The Invisibility War

“You look really well” those four words again, the cynicism builds within me.
‘Do they think I’m making this up or something? I may look okay but I certainly don’t feel it or maybe they are just being nice”.

 
In society, there is a mainstream idea of what illness should look like, how long it should last for and expectations of recovery, when you will suddenly become ‘well’ again. Unfortunately, for those living with chronic illnesses we will never meet those expectations, we may fluctuate within them, learn new ways of managing our conditions, go into ‘remission’, our symptoms may wax and wane but we will not get ‘well’.

 
Many chronic illnesses whether it be EDS, depression, anxiety, lupus or Crohns disease, to name but a few can be invisible either on the most part, on days, weeks or even months. This invisibility can make it extremely difficult for even those close to us to be able to relate, acknowledge, understand and empathise with our individual daily battles with illness. It can be hard for people to relate when something isn’t always apparent or visual. However just because you are unable to see it does not mean it is not there, it’s very real.

 
It is estimated that 69% of those with invisible illnesses were told their symptoms were down to anxiety or not even believed, before eventual diagnosis. Many people with invisible illnesses have spent years being disbelieved, poor treatment and care so you can see how the words of others can have such a significant impact on their wellbeing.

 
Over the years, I became somewhat a master of hiding behind my pain. I did not have a name for all the symptoms I was enduring, I could tell at times certain people doubted the validity of my symptoms and suffering. It was somewhat easier to hide behind a smile than attempt to explain just how unwell I was feeling when overall physically, I looked fine. I even took this stance often with certain doctors or consultants as I felt their willingness to help was not there, they could not find the cause of my problems therefore to them they did not exist or exist in the capacity I presented to them.

 
Since my diagnosis’s my attitude has changed to a certain extent, I am much more open about how I feel both physically and emotionally, I no longer actively hide my symptoms behind a smile, I choose too at times. Validation of a diagnosis can help enormously in the ‘believing’ stakes but not necessarily in the ‘understanding’ stakes. Living with a chronic illness is unpredictable, literally not knowing what is coming hour by hour, even minute by minute. It is a hard task constantly adjusting to this unpredictability. What we can do one day we may not be able to do the next day or even again that same day which those without illness can, not always, but can find hard to comprehend. If we have one activity that day (usually hospital) everything pivots around that. We could be seen out ‘looking well’ but it’s not seen that to enable us to make that outing we had to stay in bed until it was time to leave, we couldn’t possibly wash our hair too and afterwards when we get to our homes we crumble with pain and symptoms, all behind closed doors. When you’re seen out, it’s just a snippet, a snap shot, a moment, not your actual reality.

 

In life, we only see what we want to see and only let people see what you want them to see. We often live our lives through many different faces and smiles that may not necessarily reflect how we are feeling. That good old British stiff upper lip!!!

I am ‘a smiler’ it’s my way of coping, almost like my default mode. I don’t want the conditions to define me, I want to hold onto as much of ‘Ali’ as I can. I will often feel terrible but put my make-up on and some nice clothes just to sit in my house for a feeling of normality and to break up the horrendous ‘my heads falling off hobo look’ of late!! I may take a ‘selfie’, I may post on social media, you may see me smiling however don’t be fooled by the smile, it does not mean I am healthy and miraculously cured. It means I am happy in that moment or I am trying my best.

 
It’s strange that we all, myself included on the most part relate more to the visuals in life. Some days my condition can be very visual, will I let you see that? On the most part, no. I’m not ashamed of my illnesses but I still feel uncomfortable allowing people to see me look so sick, on these days, moments, weeks, I will stay in. I could be in numerous braces to steady my joints after dislocating, my neck brace on, using my walking stick or wheel chair, underneath the make-up looking like I’ve done 12 rounds with Mike Tyson with black eyes from brainstem compression, black and blue with bruises, resembling a real-life mummy wrapped in KT tape and on those days, I find I don’t actively talk about my symptoms as much because they are VISIBLE, often speaking for themselves. Does this mean the days I am not in a wrist brace, means I am pain free? If only, the pains in my wrist are constant it just means on the day I’ve worn the brace the bones have disconnected and its dislocated so I need to rest to it.

 
I would say for anyone living with chronic illness, whether invisible or not to go easy on yourselves. Do not feel you need to justify your pain or your symptoms. What you are experiencing is real. People will never fully understand, even people with the same conditions as you, as we all experience pain differently, we all have different symptoms and coping mechanisms. Our lives are already filled with challenges, we may also be in pain from a life un-lived or lived how we had not imagined so be kind to yourselves always.

 

It may at times be invisible but that does not mean it’s not there. See into the invisibility and help make our invisible, visible.

 

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Where have I gone?

Cushions strategically placed; under my knees, two behind my back, one around my neck, one either side of my shoulders and one under each arm, finally after looking like something from a Bensons for beds advert I’m ready to start blogging from my bed. Five minutes in I must stop, my shoulder feels like its hanging by a thread, wrist throbbing, head pounding and eyes stinging. Frustrated I reluctantly place my laptop to one side, resigning myself to the fact my body will not play ball. I quietly tell myself those 4 words that have become somewhat regular in my vocabulary “I’ll try again later”.

 
Imagine starting each day with a certain number of spoons, sound strange? The spoons are a metaphor, they are a measurement used to track how much energy you have throughout the day. Each activity requires a certain number of spoons which can only be replaced when you recharge until the next spoon becomes available, this could be minutes, hours, even days. Bear in mind you don’t have an unlimited supply of spoons like a healthy person, you’re very much rationed on the said spoons. You don’t have an unlimited amount of possibilities or energy, you are unable to do activities without effects and consequences. You have constant choices to make and consciously have to think about things the majority don’t. Spoons are used for the most mundane of activities such as taking a shower, drying your hair, getting down the stairs and even being upright. You can see how rapidly your spoons are consumed within the first hour of the day. Frustration builds as you have run out of spoons, you’re stuck in bed, waiting for the next spoon to arrive so you can load the dishwasher. Suddenly every aspect of your life is a challenge. Everything that was once simple, not even entering on your radar becomes a task, difficult, hard work……. a spoon taken. Every tiny aspect of your life must be thought out and planned, for every action no matter how small there is a consequence. Living your life with a limited and unpredictable amount of spoons each day isn’t easy. There is always a block you have to navigate whether it be adapting an activity as simple as taking a shower, allowing someone to help you or simply having to wait until the block decreases in size and you can just about clamber over it. You can imagine with the number of spoons used up on what were once, easy everyday tasks that there isn’t too many left for doing the things you love, the things that make you, you.

