Rare Disease Day 28 Feb 2014
The Rare Disease Day website states ‘a disease or disorder is defined as rare in Europe when it affects fewer than 1 in 2000‘. Ehlers-Danlos Syndrome falls into this category. It is estimated that about 1 in 5000 people have Ehlers-Danlos Hypermobility type with around 1 in 15000 people being diagnosed with Classical type and fewer with Vascular and other types (Dr Alan Hakim, 2013).
We asked you to send in your stories for Rare Disease Day 2014 about how living with a rare disease affects you. Here are a few stories about life with Ehlers-Danlos Syndrome:
I was diagnosed with Ehlers-Danlos Syndrome (EDS) in 2011. Whilst I’ve had problems all my life, my illness really started in January 2003 when I suddenly went downhill at 14 and no one knew what was wrong. A few months later I was diagnosed with Chronic Fatigue Syndrome (CFS) and a lot of my problems (like my stomach issues and fast heart rate) were passed off as stress and anxiety. Thankfully I had very supportive parents and was able to slowly build myself up to the point where I could live independently and go to University.
All this changed in 2009 when after starting an intensive Masters course I had a massive relapse, which was then added to by some very bad advice by a CFS psychologist. It was always so hard to get a doctor to listen and not just say that any new symptom was obviously the CFS and couldn’t possibly be anything else! Then a friend of mine was diagnosed with EDS. I went online to find out more about her condition, looked at the list of potential problems and felt like I’d been slapped in the face. It really was such a strong reaction. It seemed to explain my entire life, even small things like why I’d had such problems with my teeth when I was younger because of a ‘somewhat oddly shaped palate’ or why I’d bleed after the operations that I’ve had. I called my partner in and told him to look at the list and he couldn’t believe it either.
I went to my doctor with a list and whilst she said she didn’t really know much about it she was happy to refer me on. I went to the University College Hospital in London (UCLH) a few months later and after about 40 minutes of going through everything the doctor said I clearly had EDS as well as Postural Orthostatic Tachycardia Syndrome (POTS). I couldn’t believe that after all that time they finally knew what was wrong with me. Since then the level of help I have had from the specialist professionals has been phenomenal and my symptoms have now been properly investigated. They have found out what’s actually going on, rather than it being all in my head. I’m now on a proper pain management regime, have found the right medication and been able to access the correct physiotherapy too.
Obviously there have been ups and downs with all this and knowing that you have a genetic disorder that has no cure isn’t the best but actually knowing what’s going on and being treated seriously by my doctors has really improved things for me. Hopefully now I can avoid major crashes in the future!
My EDS Story – Zoe Lewis
I have Ehlers-Danlos type 7 – Arthrochalasia, there are believed to be around 40 confirmed cases in the world which I suppose makes me pretty rare. Ehlers-Danlos is a connective tissue disorder and there are many types which share some complications. The severity of the illness can vary greatly from person to person and type.
I have known all my life I suffered with this rare syndrome. When I was born My Mum said she knew I would be trouble, born breach like a small package all parceled up, arms and legs and head folded in like a ready packaged chicken, little did she know! My Grandma commented I looked like a Pekinese dog which sounds funny but actually I had a very tiny little face with big eyes, but my skin was so loose and wrinkly like I had skin two sizes too big. It was said at first that I was very overdue, but it turned out not to be the case, I just had the cutis laxa type appearance of wrinkly see through veiny skin all over my body.
My Mum recollects that in the early days they were never told the true full picture, but snippets. I had a scoliosis and congenital bilateral dislocated hips and clicked in every joint. At 12 weeks old I was put into a solid frog plaster cast, which was changed every month under general. This was very difficult for my parents, as they had to watch me in pain having to starve to have the general anesthetic. It’s funny because when I look at pictures of myself as a baby pre cast I am gaunt, dark rings under my eyes and look very ill, after casting I look like a completely different baby. It took 9 months for my hips cast to come off. I walked at 13 months old despite my parents being told that I may never walk and if I did expect it to be very delayed. I defied the odds.
