First of all sincere apologies for the lack of updates lately. It’s been such a busy time, that writing has fallen by the way-side a little.
I just thought I’d give you a small update about what I’ve been up to lately.
I recently wrote about the process of obtaining a Personal Budget to employ a Personal Assistant. I’ve had that in place a couple of months now, and it’s been brilliant. She’s been able to help with a range of things, from the obvious helping with care, but also driving me to appointments, getting my wheelchair out, cooking, sorting out areas I haven’t been well enough to do in my home, etc. It’s taken quite a bit of stress out of my life and I’d recommend it to anyone.
Medically I’m still very much in no-man’s land. The Pain Clinic have declared me incurable and untreatable, and discharged me. Apparently they only help people who will get better, they’re not interested in actually managing pain. My specialist consultants also are no longer able to keep me on their books due to over-subscription, so I’m consultant-less. It’s quite a scary place to be.
My GP has been trying to help with this situation, and wrote to a local Rheumatologist who lists himself as a connective tissue specialist on the hospital website. He rejected my referral, and wrote back saying, “I have no interest in managing Ehlers-Danlos Syndrome.” Although he did refer me to a Hypermobility Specialist Physiotherapist instead. Silly, silly me actually got my hopes up and thought she might be interested in working with me to move forward. Instead without any examination she declared there was nothing she could do, so I’d have my final batch of Hydrotherapy and that would be it. She then spent the rest of the session basically telling me I should be perfectly okay in the mornings, as other people with the condition manage. I tried to explain I have severe insomnia and sleep issues, and am a total mess in the mornings, but she wasn’t interested.
I then finished my final block of Hydrotherapy, which I really enjoyed. I do find the warmth means I can do much more than out of water. I would love this on a rolling-basis, and will keep trying to pester the hospital to make this happen.
I’ve also just had a sleep study done, but I’m waiting for the results. It was a machine I had to take home, that strapped to my stomach and chest, a tube went up the nose (nice!), a mask for your mouth and finally a clip for your finger. They have to rule out Sleep Apnoea before they’ll send you to a sleep consultant, but I am a little worried they’ll tell me it’s not Sleep Apnoea, and then not let me see anyone at all. Hopefully I’ll get the results soon, and this long-term issue is explored.
I have been lucky enough to find a private physiotherapist, however, that has an interest in Ehlers-Danlos Syndrome – and he genuinely does. It’s a lot of money, but the only treatment I’m now getting. He hasn’t promised he will be able to help me, but he’s going to try. If I can even maintain what little mobility I have, that would be a start. I’m diligently doing all the exercises he’s giving me (not a hurriedly printed out sheet, but thoughtfully targeting specific issues) and he’s easy to contact if things go wrong to provide advice, and alternative exercises. He really seems to know his stuff, so that’s been great.
I also recently took part in a study on Ehlers-Danlos Syndrome that’s all about what triggers the condition in the first place (obviously it’s genetic, but there is often an occurrence in your life that makes it much worse, often puberty, having children, an accident or something else. Others just have it severely from birth, so it’s exploring all of that.) The research also looks at flare-ups and the triggers.
The research sounds really interesting, and when it’s published it will obviously go into medical journals, but particularly GP and physiotherapist journals, to catch front-line staff.
One thing I truly believe is that the fight for decent rates of diagnosis lays almost entirely with GPs and Physiotherapists. They’re the ones that have to see signs, and see past one problem joint, or a history of chronic pain without a solid diagnosis and make a referral to a specialist. Once they have that awareness, only then can diagnosis rates climb.
I’m also preparing for Christmas, and this year have decided to have my first ever real Christmas Tree! I’ve also been making festive bunting with the help from my Personal Assistant, and it’s the first year in a really long time I’ve been genuinely excited for Christmas!
Please comment below to tell me your news, and whether you’re looking forward to Christmas or not?
What is Ehlers-Danlos Syndrome?
Ehlers-Danlos Syndrome is a connective tissue disease, caused by faulty collagen. Collagen is the ‘glue’ of your body and is part of your skin, organs, ligaments, tendons and much more. The condition is multi-systemic, which means it impacts almost all of the body in some way, including the heart, lungs, auto-immune system, gastrointestinal tract, eyes and in lots of different ways that are too numerous to mention. There are different types of the condition, which range from highly disabling, to some types which are even fatal.
