Tag Archives: Ehlers-Danlos Syndrome

A Day with Ehlers-Danlos Syndrome : Part 2 (A Bad Day)

I’ve been awake for a while and aware that it’s going to be a bad day. My head is pounding, I feel horribly sick and it feels as though my body’s been hollowed out, filled with petrol and set alight – nasty, burning pain all over. I’m stiff and uncomfortable so I’d really like to turn over, but I know that will only bring on the sickening lurch of a pre-faint so I lie still. Eventually, my electronic pill box sounds and I reluctantly open my eyes. The room is spinning and my eyes seem filled with wriggly lights (I don’t know how else to describe it). I manage to lift my head enough to take the pills and then collapse back on to my pillow. It’s too soon after taking the pills so now I have heartburn. After a few minutes I realise I really need the loo. I carefully pull myself into a sitting position using the rail by my bed and groan as the nausea becomes more intense and the room spins with increased vigour. My bladder is growing more urgent so I pull myself up into a stand. Massive mistake: my knees buckle and I hit the floor. After a couple of minutes I manage to muster the energy to slowly slide to the bedside table and press the alarm call. The safest thing would be to wait where I am, but then my Mum would have to clear up a puddle and goodness only knows she has enough to do already, so I start to slither towards the bathroom. I can’t crawl because my wrists, elbows and shoulders give way too easily. I’m about half-way there when I hear my mum’s key in the door; she’s responding to the alarm. She helps me up and takes most of my weight as I shuffle slowly and clumsily to the bathroom. To me, everything seems hyper-real and yet curiously unreal, as though I’m really far away. Mum helps me on and off the toilet seat and holds me steady as I wash my hands. I’m past worrying about my dignity after all these years! She helps me back to bed. By this time, I’m talking nonsense and occasionally laughing for no reason. My floppy blood vessels mean there’s not enough blood in my brain so I can’t function normally. It feels like the functioning part of my brain is locked away in a cell, screaming to be let out. It’s scary and disconcerting. I drift off to sleep. Mum will take my daughter to school. At least I know she’s safe.

 

My mum comes in a couple of hours later and holds the cup to my lips so I can drink. Can I manage a shower? I’m desperate to get washed and dressed but it feels so daunting. Mum helps me to the shower and washes my hair. The water feels good. Mum helps me out. I manage to dry myself but dressing is beyond me. It’ll be a pyjama day. I manage the top but Mum has to put the trousers on me; leaning forward makes me reel. The effort of showering has exhausted me, but I’m sick of being in bed so Mum supports me as I shuffle slowly to the living room and the sofa. She makes sure I have plenty to drink, the phone, my walking stick and the alarm call button. She makes me some toast. I feel too sick to eat but I try. The food gives me some energy but my stomach aches; there’s not enough blood there to process what I’ve eaten. I try to watch TV but it seems so loud and bright that I feel I might explode. I switch off the TV and doze.

 
I wake with a start. What’s the time? By now it’s almost lunchtime and I realise I’m going to have to start making calls to cancel the plans I had for the afternoon. An appointment, a meeting, a coffee date – I hate having to flake. I call and explain I’m too ill to make it today. I talk slowly and it’s hard work. I’m tripping over my words, using the wrong word in the wrong place, slurring and stammering. I’m grateful I’ve taken the time to explain to the people in my life on the good days what a bad day can be like; strangers just think I’m drunk. I hang up, exhausted again and decide to shuffle back to bed. I call my parents first so that they know what’s going on. They confirm they’ll collect my daughter from school. I collapse into bed, barely strong enough to pull up the covers after the effort of moving from the living room to the bedroom.
 
It’s 3.30 pm and my Mum brings my daughter to see me. She hardly bats an eyelid at the state I’m in; she’s used to Mummy having days like this a couple of times a week.She tells me about her day and we hug. Then she goes back with my Mum to have her dinner and a bath. She’ll have to sleep there too. 
 
I’m stiff from lying still for so long so I decide to potter about the house a bit. After a couple of minutes I’m short of breath and so it’s back to the sofa until my husband arrives home from work to cook a meal I’m too tired to eat – using cutlery (even adapted) and chewing is surprisingly hard work.
 