As a baby I was seen by multiple consultants, all had an interest in different parts of my body. From early on and throughout my life I was prodded poked and stretched to see what I could do. I was extremely bendy and could contort my body in all sorts of positions, I have stiffened up a little but still I am told I am very hypermobile.
With EDS the collagen is defective; collagen makes up 80% of the body and for me effects most of the bodies systems. My hip joints and vertebrae as a baby were non-existent and grew, my scoliosis as a child was bad but as I got older it began to improve. I had no idea later in life my back would degenerate.
As I moved into being a toddler I had a lot of injuries, I was always covered in big black bruises and would dislocate my joints a lot. I also had a lot of nosebleeds and when I fell or hit my body, the slightest tap would cause my skin to split open, resulting in another trip to the hospital. My Mum says I clicked and clunked in every single joint, she could not hold me without joints slipping out. Early on my parents were told that I had a syndrome known as the Indian rubber man syndrome or EDS. At that time not much was known about EDS and they were left to get on with it. Consultants were always very good to me but I never was told the whole picture. I was always surrounded by trainee doctors wanting to sneak a peek at what EDS looked like. I had photos taken for journals to help educate medical professionals. I spent a lot of time in and out of hospital as a child not only with back, hip, joint issues but also with stomach and kidney problems. I was an extremely sickly child who would not eat for weeks on end, the doctors believed I was internally bleeding and was suffering but couldn’t tell anyone. My parents were I must add extremely lucky that the consultants that took care of me knew what was wrong, as I am sure constantly being in A&E with bruises and injuries from head to toe could have posed issues. I often dislocated vertebrae and went into spinal shock; these were the worst times for pain for me as a child.
Despite being constantly ill with EDS and constant viral infections I was a very lively outgoing little imp. I was tiny weeny as a child, part of my type, so at 5 I looked like a 2 year old. Arthrochalasic types are small in stature compared to other types. I am 4 ft. 8 now; we are known to have dwarfism type stature. Arthrochalsia type A and B diagnostic criteria takes stature and congenital hip dislocation into consideration when diagnosing, however biopsy is the only true confirmation as other types may be born with dislocated hips.
My parents were told to wrap me up in cotton wool as a child but I was so feisty and outgoing my parents learnt to play it by ear and see how I coped with activities. I believe they did the absolute best for me in this way. They didn’t want me not to experience what others did, and it allowed me to decide what I could and couldn’t do for myself, I usually learnt the hard way, on the trampoline one time only, collapsing in spinal shock. I learnt very quickly pain sometimes was not worth it! I never had the advice not to do contact sports until I was a much older child, and actually my frame despite the injuries made me very good at running and gymnastics in the early days. I soon had to stop! Although I loved disco dancing, as I seemed to be able to move like no other kid, so EDS did have its advantages.
At primary school I missed in total 2.5 years of schooling but I still loved to learn. I did have a lot of injuries and learnt to pace myself. I had to wear special shoes and insoles to keep my ankles from pronating. But no other splints were available for me, so I would often have crape bandages and tape around my body parts.
Bouts of glandular fever, kidney infections, viral infections, chest infections, hearing loss from infections, meant that I was forever ill and my immune system seemed to take a real battering, my blood results were always out of kilter. One of the other complications of EDS for me was my very fragile degenerative teeth, they cracked, decayed and the dentine flaked off, despite my roots being the opposite, calcifying to the bone and other teeth, which in itself caused issues for tooth extraction, every tooth had to be taken out in hospital under General anesthetic and twice this resulted in a broken jaw, and bone broken through to my sinus. I couldn’t eat very well as my top jaw never grew which meant there was a 2.5 inch under bite from top jaw to bottom. I was referred to consultant care at my local dental hospital to try and come up with solutions for me. At age 12 after 4 years of braces, treatment of every new procedure and wire known to man, including space station technology, I was given a denture over my teeth to help me, as my teeth roots just would not move. This really helped to stop my face caving in and help me eat. I was ecstatic, although I hated having to wear a denture at the age of 12.
