Having written about organ donation in The Huffington Post in February, I was contacted by the makers of a new documentary called A Love Worth Giving to ask if I would help to spread the word about their Kickstarter campaign.
The documentary follows a newly married couple Sam and Luke. Sam has Cystic Fibrosis and needs a double lung transplant urgently. The documentary follows her battle as she has to deal with false alarms and her health going downhill.
The makers have started a Kickstarter Campaign to fund the post-production and release of the film. I’ve watched the trailer and already it looks incredibly professional and well-done, and looks to be a heartbreaking story. (You can watch this by clicking on the link to the Kickstarter.)
The makers of the film have also set up a page on their website where you can submit a photo of one of your loved ones – if they have either died waiting for an organ, or if on their death they donated organs to help save other lives. This is a really lovely touch, and it would be great to spread the word to get the film made, and to hopefully help change attitudes about organ donation.
While 9 out of 10 people in the UK support organ donation, only 3 out of 10 actually sign the register. And many family members override their decision on their deaths.
This has to change urgently to many more lives can be saved.
There are only three days to go to get the documentary funded. Please dig deep and spread the word!
Edit August 2015: I’m really pleased to say this project did get funded, with 380 backers pledging£15,435. As of this month the documentary is complete, and I can’t wait to see it! The documentary will have its world premier at the 2015 Rhode Island International Film Festival – which is excellent news. Well done to them!
Credit to the Kickstarter Page.
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?
Let’s start with a fundamental truth: having a chronic illness sucks. There’s no denying that.
However, looking at the people I know who live with a variety of long-term conditions, a lot of them have developed certain skills that can help them.
When you have a chronic illness, your body often fails you. You frequently have to stop doing normal everyday tasks, activities and hobbies that you once enjoyed. Your illness can change on a daily basis, or even multiple times within an hour making it very difficult to plan anything in advance.
Resilience is defined as the “ability to spring back into shape and recover quickly from difficulties.” It’s something those of us with chronic illness have to do every day. Any day can bring a new symptom, a flare up or even a new diagnosis. We have to learn to live with that, and deal with our new circumstances, whatever that may be.
We’ve been dealt a certain hand of cards, and we can’t give up or take a break from it – we just have to keep fighting.
Strength is a fundamental facet of chronic illness. The strength to drag ourselves out of bed even when every cell in our body is screaming ‘what the hell are you doing?’ Or strength to put a happy face on when you’re meeting friends, or going to a family function. It takes a lot of strength to keep going, no matter how bad things get.
But there’s also the strength needed to advocate for ourselves – because even if you’re fortunate enough to have supportive family or friends, you know your body best and you will have to fight for what you need, often over and over, and have to be brave enough to ask for help when enough is enough.
Having a health condition means we need to become an expert in lots of different areas. Whether it’s our condition, treatment, benefits/welfare, how to obtain assistance, relevant laws and much more – we have to know it all! We’ve constantly got a new battle on our hands, as it’s rare help gets handed to us on a plate.
And having a rare condition brings added pressure, as we’re often also having to educate our doctors and medical professionals in what exactly our condition is in the first place (while trying not to annoy them!) before we’re even able to get onto treatment and management options.
Knowledge is vital for us to understand our own circumstances, and can also be very empowering.
Having health issues tends to open your eyes a lot more to other people’s struggles in a way you may not have even thought about previously. Compassion for other people comes more readily when you can relate to them due to your own experiences. Empathy is defined as, “The ability to understand and share the feelings of another.”
Chronic illness can cause an amalgamation of issues that aren’t just medically related, and it can make it easier to have an insight when other people experience their own difficulties.
In my own personal experience, I find when I mention I’m having a bad day, or share a fundraiser for a local charity – it’s my friends who have their own health issues who are the first to respond offering support.
Creativity comes in lots of different forms, from the traditional arts and crafts type, to the way you live your life. Through my own conditions I’ve tried to embrace my inner craft goddess, and discovered so many other people with disabilities also take part in similar pursuits. Perhaps because it’s a great way to distract yourself from pain, and also because enabling yourself to create something to be proud of brings a positive element to your life.
But creativity isn’t all glitter and glue – it’s also having to be creative with our own lives. You have to learn to think outside the box, find ways around issues and constantly problem-solve. The ways we used to do something may not now apply to us, such as hopping on a bus to go shopping. We may have to use our imaginations to devise easier and shorter routes, alternatives to carrying heavy items, and keep the trips out short and sweet to manage fatigue and pain. Instead of saying “I can’t do that” it becomes “How can I find a way to do that?”
If you know somone with chronic illness is having a bad day, why don’t you share this with them to give them a boost?
What would you add to this list? Please comment below with some of the skills you’ve gained, or skills that you see your friends/loved ones using.