At 8.30 pm my husband helps me into bed. Despite resting all day I’m still exhausted and in significant pain. As I fall asleep I hope to have a couple of good days before my next bad one.

A Day with Ehlers-Danlos Syndrome Part 1

I didn’t even hear my husband’s alarm go off this morning but I’ve woken up now my electronic pill box has sounded at 7.00. Groggily, I tip the pills into my hand and take them. The little white one is to help my body process whatever I decide to eat for breakfast and the orange one is to raise my blood pressure enough so that I can get up without falling over. I go back to sleep.
It’s 7.50 now and my alarm has just gone off. The meds have worked so I get up slowly, sitting on the edge of the bed for a few minutes until the room stops spinning. I walk wobbily to the bathroom to use the loo and brush my teeth.
At 8.04 there’s a knock at the door – my carer’s arrived. She helps me into the shower and then opens the curtains and puts away clean laundry until I’m ready to get out. I call for her help and she supports me as I rise from my shower seat and back to the bedroom. I’m able to dry and dress myself independently today which is great.
It’s 8.35 so go to collect my daughter from my parents’ house next door where they’ve been getting her ready for school. I can manage the walk to and from school today but I’m still very sore and tired by the time I get home. I’m feeling too nauseous for breakfast so I just sip water before setting off for the facial I’ve been looking forward to, thanks to a voucher I received as a gift! The facial is lovely, but it’s hard to enjoy it as by now my feet are burning and I’m starving from missing breakfast! I come out of the spa and buy a sausage roll before driving home.
Once home, I settle down to work for a couple of hours. My carer arrives at lunchtime but I’m not hungry (due to the sausage roll!) She hangs out the washing and washes the dishes for me.
Next it’s into town to do some shopping. I park at the supermarket first and then drive to the centre of town. Having completed most of my shopping I have to leave visiting the chemist as I’m too tired to walk there and I worry I might not make it to my physio appointment if I attempt it.
The physio puts me back together – my pelvis, rib cage and jaw are all out of alignment and my muscles are stiff and painful. I feel much better after the treatment and the physio comments on my improved colour!
I head home and have half an hour to work before I go to collect my daughter from school. I’m tired and my feet are still burning but I’m 80% confident I can manage the walk there and back nevertheless so I set off. Arriving in the playground I have to sit down as I’m really beginning to flag. I chat with a friend until the children come out.
It’s a Wednesday, which is when my daughter stays at my parents’ place overnight. She wants to go there straight away to play so I drop her off and say goodbye.
At home, I feel I should take the opportunity for a nap but decide I have too much work to do so I get on with that. At 6.00 my carer arrives and helps me into my pyjamas as well as closing all the curtains and bringing in the washing.
At 08.00, my husband returns from work and stir fries loads of veggies and I tuck in hungrily using my foam-handled Nelson knife (using a normal knife causes my wrist to partially dislocate and or sprain).
I start to feel spaced out and a bit vague so my husband helps me into bed. I lie there for a bit feeling ‘unreal’ and compulsively repeating the same meaningless phrase (my brain is lacking in blood flow) until gradually my circulation settles and I fall asleep.
This was a good day.

Fit to be fit?

Fit to be fit

I’ve never been one for exercise. As a child, my ideal activity was sitting playing or reading and outdoor games were similarly sedentary – playing house, making mud pies etc. School P.E. lessons did nothing to convince me to change my mind, particularly the hysterical life-and-death approach to so-called team games. It also seemed that if you were not gifted at competitive sports you were not entitled to proper teaching; my 6th Form games lessons were spent working out to the same Jane Fonda exercise video circa 1982, which, although mildly amusing (I can still sing the theme song), was hardly quality Physical Education.

I now understand that it was perfectly natural for me to feel disinclined to exercise; Ehlers-Danlos Syndrome means that tissues are weak, joints unstable and proprioceptive ability poor. The nausea, headache and shortness of breath I experienced were not signs of my lack of fitness or anxiety at having to partake in activity I found un-enjoyable, but symptoms of my autonomic nervous system being unable to cope with the demands of it.

