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!


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.

Why I Struggle to Say that I’m Scared

Throughout my increasingly long battle with chronic illness, I have noticed just how much I hide from the outside world. What starts off as one day with a headache and an ‘I’m fine’ has turned into years of feelings all neatly held out of view, sometimes even from myself. It is so easy to put on that practiced smile, and pretend to the wold that everything is still ok, perfect, fine. Non of it matters, although all of it really does.

Having recently gone through a partial thyroidectomy, I found myself back at home for christmas.

The surgery itself went relatively smoothly, tho I ended up staying in hospital for longer then I should have done because my ability to swallow solids has been messed up thanks to being intubated (oedema inside my throat coupled with swelling outside on top of already having oesophageal dysmotility), and it proved pretty tricky to find the right pain control. I ended up seeing an ENT specialist, the speech and language team (apparently it will take about 3 months for my swallow to settle) and the OT who suggested both a wheelchair so that I can get out more, and also the possibility of looking into supported living through my social worker – it seems that not being able to leave the house much really isn’t all as normal as I pretend it is.

Barely a week after my surgery I was becoming increasingly frustrated that everyone in my family, and I mean everyone, was telling me that I looked fine, and was obviously better (even though I am STILL living largely off rice pudding). It made me so angry because it made me realise just how much I am used to hiding all of the symptoms I experience daily. I am so used to hiding feeling like crap that even after surgery I can’t bring myself to break the illusion and allow myself to be ill. I wanted nothing more then to crawl into a hole. I wanted peace and quiet, I wanted to be allowed to cry and experience the pain, to be allowed to be the Charlotte that has just gone through surgery. Yet instead of this, I found myself being guilt tripped into helping my mother clean the house, care for my Nanna, and play the happy and bubbly woman that I know my family prefers to see.

Having got back to my little house, with just me and the dog, it is really starting to hit me. The emotions have all been so held back that I genuinely believed I didn’t feel anything about this surgery, or even about all of the other crap that my many other health conditions bring with them. I had no feelings, no describing words, just ‘fine’. This is not altogether true tho.

I am not fine. I am scared, I am lonely, I am in pain and I am sad. I am frustrated, I am angry, I am exhausted and I am fed up. I am a ball of mixed up emotions that have all been funnelled into nothingness and masked by spending my time learning to crochet – ever the practical one. This way of living is so normal to many with chronic health conditions, but it is truly an unbearable way to be.

Why is it that we feel such a need to hide from the outside world? Our reasons will be individual to our circumstances, but I know that for me there are a few.

A lot of it is that I was conditioned into it from very early on. We don’t talk about feelings in the family I have grown up in, and this extended out to physical feelings as well. I often attended school with a raging temperature, sick with the flue or ill with tonsilitus as a child. I went on countless music courses in excruciating pain with repetitive strain injury as a teenager, but it was a case of taking some pain killers and shutting up, so that is what I did. By the time I was an adult, I figured thats just how people deal with pain, and it has become so ingrained that I find it very difficult to sit down and actually tell anyone how much pain I live in every second of every day.

Some of it is protection, and this works two ways. I protect others from my conditions by brushing things off and downplaying the symptoms. I protect people because I don’t want them to have to find a way to deal with it when there is already so much going on in their lives, or if it would make them feel uncomfortable. I don’t like being the awkward one, so if I can sense that its a difficult subject for someone else, or that they wouldn’t want to depend on me like they would a normal friend, then I will withhold the reality. I also use it to protect myself. I have sat through so many awkward or difficult conversations where someone hasn’t understood the impact of my conditions that it is simply easier not to tell them. I don’t want to feel angry when I’m misunderstood, nor upset when they say the wrong thing, so I don’t say anything at all. It seems easier all round on the face of it, tho it doesn’t half put you in a lonely position.

Another part is that I don’t want to be perceived by others as weak for being ill. EDS and POTS seem so easy for doctors and relatives to discard as laziness that I have become used to not mentioning them at all. I don’t want people to tell me that I embody the one thing I already fear most, which  is that I choose not to work, and somehow choose to be ill. That if I just put the effort in, I could magically get better and be well enough to work. I know that non of this is a choice, yet the fear of being perceived this way is enough to keep me quiet to everybody around me.

