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.

Part Two of ‘The Not So Sexy Side’…

Part Two of ‘The Not So Sexy Side’…

As you may remember from one of my older posts, EDS causes problems only a woman can get. Pelvic organ prolapse is usually something only reserved for those lucky women who are older and/or have had kids, yet with EDS we often get this thrown into our 20’s free of charge!
Pelvic organ prolapse referes to a prolapse within the pelvic region. A prolapse means that the muscles and ligaments (in this case within the pelvis) have stopped being able to support the organs sufficiently, and this leads to hernias. In pelvic organ prolapse this can be concerning the bladder (a cystocele), the rectum (the rectocele), the small bowl (an entrocele), the uterus (utrine prolapse) and a vaginal vault prolapse (a prolapse following a hysterectomy). With pelvic organ prolapse these organs bulge into the vagina causing an array of fairly uncomfortable symptoms.


Having finally been diagnosed with a prolapse after a 2 year fight with medical professionals, I was eventually referred to a local gynaecologist to get things sorted. Being of typically English sensibilities, it was not exactly the least embarrassing appointment I’ve ever attended!

I spent the few days before my appointment in obligatory panic mode. What if I was brushed off again? What if it wasn’t a real illness and I had made everything up? What if I would be laughed out of the clinic in shame?! I am aware non of this was a logical thought process seeing as a professional had already diagnosed me, but with EDS a diagnoses is often brushed aside when it seems inconvenient for the next professional to take into account. I am so used to having to fight my corner that I get huge anxiety before each and every appointment. With this appointment, I thankfully needn’t have worried.


As soon as I stepped into the room, the doctor was friendly. The more nervous I am, the more I spout random nonsense, and what with this particular appointment concerning my lady bits, there was a lot of nonsense being spoken. Thankfully, she was more then happy to join in with my nervous chatter.

She started off with some questions concerning my symptoms. We spoke about pain and a downward heaviness down below, issues with incontinence, issues with intercourse etc. She kept the questions going, so there were no awkward silences in which I could get painfully embarrassed.


Next came an internal exam – I was expecting the cystocele, but it did come as a bit of a shock when she also diagnosed a utrine prolapse and rectocele. It seems the party for one just turned into a party for three down there.

My options for treatment at this stage are limited. I will eventually require surgery as these issues don’t fix themselves, but as I am still to have children, and own particularly crappy collagen, they would rather try ‘conservative measures’ first. These consist of specialist physio therapy focusing on training up my rather lax pelvic floor, and the use of a pessary – a silicone device inserted into the vagina that helps to hold everything back in place.

A selection of different pessaries


The physio doesn’t sound like a whole lot of fun – obviously to retrain your pelvic floor, it requires feedback, and from what I can gather this will be via another person and via specialist equipment. I am not by nature prone to flashing my bits to random strangers in hospital settings, and this will be no less embarrassing then turning up to my own wedding naked. I know that. The physio will largely be aimed at trying to halt the progression rather then curing it, and that is a thought thats hard to swallow.

The pessary is probably the thing that I am struggling most with. There are many different options from your doctor to choose from, each supporting different areas and providing different levels of support. Some will support just the uterus, the bladder, the rectum – some will support more then 1 area. There are things like a ring pessary for those that don’t require as much support, and then there are ‘space filling’ pessaries for those that require a little more help. I am unfortunate enough to end up with the latter. I have been given a cube pessary. This is basically a silicone cube which has indents in that form suction to support the walls of your vagina, complete with a rather undignified silicone cord – just incase you manage to loose where it is up there! For the cube pessary (all pessaries require different care), it needs to be removed every night, washed, and left out until the morning, when you have the really fun job of trying to stuff it back up there whilst trying not to swear – really not an easy task, especially when you fling it half way across the room and have to race your way there before the dog thinks he has a new chew toy (yes, that really happened)!

