196 Days later….

This isn’t a sequel to 28 days later, although at times it feels I am in a zombie movie!! We have hit the 196th day since I was wheeled down to theatre for my cranio-cervical fusion. As my Dad says to me each year on my birthday “Where has the time gone”?

 

In the weeks and months following surgery I have had an overwhelming need to be quiet, be by myself and alone with my thoughts. Initially I had no idea why, it was just an overwhelming feeling that I had no choice but to run with. During this time I was in quite a negative mindset but struggled to articulate to those closest to me how I was feeling. I could divulge my symptom list no problem but I just could not share what was in my heart and head at the time.

I have mentioned previously that when I was diagnosed with EDS, aged 31 after a lifetime of symptoms I was actually initially elated. I felt validated. All those years of suffering and not knowing why, I finally had an answer. Of course at this stage I did not truly know the extent EDS had ravished my body. Not long after the diagnosis I was diagnosed with POTS , a condition within itself but often seen along side EDS. Another debilitating and invisible condition. Then followed the diagnosis of cranio-cervical instability. You simply do not expect to face your own mortality aged 32. We all have ideations of being old and grey, tucked up in our warm beds, myself included. I was petrified, I was scared my life would be cut short and there was still so much I wanted to do. I was declining so rapidly it was alarming. I could no longer hold my head up, walking became difficult as I lost my balance and once severe unremitting vertigo set in I felt like I was on the Sea Cat crossing the Irish Sea, only minus the Guinness and giggles!! I was struggling with my bladder, nausea, tremors, breathing and heart rate. All these bodily functions we take for granted were fading, malfunctioning….I was in bad shape. We had to fundraise for the surgery and my campaign and health issues could not have been greeted with anymore kindness, warmth and love from those closest to me to absolute strangers reaching out. This was a driving force for me to keep going however I also struggled being thrust onto social media, newspapers and television. I felt vulnerable, exposed and more than anything embarrassed. I am such a proud person and was so scared people now looked at me differently, pitied me and did not see me for me anymore. I felt lost in it all and at the same time full of gratitude. I was a basket of emotions but I was too unwell to work through those emotions, too unwell to think about them. My main aim was to make it to surgery and get on that flight to Washington. I had no expendable energy to concentrate on the impact being diagnosed with three major life altering conditions had on me within less than a year….

The impact…I remember walking into my physios room, I was having a bad day to begin with and she asked me how I was getting on. Before I knew it tears were rolling down my face, it felt like I was letting go of months upon months of emotions. A box of tissues later, I composed myself enough to talk and I was surprised at what was leaving my mouth. I never would have envisaged my physio being the person I shared all my thoughts with but it just felt right, it was time. I told her how lost I felt, that I didn’t feel like me anymore, I had no confidence, I didn’t fit in anywhere, I didn’t like myself, I had nothing to give, I couldn’t do anything, I wasn’t better, I felt under pressure, I felt a failure, Id let people down, I couldn’t look at myself in the mirror as I didn’t look like me, I felt a burden, I couldn’t get used to my new neck movement and that I couldn’t accept this is to be my life. I told her how hard I was trying but not seeing the results I expected, that the recovery was tougher and slower than I imagined, I told her I’m pushing myself daily to the point of huge amounts of pain out of frustration with myself. She gave me the time I needed to get this heavy weight off my chest. She then told me I am grieving, grieving for the life I had and for the life I had envisaged. She told me its a process, a process I didn’t start as I had received multiple diagnosis which hit me like a bowling ball one after another yet I didn’t have time to think about what any of it meant. We talked about how negative I was feeling about myself and how I was presuming others felt the same about me. I was in a negative thinking pattern. She informed me this is one of the toughest surgeries to recover from in relation to both time frame, pain and adapting to your new neck movement or lack of. She thought I was doing extremely well and pushing myself too hard, too fast and to be more gentle and kind to myself. We discussed how anyone before a surgery no matter mild or major has a two pronged thought process, the first being realistic expectations and the second being the little voice of ‘hope’ in the back of your head that this will cure everything, I will go back to ‘normal’. I most definitely had this, the sensible side of me knew the surgery was to ultimately save my life however the little hope thoughts were there and I had inadvertently set myself up to fail with unrealistic expectations. She told me I could not fully move onto acceptance until I had allowed myself to grieve, to sit back and allow myself the time to actually really think about the magnitude of the last year especially. I was given a little homework sheet to challenge the perception of myself and thoughts. Needless to say no physio took place this day but in so many ways it was so much more productive. It was a turning point for me….