Age 11 onwards is where my health declined the most, I started to have heart issues, kidney complications, and I was 10 months home schooled because of months of pyelonephritis. I had a lot of extra internal complications, such as urinary reflux, urgency and retention of urine, mitral valve prolapse/ heart murmur, POTS, costochondritis, lots of joint issues and pain became a real issue. It was because of collapses and admissions to cardiology departments that it was felt that I should get genetic testing and see a world specialist in Ehlers Danlos syndrome, there were concerns over bleeding, veins and heart problems and the general look of my skin and vascular complications which were thought to point to vascular type EDS. I saw the consultant several times and biopsies were taken from Mum, Dad and myself. My siblings were also examined and the family were all found to be able to do the Beighton scale and were hypermobile. After months we got the news that I had 7a/ Arthrochalasia type EDS, a new form which was recessive as my Father and Mother both carry a defective recessive gene of which I had inherited both from each, lucky me !!!!! So, relief for my family that at last I had a name to my condition and all the strange symptoms and complications were due to this.
From age 11 to today I have suffered day in day out, I cannot say I have experienced one day pain free or exhaustion free, this sounds a really dramatic thing to say but for me it’s just the case of which is the case for many sufferers of EDS of all types. In my adult hood I continued with terrible bouts of infections, shingles, glandular fevers everything going I caught, complications got worse with, dislocations/subluxing, pneumonias, asthma, scarred lungs, POTS flare ups, kidney problems, heart problems, stomach ulcers, severe allergies/ mast cell issues, stomach complications, menstruation problems, teratoma ovarian cyst, countless knee and abdominal operations to name but a few.
At 23 after spending 3 years at university and working I became very ill and bedridden. I believe at this time the autonomic dysfunction part of EDS became worse, little was known in the 80’s/90’s about such complications. Overnight I had a horrific migraine type headache with numbness down the side of my body and in the morning I could not walk, had changed sensation in limbs, my brain seemed to not send the correct messages to my body, and it was quite shocking. I got many opinions over the years on what was happening to me, from fibromyalgia, to M.E. to CFS to autonomic dysfunction of some kind. To me it really didn’t matter what the name or diagnosis was, whether it was as they believed part of my EDS or not, I just knew I was very ill and needed treatment. I could not feed myself, lift a cup, sit up for longer than 5 minutes at a time without being dizzy, falling asleep and generally feeling so dreadful, I vomited 5 plus times a day and would look a beautiful colour of green. My joints and dislocations were worse as was my memory, coordination. All my systems seemed to say I’ve had enough, and so at 23 I was totally reliant, wheelchair dependent and bedbound. It took 8 years to manage to build up with small /tiny bits of physio and rehabilitation. I had become very weak and had muscle hypotonia. Everything was 5 times harder to do. With EDS our bodies have to work hard to do the normal things people take for granted, like standing and walking. Daily chores make you exhausted and cause severe fatigue, along with joints that never do as they are told. I suffer with spasms and twitches, falling and stumbling which is due to dysautonomia. I did manage at 30 with a lot of determination to be able to get back to work, I would have liked to have done something with my degree but just working part time again gave me such pride and made me feel like I wasn’t a drain. I always felt awful having to claim benefits even though I didn’t have a choice. I hated this time of being bedridden, being totally dependent on others. I still stayed positive and kept myself as cheerful as possible and used art to help me to keep motivated.
Once I started work even though I was fitter physically the illness and complications carried on. I think I learnt to cope well with the constant agonizing pain EDS causes and just try and focus on positive things. For me I feel lucky, this seems a really bizarre thing to say after writing some of what my illness has caused. But yes every year I seem to pick up a new diagnosis, with Gastroparesis and worsening autonomic dysfunction, neurological complications and now rheumatoid arthritis, but because this has been a gradual process of adding illness after illness I don’t see the whole picture. I just cope one day at a time, work hard and try and enjoy my life as much as I can.