One activity I had always enjoyed though was walking. I walked surprisingly quickly and in fact was well-known amongst my colleagues for being able to walk from Plaistow to East Ham in just under 15 minutes! My husband and I would go out walking at the weekend – not rambling you understand, but wandering around London with our book of guided walks learning all sorts of tidbits and of course stopping for the odd drink.

In 2006, all that changed. Suddenly, the one form of exercise I could do and enjoyed became a painful and exhausting ordeal with the threat of collapse constantly hanging over me. I started to avoid walking as much as possible. My extremely painful left leg kept giving way underneath me so I stopped putting weight through it as I moved. I propped myself up with my equally painful but slightly more reliable right leg and dragged my left behind me, stooping because of the pain and weakness in my back. I could walk only very short distances and relied on my husband (and occasionally a friend) to push me in my wheelchair (my arms were too weak and painful to be able to self-propel). I gave up on exercise completely.

In 2010, I was referred to the three-week inpatient Pain Management and Rehabilitation Programme at the Royal National Orthopaedic Hospital. They made it clear from the start that it would be hard work and that exercise is an integral part of the programme. I was desperate for life to be better and I took a leap of faith with both feet, determining that I would do whatever was asked of me. Each day in the programme began with gentle stretching. Well, it was called “gentle” but, as anyone with chronic pain who is unaccustomed to exercise knows, that translates roughly as “bloody agony.” But it was a routine and it did get easier. We also had individual and sometimes paired sessions with the physiotherapists and I benefited particularly from gait-training with the use of a walking stick. After three days my parents came to visit me and I walked across the ward to greet them. My Dad didn’t recognise me at first – he’d got used to seeing me stooping and had forgotten my true height. By the time I was discharged, after three weeks, I knew that regular exercise had to be part of my routine and I worked diligently at my programme.

By 2012, I’d become very ill both physically and mentally. I was on long-term sick leave and applying for retirement on medical grounds. I was disillusioned with physiotherapy and in any case, I was too depressed to re-commit to exercise. We moved in with my parents and they persuaded me to visit a local, private physiotherapist named Claire Sanderson who has a really good reputation locally for her work with chronic pain. Claire was, I think, pretty horrified at how much pain I was in and that I had been told repeatedly by numerous health professionals that nothing could change it – I was simply on a quest to try and learn to live with it. Claire told me that she could help me to improve my pain. I was sceptical (yeah, right lady!) but willing to try anything, and she explained that she would spend half of our first appointment assessing my lower body and the other half treating me. Yep, she used the ‘t’ word: this physiotherapist was going to treat me. Not assess and then tell me to go and live with it, but actually treat my symptoms. She touched me. She felt my tissues and manipulated my joints. It was agony (by this stage even the sensation of my clothes against my skin was unbearably painful at times), but I felt the whole-body cramp I’d been in for years actually begin to dissipate in that very first session. And better than that, she taught me right away an exercise to help relieve my leg pain. An exercise I could use as “first aid” whenever the pain was particularly bad. I quickly found that the exercises Claire prescribed made me feel better. It was refreshing and deeply satisfying to be exercising to improve pain rather than because “I know it must be doing me good on some level.” I started to look forward to my appointments with Claire because I felt so much better afterwards. My posture started to improve and Claire suggested I join her weekly class for people with chronic pain based on Pilates. It felt like such a ‘normal’ thing to do – to go to an exercise class – but with the bonus of knowing that everyone else in the room was struggling just as much as I was. With the weekly class, and by carrying out the exercises at home, I have been able, gradually, to space out my individual appointments with Claire so that I now see her just once every six weeks for direct treatment. But I can also book in to see her for the acute problems which are common in Ehlers-Danlos Syndrome.