From this point I have allowed myself to cry and not drown in my thoughts. I am learning to open up about my ‘real’ thoughts. I am working through them day by day. In society we can be so hard on ourselves, I need to put extra hours in, Im not doing what I should be doing, Im no good, I could have done better and miss our achievements no matter how small they maybe. I am definitely making myself take note of my achievements, reflecting on how far I have come. Six months ago I was losing the ability to walk and living my life from my bedroom and today on certain days I can manage short walks, my headphones go in and I lose myself. I have been to two family events and managed a few hours of utter enjoyment and normality. I am starting to find myself again, bit by bit the pieces are coming together. Some days are easier than others but I am moving forward. Am I still grieving? Very much so however I am a million miles away from what I was six months ago and for that I am eternally grateful.

This past year has taught me that I am stronger than I ever thought I could be, I didn’t give up no matter how much I wanted too. I have no idea from day to day what I will wake up too but the one certainty is that I face whatever it is head on, with my head screwed on, literally!! Having multiple chronic illnesses is like trying to extinguish a major fire, you may extinguish one flame and another rises and another. You just cant get on top of it and at times it burns you out, drains you and can suck the life out of you. My life will always have challenges, there is no getting away from that but I will always try my best to face them.

I am healing well from the surgery, my wound is remarkable. The mans a genius, he should really take up embroidery as his needle work is phenominal !! My undercut is growing back nicely. I had my 3 mont follow up scan and the hardware was still in place. I will be having a CT scan in around 3 months to check that my bone is actually fusing which is vital for a successful fusion. It feels strange living without a rib and a fused neck as its changed my whole posture and body, there is a definite trade off when you lose the ability to move your head/neck however I take the trade off happily to be sat here able to write this. I have been having many other investigations over the last few months which haven’t been easy and I will post about themsoon.

I truly cherish the good days/moments, Suck it in like its my last breath making the most of them and manage the bad ones as best I can. When faced with so much uncertainty time becomes a precious commodity, its important to make the most of what you have when you have it. I cherish those around me even more than I did before, I take note of my surroundings more, I embrace the capabilities I have that day. I look at the world through different eyes now. For the first timein two years I am starting to feel alive again.Unknown

My escape before surgery 

It was essential in the days leading upto surgery that I kept myself occupied. I could not do too much however we managed to get out for an hour here and there in the wheelchair which was heaven. 
A place we soon discovered was ‘ the waterfront’ which on first visiting instantly became my “go to” place. I found myself gravitating to the steps which led into the water where the ducks would gather. Just to be able to be outside, hear the relaxing sound of the water and ducks splashing was truly heavenly, it often brought a tear to my eye as I really took my surroundings in. It became my all time favourite place and I found myself very relaxed when there. I would sit and just watch the water whilst listening to music. It was so peaceful it gave me the time needed to think about the surgery, the journey ahead and reflect on the journey so far. 
My head felt cluttered regarding the surgery. Most people go into surgery and ultimately come out cured or significantly better. The main aim of this surgery was to save my life, stop my neck from dislocating, stop the artery and brainstem from being compressed. Ultimately saving my life and enabling me to move again without risk of falling over and causing paralysis. Stop the symptoms in there tracks before they became even more disabling and serious. Having the neck brace as a tool when needed instead of it being a necessity. Any symptom improvement was always going to be a bonus from the long term damage already done and the recovery was going to be mammoth over the course of 12 months plus. There were fears of getting through the surgery safely especially with it being in such a crucial part of the body to developing further instability in my spine over the course of my life to the day I’ll be weaned off the brace and have to learn to do quite basic things again with little head/neck movement. We all in life want a quick fix, myself included and sadly this wasn’t going to be that. I had so many day dreams of waking up ‘cured’ and symptom free, I dreamt of doing everything I had missed out on over the course of many years but I also had to bring myself back down to earth and remember I had EDS, dysautonomia and other health issues that sadly weren’t going away. On the other hand I also wanted the opportunity to live and be able to fight those illnesses as best I could. I wanted a chance at a life again. 
Two days before surgery I sat by the water reflecting on the past two years from the loss of my job, becoming virtually housebound and thinking how much I had lost. I do find it too painful to talk in great detail about this as it’s all very raw. I then as always counteracted those thoughts with the hundreds upon hundreds of people who came together and supported my campaign, the amazing fundraisers, hard work of friends, colleagues and people I had never even met before. Receiving the warmest messages of love and encouragement that pulled me through some very dark days. Human kindness at its finest. 
I started to become mentally focused, I’d be lying if I said I didn’t cry, panic and have 101 fears running through my mind but strangely at the same time they were accompanied by an air of peace and rationale. I was ready for this surgery and the chance to move forward with a secure head and neck!!! 
It’s very important to me to have an escape, often over the course of the last few years it’s been my music I’ve fallen into or imagining going to various places in my mind and almost getting lost there. I was so fortunate to find this beautiful place that at times made me feel like Ali again, it gave me such comfort and peace to enable me think. It’s a place that will be etched in my memory forever. 