Staying positive is so important to me, when people say to me, wow you have been through a lot I always tell them ‘ I have excellent eyesight 20/20 vision, not everything is bad’, and laugh. I have learnt to pace myself, bracing and acupuncture and the right medications for my complications has allowed me to keep working part time as a lecturer, of which I’m so proud and grateful. I try every day to focus on my work and what I can do, however small. I have to have days in bed completely flat out not able to do anything, my body at times gives up on me. I do have a very good team of doctors looking after me and I am for the first time in a place where I can be me, accept what I have fully and know whatever happens to me I can get through it. I have learnt I need to take it a little bit easier, after breaking my back last year and tearing discs, osteopenia and osteoporosis can be a complication of Arthrochalasia type.
People are always telling me how stoic I am. I put that down to having amazing parents who taught me to cope, whatever. EDS is very hard to live with because its complications are wide and varied and can stop you in your tracks, but I have found a lot of EDS sufferers have the ability to be very resilient despite it all and live a good life. We as sufferers have an excellent community and the HMSA has been invaluable in bringing us altogether as has social media, it has changed how sufferers communicate about their illness, and has lead to great support for all of us. Something that was not possible for me as a child and late teen.
I wish for the future that doctors, schools and medical professionals will take time to understand the condition and realize it is not just about being bendy/hypermobile, doing tricks and having stretchy skin. It is actually a serious syndrome that can cause the sufferer to be completely debilitated and should be taken seriously. It is difficult because people with EDS, whatever type, usually look completely normal there are signs if you look for them but it takes a keen eye!
Hannah Ensor’s Story – HMSA Patron for Kids and Teens
Hannah was diagnosed with HMS aged 24, having been symptomatic all her life. She did well in school and university, but a year after starting work as an Environmental Health Officer her symptoms reached a disabling level and her Postural Orthostatic Tachycardia (POTS) became severe.
After working part time and flexible hours for a while she was medically retired in 2010 – age 28. By this point, while in hospital and unable to put a sentence together, she had discovered a talent for stickman cartoons. What started out as a way to tell others about her condition in a fun and stylish way naturally grew into a business – communicating about disability with charm, humour, and unerring accuracy.
And so ‘Stickman Communications’ was born – and Hannah officially became an author and illustrator. Light hearted and useful products, all aimed at communicating about awkward/difficult things. From booklets explaining HMS to keyring cards saying ‘Warning: High pain levels. Excessive Grumping Likely’. From car stickers to children’s picture books. Many of her products raise money for the
HMSA – and many are useful to people with HMS. In addition to the awareness raising work of her products, Hannah is increasingly in demand at events, disability fairs, book signings, and schools, as well as, of course, the HMSA residential conferences – working to create a better future for all.
A combination of physiotherapy, learning to control her movements, pacing,
medication, respecting her limits, pushing her boundaries, making the most of everything and generally choosing to focus on the positives means that she lives a full and happy life. So what if she uses wheels to get out and about, rests frequently and owns enough supports to create an entire exoskeleton?
Over the past 3 years, using the support of the HMSA and some really amazing medical professionals, through pacing and careful and consistent exercises Hannah has gained strength and improved control over her bendy joints, which led her to discover the ability to dance – and she loves it! She now dances with ‘Dugout, Oxford’, a dance group for people of varying abilities. You can view a video of one of their performances here with Hannah’s dance solo at 10 minutes into the video.
Although proud of her Stickman empire, Hannah thinks her greatest achievements have been in learning to manage her HMS/EDS and POTS and keeping up with her physio regime. It is an on going learning process, but has made so much difference to her life. Having said that, her proudest day has to be 26th April 2012. The day she was officially appointed HMSA Patron for Kids and Teens.
I’ve been hypermobile all my life, but was only diagnosed with HMS last August, in my mid 40s. As a child I was just ‘bendy’, and had various party-tricks like standing in queues with my feet pointing in opposite directions, and turning light switches off with my toes… The only problem I was aware of was that I couldn’t push drawing pins in because my thumbs just flipped backwards!
