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.

My Brain’s Falling Out and My Head’s Falling Off: Chiari, Instability and a Whole Host Of Swearwords.

My Brain’s Falling Out and My Head’s Falling Off: Chiari, Instability and a Whole Host Of Swearwords.

This particular post is one that I had hoped I would never need to write. EDS up till now has been frustrating, painful, intrusive and unpredictable, but now it is darn right scary.

As EDS leads us to produce faulty collagen, its effects can become apparent anywhere that collagen is involved. Joints, skin and organs are all affected, with further effects often reaching to hormonal systems, the autonomic nervous system and pretty much anywhere else you can think of. For many people with EDS these effects will be difficult to live with, tho it tends to be something you can adapt around – usually it is life altering, but not life threatening.

However for a relatively small percentage of us things can get a little more precarious when the brain, neck and/or spine decide to join in the party.

This particular tale for me started in mid January (barely 3 weeks after my thyroid operation). I have been experiencing headaches pretty much since I can remember. I grew up with daily tension headaches (not helped by a jaw that loves to snap, crackle and pop), and from the age of 12 I have experienced ongoing neck and back pain. Having been brought up to resemble a musical robot, I had always attributed this issues to spending hours a day in front of a music stand rather then anything linked to EDS.

Over the years my headaches have changed. I developed migraines, headaches when I cough, laugh or strain, and usually feel like my head feels too heavy for me to hold up by the time Ive been up for more then half an hour or so. My neck over recent years has also started to feel noticeably more unstable – along with the usual subluxes I am accustomed too, I do get the occasional clunk that leads to extreme burning pain radiating  up and down my head and back which lasts for days at a time. In general the change has been so gradual that I rarely stop to think about it. I am used to the headaches not going away and the need to rest my head. I’m used to fending off migraines on a weekly basis, and the pulsating pain felt when I cough, bend over or laugh. All in all, I have always just assumed its nothing more then an annoying symptom of EDS rather then anything to be hugely concerned about.

Having mentioned these issues in passing (largely focused on the migraines which are extremely disruptive), my GP decided to refer me to a Neurologist for a formal diagnosis. This finally came through for January, and as I wasn’t feeling particularly worried about anything, I attended the appointment alone. As expected, he diagnosed me with migraines (no surprises there, tho its taken years for any GP to acknowledge them), and when talking about the headaches relating to straining he very cheerfully dropped into the conversation “Oh, and you probably have Chiari Malformation which is where your brain is herniating into your spinal column – its really common in EDS, but just forget you have have it!”

Now Im pretty sure you have all heard the example ‘Don’t think of the pink elephant’. If you don’t want me to be thinking of the pink elephant, then maybe don’t tell me not to be thinking about the elephant – or better still maybe suspect that the words brain, herniating and spinal column may come across as just a little bit terrifying.

This is not the first time I have heard of Chiari Malformation in EDS. A couple of years back, a very dear friend of mine ended up extremely unwell with Chiari and Cranial Cervical Instability that led her to need to fundraise an insane amount of money to cover the costs of the surgery that saved her life (no surgeons in the UK will treat these issues in EDS patients). Granted, she was on the extreme end of the scale – but she had also been told my both neurosurgeons and neurologists here in the UK to “just ignore it” and forget about it – so this line of thinking did not sit overly well with me.

In reality (although the NHS will argue there is no difference) many cases of Chiari Malformation and instability are missed when taken in a recumbent MRI. It seems logical to me that gravity plays a part – especially when you take super stretchy collagen into account. Many EDS patients have been fobbed off with a normal looking MRI only to finally get an upright MRI which suddenly shows up a whole host of fairly serious issues. As the neurologist wouldn’t even refer me for an MRI of my spine (brain and spine MRI requested by a consultant at the RNOH) I am pretty sure an upright scan would not have been on the table without an extremely long battle. So I walked away from the appointment knowing full well that the MRI I was being given was close to useless, and feeling utterly lost.

I feel so very lucky that I had my friend to call that day as I genuinely don’t know how I would have coped with that news with nobody else to ask. She allowed me to have a freak out, and then helped me get together a plan that seems much more proactive then just pretending its not there. On her advice, I booked in privately to Medserena in London for an incredibly thorough upright MRI. I was hoping that the MRI would prove that I didn’t have any problems – tho on some level I knew that this was unlikely.