It is not so much the rigmarole of using the pessary that is the problem – its knowing that I need to use it. I somehow feel less attractive. As it can’t be left in during intercourse, I am dreading the day I will need to push a hand away and shimmy off to the bathroom to get the thing out before things can get started – spontaneity in the bedroom is truly a thing of the past. I feel embarrassed knowing that its now a part of my personal life, and worried about how people will react. As a young woman of 29, it feels unfair that my body has given out now – it is rare for women that haven’t had kids and especially rare for  young woman to suffer with pelvic organ prolapse, but thanks to EDS it here, and its here to stay.  I can cope with so many of the joys that EDS brings to the table – the pain, the subluxes and dislocations, the difficulty swallowing and digesting food, the fatigue – but for it to affect my lady bits just seems like a massive step too far.

As if I wasn’t a crap enough catch before, yet more super attractive health issues are handed to me on a plater!

Tomorrow morning I have a super early appointment to go back to the gynaecologist. Pessary fitting is a bit hit and miss – its basically trial and error to find a shape and size that works for you. Unfortunately mine is shifting down throughout the day and pressing into bits that really don’t want to be pressed. With the right size and shape, in theory it shouldn’t move about, expel from the body or even be noticeable to the wearer – lets hope I find the right one soon! The doctor very kindly offered to see me after being on a night shift to try another size or shape, so I somehow need to make sure that I am up bright and early to duck in for a repeat embarrassing experience.

EDS is one pretty tricky bedfellow to manage these days.

Being Dismissed After Diagnosis

One of the most frustrating things about Ehlers Danlos Syndrome is the utter ignorance held by most medical professionals I have come across. The two areas who seem to dismiss it most are rheumatologists and physios. Both of whom I have come up against in the last few weeks, and both of whom I have been very close to wanting to hit!

I was diagnosed with EDS years ago. You would think that the label would suffice, and that treatment could then be forthcoming – this would be the logical route for things to take. The reality though is very very different.

Every time I see someone new, I can almost guarantee the comments and ideas that will get thrown about. First off, it is never taken as read. Every appointment I have with a new person leads to a completely fresh assessment – running through the beighton score, coming out with totally different scores every time. Dismissing the pain, and usually leading to comments about how its ‘just a bit of hyper mobility’ or that hEDS is just ‘Benign Hypermobility Syndrome’. Every time this happens, a little bit more of my sanity is stripped along with it.

When would this ever happen with another illness? Would someone with a broken leg need a new X-ray every time they saw a different doctor just to prove it was broken? Would they be told its just a little sprain when the X-rays came back with proof? Would someone with heart failure really need a new set of tests so that they would be believed? Why is it that EDS is treated so differently?

Another idea that repeatedly comes up is that you aren’t that bad if you don’t score the whole 9 points on the Beighton score system. In reality, if you score over the accepted number, and/or have hyper mobility in other areas then it should still be counted. Just because someones knees may not be hypermobile does not suggest that issues with all other areas, and areas that aren’t included in the scoring system are not causing problems. This woman genuinely had the guts to tell me that my shoulders and hips were not a problem as they aren’t included in the list of hypermobile joints in the test! Apparently the fact that they continuously cause me pain, and sublux constantly are not issues worth considering. She made it clear that she had other patients who scored much higher, and that obviously as I didnt have the whole 9 points, it was ‘benign joint hypermobilty syndrome’. When I explained to her that a) I fit the criteria for hEDS (taking time to explain the other criteria she hadn’t touched upon), and b) benign  joint hyper mobility syndrome is no longer a term being used anymore, she came out with a very sarcastic ‘Well you’ve been reading up then’.

I was also told yet again that my pain was obviously effected by my mental health. In all honesty, with EDS it is more then likely that this is the other way round. Sure, I have had mental health problems in my time, but I can quite safely say that I have the same levels of pain when I am happy as I do if I am depressed. The pain levels don’t change – but my ability to cope with it does. Our pain is not psychological. It is caused my recurrent joint subluxions and dislocations – in no other area would someone with a dislocated shoulder be told that it doesn’t cause pain, so why is it that we are told this all the time? It is not the first appointment that I have been to where I would have gladly offered to dislocate their joints and asked them if it was really pain, or if it was just psychosomatic. By repeatedly insisting that we don’t really have pain, they are belittling the illness and continually knocking our confidence. Just because our joints are capable of moving further then most does not mean our bodies are designed to cope with it – it still damages, and it still hurts just like it would to anyone who isn’t hypermobile. Alas, I am not the incredible stretchy woman, as much I would like to be.