 
Over the years my ability to live a ‘normal’ life was fading yet I didn’t know why. The same flakey response “I’ll have to see how I am” when asked to do something. I found myself constantly having to justify my symptoms and just why I had to see how I was. I didn’t have a name for my symptoms but I knew the ability to commit to anything had gone. I had no control over my body from one day to another. I felt sitting on the fence with the above flakey answer was far better than 100% committing only to let people down or outright say no as on the contrary, I didn’t want to miss out. If I managed to make said plan, military planning would ensue “I can’t come for the meal but I’ll meet you after, I can’t drink either but I’ll have a brew”, “I won’t come for the meal but I’ll meet you at the cinema”, everything had to be altered in some way to enable me to make it. Boy did I then suffer for days after, I knew it wasn’t normal to be so sick, exhausted, pain so high from sitting in the cinema or going to bed at 12pm , nothing majorly taxing for someone in their late 20’s\early 30’s. Weddings and Christenings would be hard as they were long days, I often sat at the table as people were tucking into the wedding breakfast whilst I handed my food over to Nick (he loved it) filled with paranoia that people were watching me and wondering why I wasn’t eating. Fearful people thought I was being rude and shunning the gorgeous food before me, feeling awkward as to where to put my face whilst everyone was tucking into their food like normal people. I would then slip off to the car or hotel room if we were staying over and tuck into a light gluten free snack and having to wait a while for the pain to die down. Everything was hard work, I was so scared people thought I was being fussy and awkward that when an invitation came in a sort of dread and anxiety came over me. I found it difficult because before I got ‘really’ unwell 7 plus years ago I would eat anything, attend the opening of an envelope as I didn’t want to miss out, loved a glass of red, would do anything for anyone and would well and truly go with the flow. I had experienced that life, that fun, those happy times and now they were becoming few and far between. I missed so many outings, weekends away, holidays, gigs, meals out, everything social that brought people together was passing me by, my only connection through looking at the pictures on social media. I would often cry, get angry and bitter that I had missed yet another event. I began to feel like a spectator of life, it was lonely.

 
In recent years as my capabilities have diminished further It feels like I am watching myself go further into the distance, out of reach. Each day my world gets smaller, I don’t recognise myself. Im a million miles away from who I once was. I feel lost.

 
What makes us who we are? Apart from the obvious genetics and without getting too deep into psychology. What are some of the building blocks and fundamentals that help form our individual personalities? Hobbies- the things we like to do in our spare time often to relax, break a tough week at work up and the things we take enjoyment from. This might be going for a few pints with friends, painting, photography, running, swimming, going to Old Trafford (of course) to watch the footy, spa weekends…. the list is endless. Our network of friends- chatting with them, socialising with them or discussing the latest episode of line of duty, our ability to function and communicate. Our jobs/careers- how they make us feel, a sense of purpose they may give us not to mention the wage to ensure you are independent and can take care of your needs. Our families, children, our individual looks, hair colour, fashion- whether you’re a jeans and t-shirt girl or a glam chick, it’s part of you. We all hold these various blocks in different regard, some are more important to us than others but essentially, they are a variety of blocks that make us who we are. Imagine those fundamental blocks being taken away from you one by one like a game of Jenga. Eventually and inevitably the tower you’ve spent your whole life working hard to build falls, the structures lost, this is no different to what it’s like for people living with a plethora of chronic illnesses, people like me.

 
I held my job in high regard, I worked hard and I was good at what I did. I felt very lucky that early on in life I knew what I wanted to do and that was to help vulnerable children and families. I had 14 glorious and at times trying years doing this in various capacities. It gave me great pride and a sense of achievement every single day, this was what I was meant to do. Being too unwell and losing my job was devastating. A devastation I kept very much to myself, there was so much going on with my health there wasn’t room or time for even more emotions then. At this point my world felt like it had crashed at speed, I’d lost everything I had worked for. I had grappled onto my job and worked through the most awful symptoms and tests the best I could for such a long time but I could no longer manage. For those that know me well, I do not give in or give up easily so when my hands are held up it really does mean the white flag is out, I surrendour. I cried for days, I felt I now had no purpose and no position in society. I loved my job and it was another thing robbed, at times I felt angry and bitter. The ‘why me’ and ‘it’s not fair’ came out a lot. I worried what people would think, I had never been without a job in my life, sometimes having three on the go, would people think I was lazy? A scrounger? Always being financially independent, being able to purchase what I wanted, within reason obviously (council wages aren’t huge) I suddenly found myself without money coming in, no monthly wage hitting my account. Although only money, when you’re used to having your own and taking care of yourself the truth is I felt degraded, I was indepenant and now suddenly my partner was having to take on full responsibility of our home, mortgage, bills and other outgoings, it felt rotten and there was absolutely nothing I could do about it.

 
Before the illness really took hold 7 plus years ago, I enjoyed going out for meals, drinking, partying, random weekends away on the spur of the moment, running and horse-riding. When I was at college my dad used to say ‘You’re burning the candle at both ends’ I’d race home from a 12-hour shift on a Saturday at Tesco to be greeted by a big pizza and a bath run curtesy of my fabulous Mum. It was like challenge Anika, I Would have a 45-minute turnaround to get said pizza consumed, bath, hair done and makeup on before I met the girls at the Hare and Hounds or ‘the dogs’ as it was nicknamed, It wasn’t a normal week without going there at least three times!! As the years passed and these activities became harder to undertake I sort of ‘trouble shooted’ what I could do from home to keep myself occupied. I began crafting and I loved It, it was relaxing, it took me into a little world away from my symptoms. I made items for people and people were very complementary. As time went on I found I had to change the craft projects, they evolved along with my symptoms. My head and neck became so painful that knitting and button pictures were out, next to be struck off was my sewing machine, painting and drawing. The final craft I tried was candle making until my shoulder continued to buckle and tremors too great to carry and pour hot wax. I valued my nice kitchen and my skin!!! All the crafts I did through pain from the start, but there are levels of pain and when the pain was too great I knew it was time to hang up my needle and thread. I had tried to create a positive from being unable to go out and now I was unable to even do this. “I may as well just sit in bed and not move, I can’t do anything”. Angry, upset and defeated. Everything had been taken away, everything.

 
Socialising with friends out of the house was out, my days taken up with hospital appointments and pain that demanded to be heard my only option was to have friends round. Even now I have to cancel as the symptoms are all too great, flakey Ali again. Letting my friends see me behind closed doors was hard at first, I felt embarrassed, they never made me feel this way, this was my problem. I could no longer go the hairdressers, Nick became the new Nicky Clarke, often too poorly to wash my hair and put my nice clothes on I resembled Worzel Gummidge!! I’d lie flat and talk with friends, catching up on their life and them on mine. My voice echoes in my head when I talk so it’s not easy having a conversation and because at present my hearing is disturbed from the compression I can either shout or whisper unbeknown to me!!!