The most common type of Ehlers-Danlos Syndrome is Hypermobility type. This is because the most prominent sign is the Hypermobile joints – colloquially known as being ‘double-jointed’. This can occur in any joint, and means the ligaments and tendons aren’t holding the joints in place as they should, and allows the joints to over-extend. This causes damage in the connective tissues surrounding the joints. Joints can also dislocate, or semi-dislocate (subluxate) which is very painful.
As the connective tissue isn’t doing its job, the muscles take over. This means they’re usually tight and in spasm, and overwork which causes additional fatigue.
The condition is genetic and two-thirds of sufferers inherit it from one of their parents. One third get it through a genetic mutation.
How does it impact me on a day-to-day basis?
While I was born with it, the symptoms throughout childhood seemed random, so no one ever realised there was an underlying condition. I had lots of bouts of pain throughout my childhood that doctors dismissed as growing pains, and often my legs or ankles would give way and I’d fall to floor for no reason. My healing was poor and left scars easily. Simple tasks like tying my shoelaces were difficult, I struggled to write for long periods, and my handwriting was awful.
At the age of 15 my pain became constant. I now know this is a very common time to trigger the condition more seriously, but it took almost nine years before a consultant finally stopped shrugging their shoulders at me, and put a name to my condition. For all those years – the years most people are growing up, and having a life – I was getting home from college, or university and collapsing in bed, and spending all my money on alternative therapies to try and get some relief as the doctors couldn’t offer any help.
I have never had a pain-free day for almost twelve years. It’s unrelenting, and even worse is unpredictable. One day I could be limping from my left leg and unable to move my arm, and the next day they’ll be fine but my hip feels like it’s on fire and my neck is locked in place. This is makes it difficult to know what I’ll be able to do that day – and how much help I’ll need.
The condition impacts the auto-immune system and produces too much adrenaline, so I sleep really badly, and have insomnia. The fatigue is crippling, and I often have to spend all day in bed, or unable to function. Often just being able to do a simple task like have a shower, or get dressed is an achievement and all I’ll be able to do that day.
To be able to do something else – like go to a friend’s house, have a meal out or even worse – attend an all-day event is a bit of a nightmare, and involve lots of planning. For days in advance I’d need to rest to conserve my energy, then on the day take every painkiller available, and use every bit of my strength to get through it. Recovery will usually take weeks.
My hypermobile joints are particularly bad in my spine, and so I’ve developed bulging discs, arthritis, degenerative lumbar disease, scoliosis and cysts on my spine due to the over-extension there. As a result my mobility is impacted, and I can only walk with extreme pain, and so use an electric wheelchair.
Why is awareness so important?
The diagnosis rates of the condition are really poor. The average time of diagnosis for sufferers is over ten years, but for many it’s much longer, and some even live their whole lives without knowing what’s wrong with them. Many are also misdiagnosed with other conditions, or told it’s all in their heads. Ehlers-Danlos Syndrome is estimated to affect one in 5000 people in the UK, however according to a recent study, around 95% of cases go undiagnosed or misdiagnosed every year, meaning this figure is in reality much higher.
EDS is treated terribly by the medical community. I wrote a little more about this topic in Rare Disease Day and Ehlers-Danlos Syndrome. In general most doctors spend a few minutes covering the condition in their studies, and so don’t recognise it when they come across patients who are looking for answers. It’s also highly neglected in terms of research, care, NHS services and provision of medical expertise. There are only a couple of specialists in the whole of the UK for the condition, which are usually oversubscribed due to the demand.
There are some quite simple ways of looking out for the condition, so we need to get the word out to front-line medical staff – GPs, nurses, physiotherapists – as well as members of the community, so they can spot the signs as early as possible, and get help for people before they have to live years in pain without understanding why.
If you recognise any of the symptoms mentioned above in yourself, your child or someone you know – particularly hypermobile joints, long-term chronic pain and other unexplained symptoms – please seek further medical advice, and request to see a specialist as soon as possible.