Today I went alone for a cardiac MRI. My back seized up to the point that it had me in tears (even on tramadol), and took 2 nurses to get be on my feet. The results of which I am petrified of because I really don’t need anything else on my plate at the moment. On Monday I will be going alone to a neurology appointment and to see a GP (a new GP was mine has now left which is terrifying) about the fact that I am still struggling to swallow post surgery, and the nausea I am experiencing is pretty close to torture. I have barely managed to eat anything since getting home, and I have no idea how on earth to sort it all out whilst still maintaining good pain control. Then on Thursday I will be seeing my ENT consultant and getting the results of the biopsy from my thyroid (there was a 30% chance that the nodule had cancerous cells). All of these things scare me. All of these have different implications on my long term health and wellbeing, but I have no choice but to turn up and deal with them on my own and then crawl back home and try to ignore the bigger picture.

Thats just 1 weeks worth of appointments, yet the rest of January looks set to follow on as its started.

I am so tired of pretending that I don’t care about any of these things when the reality is that I am petrified of the body that I’m living in, and there is not a thing I can do about it.

Another Spanner in the Works

IMG_0258Just when I thought that everything was finally starting to fall into place, my body has yet again decided to do what it does best – and royally screw up, just in time to get in the way again! I am seriously starting to think that I was created as one big walking disaster area – just for the comedic value of whoever decided to create me. So what is it this time, I hear you ask – well, sit tight, here is the story of why yet another condition has gotten in the way of Stanmore!

As some of you will know, my thyroid has been a little special since my little love affair with high dose steroids (seemingly this was just another unlucky coincidence).  Although my symptoms seem to have played opposites to the diagnosis that fluctuates between subclinical and clinical hyperthyroidism. Rather then the weight loss, heat intolerance and issues with having too much energy, I am colder then ever, exhausted beyond belied and my weight has ballooned into something resembling a small hippopotamus. Just my luck following weight gain from steroids!

After over a year spent asking my useless GP to do more then blood tests, I was finally referred to a specialist by the amazing GP who has been covering her maternity leave. She also referred me for an ultrasound to look at my thyroid.

The specialist found a nodule after checking my neck (something that should have been done when my blood tests first came back abnormal), and the ultrasound confirmed a single nodule. The theory was that this was the thing causing my thyroid to be overproducing – however, my body is never that simple.

So, she ordered a radioactive thyroid scan. Due to the thyroid being the only part of you that takes in iodine, this is rather a nifty idea. I ate a low iodine diet in the week before, and duly turned up to the imaging department with no idea what to expect. Sat with a magazine for a short while before a nurse all garbed up with gloves and apron shouted ‘Next victim please’! He was fab! Out popped a metal box which encased the radioactive iodine, and this was injected into my arm – a little stingy, but not horrific. I waited around for a half hour or so, and then was led into the room for my scan. It took about 40 minutes in total, lying with my knees bent and head back. I did have to get some help from the nurse to get up again as my back had seized up – I felt like a 90 year old! Considering I spent 40 minutes having a lie down, I was surprisingly tired, and it took me a heck of a long time to get over. There aren’t often reactions to this test, but it turns out I was starting up a flare up of suspected Mast Cell Activation Syndrome – I spent the next 6 weeks literally not being able to stay awake, eyes running and so dizzy I couldn’t stand up, all of which started to dissipate as soon as I started taking antihistamines on the off chance they would help when the doctor drew a blank!

The scan was expected to show a hot nodule. This would mean that as the nodule would be overproducing, it would be absorbing more iodine then the rest of the thyroid. With my body being my body however, it actually showed up as a cold one!

So, after a month of not knowing the result, I was surprised to find a letter in the post for a second ultrasound. On calling up to question why I would need another so soon, it turns out this one would be with a fine needle aspiration.

