets 3 times a day to try to control it. I regularly get pins and needles in my limbs, and at random points in the rest of my body (most recently I had 3 weeks of pins and needles on my chin and at a point on my back). This can be short lived, but can also go on for weeks at a time. I also experience periods of time when I get numbness and decreased sensation in my limbs and face. Looking back, this started way back in music college, and even during my final recital, I had no feeling in my hands at all! You get so many odd sensations – burning and tingling amongst other things happen almost daily. My breathing is far from perfect – I often wake myself up gasping for air as I feel like I forgot to breath when I was falling asleep. I am breathless most days, even when Im doing nothing more then sitting on the sofa. My memory is much worse then it used to be, and I find myself loosing words and ideas more and more often. My speech feels clumsy, and tho talking has always been my strongest attribute, I am conscious that I am often mispronouncing words and tripping over syllables I would never have had an issue with a few years back.

All in all, there are a lot of symptoms that I manage day to day, and logically I know that this means Chiari and instability are having a far bigger effect then I care to admit. I also know that I am getting worse over time, and it  is a little scary to think that this deterioration may not stop.

Following on from my last post, I decided to be proactive in attempting to get views on my scan result. Things that go through the NHS are typically snail paced, and often not particularly helpful for those of us with rarer conditions. There are 2 countries with specialists who will work with EDS patients with these comorbidities: Spain and the USA. With this in mind, I made contact with the consultant in spain (largely due to him being within closer range then the USA).

I sent across my scan results, and filled in about 5 extremely detailed questionnaires. The secretary was brilliant, and explained the process and costs very clearly. Within 3 weeks, I had an email into my account with a very detailed report and suggestions for next steps.

He has identified chiari and Atlanto Axial Instability, but has also stated I meet the criteria for Crania Cervical Instability (Instability between the skull and first vertebrae), and suspects that the instability further down my neck goes right down from skull to C6-7 rather then just instability at C3-4. He said that I Am showing a lot of signs of brain stem compression (based on the questionnaires answered), and would also want to complete further testing to check for tethered chord syndrome. His advice as far as treatment goes would be to fuse from my skull to T1 (my entire neck) and decompression surgery to treat the chiari. He said I will likely continue to deteriorate if left untreated which as much as I don’t want to believe it, seems reasonable based on what he’s picked up on.

The next step from here for me is to head over for a consultation. I could wait and seek other views, but I would rather do the tests he requires and get a more thorough view of what treatment should be for me, and then take all of that information to another specialist to look at. At least it would give me a real starting point rather then things being based on guess work.

So, on the 19th of this month, I am booked in for cineradiology, urodynamic testing and a consultation. He also wants to see a thoracolumber MRI, but as I *should* be able to get this done on the NHS, I will hold off paying for one privately for now. As much as I am nervous about all of the above, it will be good to be able to actually talk to a specialist face to face about what my body is doing, and which options are available to me. I should be able to get an idea of how quickly I may deteriorate, and also be able to discuss through when the right time to think about surgery is. I have no idea if I will be ok to hold off for a few years, or if this would need to be done as soon as I could.

The scary thing in this situation (other then my body’s rather spectacular inability to fulfil its purpose in life) is the prospect of paying for such a complex procedure. Although I won’t have a full idea of cost until seeing him (and until all tests are complete), I know that people seem to be needing to cover anywhere from £60,000 upward. This figure, bearing in mind I am subsisting on disability and sickness benefits , is so outside of my comprehension that I can’t even begin to get my head around it. Obviously I don’t have it hanging around in my bank account, and I don’t have any rich friends who could offer to cover it for christmas, so it looks like I will have to do the one thing I NEVER wanted to do: fundraise.

My head despises the idea that I will need to show the world an ill person. I don’t do sob stories, and I especially don’t like people feeling sorry for me. This blog took so much for me to start writing, and I do it with the view that more information needs to be out there for doctors, friends and families to read. I don’t cover up the embarrassing bits, and I’m not hiding the darker thoughts, but this is with the hope that at least one doctor may be able to read this and get a better understanding of the next stripy patient who walks through his door. The idea of begging people to help me raise money to pay for my own surgery makes me extremely uncomfortable… I may be short and sarcastic, but I certainly don’t feel amazing enough to expect that sort of cash from people I don’t know! If only there was a rich fairy godmother just waiting to make her entrance into the worlds most boring fairytale right about now!

For now tho, I am trying to ignore the implications of the meeting with the consultant, and just focus on finding the energy to get there in the first place. Its been a mighty long time since I’ve really even left my house for anything over a few hours, let alone travelled on a plane to a foreign country, and be up and ready for tests to start at 8.45 the very next day (help)! On the recommendations of many that have been there before me, I have booked in to a place set up to cater specifically to the needs of the not so able (or special as I like to think of myself). Everything in the apartments will be accessible, there will be rails and a shower chair in the bathroom, the beds are electrical (which thankfully means I don’t need to panic at having enough pillows for my nightly game of pillow jenga), and there is a hydrotherapy pool available for use which will massively help with pain. Best of all, they will provide me with a free power chair for the duration of my stay which means I will be able to go out and be a tourist without needing to crawl everywhere on my hands and knees – I’d say thats a win! I have booked in a couple of extra nights so that I can cope with the travel, and also make the trip more then just a hospital visit. You have to make the most of it somehow or other!

No doubt I will report back at some time in the near future with yet more stripy ramblings. Maybe I’ll even treat you to a picture or two from Barcelona if your lucky!