 
Just from the limited examples above I hope they can give you an insight into just how it feels when those building blocks are taken, the fundamentals of you gone. You feel a great sense of loss. I look over old photographs with mixed emotions, I cry at the thought of not being able to do that, dress like that ,look like that and at the same time look on them with happiness that I did actually get those chances, as many don’t. It’s a sort of grief, have I come to terms with this? Absolutely not. I would be lying if I said otherwise. I always wanted this blog to be a true reflection of my journey and that includes the good, the bad and the ugly. I still feel angry, frustrated, sad, an actual empty pain in my heart and even jealous at times, I don’t think I’d be normal if I didn’t think and feel those things but at the same time I have hope. I have to believe I will get a better quality of life back, I have to hold on tight to that hope everyday as without it I may as well give up now. Do I feel like giving up? Of course, “I can’t carry on like this”, “I can’t do this anymore its cruel” however I have not been brought up to give up. No matter how hard this journey, I will continue to pick myself back up and get back on the path. I always refer to one of my favourite quotes “I don’t know how my story will end, but nowhere ever in my text will it say ‘I gave up’” I have too much I want to do with my life, too many hopes and dreams.

 
Although my body is unable to keep up with my minds desires, I still have hopes and dreams like anyone else. I’d say they have changed over the course of two years and become what you’d class as a bit ‘none grande’ instead of my lifelong dream of going to the Seychelles and staying in one of those beach huts on stilts, I now dream of the day I can get out far enough to Saint Anne’s Beach and feel the sand under my feet, granted I may step on a shard of glass from a bottle of Becks!! but that is the hope right now. My current dreams are more realistic in order not to set myself up to fail, for further disappointment. I have a little bucket list going and each week something else is added. I take myself off there in my mind and imagine what it will be like and hopefully one day that image will become reality.

 
When faced with so many challenges your priorities change, as cliché as this sounds you realise just what’s important in life. It’s not possessions, houses or holidays it’s the people who truly stick by your side in your time of need. I am very fortunate to have the best family, my parents, brother, sister in law, my partner Nick, his parents and sisters would do anything for me. They understand the conditions, my limitations and constantly support me, as do a very close network of friends. When you take away the shell its who’s inside and who stays that really matters.

 
This isn’t how I planned my life to go, this definitely wasn’t a destination I placed a pin on my map to visit but it’s where I am. None of us in life know what’s around the corner, life isn’t fair at times and life can be cruel. I’d say it’s how you take on what’s placed upon you, how you tackle it, fight through it and more than anything have hope and believe a better day will come.

The POWER of people

I sat in disbelief, faced with the daunting prospect of fundraising, I could not fathom what was happening both with my body and the fact the surgery was not undertaken in the UK. We had at the least £55k to raise to make this possible, whilst my condition continued to decline. The mountain that I was already trying to clamber, suddenly became much steeper.
On the evening of 27thOctober 2016 myself and Nick opened the crowd funding page. Sat at home we thought of the name ‘Ali’s fight for fusion’, we worked together on writing the information for the page. We initially found this quite a challenge, there was such a long history regarding my health and what brought us to having to fundraise that condensing it seemed impossible. Emotions were high as we went through everything, many hours later we had finished. We read it repeatedly, it was imperative the seriousness of the situation came across along with a brief history of what brought us to this point. I had such a sense of unease, very weepy knowing in the next few minutes this page would go live and be out in the public domain. On the whole I am quite a private person, this wasn’t just going out to my friends list on social media this was a public page, open to all. I suddenly felt very vulnerable, unsure of how the page would be received, worried what people would think of me and the true extent of my health issues out there forever. At the same I was determined to fight for my life, a better life and to raise as much awareness as possible of these rare conditions.
Enter…the page went live. I remember walking to the kitchen with my cup of tea, sitting at the table staring into space. It felt surreal, never did I imagine I would be in this situation. This is something you read about and donate too, not something that happens to you. All the charity runs I had participated in over the years, raising money for causes close to my heart and now the tables were turned. Nick called me into the room, gave me a cuddle and told me to look as he turned the computer screen towards me. Within minutes the page had been shared countless times, donations and messages of support came flooding in. Tears rolled down my face as I began to read the heartfelt words from people.
So many people came forward with the most amazing fundraising ideas, messages of support and continually shared the page on social media thus spreading the word further afield. I was taken aback by the response from friends, old school friends, past work colleagues to people I had never even crossed paths with in my life.
Within the week media were contacting Nick asking if they could cover the story. I didn’t like the thought of being in the paper but knew it was essential to spread the word and raise awareness. Nick had to give me many’ little talks’ of encouragement along the way, amazing chappy. Nick spoke with a journalist from our local newspaper which was the first article to go to print followed by journalists from other papers. Within a matter of 10 days the story had made 3 local newspapers and national news online. It felt very strange seeing my face in the newspaper and reading each article, again like I was reading about someone else. No matter how hard I tried I just could not get it to sink in.
The week we returned home from the consultation in Barcelona a reporter and cameraman from Granada came to the house to interview myself and Nick. I was exhausted from the trip; my symptoms were out of control. I had to dig deep as to get some coverage on regional news could really help the fundraising and awareness. I was grateful for the opportunity. Both the reporter and cameraman were lovely, they instantly made me feel more at ease by saying they were just going to informally chat with us about the condition and fundraising. The cameraman had set up, I remember him struggling with the glare coming off my glasses, he had asked if I could take them off for the interview “I can but I’ll be talking to the wall, I can’t even see the reporter sitting right next me” I said, we all had a giggle and decided the specs were to stay firmly on!! We chatted for well over two hours as the reporter made notes and asked questions, sometimes he asked the question a few times as I was nervous and my voice a bit wobbly. I went through everything that had led us up to having to fundraise, knowing full well 95% of the content would not be included as this was going to be a very short segment. Throughout the time, I thought the reporter was gathering information and getting me used to talking with less nerves however to my amazement when I thought the ‘practice’ was over and we would do the interview I was told we were finished. They explained they will cut/edit and piece an item together when back at the studio in the hope it would be aired that evening. Sure enough it was aired that night and cut short due to the football, absolutely fine by me I thought as I could not bring myself to watch it. I sat with Nick on the sofa whilst he watched it and I covered my eyes, not many people like to see themselves on TV. Within minutes the segment had been shared on social media by many people, still having not watched it I found myself in tears again. It felt very surreal, I took myself off for a little cry and had ‘words’ with myself. I told myself to toughen up a bit that the story was out and people were helping and wanting to help, this was positive. On reflection, I think my issues were more with the fact I am such a proud person and having spent years hiding so many symptoms that suddenly I was in the public domain with an unmissable neck brace on, looking quite thin and the true extent of my health issues finally out there, it made me feel quite exposed and now I couldn’t run away from what was happening.
Over the weeks and months countless people came forward organising fabulous events. Never did we envisage the level of support that we received. Friends, family and people I had never even met generously gave their time, effort and love to host various successful fundraisers. Everyone was mucking in and helping in any way they could. It was very emotional for us to see first-hand the lengths people were going too to help and I can assure you many a tear was shed over the fundraising period, I was so overwhelmed and taken a back. Although I could not attend the majority of events due to my health I took great joy looking at the pictures when they came in of people having fun, giggling, munching on cake, having a drink and a good chat, zombie walks, teachers being pied by students, people cycling, swimming, playing football, walking in gale force winds on Blackpool front, auctions, collecting in the community, beard shaves…the list is endless. More than anything, I loved the fact it was bringing people together.
I will never forget the day I was on route to the hospital, stopping for some water at the shop when a dear lady came over to me and placed £20 in my hand saying she had seen the story on the news but didn’t know how to donate online. I told her I could not accept the money but thanked her so much. The £20 note was passed between us for a few minutes to the point we both started laughing and the shop assistant said he would settle it by taking it himself. I gratefully took the kind donation and gave the lady a big hug thanking her from the bottom of my heart for being so kind and thoughtful. We had a little chat together before I had to make my way to the hospital.
Over the month’s cards would arrive through the post, sent from my local newspaper office where people had gone in with a donation as they did not have access to a computer. I was so touched that people had taken those lengths of making their way over to the office and handing in what were the loveliest cards with very moving words inside.
Donations continued to come in and I made sure I looked at each and every name and in my head gave a little thank you each time. So many names were unfamiliar, donations from total strangers that had read the story and taken the time to donate. It meant so very much.
In November, a shock anonymous donation of £10k came in, we could not believe our eyes. Initially we thought they must have entered too many zeros on entering the amount and were awaiting contact from the crowd funding site we had used to tell us it had been a mistake, no such call occurred. We sat in astonishment, again tears from many, this gave the fundraising such a boost. Around this time we thought we could actually make this target, we could do this. The newspapers took the anonymous donation story on and again it was shared around social media keeping up the momentum.
My family, Nicks family and friends went out into the community handing out collection boxes, posters and events posters. So many local businesses kindly agreed to have them in their shop, the staff were lovely often asking how I was doing and what else they could do to help. This was amazing, not only increasing donations but also raising much needed awareness.
Awareness….one of my main aims. As the fundraising went on I found myself receiving more and more messages on social media from either people with EDS wanting to connect and chat which was fantastic to quite a few parents contacting me with concerns about their children’s EDS or suspected EDS. I would respond with advice to the best of my knowledge and sign post them to EDS savvy consultants around the country to try and save them some time on the merry go round. More and more people were talking about EDS and sharing articles on social media. This meant an awful lot to me as EDS can be a very invisible condition, yet extremely wide spread and cruel, the fact people were coming across articles and recognising those three letters meant awareness was actively taking place.
Having to fundraise is such a daunting prospect, you worry you won’t make the target, you worry what people will think of you, you feel exposed and vulnerable, stressed you can’t help more and get involved, sometimes even feeling guilty. However, as time went on it became apparent that nothing but positivity came from our fundraising experience. As I got used to the fact my health conditions were now out for all to see and read about, I realised I have nothing to be ashamed of, I have not asked for these cards to be dealt and I’m trying to play them to the best of my ability. A weight felt lifted from my shoulders, all those years of hiding symptoms and pretending to be normal it was a fulltime job in itself, that could now stop. The real driving force behind the change in my thinking came from the way people came forward with heartfelt messages of love, fundraising ideas and donations. I don’t think I will ever be able to articulate just what that has and does mean to me. It has been the driving force in keeping me looking and moving forward, never underestimate the power of human kindness because for me it has been a magical medicine on those days I feel I can’t do another minute with the pain.