There are lots of these special ‘days’ – Talk Like a Pirate Day (19th Sept), Hairstyle Appreciation Day (30th April) and National Sandwich Day (3rd Nov) but a really important day for me is Rare Disease Day, which is today.
A disease is defined as a condition that’s abnormal, and affects the body of a living being.
Here are some facts about rare diseases:
- There are over 6000 rare diseases
- A disease is classed as rare if it impacts fewer than 1 in 2000 people
- 30 million people live with a rare disease in Europe
- 80% of rare conditions have a genetic origin
I have Ehlers-Danlos Syndrome, a genetic connective tissue disorder.
There are various types of the condition and the most common type is estimated to impact anywhere from 1 in 5000 to 1 in 10,000 and the least common types have only been diagnosed with a small handful of people.
Rare diseases are neglected – in research, care, NHS services and provision of medical expertise. There is a lack of understanding and awareness in every part of the medical community. But rare doesn’t mean we don’t matter.
While I had mild symptoms from birth, the real trouble started when I was 15 – and it took me another eight and a half years to be able to put a name to my pain, fatigue and countless other symptoms. I’m lucky – many others don’t get diagnosed at all. That’s why recognition is vitally important.
Many seeking answers are instead labelled incorrectly. The word hypochondriac sneaks into their medical notes. They’re told their attitude is too negative, or point-blank informed it’s all in their heads. People with the condition usually look perfectly healthy, which makes it even easier to get dismissed.
Some people don’t live long enough to have the reassurance of knowing they were completely right, and there was a reason they felt the way they do like in this awful story.
Once I knew what was wrong with me, it changed everything. I was able to look for the national charities, find other sufferers and do copious amounts of research. Everyone should be able to do that – and from as near to birth as possible. Simple changes like strengthening your muscles before they become locked in a cycle of spasms, wearing orthotics to protect flat feet and looking out for the myriad of complications that can arise from the condition can make a big difference.
Awareness for rare conditions isn’t the same as for many other conditions. To me it can be summed up in the conversation that usually goes,
“So what’s wrong with you?”
And the response is a blank face.
I don’t expect people to know specifics – but if you said to someone you had MS, they’d probably at least gather it’s quite debilitating without having to try to explain further.
While that may not feel so bad – imagine that lack of recognition continues with your GP, your rheumatologist, physiotherapist – the people meant to be supporting your care, as well as everyone else. You have to spell the words out to them carefully, and they promise they’ll look it up – but yet the next time you see them they’re none the wiser, and you know they forgot to pop it into google. We deserve better, and it makes you feel very alone.
When I was diagnosed I spent an hour with the specialist consultant. He was the first person who nodded when I mentioned symptoms, and pointed out a lot I hadn’t even realised were significant. He literally started from when I was born onwards, and put together a pattern that led to my diagnosis.
However, an hour isn’t enough -not when you’ve just been diagnosed with a condition that impacts 95% of your entire body. Every organ, every joint. No one talked me through the heart issues, the gastrointestinal problems, bladder, muscle tone, nervous system, eye sight, breathing issues , and the terrible sleeping problems. No one told me that while you’re given a ‘type’ within the condition – there’s lots of overlap, and it’s mostly just guess work.
To this day I’m still finding new information, and putting facts together – and I usually find that out from other people with the condition, not a medical professional.
So this is what I want from Rare Disease Day…
I want every medical professional to have a brief understanding of the condition, and realise it’s only rare because they aren’t spotting all the undiagnosed people with it. Professor Grahame, a Ehlers-Danlos Syndrome specialist consultant carried out research that suggested “for every one person in England who is diagnosed by a rheumatologist as having hypermobility syndrome, there are probably 19 that aren’t yet diagnosed.” That’s around 85% of cases that are going untreated, being told it’s in their head, or being misdiagnosed. This has to change.
The most common sign of Ehlers-Danlos Syndrome is hypermobile joints – something that should be second nature for GPs/physiotherapists to have a quick look for if someone is presenting with wide-spread pain. I first heard the word hypermobilty from a physiotherapist I was lucky enough to come across. She asked me if I knew I was hypermobile, and I said I didn’t know what she meant. She explained it was also known as being double-jointed, but the only time I’d ever seen people mention that was when people did funny tricks with their hands. My hands looked normal. “Of course you’re hypermobile,” the physiotherapist said to me. “Just look at you, look at your toes, you just are!”
That’s all it takes. One person to have the knowledge. To be able to spot hypermobile joints, to notice the person with pain in every joint that is getting negative results to every test, to the person gesticulating with their hands as they speak, with bruises or injuries they don’t remember getting, that might be a bit clumsy. If you spot just one of those things, just have a think about mentioning Ehlers-Danlos Syndrome and you might change someone’s life.