With hindsight however I realise I had a lot of subluxations, and although I just assumed it was ‘normal’ and didn’t tell anyone, I didn’t like it. My knees would lock and I’d get stuck kneeling on the floor… Or the little bones in my feet would go out of place and feel really weird. Without thinking, I avoided movements that caused pain or problems… And over the years, I became quite inactive…
During two pregnancies my arches dropped, and other problems that were probably related to undiagnosed HMS: shockingly low blood pressure; resistance to local anaesthetic (and a cross midwife who told me I COULDN’T feel her stitching me up!) and a Caesarian because my second son was doing somersaults inside my slack womb at 38 weeks and they were worried he’d strangle himself.
Then in my 40s, all sorts of problems started suddenly, over a frightening period of just 6-12 months: widespread pain, joint stiffness, more subluxations, early onset multi-site arthritis, palpitations, blood pressure changes (very low, then quite high), circulation issues and bowel and bladder problems… It was as if my body had had some kind of a breakdown. I went to my GP several times but was offered anti-depressants and fobbed off. I started internet research, and found information on the HMSA website and elsewhere that suggested to me that I might have HMS. I asked my GP to refer me to see a rheumatologist, and HMS was confirmed at Chappell Allerton in Leeds just last summer, aged 48. X rays taken at the same time confirmed osteo-arthritis all round my body, too.
Oddly, at the time of diagnosis, my Beighton score was 5. I know it was 8 when I was young, and since just last august it has dropped from 5 to 3 and now to just 1. My ‘bendiness’ has almost gone, as the arthritis has taken hold, though I still click and sublux, and have other problems.
Now, just six months on from diagnosis, my life has changed quite dramatically. Diagnosis was important because I don’t feel like it’s “all in my head”, and various services and support are now starting to happen. I have a Blue Badge and am on the list for a hip replacement. I am swimming like a mad-woman, 5 times a week, because I know it will help and because I can’t even walk let alone exercise on dry land. I’ve learned various ‘tricks’ to help manage my condition, including drinking lots of water and spending time in saunas and jacuzzis. I can’t quite see past my operation to what life will be like once I can walk again, but I hope the next few years will be much better than the last!
Tiah Beautement’s Story
I wrote a piece the other week on managing my HMS and the obsession the general public has with ‘are you better, yet’ when dealing with a chronic condition. (I have HMS and fibro).
In the past I’ve said to people, ‘There is no cure.’ ‘You can’t say that,’ most have replied. As if being practical is now on par with pessimism. In the past I’ve added, ‘Of course science is always making strides, but for now, yes, sorry, it is what it is. But the universe is welcome to surprise me.’ ‘You mustn’t give up hope,’ so many say.
I blink. Or nod. Or say nothing. What I want to say is: Let me tell you about hope. False Hope. This False Hope whispered on promises, the tantalising gifts on the tongues of magic bean pedallers and optimists afraid of crushing a person with the truth. False Hope arrives prettily, dressed in ribbons, packaged like a gift. It reaches out and rips open the boxes where a person stored all the things they tried to tell themselves they didn’t really miss, that life is now fine without. False Hope dangles these past dreams and pleasures in front of the face. Suddenly it all seems so real, so possible, so close.
Tiah’s entire story can be read here. The conclusion reads:
At the children’s party the acquaintance asked, ‘Are you working on something new?’
‘Ah, then you really are doing better, then.’
‘Yes,’ I’d said, standing there, left index and thumb throbbing, with two braces visible on my wrists, my back and left arm criss-crossed in blue tape while under my wide-legged trousers lurked a brace wrapped around a knee.
Yet in that moment, I’d answered honestly.