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As I write this post we have reached £65k and far exceeded our initial target. We are so grateful for this as a few months back we were told our crowd funding site take 8% from each donation and with insurance to purchase and after care the extra money raised will go towards that.
We have been very fortunate along the fundraising journey that an abundance of people came forward to donate, fundraise and offer there unconditional love and support, it is something we will never forget. I am quite a sentimental girl at heart and have started to make a scrapbook documenting the fundraising journey to be able to look back on all the amazing people who supported us.
Nick has put together a short montage of some of the fundraising events that took place over the 6 months, we hope you enjoy watching as much as us. A lovely reminder of the generosity of many.

 

 

 

 

Barcelona Bound

Deep breaths in and out as we taxied the runway, trying to keep my nerves in check. I hadn’t flown in years and knew it was risky with my condition due to intracranial hypertension. I’d already achieved getting to the airport and through check in, this was the next leg. Braced up I closed my eyes as we took off.

A few weeks earlier I had sent my images off to Dr Gilete in Barcelona for review. He had reported Craniocervical instability and recommended a consultation to discuss my measurements and findings further. The lead up to the trip was awful. I did not know how I was going to make it, I knew how unwell I felt going 5 miles down the road to an appointment. Weeks of nerves and military planning ensued.

Feeling the bumps of the wheels hitting the runway hurt my neck but at the same time a huge sense of relief came over me, I’d got there. I’d had a worsening pressure headache about one hour in from the altitude but nothing I couldn’t handle. We collected our bags and headed straight for a taxi to our apartment about 30 minutes away from the airport. Once we reached the apartment I was so proud I’d got there. Any kind of travel and movement is diabolical on my body. I break everything down into stages in an attempt to make it more manageable. I stayed in the apartment for the evening laying flat, my nausea was at an all time high. I went through the various questions I had for my consultation the following day.

Sat with a cup of tea I read my messages from people wishing me luck for the appointment. I hadn’t slept a wink, not a wink. Often adrenaline kicks in when I have to travel which is hard to switch off. Around 2 am I resigned myself to the fact no sleep was taking place and I lay on the sofa with a dim light on staring at the ceiling with the same questions running over and over – When did it all come to this? Why was all this happening to me ? I couldn’t compute the last 18 months.

We arrived at the hospital, the grounds were beautiful donning tall palm trees on either side. As we walked into the entrance we felt like we had walked into a hotel lobby with marble floors and the sparkly clean appearance. We were guided back to the villana entrance and made our way down the stairs to Dr Gilete’s consultation room. After a very short wait we went in. Dr Gilete shook our hands, he was a very warm natured man. We asked if we could record the consultation as I was unable to take everything in at present. This was not a problem. Dr Oliver then entered and we were introduced, again a lovely gentleman.

Dr Gilete guided the consultation initially asking me to discuss my symptoms. I explained to both Drs that I had headaches 24/7 based in the back of my skull with a pulling down sensation and neuralgia. My temples, face, eyes and teeth often had pressure and pulsating pains.  Frequently I could not talk for the pain, my mouth would droop and I would lose sensation in the right side of my face. I went onto explain about the severe vertigo in detail and constant dizziness with the feeling my head is not attached to my neck. I described my head as feeling like the weight of a bowling ball and it physically hurt to stay upright.  Pins and needles and loss of sensation on my right side of my body and I had began dropping items. My nausea and dysautonomia had worsened in recent months. Erratic heart rate and oxygen levels at times. The visual disturbances had increased along with a change in hearing. I told them my neck pain was now 10/10 the muscles like rock, constant muscle spasms and pain deep within the joints. Shoulder pain and weakness in the shoulder and arms, particularly my right arm. I could not bear anything touching the back of my head as the pain would intensify. Choking had become an issue particularly on liquids. I was having trouble recollecting words during my conversations.  “I have lost my life, having to spend 80% of each day lay flat and still”, I said. holding back the tears. Both Dr G and Dr O listened intentivly as I spoke and gave me that time to share my symptoms.