This particular referral was at the request of both my physio and GP. They both thought it would be useful to continue the work we had been doing, so I wasn’t expecting the reaction I had from this woman. She took great care to tell me that I had already had my fair share of appointments, and that this service is for everyone, and not just me. She told me that it wouldn’t really help me, and that they couldn’t see me long term (which I am quite aware of thanks), and really its just about ‘teaching you the skills to manage your condition’. She made me feel like I was some sort of greedy NHS hypochondriac rather then a patient – as if spending my time at a physio is what I really love doing in my spare time. I am fully aware that this is not long term, but I am also under the impression that I would have been refused a referral if it was deemed unnecessary. They don’t hand these appointments out like sweets. You wouldn’t leave someone recovering from a broken leg halfway through their treatment, so I find it quite unnerving that that is precisely what she wants to do here. Just because this is a long term condition really shouldn’t mean that you aren’t allowed to access treatment that will help you manage better in the long run.

The last big thing she said that was actually extremely upsetting was concerning the care that the social services have deemed appropriate for me to have in place. She looked at me like I had 5 heads when I told her about it, and then proceeded to tell me that ‘you shouldn’t be using it for things that you can do yourself like washing and cooking, you should be using it to better your life’. This was about 2 seconds after lecturing me about pacing for fatigue. Does she really think the social services have spare money flying around to give to people who don’t need the help? The hours I have been awarded are for things like personal care, assisting with cooking/cleaning and getting out and about. They were awarded because I can’t manage to do those things an awful lot of the time, and when I do manage them, I don’t manage to do anything else. In short, I need the help with the things I struggle with so that I can ‘go out and better’ my life. It was not an easy decision to reach to allow the assessment to take place, and it isn’t something I am jumping for joy about – as a 28 year old woman, I would rather do everything totally independently, but the reality is that at the moment, I have no energy left to have any sort of life, and that is far worse then accepting I might need a little bit of extra help. I know that I look well, but not everything is skin deep.

Every single time I have an appointment like this, I want to run away and hide for the next 20 years. It makes me question my illness – makes me consider the possibility that I have made everything up, even tho I have enough medical evidence to sink a ship. EDS isn’t all about the outside of your body – pain and hyper mobility are a big part, but I have a list as long as my arm of the internal issues my faulty collagen has caused. I wish that professionals would read the info as much as we have to, or at least listen to us when we have read more about it then they have. Judgement held over a patient for knowing about their health conditions seem laughable – surely an informed patient isn’t a bad thing.


Too Expensive to Treat

This last week has been testing for me.
After finally having a referral sent to the Royal National Orthopaedic Hospital in Stanmore to see their specialist EDS team, I have come up against yet another wall.

Stanmore is one of the top places in the country for patients with Ehlers Danlos Syndrome. They have a team full of specialists who unusually know about, and manage those with EDS. Their books have been closed for years, and finally the doors have been opened for new patients, all of us in dire need of a team that understand and cater to our needs.

Last year, after 6 months of pushing and a formal complaint, I had a referral sent through to Dr Kaz-Kaz at the request of my cardiologist. Unfortunately as my GP surgery had point blank refused to send the referral, by the time it was sent out they too had books too full to take new patients.

I knew that there was a reason behind my referrals not being sent out, but at the time the GP appeared to be doing this out of complacency rather then anything else.

This week I called up Stanmore to find out the status of my referral when they told me it now has to come from a local rheumatologist rather then the surgery – I knew this could be an issue, but wasn’t quite expecting to hear the reasons behind this.

I called up the surgery and explained my situation. The reaction was a flat out no to a local referral, followed swiftly with an angry “Your costing us a lot of money”.