The Awareness Ribbon for EDS features a Zebra Print. This is from the phrase taught in medical schools – “When you hear hoofbeats, think horses, not zebras.” This means when presented with a set of symptoms – the most obvious answer is usually a common condition.
EDS adopted the zebra to say – hey, rare conditions exist too!
I found learning to drive really difficult. I had lessons over two years, and I don’t even want to begin to work out the cost of that. Neither of my parents had a car, and so my lessons were my only chance to practise.
I found it hard to coordinate everything due to my then undiagnosed Ehlers-Danlos Syndrome, and had awful muscle pain from using them in a different way. I could never have more than an hour lesson as my ankle and foot would go dead, and I found it all very exhausting. In addition, I was also very nervous/anxious, and just thinking about my test caused me to shake like a leaf, which then made me make loads of stupid mistakes.
But my worst mistake was my choice of instructor. I picked a lady who stated she specialised with nervous drivers – but this couldn’t be more wrong. Her style of teaching was to keep quiet while I drove, but then ask me to pull over and ask “Where do you think you went wrong on the roundabout five minutes ago?” When I didn’t know/couldn’t remember she’d get cross. She spent all the lesson pointing out my mistakes, with the atmosphere getting more fraught.
As time went on, I found instead of growing in confidence I became totally convinced I would never pass. It felt too awkward to ‘fire’ her, so I continued with this situation for far too long, ending numerous lessons in tears.
I then went to university, which gave me an opportunity to get a new instructor. He was completely different – calm, more positive and quickly my confidence began to increase. I decided that before I took my test I wanted some more intensive lessons – more than the one a week I was having, but my packed university schedule meant that was impossible. So I spent my Easter break having lessons every day for two weeks, with a test at the end.
However, it wasn’t the total disaster I thought it would be. I got a major for hesitation because on the way back to the test centre a bus stopped in the middle of the road. I couldn’t see around it, and being quite a busy road I thought I’d be likely to meet on-coming traffic if I went ahead. We waited about thirty seconds, and then continued – but I apparently should have gone. I think it was a little harsh, but there you go.
My second test was back in my university town. I was a hundred times more terrified. My legs were shaking so hard I kept stalling, and I made silly mistakes. At one point I tried to start the car three times in a row, and it kept stalling. I couldn’t understand why, until I realised I hadn’t put it into gear.
As we pulled up back at the test centre, my instructor who was waiting outside looked over to see how I’d done. I shook my head. I’d been a mess. My test examiner asked what I’d just said to him, and I said I didn’t think I had any chance of passing. She responded, “Well I disagree.” She then handed me the pass certificate.
I can’t even begin to explain how I felt. I know everyone feels joy at passing their test, but I had convinced myself so thoroughly I would never ever pass, and it had taken so long, and being able to drive was completely life changing. I think it does take on an added meaning when you have mobility problems.
At that point my mobility was going downhill rapidly, and I was struggling to get around the university, and also the long trip home on the train (through London on the tube). But overnight my world opened up – I could get to the shops, see friends, and go home whenever I wanted.
That was almost six years ago. I was lucky I took my test before my condition got like it is now – I’d really stuggle with the lessons, but there is lots of help available to help people with disabilities.
Here are some tips I learned in the process to help you pass when you have a disability:
- You need to be comfortable with your driving instructor. Even if they’re nice, or you’re scared of hurting their feelings – it’s your money, your time and it needs to be right.
- Make sure they fit your learning style. I tend to do a lot better when someone tells me what I did right, instead of making me feel bad by making a big deal out of everything that went wrong. Either explain that to them and hope they’ll adjust to your learning style, or move on if they don’t.
- Explain your health issues to them. They can make adjustments – such as trying to make the seat more comfortable, mixing in practical parts of the lesson so you get a break, or pointing the direction they want you go as well as saying right/left if you have any processing issues.
- Your instructor can request you assessor points the direction they wish you to go in your test as well as saying left/right if you have dyslexia or processing issues.
- If you can provide supporting evidence, you can request additional time while taking your Theory Test or Practical. Make sure to explain any physical issues to your examiner so they’re aware if you struggle to turn, or whatever the issue is. You can also ask for a disability specialist assessor, who should have had additional training in this area.