Dr G already had my medical history and existing conditions to hand as I had to send those over prior to the appointment. Dr G said he wanted to hear me explain my symptoms to ensure they correlated with the imaging of which he said ‘they most certainly do’. Dr G went onto explain my images, going through them individually on the screen. He explained that Craniocervical instability was defined by a set of measurements that reach what they refer to as pathological.

Below are a few examples of my images, there were hundreds taken.

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Image of my cervical spine in neutral. The area marked is called the ‘Grabbe Oakes’ measurement. The angle is drawn from the basion to the tip of the posterior inferior C2 Vertebra. Anything 9mm or above (8mm with some surgeons) indicates ventral brainstem compression. In neutral I am 9.2 mm and in flexion 9.4mm thus suffering ventral brainstem compression.  The odontoid (the dens/peg which serves as a pivot point for turning the head) is retroflexed (kinking back) and a mass of inflammatory tissue has grown around the joint known as a pannus formation adding further pressure on the brainstem.

 

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Image of my Cervical spine in flexion. The angle marked out is called the Clivo-axial angle. This angle should be between 150-170 degrees. Anything below 150 is abnormal and below 135 degrees is pathological. Mine is 131 degrees.

 

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This measurement is called the Atlantodental interval (ADI).  This is the distance between the arch of the atlas and dens of the axis. Normal measurements are below 2mm and mine is 5.4 mm. The ligaments within my neck are weak and failing to work as they should causing the atlas to move too far forward on the axis narrowing my spinal canal and causing impingement of my spinal cord.

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Above is some measurements that go towards the Harris measurement. This measurement quantifies how much your skull is sliding in translation. In a ‘normal’ person there should be NO measureable translatory movement. A translation of greater than 1 mm between the basion and odontoid reflects craniovertbral instability. My skull slides 2.3 mm.

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Axial cut which shows I am dislocated even in the neutral position 9.8 degrees this then becomes greater on rotation left and right.

Both Dr G and Dr O explained how there is an ‘abnormal’ category of measurements and then there is ‘pathological’ they told me every measurement was pathological and in no uncertain terms I had Craniocervical Instability and Atlantoaxial Instability. They went onto explain to Nick and myself that this is both progressive and serious in nature. The main concerns being permanent damage to the nerves and spinal cord, the fact my brainstem which controls so many functions within the body is compressed and the risk of vertebral artery occlusion on rotation. As I rotate so far (over double the norm) and subclux I am kinking the artery which interrupts blood flow to the brain which can cause stroke and even death.

I sat in disbelief, when given the opportunity to ask any questions my mind went blank, despite having an A4 list of questions in front of me I could not seem to get my words out.  Nick took over for a time until I got myself a bit more together.  We discussed the surgery and what it entails. Dr G informed me that given the pathway of the artery which winds through the vertebrae and that its the skull and spinal cord it makes it a very complex surgery which doesn’t come without risks, at the same time each day living this way is a risk only for things to deteriorate and the surgery to become even more complex.  It would only take one minor trip, fall or knock which could have grave consequences. He went onto explain that I would need a fusion from my skull-C1-C2 whereby titanium bars, rods and screws would be placed. The surgery would be long and afterwards I would be taken to intensive care.  He described the first few days after surgery as a tunnel whereby the pain is so great you cant see the light but the extreme pain will pass to that of a more manageable pain.  I was told I would lose 50% movement within my neck for life limiting my ability to look up, down and side to side.  Dr G said the recovery is long and I may not see improvement until a few months in once the swelling has gone down. To support the fusion process bone will be taken from my ribs and hip. We discussed surgical fees and timeframes. The consultation ended and both Dr G and Dr O said any questions or concerns I have to not hesitate in contacting them.

On leaving the hospital I contacted my parents, I sat on a little stone bench and gave them a watered down version of the consultation. I felt it more appropriate to tell them the severity in person rather than over a phone call dipping in and out of range.

Once back at the apartment I went straight to lay down the vertigo after the taxi ride had returned with full force.  I felt very numb, almost like the consultation wasn’t even about me and I sat in it on behalf of someone else.  I encouraged Nick to go out and get himself some paella whilst I had a lay down.  As soon as the door shut the tears came flooding, I must of cried solid for over an hour, I couldn’t compute anything, the only emotions I was feeling at that time was overwhelming fear and sadness.  I didn’t want to talk about how I was feeling, I just wanted to go home.

I can only describe the journey home the next day as a cross between a carry on film and hell. The vertigo rendering me unable to walk I crawled (literally) through Barcelona airport, was stopped at security where my bags were searched and had to stand on a podium whilst i was searched thinking that any minute I was going to hit the deck.  I sat in the lounge with my head in my hands, I could not focus on anything as everything was spinning at high speed. Once we boarded I was so scared of being this unwell, so high up in the air we contemplated getting off the aircraft and booking a flight for the next day.  The strong urge to get home overode any possible complication that could occur mid flight. My eyes closed, I took deep breaths waiting for take off, Nick holding my hand as we headed towards the run-way, we had been taxying it for 45 minutes finally we were ready.  Quite literally as the plane was about to take off a passenger pressed his emergency button and we had to return to the gate, the passenger was having a panic attack.  We were another hour whilst the passenger disembarked the aircraft and his luggage was removed.  Normally such a patient and understanding person I found myself frustrated and weepy, I just wanted home.

We arrived in Manchester and my phone was throwing out beeps like the clappers.  Little did I know mine and Nicks family and friends were tracking the flight home and it came up “Aircraft returned to the gate, passenger welfare”, everyone thought it was me god love them.  Nicks parents collected us and his mum got me into the front, they had brought a lovely snuggly blanket for me to cuddle into on the way home.  We had a giggle in the car as Nicks dad had put the heated seats on to keep me warm without telling me. I sat quietly for 20 minutes thinking I’d peed myself and thought how could I get around this without anyone knowing.  It was only when I opened the window as I was hot, Nicks dad said he’d turn the seats off. “Thank God, I thought Id wet myself and thats what the warmth was, Ive been trying to think of ways I could get out of the car without anyone noticing for 20 minutes”!!!!

Never had I been so happy to be home.

Sadly the journey took it out of me for months and my symptoms have continued to decline ever since.

Over the next few days realisation set in of what was ahead of us. The power of people and human kindness came to fruition…….

 

A Real Life bobble head

We got off the train at Euston station, severe vertigo rendering it impossible to walk. I could not see straight nor gage where the floor was. This was to be the start of many more attacks of vertigo, little did I know what the cause was going to be. My partner, Nick, virtually carried me across the station to a coffee shop where we remained until the spinning settled down enough for us to navigate our way to Medserina for my upright MRI scan. It was August 19th 2016.