Yes, thats right – never mind the fact that I am a patient with the same rights for help as any other chronically ill person on their books – this entire continual struggle is fuelled by the surgery having me down as either a patient not worthy of help, or a patient who is too costly to bother with.

This type of comment seems totally unacceptable to me. Regardless of the cost behind treatment and referrals, it is not a comment to fling at a patient who is sick. It is not a comment I have ever heard those with other chronic illnesses mentioning – in fact I know several severely disabled people who have fantastic support from their GPs who put in the care that patient needs regardless – because they NEED it. Yet with in the EDS community it seems this is not an uncommon thing.

This illness that effects our every moment has a hugely detrimental effect on us. It steals away our ability to move about safely, it causes great pain and often leads to a point where we are physically unable to work. It steals our ability to eat normally, to sleep normally, to function socially or function at all. It causes heart problems, urinary problems, swallowing problems and severe fatigue. It causes so many issues that we become depressed and anxious. It can effect our lives so much that we literally can not function, and without treatment these effects are life long.

We may not be dying, but living with EDS that is not being managed intrudes our lives just as much as it may with a terminal illness. Every shred of our being is slowly stripped away – and all of this without the support of the medical profession.

Why is it that this is OK? Why are we the illness that everybody ignores and disbelieves? Money should not come into this when a persons life could be totally turned around with the right input – and with most other chronic illnesses this would indeed be the case.

I am exhausted of fighting the system. I have bitten the bullet in the past and gone privately for help, but unfortunately living on benefits doesn’t leave you with a lot to spare, and Stanmore won’t accept a private referral for their services anyhow. I do not need this continual fight on my hands when I am already sick, in pain and exhausted, yet I have no option but to carry on pushing hard if I want this referral badly enough, and I do. I want the chance to function again, to live again and to work again. I want the opportunity to learn how to self manage effectively and understand how to cope with my illness. I want a life.

This is all very difficult to remember when you are continually written off by those that should be supporting your needs. It is a very damaging way to treat a patient, and really does beg the question; Why am I so unworthy?
I feel lucky that I am mentally stronger then ever before. I can just about cope with this – it is healthily getting turned into anger rather then self hatred, but 2 years ago this would have had me on my knees and added fuel to the fire of depression, anxiety and PTSD. It scares me that there are so many other out there dealing with the same comments who may not cope with them so well.
It is not acceptable.

Another Day, Another Diagnosis

Yesterday I took yet another long journey down to London for another hospital test. This time around, I had the joys of a gastroscopy, which too be honest is not the nicest thing I have ever experienced – 6 hours nil by mouth may be tolerable if you live right by the hospital, but doing this along side a 2.5 hour journey was particularly unpleasant – I never realised how much I love drinking water!

I elected to have sedation as I am not a fan of feeling like I can’t swallow or breath, and judging by the bits I remember (namely choking), I’m pleased I did! They also took a couple of biopsies whilst down there, and again, the idea of knowing someone is taking a small chunk of my digestive tract isn’t something I want to be aware of while I’m unable to run away.

Sitting on the ward with a cannula in my arm felt odd. I don’t often consider myself to be ‘sick’, but each time I’m faced with a hospital test, it suddenly becomes real. I become painfully aware that these tests aren’t given to healthy people, and the number of tests I am in the process of having are certainly above the average for a healthy person of my age. This last year I have had far to many tests. Scans, an MRI, an echo, cardiac stress test, lung function test, 7 day halter monitor, numerous ECGs, tilt table test, a sleep study, 2 weeks wearing an actigraphy monitor, many hospital appointments, too many blood tests to count, and now comes the barrage of tests for my digestive tract and bladder. EDS really doesn’t let up. The worst thing is that I am fully aware that these are by no means the only tests that will come up in the near future as there are still other referrals to be sorted.

