I only bloody got to London twice in one week!

Last night’s blog post was quite cathartic for me. After last week’s London adventures I have been so exhausted I hadn’t really had a chance to organise my jumble of a mind and I think I started to do it. It has a bit of a strange tone to it I know, but that’s because this week has been so strange. Not only did I actually get to London twice in a week (I still can’t quite believe I did this) and stay at my parent’s house for the night which were major achievements, but I also had two pretty major and massively disappointing appointments.

I’d like to start with a caveat, I think the NHS is freaking fantastic. The fact that I am able to access any treatment and I don’t have to have the stress of the cost on top of it is amazing, I dread to think where I would be mentally and physically if I was in America. However, the NHS is not perfect, a point on which I think we all can agree. I also understand that most doctors do care, and do want to help, but are tied into a difficult system that they can't necessarily change. Now that is over, I'll get started.

People with EDS are known as zebras in the medical community. Apparently medical students are taught that when you hear hooves, think horses not zebras. That is, the most likely cause is the most common. However, every now and then you get someone like me who comes along, who is most certainly not a horse. I am a loud and proud zebra, but it’s taken them over 10 years to work that out. Over the years I’ve been passed along to various specialists; rheumatologists, psychotherapists, endocrinologists, pain specialists, physiotherapists (and the list goes on). I’ve had MRI and CT scans and am more familiar with bloods than a phlebotomist. I’ve seen private psychiatrists (who told me that I was anorexic, which couldn’t be further from the truth!), osteopaths and counsellors, and I even had a yoga instructor for a while with whom I spent an hour a week breathing. And yet, it took over 10 years for a doctor to ask me to bend my body around, to touch my extremely soft skin and noted the scars that appear from nothing. It took them ten years to take notice of the fact that 80% of the time when I stand up my vision blacks out slightly and that it was more than just being tired all the time. This was ten years after the first osteopath ever remarked on quite how hypermobile I am and two years after Mr Spoonie Foodie (a biomedical scientist) suggested I had EDS and Marfans. As you can imagine, it’s been very frustrating decade, but that is now behind me and I have been diagnosed, which gives me access to a lot more treatment.

As I said before, Professor Graham is the specialist of all specialists when it comes to my condition. So you’d think, that once he referred you on, your next consultant would read his notes about you and then take things from there. But no, apparently the NHS is incapable of speaking to one another, so instead they waste my time, the consultants time and their money by repeating this same consultation again and again and again. They're cutting services left right and centre and yet there doesn't seem to be enough communication within the NHS to prevent unneccesary and repeated trips. Why can’t an initial contact like this between patient and consultant be done over the phone, or by skype? Surely the specialists understand that getting to/from London and these appointments takes everything out of people with these conditions?  In the past year I have had no less than five appointments which were EXACTLY THE SAME! Well, six if you include the one with a geneticist who was a prick and refused to do the gene test and also said she didn’t think the Professor Graham was right. But that’s beside the point.

So (and I can imagine you can see where this is going) last week’s appointments turned out to be the biggest disappointment ever and actually a fairly unpleasant experience. My referral to Dr Ingle’s clinic at the national hospital was, from what I understood, the start of a set of autonomic tests. At Dr Ingle’s clinic I was met by a consultant who didn’t introduce himself to me. He then marched off along the corridor far quicker than I was able to walk and when I explained that I couldn’t go that fast he didn’t slow down, so I was left peering into each doorway trying to see where he had gone. It was pretty humiliating to be perfectly honest. The appointment ended up being only 20 minutes long and wasn’t actually the start of testing at all, but basically him talking at me about the condition. Now, don’t get me wrong, this was massively useful and quite interesting. It turns out new research suggests the POTS is actually connected to the hypermobility. In most people, blood pressure is maintained by constricting and relaxing of the blood vessels, thereby forcing blood around the body. So when we stand up, the vessels in our legs should constrict in order to maintain blood pressure around the body and counteract gravity. It seems that what happens in hypermobile/POTS patients is that our vessels don’t constrict as they should and instead, in order to maintain a steady blood pressure and not faint, the heart pumps quicker. This is linked to many of the fatigue symptoms, as well as palpitations and of course, fainting. There has been a lot of new research going into this and there are quite a few new treatment options starting with drugs, but of course before you can get these you need to confirm that the patient has POTS. To do this they run tilt table testing, which is what I thought I was going to start last week. Well, no, once he’d done talking to me that was it. I was sent on my way and told I would have to wait EIGHT TO TEN MONTHS just for the testing to begin. Oh, and he also casually dropped it in that this might of course not be POTS and could be something more serious like Addison’s disease or a tumour (but you know, we’ll just wait a year to find out!). I mean seriously? What the hell was the point in that appointment? Why didn’t they just call me up and have a chat over the phone instead? And waiting another year? I know that the equipment for these tests etc is expensive but that will be nearly 2 years since I got diagnosed before even getting tested, let alone beginning treatment! I shouldn’t complain, at least I’m on the waiting list finally, that and there is nothing I can do, but it is so incredibly frustrating to be told to just keep your life on hold for another year before we can even think about starting to get you better. I left feeling horrendous, in loads of pain and absolutely exhausted. And all that to be told I’m just going to have to wait for the best part of a year.

As you can imagine, I wasn’t massively looking forward to Thursday’s appointment. This time I was under the impression that this was the assessment to get into the inpatient rehab clinic that seems to be the only major treatment available for EDS. I was prepared for another long waiting list, but what I wasn’t prepared for was to find out that this wasn’t the assessment. No, this was the pre assessment before I actually have the assessment for the rehab clinic. Um...what? Surely the referral from Dr Graham was the pre assessment needed by a rheumatologist that would then be followed by the assessment for the clinic? Apparently not. So instead, I had an hour long appointment (to be fair she was quite thorough) where she literally repeated everything Professor Grahame had done, down to the point that where I missed something out that was in my notes she’d then ask me about it...I’m sorry, but I REALLY don’t understand why on earth I went through that appointment. She again checked my arm span/height/hand/foot ratios for Marfans and came up with the same conclusion as the Prof that I had incomplete marfanoid habitus and confirmed I had EDS (though gave me a lower 6/9 on the beighton criteria than his 8/9). So now I am going to have to wait another 2 months before I can see the assessment team who will decide whether or not I can actually go on the rehabilitation programme and then another 6-8 months before a place becomes available. I mean, thank goodness I’m finally in the machine and chugging away but I feel like my life has been on hold for so long. I just want some practical help with how to move it forward just a little.

On that note, I’m doing my exercises that the physio gave me. They are basically very slow resistance based exercises that strengthen the muscles, and they seem to be working! For the first time in my life I actually have some bicep definition and I am noticing how I am now able to do more of the exercises as well as feeling less wobbly whilst I’m doing them. It’s really exciting to actually see and feel the progress I am making. I’m still not able to walk for much more than a couple of minutes and there are days where that is still a struggle, but I’m seeing some improvements and that’s all I need.

Although its slow going, things are definitely heading in a good direction at the moment. Mr Spoonie Foodie moves in in two weeks which is ever so exciting! I’ve got some sorting out of my shit to do first and I’ll definitely have to be tidier(!) but I really can’t wait. More and more I feel like we are a proper team. We haven’t had the easiest of relationships, neither of us are the easiest of people, but I think that’s what makes us so strong. Also, my parents absolutely love him which has got be a bonus!

So perhaps my odd little metaphor at the start of the last post makes a little more sense now. I am definitely on the verge of some very big changes, but some of them are taking a bloody long time to come.