- Make sure you detail all your needs when booking either your practical or your theory. The last thing you want when you’ve just arrived at the centre when you’re so nervous you can’t think straight is to start having to point out things that aren’t accessible to you. And if you’ve already told them, you’ve covered yourself and it isn’t your problem – it’s theirs.
- If you can’t make it to the test centre for your Theory Test (for example it’s not accessible to you) it’s sometimes possible to take your theory test at your home.
- There are specialist instructors/cars out there if you need an adapted car, or someone used to working with people with disabilities. Ask for recommendations, or ask bigger driving schools if they have an adapted car on their fleet.
- If you receive High Rate Mobility Disability Living Allowance or the Mobility Component of Personal Independence Payment you can apply for your provisional driving licence at the age of 16, instead of the normal 17, which is really helpful.
- Motability and other charities sometimes offer assistance towards learning to drive when you have a disability. If you’re working then it’s also worth approaching Access to Work if driving would enable you to remain in work, or make life easier for assistance with lessons.
- If you need help to find the right adapted car for you, places like the Forum of Mobility Centres or the Queen Elizabeth’s Foundation for Disabled People can help.
Remember it may take you longer than someone else, but you will get there in the end.
Also check out this great post by Disabled In Scotland who details adaptations that can be made in the car, and the different route he took to find the right instructor for him. There are also some great tips here for deaf drivers.
Do you have any tips to share that helped you? Did you find it a struggle to pass your test, or was it a breeze?
Credit to: Michel Meynsbrughen
I belong to some Ehlers-Danlos Syndrome groups on Facebook, and they can be a fantastic resource for information. I wouldn’t know half as much about this complex condition if I didn’t have that connection to others living with it. Today I found out just how powerful groups such as these can be.
It started when a member posted a video of a lady on YouTube called “My Naked Protest.” In the video a lady called Kateryna sits in her chair, naked – and explains that she has been left without care for about a month – and cannot look after herself.
She is now covered in pressure sores and rashes, and doesn’t have any pain medication for her Ehlers-Danlos Syndrome. (She also has brain injuries, Autism and spinal cord injuries.) She has not been able to bathe for a month, and the last caregiver she had washed her clothes in detergent she’s highly allergic to – making her even more ill, which is why she sits there naked. She’s also now having to try and rehome her two cats – one of which is a service animal, as she can’t take care of their medical issues. Making her bed is also impossible, and the sheets are causing her agony without the right pressure relief pads on it.
She explains why she has lipstick on – knowing already that people would judge her for such a thing. She had put it on a few days previously to make herself feel better, but then the carers had failed to show up as promised, so she’d been unable to wash it off.
And so – after being totally abandoned by the care agencies, and after phoning constantly asking for help, and being told over and over that they’ll be there tomorrow – she makes one last desperate attempt to get help. When you have to take your clothes off to get help, something has gone horribly wrong.
The video was published on the 8th January, so she still had a wait – but when the video was posted in an EDS group, a few people jumped into action. Members called to report the problems to Adult Protective Services. Others called the police.
The police visited her – and were appalled by her situation, and made their own report to Adult Protective Services. They brought food for her, food for the cats and toilet paper, and even made her bed. Someone asked a local pizza shop to donate a meal – which they did, along with some large bottles of water. Her medical insurance company saw the video and have arranged for alternative care from tomorrow.
What’s terrifying is that this situation is repeated across the world. So many people with disabilities are hidden away, suffering without carers in place – struggling just to get by.
While this is an awful story – the silver lining was that it was lovely to people pulling together to get help in place so quickly and I hope the next care company don’t let her down again so badly.
Kateryna would like her story to be passed around so others do not suffer as she has. I hope she has a happy ending.
I have pain all over the place, but ranking at the top of the list is my lower back. I’d previously had facet joint injections in my neck and shoulders some years ago, but they didn’t help. However, it was before I’d had an MRI (as it took around 8 years of arguing to get one) and so the consultant was ‘guessing’ at what the issues were. Later on the MRI did clearly show an area in my lower back that had degenerative lumbar disease in the facet joints, arthritis, and others issues. It made a much better target for injections than anywhere else, so I thought it was worth a try.