Manchester to Washington 

Well……what a journey with a long way yet to go. It’s been some time since my last blog post and so many things have happended I am going to attempt to add some as-semblance of order and take you on a little retrospective back over the last few weeks when I feel able.  I guess beginning with the journey is as good a place to start as any. 
On Sunday 3rd September we made the trip to Manchester airport. I hadn’t slept a wink the previous night, I resigned myself to the fact no sleep was taking place as the nerves were too great. I had no idea how I was going to make such a mammoth journey in my current state of health, I knew how unwell getting to a local hospital appointment made me but at the same time I had worked so hard on my mindset leading up to the journey that I felt very focused on the task in hand and that is how I looked upon the journey- a task, a task with numerous phases and challenges within it. 

Phase one was making it to Dublin airport where we would stay overnight ahead of the “biggy” to Washington the next day.
We arrived at Manchester airport safely and said goodbye to my brother and his lovely wife who had kindly driven us. I got quite emotional saying goodbye and had a good cry clinging onto my brother. We headed through to airport security. There was none of the usual excitement you’d associate with the airport before embarking on a holiday, this was a mission. I was in my wheelchair due to my vertigo and it was a new experience going through the airport and customs this way. I bleeped (as always) at the scanner and was wheeled to one side to get “checked” then the task of trying to gather all the hand luggage the runner spits out at the speed of light, I felt frustrated I couldn’t do this myself and look after my own belongings. It was all too fast for my head and my body to keep up with. 
We soon got into the airport and had a cuppa, which is my answer to most things. I think that’s the Irish in me. I was nervous for the flight, although such a short flight I am not a keen flyer at all. It’s more the fear of not being able to get off the aircraft than anything else. My dad took me over to look at some of the planes on the runway, whilst we waited for our gate to open. Eventually the gate opened, I was struggling being upright by this time and was wanting to lean my head against something to take the weight off. Nick wheeled me to the gate and I remember my first thoughts were that of dread!! This was not a plane before me this was a coach with wings i thought!! My dad made a joke the Tiger Moth plane he had been in was bigger. I think given the time of day and the few people on the flight they were using the smallest plane!! I remember commenting on the 4 steps up to the plane and then getting stuck in the toilet. As soon as I got to my seat I put my headphones in and got myself in “the zone” I was nervous but this was the first leg of the journey and in 50 minutes I’d of made it. The plane taxied the runway and quickly took off which admittedly was a little bumpy but given it was a coach with propellers it wasn’t as bad as anticipated. 