About a year ago, I started to feel that I wanted to get involved in some kind of cardiovascular exercise. I purchased a pedal-exerciser on Claire’s recommendation and sit happily in front of the TV, pedalling away. I enjoy the buzz of endorphins I get from it and a few months ago I decided I wanted a bit more of that. I realised I wanted to join another class but it felt so daunting. For a start I’d have to explain my medical situation, and risk not being believed or taken seriously, because I “look fine.” Then, I’d have to actually go to the class and work out with people who aren’t necessarily disabled, ill or in chronic pain. It was scary so I did nothing. Meanwhile I was getting more and more overweight.

Then, one day, a good friend mentioned a Legs, Bums and Tums class she’d been attending. She said it involved a fair bit of floor-work so I figured that, as long as I could get on and off the floor, I could probably have a go. I took a deep breath and called the number she’d given me for The Fitness Factory. Nicola answered right away. She was super-friendly and easy to talk to. I explained my situation and she said, “Why don’t you come along next week and watch? Have a go if you feel like it and see how you go. And if this class isn’t right for you then we’ll help you to find something that is, even if it’s with another company.” It was the best response I could have hoped for. I went along feeling remarkably confident. I introduced myself to Andrea – Nicola had already talked to her about me. Andrea told me just to go at my own pace and to ask for help if I needed it. And that’s just what I did. Three months on and I now attend two Fitness Factory classes a week (as well as Claire’s Pilates for People in Pain group). Andrea and Nicola have taught me alternatives to the exercises I just can’t do (I’ve had to experiment and find what works for me) and they do this without making a fuss; I’m just one of the group. I hear Claire’s voice in my head the whole time reminding me about my posture and alignment (her ubiquitous cry of “bo-ttom!” reminding me not to over-arch my back but tuck my tail under instead). It takes supreme concentration, it hurts and very often my movements bear little resemblance to those of everyone else in the room (!), but I’m doing it. I’m getting fitter and I’m losing weight. And I’m regaining control over my own body. Nothing feels better than that.

Crafty New Me

In 2010, I’d been struggling with my health for some years. I had my diagnoses of Ehlers-Danlos Syndrome and Postural Tachycardia Syndrome and I was doing my best to get on with life. I was working part-time and taking care of my daughter (then 18 months old) the rest of the time. All my energy (and I had very little) went on my daughter and my work. I did nothing just for myself and I very rarely went out. When I wasn’t busy with parenting or work, I was sleeping.

I was fortunate enough to be referred to the Royal National Orthopaedic Hospital for their multidisciplinary Pain Management and Rehabilitation Programme. It was a big commitment as it required me to be admitted into hospital for 3 weeks (home at weekends). It was hard to leave my little girl but I realised that any improvement in my ability to manage my health could only be beneficial to her. My parents were fantastic and brought my daughter in to visit me twice each week.

I learned so much from that programme, and made some wonderful friends (maybe I’ll come back to all that in future posts), but one very important thing I learned was that it’s okay to use some of that precious energy to do something purely for fun. That to save all my energy for others and for work is not taking care of my own wellbeing.

Early on in the programme, the Occupational Therapy (OT) team asked us about our hobbies. I realised I had none. They suggested card-making so I gave it a go.

 
The first hurdle to overcome was being unable to use scissors without spraining my fingers – quite a hazard in paper craft! Being OTs, they quickly sorted that out by prescribing single-loop handled scissors and it was like a revelation! I actually had tears in my eyes as I cut paper for the first time in 4 years. And then I was hooked. It was therapeutic to produce something beautiful. Well, it wasn’t really beautiful but it was certainly something – my very first homemade card. I also found that using pressure gloves and finger splints increased my manual dexterity and helped with the pain of fine-motor tasks.
 
I started to think about all the other things I could do with my new-found skills – and ‘permission’ to spend energy this way. Four years on and I make all  the greetings cards we send as a family (well, except Christmas cards as there are just too many!) I also decoupage and I’ve recently started machine sewing (with lots of help from my very clever Mum). At Christmas, I print wrapping paper and gift tags with my daughter – she LOVES to craft so it’s a real joy that we can do it together (although it does mean a rather fluffy glue-stick and glitter everywhere).
 
I also enjoy a very active social life – well, lots of chatting over cake, if that can be called active! 
 