Again, I rocked up at the imaging department (I was feeling like I perhaps aught to bring a pot plant and move in by this stage), and waited nervously for someone to stick a needle into my neck. Once in the room, I was introduced to the doctor, and had an ultrasound so that they could find the nodule. He then started getting out the equipment, and just before injecting the local anaesthetic (by far the worst bit) asked me to try not to punch him mid procedure! Anaesthetic in, they again used the ultrasound to pinpoint the right place, and then used a needle to scrape some of the cells, and then repeated this again for a second time in a slightly different position. After the excitement I was free to leave – I had a dressing over my neck, and due to being allergic to plasters, I got a different sort and was promised it looked much cooler then the others. He even suggested a few amusing stories I could give people to explain my new look – I do love doctors who are up for a laugh! My throat was sore, and my swallowing was painful for a couple of days, but nothing too major.

The results from this were most likely to be fine, and I was told it was a precautionary measure rather then anything to worry about. Unfortunately, it turns out that this was not altogether accurate. I ended up with a few critic clues in the post, from a not altogether helpful NHS. The first was that my results ‘need further discussion and have been referred on for this’. That is all I got! It is hard not to worry with that sort of letter, but I still tried not to think much of it.

The next letter took over 6 weeks to come through. With so much time in-between, I figured there would be nothing to report, but I was not so lucky. The next cryptic clue I received stated that my ‘results have been discussed, and and a surgeon will be in touch’! Just my luck. So, after an awful lot of detective work, discussion with my GP and phone calls I was told that I had a nodule classified as U3 THY3f. After more phone calls trying to get hold of my consultant, she explained that this mean that my nodule had cells that were ‘in a grey zone’, meaning that there are suspicious cells present, but they can’t be diagnosed as either benign or cancerous without further exploration. As another biopsy would show the same result, this means the need for a partial thyroidectomy.

Saturday just gone, I had an appointment with an ENT surgeon. He was lovely. After trying to remember my long list of diagnoses and medication, I was petrified that he would think I was prone to making up my illnesses, but thankfully this was not the case. He spoke about the surgery – it should take about 2 hours, and may or may not have a drain depending on the size of the cavity and bleeding once the half of a thyroid has been removed. I will also need to be in hospital for at least one night, possibly longer depending on if my body is behaving itself. He then had a feel of my neck, and then used a camera to look down my nose into my throat – not the most pleasant procedure I’ve had.

Surgery is booked in for the 18th of December, just in time for christmas. I am struggling slightly with this for a number of reasons. Mostly I just feel that my body has let me down yet again. I have finally gotten to Stanmore, and my admission was supposed to be at the beginning of the year, butagain something else has screwed up. I feel like its a dream I just can’t get to. Stanmore could mean that I can actually start getting better, but how can this happen when I can never get to it? Every time I start finding a way to get better, my body keeps pulling me back under – and this isn’t even an EDS thing. So far on top of EDS, POTS and all of the other conditions they bring with them, I have had optic neuritis which royally screwed me over, the BRCA2 gene and now this. How many more things will go wrong?

I am reaching my limit with what I can cope with in this body, and feeling truly trapped. I desperately want my life back. I want to feel well, to work and to leave the house more then once in a blue moon. I want a life without pain, without the constant worry of whether something else is going to go wrong that I have absolutely no control over. I want to be normal, but I can’t do anything about it. Living in this body is torture.


Stanmore (RNOH) (first appointment and pre-assessment)

Its been a while since I last posted, apologies! So much has gone on over the last few weeks that I haven’t really known where to start. My mental health took a bit of a bashing with my PTSD flaring up, tho thankfully its settling down again now, and sadly dad has cancer again, so theres rather a lot on my mind. Although things have been difficult, there is also rather a big positive happening at the moment – I have finally been able to get seen by a member of the team at Stanmore!!

Its taken a few years of frustrating battling, but finally with a good GP behind me, my first appointment letter arrived. I was partly jumping around with excitement, and partly absolutely terrified. In order to get a referral to Stanmore, you first need to go through a rheumatologist. With EDS this can pose an insanely large challenge, and just as always, I was not to be disappointed. Due to the rheumatologists under my local hospital point blank refusing to believe EDS could even be helped and refusing to see me, let alone refer me to Stanmore, I had to travel down to Orpington to see someone. The person I saw was beyond useless. He insisted I didn’t have EDS, but simply had fibromyalgia. He insisted I only scored 1 on the Beighton scale (far bellow the 5-9 I have been scored in the past) – ignoring my obviously hypermobile elbows, and stating that even tho my thumb could touch my forearm, they weren’t bent backward and therefore didn’t count! When I questioned him about what my internal issues were caused by, he simply tried to brush it off with ‘I don’t know, but not EDS’!! I came out of that appointment absolutely fuming. On my insistence, and the insistence of my cardiologist, he did however agree to refer me onto Stanmore – so I suppose I should count my blessings.