The scan itself was tolerable – especially as the private sector seems to arrange for pots of tea and snacks to be brought to you before and after your scan! They were great at explaining exactly what each part of the scan was for, and they did their best to put me at ease and keep me comfortable. The scan lasted for almost 2 hours, and within this time we took scans with my head in a neutral position, in flexion, extension and turning left and right. As its extremely challenging staying still (even tho each part is broken up into 2-6 minute sections) they use props and clamps to try to keep you as still as possible. It wasn’t a comfortable experience, but it could have been a lot worse. I returned home that evening with a pretty horrific migraine, and unfortunately the scan does seem to have given me a flare up in migraines and headaches. I don’t work well with my head being manipulated, nor with staying at the extreme ends of motion for any length of time. So far Im on migraine 3 in 5 days, but I trust it will settle again soon.

The report came through the day after my scan, which is just as well as I was climbing the walls with nerves. On the one hand, a diagnosis like this is terrifying, yet on the other it would give an explanation (and the potential for treatment) for so many symptoms that I experience. Just like so many parts of EDS, each new diagnosis is a double sided coin.

Reading through the report I felt like my world was falling apart. It shows not just a 10mm chiari malformation (herniation of the cerebella tonsils), but Atlanto Axial Instability and C3-4 pushing out of place and into my spinal column every time I bend my head forward. AAI is instability in your top 2 vertebrae, and mine showed that both facets sublux when I turn my head in one direction, and one side fully dislocates when I turn my head in the other. To top it all of my degree of hyper mobility in my cervical spine is also pretty darn impressive, but this in combination with the above leads to ‘significant instability’ – words that you don’t really want written about one of your most important supporting structures in your body!

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My Chiari Malformation

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Scans showing my neck in neutral, flexion and extension

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Misbehaving C3-4

It is hard to wrap my head around the information I have been given (pun intended), but more so because there are no specialists in the UK that I can go to for advice. Although both chiari and AAI are regularly treated in those without EDS (with the acknowledgement that these conditions cause symptoms for the groups that are being treated), there is a distinct lack of understanding, acknowledgement and knowledge when it comes to treating (or even consulting with) EDS patients. Just like the advice I was given, most people are told to forget about it, that it wouldn’t possibly cause any of their symptoms and that there is no evidence that treatment would be beneficial – ignoring the research carried out by world experts, and contradicting their reasoning for operating on non EDS patients. In reality, it is likely a lack of funding. The surgical technics required for treating EDS patients with these conditions differ from the norm, and so the NHS would need to plough a fair amount of money into new training. For such a small proportion of patients, there is little incentive to back the idea, and so it is far easier to claim ignorance then acknowledge  a lack of funding and knowledge.

Unfortunately this leaves me with few options for advice or treatment. Those that deal with EDS patients are based in either Barcelona or the US, and although I could fight to see different people in the NHS, the results would almost certainly be the same. I want advice on how to manage these conditions – advice on activities that should be avoided, or ways to help myself. I also want to know exactly how serious my scan results are, and whether surgery will be needed in the future in order to maintain some quality of life. The last thing I want to do is ignore its presence, and risk deteriorating to the point of paralysis or even death. Not every patient will deteriorate to such extremes, but its a possibility, and its a possibility that I would rather try to avoid. With all of this in mind, and through an amazing bunch of fellow sufferers, I have begun to make contact with some surgeons abroad. I am beginning the process of sending in my scan pictures for them to look out, and will hopefully be able to get some views from people who know what they’re looking at. If they say that surgery won’t be necessary, then I’ll be jumping from the roof tops, but if it is necessary, then I need to know sooner rather then later as I would need to find a way to self fund. At the very least, it will give me a chance to ask questions about how I can best look after myself from here on in, and what I need to keep an eye on symptom wise.

All in all things are looking pretty scary at the moment, and normal life seems to be slipping further and further away. I really did think that once I had attended the rehab course at Stanmore I’d be able to get well enough to study through hard work and determination, but now I’m not so sure. No matter how much physio and pacing I put in, I won’t be able to fix my brain or my neck. There will be no guarantee that things will stay as they are, and so how can I be sure that I would get through the year without things going disastrously wrong? Thats without taking into account the fact that I have far to many symptoms at my current level to even consider it as it is.

Each time I convince myself that theres a way to sort things out, the unpredictable and frankly horrific nature of EDS rears its ugly head to set me strait again.