Carla Spear’s Story – HMSA Local Group Leader and Forum Moderator
When I was first diagnosed as having hypermobility syndrome (HMS) in 2007, I had no idea what was involved apart from pain, tiredness and dislocations. Stumbling upon the HMSA (Hypermobility Syndrome Association charity) a short time after, I was greeted by others in similar situations and found that there was a complexity to this condition that cannot be ignored or dismissed. To me now, it seems that HMS/EDS – whatever you want to call it, is similar to that of the autism spectrum in that it ranges in severity and complexity according to each individual. It seems that specialists such as Dr Helen Cohen and Professor Graham agree.
It would seem that my decline started in 2005 when I was travelling in Nepal and became very unwell; I’ve never been the same since. Before the trip to Nepal I had been an active, sporty child and teenager who was just extra clumsy and had joints that did funny things. I also suffered from multiple infections and struggled with fatigue but to me, this was my normal – I didn’t know anything else. After 2005 I could no longer ignore that something was wrong so I went to the doctors, only to be diagnosed with depression and sent on my way. At this point I was in and out of A&E and doctors surgeries with sprains, strains, dislocations, tummy problems and infections…the list goes on.
A physiotherapist finally put two and two together whereupon I was referred to a rheumatologist. On seeing the rheumatologist I was informed that I had HMS, was just bendy and needed to get on with everything. This was extremely tough as I was at university at the time, struggling with the combination of the workload, pain and tiredness, not to mention the lack of understanding from others – you see, I “looked” normal.
On going back to the doctor after finding the HMSA, I went armed with information to ask for a referral to Professor Graham (one of the top specialists in the condition in the country). Finally in September 2007, I met the esteemed Professor Graham. The list of diagnoses I came out with was quite extensive: Ehlers Danlos Syndrome Type III*, Fibromyalgia, ME, Orthostatic Intolerance and gastrointestinal issues. He had hope for me and I was given referrals to investigate my gastro issues and to see a physio to prevent further decline. Unfortunately at this time I could no longer cope at university so withdrew from the course. This was extremely upsetting but I had to focus on my health so I moved back home.
As my list of problems continued to grow, I kept going – there was no other choice.
I had moved into a flat of my own in July 2011 and although I struggled at first, I now have access to everything on one level (apart from the 12 steps up to the front door), a cat to keep me company and carers Monday to Friday. It’s the perfect location; just opposite my doctors surgery and pharmacy, with the hospital just round the corner and friends and family nearby who I can call on if needed.
As my luck (or very bad luck) would have it, every year since the diagnosis from Professor Graham I have been floored for a few months at a time by a mixture of sinusitis, swine flu, labyrinthitis, food poisoning, ruptured cysts and other complications. These hastened my decline and after spending a month in hospital at the end of 2011 with a ruptured cyst followed by complications, it got to the point where I needed carers to enable me to function and cope with basic tasks such as washing and dressing. At first I found this demeaning and was resentful that I was unable to perform such basic tasks for myself as I’m a fiercely independent individual. Now however, I am grateful for the help, assistance and companionship they provided as their help enabled me to save my remaining energy on enjoyable tasks such as arts and crafts. It took me a long time to start getting back into my other activities such as driving short distances and volunteering as a Brownie leader.
The beginning of 2012 was one I’m not going to forget. On 31st December 2011 I suffered severe abdominal pain, accompanied by full body muscle spasms. I won’t lie…I was terrified. I had no idea what was going on and had never felt pain like it. New Years Day 2012 I was discharged and went home, none the wiser as to what had happened but hoping that it wouldn’t happen again. Unfortunately, my body had other ideas and the full body spasm attacks started happening 2-3 times a week, the majority of them resulting in me having to go into hospital because the spasms wouldn’t stop and more often than not, due to the EDS at least more than one joint had got stuck and needed relocating. At times I resembled a pretzel the spasms had me so contorted.
The next few months were very tough to get through with all these added problems and no one knowing what was wrong. It was suggested on more than one occasion that I was doing it deliberately/faking it/going in for drugs/it was all in my head etc. It wasn’t surprising then that I had a breakdown and ended up in hospital for 5 weeks. During this time the problems persisted but I kept myself busy with crafts, mind maps, goals, challenging my thoughts and so on. In my third week there I had a revelation that will stay with me for the rest of my life.