We soon landed at Dublin airport and collected all our luggage. We were greeted by our good friend Rich who we had arranged to come over and help get me and the luggage to the airport premier inn and help with food. As I’m gluten free and my diet consists of only a few foods it makes eating out very awkward. Luckily we had arranged in advance for Rich to take me over to his lovely friends house to cook some gluten free pasta to ensure I had some food for dinner and breakfast. By this time I was exhausted and went to lay down for a few hours flat whilst my family went for some food. My dads cousin, his lovely wife and there daughter from Ireland had arranged to travel to meet us at the premier inn which was just lovely to see them all and have a lovely cuppa. It meant an awful lot they had travelled over to Dublin to wish us all well. That night I didn’t sleep well, the worry overtook me a little at this point with lots of tears for the flight to Washington. I am not a seasoned traveller and had never travelled that far before well , let alone in the condition I was in. I was scared of getting unwell on the plane and being unable to get off. As the light peeped through the curtains it was around 5 am I got myself up and ready. A few hours later Rich drove myself and all the luggage to Dublin airport and my Parents and Nick shortly followed. Luckily we had arranged special assistance which meant being able to sit in a quieter area of the airport before joining a different queue to check in, I highly recommend this if you are travelling in a wheelchair with medical supplies. We were directed up to airport security, before going through we said goodbye to Rich who had been so helpful and then through the gates we went. My wheelchair was scanned as I went off again. We didn’t have too much time for a rest as we then had to make our way to customs, we chose to fly from Dublin for this very reason that you can do all the customs and essentially gain access to America in Dublin which would be speedier that doing this over in Washington. We went through a second batch of security where everything had to go through again including shoes and my neck brace was swabbed and wheelchair scanned. We then joined another queue this time my finger prints were taken and I was asked the reason for visiting the United States, “surgery” and provided my Esta and medical information, my photo was then taken and i was stamped. By the time we all got through it was time to head to the gate as the plane was boarding. I was nervous but also again very much in the zone, I had a task to do and it was going to get done. When boarding the flight I was helped on board and the Aer Lingus crew were fantastic with there care and consideration. Myself and Nick were in business class, I could not remain upright for anything longer than 30 minutes without issues arising so I needed to lay flat. Again the headphones went in and I tightened my brace in anticipation for take off. The flight seemed to board very quickly and began reversing onto the runway I remember thinking to myself “Well Ali, this is it. You got here missy there’s no going back now”. The plane was on the runway and away we went. There was a little TV where you could track the flight. I lost count of the amount of times during the flight I flicked onto this to see whereabouts we were and more importantly how long left. I did find the flight difficult but being able to lay completely flat made all the difference. Around the 5 hour mark I ran into more difficulty with a severe headache and pain I took my medication and lay back down. I sadly couldn’t participate in the food on the plane but had brought some cake and bits and bobs to tide me over. I drank a lot of water as flying dehydrates the average person and with having POTS you require even more. As well as my wonderfully sassy flight socks which felt like they were cutting off my blood supply I took my doctors advice and took regular walks through the cabin (usually for a pee from all the water)!! I more or less stared out of the window the entire journey listening to my music thinking about what was about to happen and that it was finally happening. It all felt very surreal.




After around 7.5 hours we touched down at Dulles airport. I shed a tear when disembarking and everyone said how proud they were. I was suffering quite badly with vertigo at this point which was unsettling but expected. We made our way through the huge airport to baggage collection and onto airport travel. It was around another 1.5 hours before we reached the house. I was carried out of the taxi and put into my wheelchair.

As I looked up at the house, teary, in agony and with no concept of time I thought to myself “I’m here, I did it. This will be my home for the next 6 weeks”. 

 

Turbulence

As I reflect on the last two months, i do so with mixed emotions. Each day has been very turbulent, almost like a pick n mix bag of emotions, thoughts and feelings. I think the phrase used for Marathon runners when they have hit the 20 mile mark is most fitting “hitting the wall”. I have most definitely over the course of the last two months ‘hit the wall’, repeatedly.

The last eight weeks have seen my family and I having to seek opinions from numerous Neurosurgeons overseas and here in the UK in relation to the rest of my neck. I received a call at the beginning of May informing me I would require a full cervical fusion from the surgeon I was due to have my surgery with at the end of May. My initial reaction was panic, floods of tears and fear, I could not imagine my life with a fully fused neck, not being able to move my head and all the limitations that would bring. This isn’t what we had prepared for. Can people live fulfilled lives with a fully fused neck? Yes, I am fortunate through this illness to have met some wonderful people some of which are fully fused through the neck and some of which beyond that. It isn’t easy both on a pain level and having to adapt but it can be done. Once the initial shock dissipated a little, questions started to flood my mind. The first being, why? This had never been mentioned before in my consultation nor had it in subsequent dialogue over a seven month period. I felt uneasy, I discussed it with family and friends and we came to a unanimous decision to seek further opinions as I could not roll with something I did not 100% believe in. This would be the biggest surgery of my life. We sent my images over to numerous neurosurgeons in America, all independant of each other and the waiting game began for the opinions to come in.

In the midst of this unease and uncertainty saw me having to attend over seventeen appointments. Now, my life of late is a sort of one large hospital appointment, there is rarely a week that goes by where i don’t have at least 2-3 appointments however this time things were a little different.

My first appointment during this time was with Ear, nose and throat as I am unable to breath through my right nostril, a problem I have had for some years but ignored. The appointment was to discuss the results of a CT scan I had of the temporal bones looking for a particular condition, the results came back clear, YES i thought to myself there isn’t yet another problem. The consultant then proceeded to get an endoscope out of the draw and placed some jelly on the end and began feeding it up both nostrils, I was not expecting this and nor was it a pleasant experience. I had just about had enough of these mini hose pipe tests over recent weeks!! He informed me I had a severe sinus collapse and deviated septum which would require a surgery. “You have to be kidding me” off memory were the words of choice. He explained what it meant and what the surgery would entail, we agreed my neck needed to be fused first as no anaesthetist would put me to sleep with an unstable neck so we agreed we would follow up to discuss a time frame in August with the view of the surgery in the new year. The surgeries were just piling up from fusion surgery, impending shoulder surgery for tears and now a sinus surgery. 2018 is going to be great !!