With every test comes the chance of yet another diagnosis, and this gastroscopy was not to disappoint. I have had digestive issues for so long that I forget it isn’t normal – recurrent issues with acid reflux, dysphagia, bringing up food (either undigested or partially digested), feeling sick every time I eat, bloating, pain, food intolerances, IBS type symptoms and only managing small amounts of food in one go before feeling like I’m going to throw up. I spent years trying to get help for it, but have repeatedly been told that its ‘probably just IBS’, or other equally unhelpful comments.

It wasn’t until I paid for that private appointment with a POTS specialist that these issues were taken seriously, and I after a long fight with my GP, I finally got a referral to a gastrologist.

With Ehlers Danlos Syndrome and POTS, gastric issues are common. A body thats too stretchy often leads to complications, and POTS is no better. Common issues include dysmotility such as gastroparesis, GORD, hernias and dysphagia, alongside issues with constipation, diarrhea, pain and cramps. To put it bluntly, its a whole bundle of fun.

I hadn’t really put much thought into weather they would find anything wrong during the gastroscopy – I think things have been belittled so often when I have gone for help that I had thought that there was nothing really wrong. If you spend enough time telling a patient that it must just be because they are tired/stressed/nervous then they start to believe it. I genuinely thought that perhaps everything was in my head rather then a real problem, and that these tests would only highlight this.

So after I came too, I headed into the nurses office who went through the exam. To my surprise I have a fair sized sliding hiatus hernia (and some damage done by acid reflux) to add to the list of weird and wonderful conditions caused by my faulty collagen!

The thing that has thrown me off most about this is that according to the nurse, this doesn’t explain a lot of the issues I have with my stomach, and so chances are there will be another unexciting diagnosis right around the corner. I have a couple more tests for these on the horizon, and it is safe to say I am looking forwards to these like a hole in the head.

Sometimes I think that I don’t really want to know what is going wrong anymore. I have a list of diagnosis fit for any 90 year old, and its scary to think just how many more may be added to the list. Ignorance is bliss as they say.

To add another spanner into the works, my blood test results for my thyroid levels have come back wrong for the second time – so no doubt that will be the next thing on the list to figure out.

For someone that looks so well, I certainly have a talent for being broken!

What I Wish I Could Say To My Doctor

Well its been a short while since posting, but as many of you know first hand, the life of a stripy person is never dull.

Over the last few weeks I have come up against the joys of the national health services again – the most tricky of these services seeming to be the good old general practitioners. Yes, thats right, those smiley happy (usually) pointless people that you have to deal with every time your health takes a turn.

In general, I tend to avoid going. Dealing with Ehlers Danlos Syndrome has taught me a lot – how to grin and bare it, how to put on a smile, and most importantly how to ignore the pain in order to find some way to get on with the life you should be having. Unfortunately, the biggest lesson I have learned upon the way is that seeking medical attention is more likely to leave you labeled with unsavoury terminology rather then give you any help in dealing with the symptoms that EDS creates.

If I could openly talk to a GP who I knew would actually absorb the information, there are a few things that I would like to point out to them, and today these have been brought into the forefront of my mind after yet another frustrating and pointless appointment. So – brace yourselves – we may be here for a while!

First and foremost I would like to make it clear that Ehlers Danlos Syndrome is a very real illness. It is not the stuff of legends, nor is it something out of a fairy tale. No. Ehlers Danlos Syndrome is an illness caused by faulty connective tissue. As a doctor, I would like to assume that this is a conceivable idea, and shouldn’t pose too many issues so far as forming some understanding of what may be affected. Most importantly, I would love to be able to assume that you didn’t get your notes mixed up in med school and come away with the idea that having said faulty connective tissue is not possible, and is in fact something only a hypochondriac would say.

Second, I would like to make it clear that we do not tend to frequent your consulting rooms for fun. Believe it or not we are people that do have better things to do with our time. Speaking for myself I can quite safely say that I would much prefer sitting in the pottery studio then waist my life sat in you’re waiting room. We live our lives in a lot of pain, and for the most part we tend to get by and get on with things without needing your continual attention.