Despite the multitude of symptoms I’d had over the years, headaches were never one of them. In May 2015 I began to develop the most excruciating pain to date. I had pains in my head 24/7, they ranged from pressure, occipital headaches/neuralgia, pulsating temples, frontal head aches, pulling back of my skull, teeth and facial pain. At times I lost my speech, my mouth would droop and my face go numb. These were headaches like NO other and the fact they were unremitting, I knew something serious was at play. Debilitating neck pain accompanied the headaches along with clicking, crunching and spasms. I became very aware of the weight of my head. Suddenly I felt like I was carrying a bowling ball on a tooth pick and my shoulders became equally as painful. It was terribly debilitating.
The next 15 months were spent in and out of A&E, neurologist appointments and testing. It was a long, complicated and traumatic 15 months, the worst time of my life. I will revisit this time again in my blog but at present its still too raw.

The oh so familair diagnosis merry-go round commenced, starting with a suspected stroke, due to weakness on my right side and facial droop then followed bleed on the brain week. As time went by and more scans were not showing the cause (as they were supine) the avenues of migraine, occipital neuralgia and hemiplegic migraine ensued. I had injections into my skull without anaesthetic due to allergy, all to no avail.

I was losing hope. I had no idea what was causing such pain but I knew living my life like this was not an option. I couldn’t carry on. As time went by the headaches increased in intensity, shoulder pain worsened and the ability to carry my head was becoming impossible. When upright I’d physically hold my head up with my hands at times. As strong willed as I am, at this point I felt I could not go on another minute. I was still employed but unable to attend work due to the severity of the symptoms.  I spent 3 months mainly bed ridden, feeling very isolated and alone. I was losing every aspect of my life, everything I worked for, everything I enjoyed, everything that made me, me was fading away and I didn’t know why.  My world became very small.

Over time I developed weakness down my right side, I was dropping items, severe vertigo, dizziness, vision problems, increase in nausea, my hearing changed, tinnitus, I started to forget words and have problems communicating at times.  I began choking on liquids and solids and found it increasingly hard to swallow. The list of symptoms increased as the months went by.  I had never felt as frightened as I did at this time.

It was a chance conversation with my Shoulder surgeon that put me on the right path. I’d had right shoulder pain for years and was told by many physios it was just a strain. An MRI scan revealed I had 2 shoulder tears (EDS for you) and I was referred to a surgeon. As well as my shoulder the surgeon was very interested in EDS, asking me about my various symptoms. I told him about the current symptoms and the impact they were having on my life. He immediately said it sounded like neck instability as the way I was describing my symptoms was almost identical to that of rheumatoid arthritis patients he had come across with instability in the neck. 

Once home I took to google, I came across articles on Craniocervical instability and the similarities were far too canny. I found a support group on Social media (they have proven invaluable) it became apparent from other sufferers and medical information that the only way to find Instability in EDS patients, due to the mechanics of why we have it is through an upright Motion MRI of which there was only one in the country.  My GP made the referral.

We arrived at Medserina and were taken into the waiting area. The vertigo was so extreme I had no idea how I would sit in a scanner for the next hour, but I hadn’t come all this way for nothing.  I sat and sipped on water trying to focus on one place in the room as it span around me. Nick completed the paper work for me and I signed.

Nick walked me to the scanner and stayed in the room with me. I was strapped into the scanner with a cage placed over my head. First of all, scans were taken in neutral followed by flexion, once I was flexed a bar was placed to support my head and to limit movement to enable a good clarity of picture, the cage was again placed and the radiographers left the room to take the images.  I then had to extend my neck and it was held in position.  It was very painful but I tried to remain focused to get the job done. Then came rotation both left and right. On rotation right I lost the ability to swallow, this frightened the life out of me.  I could not speak as I could not swallow, a groaning came from me and the radiographers ran in and released the bar. I had no idea at the time why that had happened. The scan lasted just over an hour.

We sat in the waiting area afterwards as I felt too unwell to get back to the station. Myself and Nick got chatting to a lady who felt equally as rough after her scan and it turned out she also had EDS and not only that she lived less than 10 miles from me, small world.  We exchanged numbers.  We left the facility after 30 minutes with my images on disk and was told the report would arrive within the week. The journey home was horrific. My head and neck agony after the scan, vertigo still lingering and now nausea.  It felt like the longest train journey of my life.

A few days later I received a copy of my report, it was clear from the imaging I had craniocervical instability. I did not understand everything in the report so I arranged a telephone consultation with Professor Smith, consultant radiologist.  Professor Smith explained that I had a range of motion in flexion and extension greater than 95 degrees and that instability was demonstrated in flexion and on rotation. He went onto say I would need a fusion surgery however this is not yet undertaken in the UK on EDS patients. I could not believe what I was hearing, it was 2016 not 1916!! The professor gave me a few names of world class surgeons, all based in America and said that UK/Irish patients were having to go for surgery over there.

Panic set in, how could I make it to the states? How could I fund such surgery? I read numerous stories of patients in the UK and Ireland having to fundraise to get over to America. One amazing girl had to sail as she was not fit to fly. I felt I was in a living nightmare.  I needed to get the advice of top neurosurgeons as soon as possible.  In the support group there were talks of another surgeon based in Barcelona who had recently undertaken a surgery on a fellow EDS’er.  More and more UK patients were heading over to consult with him, I felt at that time this was a viable option and we made arrangements to have a consultation over in Barcelona for November.  We also scheduled a Skype consultation with one of the worlds leading neurosurgeons in America for December.

It was only when we made the long and difficult journey to Barcelona to meet with both Dr Gilete and Dr Oliver (Europe’s leading skull based surgeon) did we realise just how severe my instability was and the risks involved…………….

A Racing Heart

Sat slumped in triage the monitor persistently beeped 156,157,158 my heart rate continued to soar. I was taken straight into resus; no 6 hour wait in A&E for me today. I was placed in a lovely gown and electrodes positioned around my body. An ECG was done promptly. The doctor on duty came and asked me questions whilst keeping one eye on the machines. He repeatedly asked  if I had chest pain or any pain in my jaw and down my arm “no, I just feel unable to catch my breath, sick, shaky, dizzy, thumping headache and extremely fatigued”. It was Sunday night, I had been going like this since Friday but the stubborn voice in me continued to say, ‘it will settle’. Sunday came around and things were seemingly deteriorating I knew it wasn’t safe for me to sustain such a heart rate so I reluctantly agreed to get checked out.
A few months previously I had seen a cardiologist who I had been referred too based on my tachycardia and autonomic symptoms. The cardiologist had done a quick bedside active stand test and felt it reasonable to book me in for a tilt table test to investigate POTS (Postural Orthostatic Tachycardia Syndrome). I wasn’t nervous about the tilt table test, I had so many invasive tests in the past, I remember thinking how hard can it be being strapped to a bed and tilted, piece of cake, I thought.
Tilt table test arrived, myself and my dad went over to the cardiology department at the hospital. I started to feel a little uneasy, not scared just uneasy, the fear of the unknown I guess. My name was called and I walked into a very dimly lit room to be greeted by two cardiology nurses. We discussed my symptoms and they went on to explain what the test entails. Electrodes and monitors would be placed to record my blood pressure and heart rate continually throughout the test. I would be strapped to a table lay flat until they obtain a reasonable baseline heart rate, once a baseline is established the table will then be tilted to an angle of 60-70 degrees where I will stay throughout the duration of the test. I must remain quiet throughout the test (hardest part of the test for me, I thought) only talk to relay any symptoms I may be experiencing.