Next came urodynamics testing. What a fun morning that was !! I have been experiencing bladder symptoms for around two years however in the grand scheme of symptoms it was lower down the priority list, yes i have a list!! In recent months it had stepped up a notch and my urologist referred me for urodynamics testing and referred me on to the head urology consultant for review. The urodynamics test was not a pleasant one for me at all, now I was not expecting to breeze through the test but I was also not expecting the pain it caused. I am going to spare you of all the ins and outs (literally) so… in a nut shell the test involved a tube being placed up the urethra and into the bladder and if one hole wasn’t enough they kindly placed one up the bottom too. After a few wails during placement I remember thinking the tube resembled a strawberry shoe lace, remember the ones you got when you were a child from the sweet shop? Theres that fond childhood memory banished!! Next i had to stand up with said tubes placed whilst the nurse tugged to ensure they were in, nice. My bladder was then filled with water and i had to state when i felt i could not take anymore and i felt full. This was testing how much fluid capacity my bladder can take. After this I had to cough numerous times to see if i peed myself on a lovely strategically placed nappy pad on the floor, i remember looking at the ceiling at this stage thinking “ When did it all get to this” I didn’t know whether to laugh or cry. Now came the emptying of the bladder, this was to see if my bladder empties fully and what the pressures are when doing so, i had to sit on a ‘fake’ toilet and pee, simple i thought. well….. it hurt, it stung and as always took me forever to pee in dribs and drabs. Once finished the tubes were removed and I was told the results would be sent quickly to my new consultant with a follow up. I greeted my dad in the waiting room like a rabbit in head lights and burst into tears. Looking back I think I had just had enough of being poked and prodded the last few years.

The next week saw me at Wythenshawe hospital for allergy testing. An MRI of my spine at Salford to review the cyst I have happily resting in my spinal canal and an appointment with a kidney specialist after traces of blood and protein were coming out in my urine and a decreasing kidney function. The specialist was lovely and showed so much interest in my conditions, she also new a lot about EDS which is always a bonus that a doctor isn’t taking to google or wikipedia !! She felt the blood loss was due to a thin kidney membrane due to the EDS and advised to have regular monitoring of kidney function.

The following week I saw my wonderful specialist bowel nurse, she’s such a lovely lady. As my bowel isn’t playing ball at all it is causing a lot of pain daily. We discussed a relatively new drug on the market which acts on the nerves to basically try and get them going a bit, we agreed I would have a trial of this. I then went to see the swallowing team later that week as i have been choking intermittently on fluids and often my throat closes numerous times during eating but thankfully it happens very quickly. My swallow was assessed and it was identified that I have an uncoordinated swallow and spasms, i was given thickener to put in my drinks. JOYS.

Genetics at St Mary’s hospital was the next port of call, this was a long appointment to discuss the possibility of other types of EDS and connective tissue diseases. My DNA was taken (Jeremy kyle style) for testing which will take a few months to come back however the geneticist was quite confident I had hyper-mobility type EDS with some classical cross overs. We saw my wonderful GP the next day to update her on my appointments and to discuss the torrid time I had been having with stroke like symptoms, irregular heart rate, dizziness and low blood pressure. My blood pressure was low that day and she decided she wanted to do some blood tests.

The day arrived for my follow up with the head of urology to discuss my urodynamics. The urologist delivered the news that my bladder was not voiding correctly, the pressure was over double for what it should be for a female with slow flow and residual water left in the bladder. He informed me I would need to start self-catheterising to ensure my bladder was being sufficiently emptied daily. He went onto say due to the fact i found the urodynamics testing so painful I would need a rigid cystoscopy to dilate the urethra as he suspects a stricture and to have a look inside the bladder however with my neck being unstable I could not be put to sleep for this which would be the normal course of action, it could be done under heavy sedation, although i would still feel a lot of pain. He explained to me the correct name for my bladder issues is neurogenic bladder where the nerves are not working correctly, this could be due to EDS or a condition called tethered cord of which i am yet to rule out. He explained I would need to attend a specialist nurse led clinic on 27th June to be tought how to self catheterise and that we would have to see if able due to the narrowed urethra and if its too painful i may need the rigid cystoscopy to widen the urethra under sedation as we may not be able to move forward without it. That day I had another scan of my bladder after emptying it which showed fluid still remained. I left the clinic feeling like i had been hit by a truck. My dad gave me a big cuddle and reassured me we will together get through this next set back. He is my absolute king.