With chronic pain and fatigue, even fun is exhausting and can have its consequences for the next day but learning how important it is for my mental health was a really important lesson and I now take enjoying myself very seriously.
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What do people with disabilities look like?

Last week was Invisible Illness week! A great chance to raise awareness of invisible illnesses and disabilities. But what does it matter? Some illnesses and disabilities are visible and some are less so. So what?

It matters because people with invisible illnesses and disabilities are abused and discriminated against on a daily basis because of how we look (and the flip side is that people with visible disabilities also experience abuse and discrimination).

“The term invisible disabilities refers to symptoms such as debilitating pain, fatigue, dizziness, cognitive dysfunctions, brain injuries, learning differences and mental health disorders, as well as hearing and vision impairments. These are not always obvious to the onlooker, but can sometimes or always limit daily activities, range from mild challenges to severe limitations and vary from person to person. Unfortunately, people often judge others by what they see and often conclude a person can or cannot  do something by the way they look. This can be equally frustrating for those who may appear unable, but are perfectly capable, as well as those who appear able, but are not”. (The Invisible Disabilities Association)

This is mainly an issue (for me anyway) when parking with my blue badge. Apparently, holding a blue badge means, to many, that I should meet their preconception of what disability looks like. To look like me just isn’t good enough. From the moment I drive into the space, I’m expected to conform to someone else’s idea of what I should be. Since I don’t meet many people’s expectations of how people with disabilities look, I am subject to dirty looks, muttering and direct comment such as “you look fine; you don’t look disabled; I don’t know why you’re parking here; you can walk then” etc. One woman actually made a nasty comment to my (then) 4 year old daughter about me. I have to take a deep breath and brace myself before I get out of the car, just in case one of these narrow-minded individuals is about. 

I’m lucky though – I haven’t experienced serious abuse and I haven’t been physically assaulted. But it’s enough to make me feel very vulnerable when I go out alone. During a hospital visit a few years ago, I met a lady with Multiple Sclerosis who had been followed into a supermarket by someone shouting obscenities and calling her a scrounger. She was understandably terrified and sought the intervention of supermarket staff (who were brilliant and chucked the nasty person out of the store).

Of course, parking isn’t the only time this prejudice raises its ugly head; I’ve experienced the same problem when using my wheelchair – particularly when going from being in my wheelchair to being out of it. Some people seem to think that it’s all or nothing with a wheelchair: if you only need to use it some of the time then you don’t really need to use it at all.

Unfortunately, members of the medical profession, social care. local government and DWP workers sometimes hold this misconception too, which means we often have to fight very hard to access the help we need. The medical history of people with invisible illnesses and disabilities is often characterised by repeated visits to professionals where our symptoms are written off as all in the mind. Ehlers-Danlos Syndrome and PoTs don’t show up on scans or blood tests. I spent many years repeatedly labelled as a hypochondriac by my GP and my teachers – since my symptoms weren’t visible they decided I was either making it all up or mentally ill. Not surprisingly, I actually became mentally ill with depression as a direct result of being disbelieved. Worse than that I began to distrust myself. I’ve since learned that this is absolutely typical of the path to diagnosis of an illness or disability which is invisible.

Pain, dizziness, weakness, fragility of tissues and a whole host of other symptoms and difficulties are often totally invisible to the naked eye. It doesn’t mean they’re not there. And of course, non-physical disabilities e.g. cognitive and communication disabilities can be even harder to spot.

The concept of not judging a book by its cover is such a cliché and yet we do it every day.

What do people with disabilities look like? People.

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Working for me works for me

I’d never considered becoming self-employed. If I’m honest, the idea always scared me. People would ask if I’d ever go into independent practice as a Speech and Language Therapist and I’d give an emphatic ‘no.’ But then, I never imagined I’d become disabled and that my life would take a wholly different route from the one I’d expected.

 
When I was forced to retire in 2012, I was very unwell. I knew even then though, that I’d have to find some kind of work I could do – I’m someone who HAS to work and I couldn’t contemplate a life without employment of some sort. In one of my previous posts, I talked about voluntary work and how much it has helped me to rehabilitate and to look to the future. But I also find myself running a Social Media Management business and in the early stages of (hopefully) setting up in independent practice as a Speech and Language Therapist.
 
