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!

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