Having been essentially written off by the last person I had seen, it is no surprise that I was feeling pretty nervous about going to one of the UK’s only specialist centres for EDS. I was fully expecting the same reaction, so had been getting steadily more anxious the closer my appointment got. What if he was right and I didn’t have EDS at all? What if they thought I was lying? What if they couldn’t help me?

Thankfully I had nothing to worry about. After the nurses taking my heart rate and blood pressure (and my POTS confusing them as my blood pressure went down upon standing), I was led into a room with a consultant. She took a very detailed history from early life to present, and then proceeded to question me about the referral. She asked why the letter had stated ‘I would appreciate your help with this EDS classification matter’ and was pretty astounded when I explained that the rheumatologist had only scored me a 1 – especially when I held up my elbows and told her he said that they weren’t hypermobile! She told me she would ‘copy him into the letter’ to teach him about EDS! After this, she gave me a thorough examination. Apparently I score a 7 on the beighton scale after all alongside my shoulders and hips being very hypermobile, and she also checked a number of other things. My skin is more stretchy then normal (which I wasn’t aware of), and I have an overcrowded moth with a high arch palette – typical of EDS. She also checked underneath my tongue, and told me that I have no frenulum – I had no idea that this was another sign of EDS – you learn a new thing every day! After all of this, we sat down to have a chat. She has asked for me to have a brain and spine MRI to double check everything as I suffer from certain headaches, so I have been referred to a neurologist for this. We also discussed what Stanmore could offer me. Although they do offer outpatient support, we agreed that as I live so far away, the 3 week rehab programme would be more appropriate, and so she referred me on for this. When I asked about timescale, she said that I would likely go for rehab within 3-4 months. I was sceptical about this, so hadn’t gotten my hopes up – it seems I may be wrong!

A few days after my appointment I received a questionnaire in the post. This was largely about they places I experience pain, and daily functioning. I sent this back, and a week or so after this, I received a letter inviting me to a 2 hour preassesment session held at the rehab centre. Feeling nervous and excited, I travelled down on Friday morning to attend.

When I arrived, I was invited into a room with a fellow 10 or so patients for a presentation. This consisted of a video followed my a question and answer session. Everybody was friendly, and a good proportion of the others turned out to have EDS or Marfan syndrome.

The program is run in 2 settings. Both contain the same materials, but one is run in a hotel, and the other in a hospital setting. The idea of the hotel program is that it replicates the experiences of normal daily live as much as possible, so you have responsibility for all medication, personal and medical care yourself. The hospital program is for those who fall into a higher risk category, and there are nursing staff available for help with personal care, and perhaps a higher level of support if needed. The decision as to which program is most suitable for you falls down to the medical staff, and although they discussed this with me, I didn’t have a choice in the decision.

After this, we were asked to wait, and were called to have an individual assessment with a member of the team. I was called by an absolutely lovely pain psychologist. We sat down, and went slowly through the questionnaires I had filled out before attending. She was extremely detailed with both my physical and mental health history and present symptoms, and not once did I feel uncomfortable or embarrassed. We spoke about how the program is run, and throughout this process she talked about both the hospital and hotel program. She also completed a couple of risk assessment forms covering things like risk of falling, personal care needs and safety with medication/mental state. As the assessment went along, she did suggest that it was likely that the hospital program would be more appropriate for me. This was partly due to my physical care needs, superpowers with regard to falling abilities and also my tendency to dissociate in certain situations. The hospital program also seemed more suitable due to its slightly smaller group side which shouldn’t feel as daunting to me. I struggle in large group settings due to PTSD left over from bullying, and this massively can trigger me to dissociate. On the hotel program, you are with a group of up to 15 people (I believe) for the entire 3 week program – obviously this can be great if you get on with everybody and don’t mind larger groups, but I can also see it being difficult if you happen to clash with a fellow patient. With the hospital program, you come in to the program in a core group of up to 4 people, and work with people who have already on their 2nd or 3rd week of the program. This means that you meet new people every week, and there are also a maximum of 12 people per week. By the end of the assessment we had spoken through my potential concerns with the hospital program – namely that I didn’t see myself as being in need of greater support then others, and that my concerns of coping with such a full timetable/living on the ward, and I felt much more at ease. We laughed and joked a lot which really helped me feel comfortable, and I even spent some time showing off my doggy pictures!