“I am NOT my illness”
The change began here. It had got to the point where everything I did or said was about my illness or problems. Slowly and surely I kept challenging my thoughts and little by little, bits of me started to re-emerge. I became more communicative, assertive, positive (not always I admit but everyone has bad days), and found that focusing on small achievable goals, I had hope for the future again.
Don’t get me wrong – it’s not a cure, I’m not “fixed”, nor will it suddenly get better. But…I am NOT my illness – I am Carla; kind, creative, thoughtful, practical, helpful, and just a bit (ok…a lot) bendy.
The reason for the full body spasms is still unknown, nor the ongoing abdominal pain but due to my change in thoughts, I am pushing through regardless. These issues may never me explained although I am awaiting an appointment with the medically unexplained symptoms unit at my local hospital.
The progress I have made mentally has astonished me and I surprise myself every day. I’ve already achieved my small goals of going back to brownies, driving and doing some crafts but most of all, I’ve started going back to the gym.
Am I mad going to the gym when I risk dislocating any of my joints with any or no movement?
Not so – you see, I found when I was in hospital and then housebound, the little muscle that I did have had wasted away. It had got to the point where all I was doing was watching TV, taking meds, sleeping, taking more meds, eating and taking yet more meds. I didn’t feel any better for it though. I was empty and living a pointless existence.
Very gently I eased myself back to the gym starting off with one visit a week, minimal exertion and stopping as soon as my body told me to. It was exhausting but deep down I knew it was the right thing to do. I had to listen to my body and although I knew I’d never be likely to run a marathon, it was essential to me to get some form of stability back in my body. In addition to this, I also started reducing my meds – they hadn’t helped much so I chose to manage it mentally through distraction, challenging thoughts and relaxation. I also had fantastic support from my CPN and GP.
I’m now at the point where on a good week I might manage to go to the gym twice a week – admittedly I only do 6 minutes on the bike at the lowest resistance, then do some physio exercises. It’s not lots but it has made a huge difference – not only physically but mentally…I like exercising!
I’ve recently worked together with the head consultant at my local A&E department to draw up a management plan that is suitable for me, explains what is or is not necessary and the plan of action.**
I’m still in a wheelchair…so what? Nothing has changed the fact that I have EDS, ME, Fibromyalgia, POTS, Gastrointestinal issues, allergies and unexplained medical problems. These aren’t going to change so I have made the choice to manage them so that I am NOT my illness!
I have recently been offered a power chair by wheelchair services but to their puzzlement, I refused. I am determined to stay fit and active and to me a power chair would be more restrictive. Think of the Paralympics for example:
What these athletes are doing in the paralympics and what they are capable of is amazing.
Please think of this though: I forget I’m disabled sometimes and I watch the volunteers bouncing on the sides and think to myself “How the hell do they have the energy to do that?”, then I remember, I’m not normal – just as I’m not like these athletes. Some of them have capable disabilities. They aren’t all suffering the fatigue lots of us have. For many of them they “just” lost a leg or the use of their eyes, it doesn’t actually prevent them from being fit, unlike me. I’m not belittling the fact they have tragically lost a limb but some people just can’t see what we go through every day. The pain, tiredness, innards that don’t work, dysfunctional hearts, emotional aspects and so on.
Not everyone with the same conditions suffer the same. I have a very complex mix of problems that has left me reliant on carers and frequently housebound. Yet I stay active and my motto is “I am NOT my illness!”Not all disabled people are capable.
My lessons from these past few years have been enlightening and will stick with me forever.
1 – I’ve learned that by moaning and not doing anything about it, you won’t get anywhere. You have the key to your own future!
2 – Don’t assume that because someone else has the same condition that you will end up in a wheelchair. It has been a combination of issues that have left me using a wheelchair but I fought long and hard to stay on crutches and even though I use a wheelchair now, I make sure I stay as active as possible to prevent further decline and keep my fitness up.