So, when we do turn up, PLEASE LISTEN with open ears. Understand that we may turn up with new, worsening or scary symptoms. Listen and try to understand that there are many things that can go wrong when the collagen in your body is faulty, and that these really do need to be kept in mind before making a decision on the cause of things. It may look like a clear cut diagnosis, but unless you bear in mind that there is a pre-existing medical condition underpinning this, then you are likely to be barking up the wrong tree.
More times then not, we leave your office feeling that seeking help is a futile experience, and for me this has led to years of avoiding your offices at all costs. This is continually reenforced every time you refuse to listen, and pretty much every time it turns out that there was indeed cause for concern years later when I finally find someone who makes the time to investigate things properly.

Thirdly, understand that we are not drug seeking. You may look at the list of medication and think it to be unsuitable for a ‘healthy young adult’, but in reality you are not dealing with someone who is the picture of health. You are dealing with people who are in pain, who have stomach issues, urinary issues, depression/anxiety, POTS, joints that sublux or dislocate multiple times a day – to name a few. You are dealing with people that have so many systems in their body affected that it is very difficult to treat in almost any direction you can come at it. Yes, heat patches, stretching and a tense machine may be of use if your muscles are sore, but they really don’t cut the mustard when your hip refuses to pop back in for days on end. Mindfulness again may help you to calm your head and become mentally stronger, but not every symptom of ours can be controlled with this – I should know, I can tell you just how ineffective it is for intense pain from sitting in a service area balling my eyes out after attempting to use it to enable me to drive for another 3 hours that day.
Sometimes medication has to play a role.
I would love to be free from my tablets, but in reality these keep me at a level of being semi able to function. They dull some of the pain, they boost my mood, they slow my heart rate – they even help my stomach acid to stay where it should be rather then coming back up to my mouth. They are far from ideal, but they are often preferable, and even necessary.
So before jumping to conclusion that we are entering your office solely to gain access to your prescription pad, please understand that we would jump at the idea of never needing another tablet ever again if there was such a treatment available.
We are ill people with a very complex illness – we are not addicts.

Fourth (and I can’t believe I am even having to put this into words), you really need to understand that Ehlers Danlos Syndrome is not a ‘phase’ that we are going through. This is a genetic condition – and just like every other genetic condition you treat, that means that it is part of how we’re built. We aren’t going to wake up one morning with a body that suddenly remembers how to fix itself – our blueprints are wrong, and we have already been built. These symptoms aren’t simply going to disappear overnight, and many of them will sadly never disappear at all.
For some people (the luckiest of an unlucky bunch), they may not deteriorate too much – but for many of us, it is a syndrome that can cause long term and severe deterioration in almost every part of the body. Its called deterioration for a reason.
By telling us that we will ‘get better’, or ‘it will pass’, you are simply reenforcing our understanding that you ignore, diminish and don’t listen to us. You are making it crystal clear that our illnesses are not cause for concern, and therefore making it evident that we are unworthy of treatment.
The damage that this can cause is monumental. Not only does it make it harder for us to get treatment, but it can have horrific effects on a patients mental wellbeing.

Lastly be aware that we know our bodies far better then you ever could. After all – we are living in them. If we come to you for help, it is usually because something isn’t right, and isn’t our normal. You’re medical opinion is only useful if you take the time to hear us when we try to talk to you. You may suspect how we are feeling, but unless you actually take the time to talk with us about our symptoms, then how on earth can you make a decision on what is wrong?
We have bodies that suffer with chronic pain – therefore, if we are saying that something feels different, then it is extremely important that you don’t assume this is just what chronic pain feels like. We know what our pain feels like – we live in it day-in-day-out. If there is a new and severe type of pain, this really does need to be looked into just as you would for any other patient. Just because we have pain does not mean we can’t tell if things go wrong.

It is exhausting to be continually pushing for care, and extremely frightening to be ignored when you know that something isn’t right. We are the experts on our body, and more often then not, we often have to become the experts in our own condition. It would be a much easier ride for those with unusual conditions if doctors could accept, learn and work with us, rather then assuming that they know everything. They are only human after all.