After this, she led me out through the ward, showing me about a bit as she led me back to the waiting area. I was then free to go!

All in all, I was there for about 2.5 hours, tho my assessment was much longer then it normally would be (my poor mother was waiting around much longer then she should have been!)

I was told that I am likely to go into the program some time in January unless they happen to have any last minute cancellations. This seems surprisingly quick to me, and is slightly terrifying. The idea of the program is amazing – it will basically give me specialist and individualised knowledge and skills that I can use to start the process of rehabilitation. There will be a lot of 1-1 physio, occupational therapy and sessions with a specialist psychologist who deals with pain. These should teach me what I need to work on, and give me the tools to begin to change my routine at home in a way that may help me learn to live with both EDS and chronic pain. The scary side to this is that once I have completed the program, the responsibility is on me to be able to maintain the things I need to work on!

Once you have completed the program, you have a review in 3 and 12 months to establish how you’re doing, so I guess you aren’t totally alone.

All in all, I feel very grateful to have finally gotten to Stanmore. It won’t be a quick fix, and there is still no guarantee that you will be back to normal in the future, but what it can do is teach you how to live with your illnesses and chronic pain in a less disruptive way.

I really do hope that this is the beginning of getting things back on track – however far  that will takes me, it has to be better then here!

Getting Sick When Your Already Sick

One thing that few of us chronically ill people talk about is being ill – and this seems doubly true when it comes to getting a normal illness on top of it all.

When you are chronically ill, feeling a bit crap comes as an every day occurrence. You are already in pain, exhausted and feeling that fluey feeling most people only experience with the actual flue. Personally I find that what I really mean when I say ‘I’m fine thank you’  is actually ‘I am in so much pain I could puke and I’m so exhausted that I feel a tad on the dead side, but thats nothing out of the ordinary thanks’. I do this because like many others, I assume that I am rarely talking to someone who would actually want an honest answer – after all, I suspect most wouldn’t be able to figure out a reply without a spectacularly awkward silence first. My illness is something I have tried to push out of focus to the world around me – I take pride in my ability to mask the bad times and make people forget that I’m ill. After all, I am a woman first and an illness last.

However, let me be the first to tell you that getting ill on top of being ill is not much fun! Today I woke up with a sore throat, a fever that has been raging all day, skin so sensitive I want to peel it off, and that general fluey fog you get with being ill. I have gone between shivering and being so sleepy that I can’t keep my eyes open, and spent most of the day feeling like the world was moving.

Now, for all you well people out there – imagine having the flu, and then adding it to all of the symptoms EDS and POTS gives someone day-to-day. Add it to the joints popping in and out of place all day, the usual constant headache, tight muscles, chronic pain and brain fog (yes, you get a doubly foggy brain!) Add that to issues with swallowing, tachycardia, postural dizziness and fatigue. On top of all of that, add in the nausea, back ache, odd temperature regulation, insomnia and TMJ (jaw) issues. I am not listing everything here, but you get the idea. We already feel like crap day to day, so when the double whammy comes, it tends to hit us hard.

Even on a day like today, I hide it, even tho its perhaps more socially acceptable to talk about this type of illness. I may mention in passing that I’m feeling ‘a bit crap’, but what I actually mean is that I feel bloody horrific. I shut myself away, and I wait for it to pass, all the time knowing that when I get better, I won’t really be better at all – I will just be chronically ill rather then chronically ill with bells and whistles.

The one upside, is that being chronically ill will feel much better then this does.

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.