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Image taken from pots.org
I lay hearing the continual irritating beeps of the machines thinking to myself ‘let’s get this show on the road and tilt me’. After around 15 minutes the table was tilted. I was NOT expecting to feel how I felt. Suddenly my heart began to race feeling like it was beating out of my chest, an overwhelming heat came over me and I began to feel sick and dizzy. I relayed this straight away to both nurses who acknowledged me and then remained quiet. After a while my legs and feet began to burn and itch, I peeped down at my feet, yep they were purple. This was an all too familiar symptom I had since being a teenager. I nicknamed it ‘The corned beef legs’ Its blood pooling in the legs and It was a frequent occurrence throughout my life. I could not stand for too long as it would happen and have to fidget and keep moving around to stop it. At High School, there was no way I was letting anyone see the corned beef phenomenon and I lived in tights, even in the height of summer id be baking but putting socks on was not an option, I was too paranoid. I of course did not know why this was happening throughout the years. Finally, the table was tilted back to flat and I was told to just lay quietly. At this stage I felt like I was going to be sick, visibly sweating, headache, dizzy and my corned beef legs felt like they were on fire. The straps were finally removed and I was sat up slowly. The nurse asked how I was feeling. “Awful, I was not expecting that at all, is that even normal?” she sat next to me and explained that my baseline heart rate was 64 BPM and upon tilting quickly shot up to 135 BPM and continued to rise, on tilting back my heart rate stayed up at 128 BPM and eventually went down to 86 BPM. The nurse said the hearts response to being tilted was abnormal and that I did in fact have POTS. I would get a follow up appointment to discuss a management plan with my cardiologist.
Back in the waiting room my Dad was sat, he had waited the whole time. “I’ve got it” there those three unattached words rolled off my tongue again. Walking out of the hospital I explained to Dad what the test entailed and what my bodies response was and that we can now add POTS to the diagnosis list. I remember thinking I had spent so many years without a name for any of my symptoms in the space of a few months I now had two. I felt terribly unwell and just wanted to get home. The after effects of the test lasted weeks.
Fed up and frustrated constantly using an oximeter to monitor my heart rate I purchased a Fit bit which recorded my heart rate all day long, logging it on a graph. It soon became apparent my heart rate was not always high it was in fact the complete opposite and very low. I contacted my cardiologist and he suggested having a 7 day holter monitor fitted.
The monitor was attached to my chest and side and fitted around my neck. It had to stay in place for 7 days and I was to record any symptoms and what activity I was doing at the time. The monitor was extremely uncomfortable throughout the 7 day period however I was told it will be minor irritation from the pads. When removed it became clear this was not the case and I had reacted badly and was burnt. A week back and forth to the hospital for dressing changes commenced.

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When the results came in, it showed as well as high heart rates there was in fact low readings in the late 40’s and 50’s causing me to feel breathless. I was experiencing both tachycardia and bradycardia. This made medicating POTS somewhat difficult as the medication I was due to be prescribed slowed the heart down and as I was experiencing both this was not then an option.
I liken living with POTS to that of having a permanent hang over. As it is caused by the dysfunction of the autonomic nervous system which controls everything our bodies should do automatically, its effects are equally as broad ranging.

The simple act of standing up must be undertaken carefully as I will either faint or feel extreme dizziness (pre-syncope) which is a sign to tell me to sit back down before I faint. On standing my heart often behaves like that of a marathon runner 20 miles in, however all I have done is stood up. The heart pounds out of my chest, initially this used to make me panic but I got used to it after a while and have come to expect this sensation as the norm. Add in orthostatic headaches, a sort of pressure within the head when you stand you can imagine standing isn’t on my top 10 list of things to do but a necessity every day.

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Reading taken a matter of seconds after standing up.

I often feel hazy, fatigued and weak. Brain fog can kick in whereby you feel your thoughts are in a fluffy ball of clouds and unclear. I often forget words mid-sentence a bit like when we go into a room for something and when we get there we forget what we’ve gone in for. It’s like that but frequently throughout the day.

Tremors are a big part of POTS for me and can be quite troublesome. My whole body can shake and hands visibly tremor ,which can make carrying my cuppa into the room a challenge (really need to ditch the cream carpets).

I can become short of breath and not necessarily on exertion either, this one is a scary one as sometimes it feels like I am grappling for a big enough and fresh enough breath in.

My body is unable to regulate its temperature which is a nightmare. I could be sat in the room with a normal temperature and all of a sudden an overbearing heat comes over me, in the space of 20 seconds I feel like I have been placed into a roaring fire and will find myself having to do a rapid ‘Full Monty routine’ (terrible sight) and cover myself in cold flannels.

Sleep- what’s that?? 2-3 hours broken sleep a night.

I am plagued by my digestive system which is also linked to the autonomic nervous system.

My bladder is also effected which can be linked to both POTS and EDS.

There are many overlaps between both conditions making it difficult at times to differentiate whether POTS or EDS is at play.

The day I was diagnosed with EDS.

Sat in the waiting room of The hospital of St Johns and St Elizabeth’s, my stomach full of knots and head whirling with thoughts waiting for what seems like an eternity for the door to open and my name to be called.

It’s August 14th, 2015. We had got a late train down to London the previous night and stayed in a little hotel, I couldn’t sleep last night nerves most definitely got the better of me.

We had travelled down to London to see one of the country’s leading rheumatologists who specialises in Ehlers-Danlos Syndrome. You see, it wasn’t the first time I had heard those three letters ‘EDS’ before in passing and who better to confirm or deny than a leading specialist.

Id complained of joint pain as a child, when taken to the doctors it was always attributed to growing pains, reasonable really considering I was a growing child. I was 11 when I had my first dislocation. I remember it like it was yesterday. I was lay on the sofa watching TV at home I turned ever so slightly and my right knee cap just popped out. I started to shout as I had no idea what was going on other than the fact it hurt like hell and looked disgusting. My instant reflex was to bend my knee (NEVER ATTEMPT TO BEND A JOINT WHILST DISLOCATED). I howled with pain, my Mum ran over whilst my brother ran out of the room (I don’t blame him). Instinct kicked in to knock the cap back and low and behold it went quite easily back into its rightful place. My knee throbbed for days and for a few weeks felt very uneasy, I was petrified it was going to pop out again.