It took another few years of me arguing for them (nothing ever just seems to just happen on the NHS!) After getting nowhere locally, my Ehlers-Danlos specialist at the Royal National Orthopaedic Hospital at Stanmore sent me to see her Pain Management college, who gave me the go-ahead for bilateral lumbar facet joints at L4/L5 and L5/S1 (so four in total).
A few days passed after being told I could have them and suddenly I thought – what the hell am I doing fighting to have needles put into my spine?! I was sedated during my previous injections, but I did have some memories of the procedure. I remember my consciousness kind of returning and thinking ‘please stop now.’ I was later told the sedation didn’t actually knock you out, but simply wiped your memory – and that you would be aware of what was happening as they did it. This memory built up in my mind until I was convinced I’d be strapped down with horrible painful injections into the middle of my spine, then I’d just forget afterwards so I didn’t realise how awful it would be. So I was about 500 times more nervous for these injections than previously.
The whole topic became a ‘do not discuss’ subject. As soon as I thought about it I went into a state of panic, so there was no research done as I normally would, and I didn’t even allow my mum (who accompanied me) to talk about our arrangements of getting there until the day before. I just wanted to block it totally from my mind.
We travelled to Stanmore the day before for two reasons. The first that I can’t make the journey there and back in one day, and the second was that my admission time was 7am. Ugh!
Although I wasn’t having general anaesthetic, I was told not eat after midnight, and no drinking after 6am. It was the latter I’d struggle with as I’m constantly thirsty.
I arrived at the hospital a couple of minutes before 7am and had a choice of parking for the first time ever! When we reached admissions there were a few people waiting outside, but when the queue got the doorway I realised the room was packed with both people queuing, and waiting in the chairs. It wasn’t the easiest getting my wheelchair in and out with no room for manoeuvring. There was only one member of staff dealing with everyone. The older lady in front of me didn’t speak any English, and was soon joined by an interpreter. The staff member made a phone call and I heard him say, “Are the wards still closed, there are a lot of patients here?” He then informed the interpreter they had a bit of a wait ahead. I was next, and was told the same thing.
I was a little cross, only because getting somewhere for 7am isn’t very easy for me and more importantly 7am = grumpiness! I did later found out the wards were opening for the first time after being closed for two weeks over Christmas – so I assume the delay isn’t normal.
I reached the ward and was met with a number of friendly nurses and health care assistants who welcomed me, and showed me my bed. The lady with the interpreter was in the bed opposite me. A nurse admitted me with a huge pile of paperwork, most of which seemed irrelevant in my case! I was asked a couple of times if I had any allergies. She then took swabs for MRSA and then took my blood pressure (which I hate, as it’s very painful. The muscles in my upper arm are very tight and particularly sensitive). I was told I was third in the queue – and the doctor would be seeing me beforehand.
A couple of other patients got in the bed and went to sleep, which seemed like a good idea – and would also make the time pass. However every time I was about to drift off I was woken by someone. The first to measure me for pressure stockings, then again to tell me I didn’t need them. Then a doctor who was working with my consultant arrived to explain the procedure. I was glad it was her and not my consultant as she was much warmer than my consultant is, which helped. She spent a lot of time explaining how much time they took to sterilise everything to reduce any chance of infection. I wanted to rudely interrupt and say that at that point, I didn’t care – I just wanted her to promise she’d knock me out! She then went through the possible risks – infection, numbness in the legs, pain flaring up afterwards and in a very tiny amount of cases nerve damage. She asked if I had any allergies.
Finally I could ask the one thing I wanted to know.
“You will be sedating me, right?”
“Did you ask for it before?”
“Yes,” I emphasised.
“Then we will.”
“Will I know what’s happening?”
“Well, some people do sleep.”
“Please make that happen with me. Please.”
Then the pharmacist came to take note of my list of medications, and asked whether I had any allergies, then a number of nurses came to tell me, “It won’t be much longer.” Then that they were on their way for me, only to still be there sometime later!