3 – There are many degrees of EDS/HMS – just like a sliding spectrum. Just because I am at the severe end it does not mean that you will end up in the same situation.
4 – Medication and surgery are not the cure. Exercise is not a cure but it is a form of managing the condition – it maintains strength and prevents decline.
5 – There are always bad days but they pass…honest!
My biggest lesson of all is that: I am NOT my illness!
EDS cannot be cured, yet it does not have to be endured. We can instead, learn to manage it.
*There is much debate as to whether Hypermobility syndrome and Ehlers Danlos Syndrome type III are one and the same thing. For the sake of this writing, I feel that HMS is less severe than EDS. I am not belittling the problems associated with HMS, however to me there seem more complications with EDSIII. Not everyone will agree but this is how I feel.
** If you are a frequent attender, I suggest you speak to the team about drawing up a plan with them that you are both happy with. Mine states that self relocation is always attempted first and that I only go in if I am unable to do it myself. It also negates the need for x-rays unless there is new trauma, and sedation is only to be used at minimal amounts as the final resort.
I often feel words like David Cameron’s put me down when he stated “we must focus on what we can do not what we can’t do”. I am a FIT person, but I will never be capable of pushing my chair at high speed or swimming faster than anyone else. I go to the gym at least twice a week and try and stay as active as possible. I am DISabled by my illness and people need to understand the difference. I have to be realistic. So please remember this before you tell someone with a disability and/or illness that they should “Just get out” or “Get some exercise and it will help” or that there are others out there worse off. No, it won’t. My body isn’t capable of sustaining energy levels required to walk more than a few feet, nor do I have the ability. It takes all the effort I have just to stay awake and talk to people.
I’m 34 years old and was officially diagnosed with HMS aged 30 and also have Raynauds and Fibromyalgia. Unlike most hypermobile tots, I started walking at 8 months old. I was able to painlessly dislocate my right hip joint, pull my leg in front off me and hop around on the other leg until I was in my 20’s. I have always walked funny but didn’t suffer any pain until I was 18 when I suddenly started get paralysing back pain and spasms. My sacroiliac joint kept slipping. I was told by physio I may be in wheelchair by 30 (I’m not) I was also told would struggle giving birth because of hips.
When pregnant at 25, was told by midwives that I would be fine. Ended up with emergency c-section as pelvis tilted back and baby couldn’t move through birth canal. Really struggled with pain in 2nd pregnancy 21/2 years ago, have been in long term physio ever since, currently take between 30 and 38 tablets a day and am also under going treatment for another condition.
Sometimes I get frustrated that I get so tired and I can’t do what I want, especially with the kids. I’m a little naughty sometimes and ignore advice not to do things like soft play and going on trampoline with kids….BUT I will not let this beat me. I am a VERY determined person and I WILL live the life I want to live, I work full time and survive on hot baths, medication, lots of joint supports and coffee (and a handy husband and helpful 8yr old.) 2014 will be a year of finally learning to pace and get better (having some surgery this year.) 2015 will be get fit and stay active!
The HMSA has lots of information available for those living with a hypermobility syndrome from the diagnosis criteria to help and advice on treatments to discuss with your healthcare provider to help manage the pain and fatigue. We have a team of medical advisors and our helpline staff are there to help and advise over the phone when support is needed. We run a number of local support groups across the UK each month and members can access any of these as part of their membership package which is priced from just £8 per annum. Our Local Group Leaders receive top-quality training every year to ensure they are up-to-date with news on hypermobility syndromes and ways to support their members.
This year we are running a number of Family Days across the UK in May to reach out to members and non-members alike in various locations with more days to be added later in the year – please visit the Events page to find out more. We also have the Residential Weekend and Conference in July and these events are a great way to meet others living with a hypermobility syndrome, find out more about techniques for managing symptoms in children and adults as well as helping family and friends understand and learn about the different ways these syndromes affect us and what they can do to support us.
If you would like to tell your story please email Jen Patchett here