Over the course of years this became quite a frequent occurrence. If my knees weren’t dislocating they were popping, clicking, crunching and giving off wild pain. I had to constantly move around, stand up, sit down, bend and straighten. They would stiffen up and throb. I’d wake often in the night having to lift each leg out of the bed and do a little circuit of the bedroom. Driving could be painful, frequently needing to pull over, get out to stretch my knees whilst holding the caps in place. Navigating the stairs was a challenge often shuffling myself up and down on my bottom. Over time my wrists, ankle, fingers and right shoulder joined the dislocation party!! What was this party and why was my whole body involved? This was one party I did NOT want an invitation too!! Sometimes I could relocate the joints myself and others warranted a trip to A&E.

Throughout these years, I had seen several specialists from endocrinologists, immunologists, physiotherapists, dermatologists, gynaecologists and so on!! Again, having a multitude of tests and going around the ‘diagnosis circle’ of spondylosis, arthritis, lupus, Addison’s disease- all sorts was branded about.

The first time I had heard of EDS was from a physio who in passing mentioned I was very hypermobile. I went home and consulted Dr Google. The information at the time on EDS was very limited and did not cover what it entails. Due to how sparse the information was I could not connect everything to it. It was then mentioned by a rheumatologist, I had gone regarding all my joints as at times they would swell and the current theory was an auto immune disease of some ilk. The rheumatologist looked at the MRI of my knees and mentioned both were subcluxed (partially dislocated) and were mal tracking. She then examined the rest of my joints saying “You’re very Ehlers-Danlos ’y’ “ She took this no further and sent me on my way with steroids for my swollen joints and a referral to orthopaedics and physio. It was then at the orthopaedic appointment it was mentioned AGAIN how hypermobile my joints were. We discussed a potential knee operation but the likelihood of the pins popping out and further surgery was high so we agreed on more physio. Once home I typed Ehlers-Danlos syndrome into the search engine again, bearing in mind 5 years had lapsed since I last consulted Dr Google after it was first mentioned. I could not believe the information before my eyes, it was me. Joint dislocations, gastrointestinal problems, bladder problems, chronic joint pain, back pain, fatigue, insomnia, headaches, palpitations…I went through the list, methodically ticking the symptoms off in my mind-tick, tick, tick. The information was MUCH more advanced than 5 years previously.

I took to social media to hunt down EDS support groups. As I sat reading posts from other sufferers for the first time in my life I could relate and connect my broad ranging symptoms to theirs. Even more evident was the commonality of years of anguish before a diagnosis was made, a very late diagnosis. I built up enough courage to write a post outlining my current situation with symptoms and my history. Many people came forward offering excellent advice and support. The one piece of advice that came up numerous times was getting booked in at The hospital of St Johns and St Elizabeth’s, London to see one of the country’s leading experts in the condition. After discussing with my family, we came to a unanimous decision that I must go and consult with this expert.

“Alison Turner” Oh heck, that’s me. In I went.

The consultant was a very warm mannered man. He listened attentively as I discussed my symptoms over the course of my life from joints, fatigue, pain, tremors, palpitations, insomnia, stomach etc. Of course, to be thorough I had sent my medical history over in advance so he was armed with all information whether relevant or not. I was asked if he could do a thorough examination of my joints. Each joint was meticulously examined, moved, and even listened too. Next I was asked if I could perform a series of movements with my joints, this is known as the Beighton scale and is used to quantify joint laxity and hypermobility. See images below-

Beighton Scale

 

Image 1- Elbows bend backwards more than 10   degrees.

Image 2- Thumbs bend back onto the front of your forearm.

Image 3- Fingers bend at a 90 degree (right angle) to the back of your hand or little finger bends at a 90 degree angle.

Image 4- Knees bend backwards more than 10 degrees.

Image 5- Hands placed flat on the floor with your knees straight.
I then had to stand straight whilst my spine and posture were assessed. Following this my skin and scarring was examination, my skin was pulled at various places mainly my neck, hand and elbow. Finally finishing up by listening to my heart.

After the physical examination, we discussed my symptoms further and family history. I felt quite uneasy with mixed emotions at this point, I had no idea what he thought and I wrestled with ‘If I’m diagnosed at least its finally an answer Vs I don’t want to have a chronic condition’. “Without doubt you have Ehlers-Danlos Syndrome Type 3 with classical cross over and Marfan habitus features. I kind of froze at this point whilst the consultant discussed why and how he came to this conclusion. On examination he said hypermobility was demonstrated at the CMCs, fingers, wrists, elbows, shoulders, hips, ankles,mid foot and patella with subluxation and mal tracking. Repeated dislocations and subluxations of numerous joints (knee and wrist in the consultation as well). Recurrent soft tissue injuries and Arthralgia. Scoliosis of thoracic spine.   He noted bruises on my skin, wide thin scarring and hyper elasticity. Gastric dysmotility, local anaesthetic resistance, postural symptoms of pre-syncope and syncope suggestive of cardiovascular autonomic dysfunction, tachycardia, headaches and urinary issues suggestive of pelvic floor weakness and bladder wall elasticity.

The consultant created a plan of action. I would need to see a cardiologist for my tachycardia to be assessed, urologist to have my bladder symptoms investigated, he recommended isotonic drinks in the interim to try and help with the syncope. A physiotherapy rehabilitation programme, possible neurologist to get the new and crippling headaches investigated. He sign posted me to educational and supportive resources of the condition. On leaving we shook hands and he told me if I needed anything this was an open appointment and not to hesitate to contact him and a report would be sent over to my GP.

The waiting room had filled up in the hour I was in the consultation, I gave my partner Nick the nod and made a speedy exit to the door. “I’ve got it” was my response to being asked how it went. We sat outside the hospital, the busy London hustle of people and traffic I rang my dad “I’ve got it” there those three matter of fact words again. My dad was upset, I found myself going into more detail about what the appointment entailed. I was very weak at this stage exhausted from the travel, lack of sleep and emotions we headed over to the Beatles café across from the hospital and had a quick cup of tea we didn’t have long before the train home and all I could think about was getting to the station in one piece. I wanted to go home.

As soon as I found my seat on the train my headphones went in, I didn’t want to talk, just think. I stared out of the window the entire way home, random mixed emotions infiltrating my system. I felt sad, relieved, validated, angry, exhausted, worried and back to sad again. I couldn’t compute the emotions, I just went with them. I shed a few tears but on the whole I was very quiet and composed.

A few days after arriving home my consultation/diagnosis letter arrived, seeing everything in black and white I suddenly felt quite aggrieved. I wanted to photocopy it and send it to every doctor who judged me. I knew I had to keep myself together as a new battle was ahead in obtaining help for my symptoms and investigating the new headaches that had seen me in hospital on several occasions with suspected strokes and bleed on the brain.

Little did I know at this time, just what lay ahead of me.