Eventually the porters did arrive and wheeled me into theatre. A nurse went through the final questions – checking I knew what procedure I was having, if I’d had any before, if I had any allergies and if it was definitely my signature on the consent form. The nice doctor then fitted the cannula into my hand, before I lay down front-first on the table. They put an oxygen mask on me, a clip onto my finger to measure oxygen (I’m sure that has a proper name) then to my annoyance – a blood pressure cuff which I was told would be taking my blood pressure every few minutes. It took it twice, before I told the nurse how painful it was. “No problem,” she told me cheerfully, and moved it onto my leg instead which was much better. She then began chatting to me about my hobbies and jewellery making, before my consultant began putting the sanitising fluids on my back. The nice doctor then began to input two injections of sedation into the cannula. I closed my eyes – trying to relax. She then asked if I could still hear her, and I said yes. She thought I’d gone to sleep, and I said I hadn’t felt any sedation yet. She said she’d keep going, and added two more tubes.
The next thing I remember is feeling a bit of pain in my back, and a nurse telling me it was all over. I definitely hadn’t been aware of it. I was taken into recovery for awhile, still very sleepy and groggy. A nurse chatted to me for awhile, and I remember telling her to say thank you to the nurses, as they’d calmed me down a lot beforehand. She promised she would. She then asked if I knew which ward I’d been in.
“It began with J…” I answered.
“Jackson Burrows is the only ward beginning with J.”
“No, it wasn’t that. Was it Diane something?”
“I guess now wasn’t the best time to test your memory?” she laughed. She then came back and said that unfortunately they’d moved me to Margaret Harte Ward. I began to worry if anyone had told my mum as I knew she was planning on getting a cup of tea while I was in the theatre (as she’d very kindly not eaten/drunk anything in front of me while I couldn’t).
As the porters wheeled me out of recovery, the nurse suddenly called for them to stop. She dug through a cupboard, and pulled out another blanket to put around me. “Sorry,” she said to the porters. “I just didn’t want her to get cold on the way back.”
When we got back to the ward I spotted my mum very quickly and realised it was the ward I’d been in all along! The nurse handed over to the ward nurse, and said that my blood pressure had risen quite a bit during the procedure, so they needed to keep an eye on it. Argh, more blood pressure checks!
I was left to sleep for a bit as I slowly came round. A nurse then came round to do the blood pressure test. I admit I groaned, and said the test was hurting quite a bit, then of course after gritting my teeth the machine malfunctioned. Just one more time, I was told. Luckily it was back to normal, and as she took the cuff off she realised lots of red marks had come up all the way round (which are still there today!). She gave me an apologetic pat. I asked if she could take my cannula out as it was pulling quite a bit. She took a look and said it was quite red, and that someone would do it before I left. (Well, one would hope so!)
I was told to stay until lunch time, and then I would be discharged. The nice nurse who admitted me then walked past and so I asked her about the cannula, and she said no problem and took it out. She also checked my ‘wounds’ (just slightly larger than needle marks) and said she was happy with how they looked.
Still waiting opposite me was the older lady with the interpreter. I heard a nurse say to her that her operation wasn’t going to be until mid-afternoon – yet she’d been admitted at 7am! It seemed mean to make her wait so long, and I assume the NHS had paid extra for the interpreter when they could had admitted her at the 11am or 2pm admission times. I noticed the little old lady chatted non-stop to her interpreter, which made me smile as I could just imagine in the 7+ hours the interpreter sat with her she got to hear her entire life story.
I left at 1:40pm, slept all the way home, slept all afternoon and very unusually for me – all night!
So all in all – it wasn’t nearly as bad as I dreaded. The staff – particularly the nurses, were lovely. There were lots of touches of kindness, like the nurse in the theatre distracting me from my nerves, the recovery nurse chatting to me as I came round and making sure I was warm, and the staff back on the ward who took care of me and offered me toast and regular drinks when I came round. They all made it a much easier experience. Also – the NHS really, really want to know if you have any allergies.
I’m in a lot of pain now which feels different to my usual pain. I was warned I may flare up, particularly where the needle touches the bone – so I’ve no idea if it worked yet. I think I need to wait for all of that to calm down over the next couple of days before I can tell if it helped.
An image of a facet joint injection