So what happened? Well, first I contacted a friend for whom I used to work in an entirely different capacity. Was there anything I could do for her at home that was low-stress? I emphasisied that I didn’t want charity, but to be useful and productive. She answered immediately: I don’t know how to keep up with Facebook. I know my business needs it but I have neither the time nor the skills.  And so I started managing her business’ Facebook account. And I loved it.
 
Not long after, I took my daughter to the library to choose some books. We arrived early (that’s kind of a pathology with me) and had to wait for it to open. There I was, hopping about from one foot to the other to keep the blood pumping (a complication of Ehlers-Danlos Syndrome can be Postural Tachycardia Syndrome) when a friendly voice said, “I love your dress!” I looked up and saw a woman about my age with two beautiful daughters about the same age as my little girl. My initial impression was of butterflies, feathers, a trilby and a big smile. I liked her at once. We got chatting and found we had chronic illness in common. We swapped numbers (I don’t usually do that with people I’ve only just met – it almost felt a bit dirty; like I was picking her up ;)) and for ages she was in my ‘phone as “Jo Library.” It was when we met at the park so our girls could play together that I discovered that life, with its sometimes poetic synchronicity, had found me a friend, just when I was starting to get interested in Social Media, in none other than the Dexterous Diva, Jo Gifford herself. 
 
I checked out her website and signed up to her mailing list. I enrolled on her courses “Creating Brilliant Blog Posts” and “Idea Generation for Badass Bloggers and Content Creators” and found myself completely inspired. I started to feel like I could build a business in Social Media Management; like I had a career opportunity here. Here was someone so like me in so many ways, making it work. I started to feel that I could make it work too. I decided to look for another client and was fortunate enough to strike it lucky straight away. I’d registered as a business for tax purposes when I first started working for my friend’s business but now I felt I had a real, proper, actual business that could really go somewhere. Victory Online Social Media Management.
 
My business is still young (and small) and I’m learning all the time. But thanks to the Dexterous Diva I’m making working for me work for me. Working from home means I can manage my symptoms and listen to and respond to my body in a way I just couldn’t working elsewhere. I’m here for my daughter and husband and I enjoy a rich social life. And now I’m looking to the future and a return to Speech and Language Therapy as a career – on my own terms.
 
Meeting Jo was an enormous boon professionally, but she has also given me the gifts of self-belief and hope. What could be more precious than that? 
 

Why resting is such hard work

One of the hardest things about having a chronic illness is needing to rest so much. Doesn’t sound so bad? We all enjoy a little R&R right?

Sure, but enforced rest because your body has ground to a halt when your brain’s still raring to go is no fun. When you have stuff to do, when people are depending on you. When you made a promise, a commitment and then a bunch of chemicals, proteins, genes, whatever, decide for you that it’s just not gonna happen.

And then, once you accept you’re just going to have to lie down, you surrender to another labour: trying to get comfortable. Chronic pain does not make this easy and with Ehlers-Danlos Syndrome, there’s barely a position where some joint doesn’t end up out of alignment. If longer periods of rest are required, muscle stiffness and weakness becomes an issue and a kind of mini-rehab has to be undertaken before you can get up again – gentle stretching and repositioning of joints that have ‘popped.’

I used to spend these rest periods frustrated; resenting my body and begging it to just step up and do what it’s supposed to. At the same time, I would dissociate from my body and perceive it as a cage, trapping me into an inert, passive existence. Rejecting my body in this way meant I was at war with it. With help, I have learned a different way. A psychologist introduced me to Mindfulness which allowed me to connect with my body again, to accept my symptoms as being part of myself and to feel compassion for my body in its pain. Now I work with my symptoms rather than against them. I take comfort in knowing that when I’m resting, I am taking care of myself and I’m teaching my little girl to prioritise taking care of herself and her health. I hope, a legacy of love. why resting i s such hard work (1)