Set backs….. that is exactly what I felt my life had become. There isn’t really a week that goes by when a new problem doesn’t arise, something new to take on, another challenge and I had just had enough. How much can one person take? I remember thinking, this just isn’t fair anymore. There was not one organ or joint that wasn’t affected. I cried a lot that day, i felt angry, sad, frustrated and full of fear. When hit repeatedly with problem after problem it becomes even harder to digest new problems, I often feel too overwhelmed to even allow them to register in my mind. I think that is why I find doing my blog so therapeutic as often instead of talking about my feelings with people i will go quiet, shut off and withdraw and blogging gives me the arena to indirectly face what is happening.

Having no control over my body is a constant worry of whats going to go next, its important to me to keep control over the one thing I do still have a hold over, my mind. I inevitably will pick myself back up time and time again because there is no other option. Keeping the mind strong isn’t easy as the capabilities dwindle, you can’t occupy yourself, you can’t do the things that once filled your time so you have to constantly find new ways of doing things to maintain some level of independence and dignity. At present I am trying to teach myself sign language, it is something I am able to do without causing too much pain, it keeps my mind occupied and i hold onto the thought that maybe one day i could use it in a capacity of helping others. It is currently giving me a purpose.

The following week i received a call from my Gps practice regarding my recent bloods and have been called in as some abnormalities have been picked up on my renal and liver function tests, hopefully nothing too serious. We travelled to Salford to meet my UK neurosurgeon to discuss the recent scan of my cyst, some good news at last, a little win. The cyst has not changed in size in 12 months, he informed me he could not fully rule out a csf leak within the cyst and the only sure fire way to do so would be a CT mylegram which in itself can cause a leak especially with EDS patients as you are piercing the dura so we agreed a watch and wait for another year for now.

There were many more appointments throughout the course of the last 2 months and with many more to come over the coming weeks and months, I am trying to look at each appointment as one step closer to a better quality of life although I am not quite there in my mindset yet with the recent knocks.

Where am I right now? On Monday we received the fourth and final opinion from the neurosurgeons. The opinions had come in dribs and drabs over the course of two months with dialogue between myself and them. Its been very difficult managing the communication, the uncertainty coupled with the above appointments and set backs but i live to tell the tale, granted a little wind swept and battered. The general consensus from all surgeons is that I do not need at present a full cervical fusion however i am in desperate need of being fused skull-C2 due to the compression of my brainstem and rotational instability which most definitely reflects in the pain levels of late. I am dislocating very mildly at C3-4 and a bit more at C4-5 however it is not impinging my spinal cord and does not meet a surgical threshold based on my imaging a year ago. Will this be my last neck surgery? Probably not, no. However that is a bridge to cross hopefully a long time into the future. My family and I thought long and hard about all the opinions presented to us and how they all correlated, we liked the consistency. Together we made the decision for me to undergo the surgery in Washington with a surgeon who has performed this operation countless times on EDS patients. This is a major surgery and does not come without major risk and we feel this is the right direction for me and my condition. The thought of getting to Washington fills me with dread but it can be done and will be done. The change of course has resulted in us having to open the fundraising again as the costs are significantly higher in America however I remain positive as this has been achieved by many patients in the UK and Ireland. I am very fortunate to have the most amazing support network of people who quite literally keep me going through there encouragement each day.

Over the next few months I hope to write about my feelings as we embark on this life changing journey and head towards the surgery in September.  Although i feel of late i am hanging on by a thread i am determined more than ever to face this head on and get a better quality of life for myself.  The surgery will not only save my life, it is life changing and will enable me to take some control back of the rest of my body.

“Do not be afraid to travel a new path, it maybe a way to find what you’ve been looking for all along”

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.

 

 

 

 

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 Zebra Life…but why the blog?

First blog post Ali…….NO PRESSURE!!

I guess at this juncture I should explain why I have chosen to blog, my reasoning behind it and what I endeavour to achieve by opening myself up in the public domain.

Since being diagnosed with EDS, POTS, CCI and AAI my world has become smaller, as my capabilities have deminished due to ever increasing symptoms. There is a constant tug of war between my mind and my body.  My broken body is unable to keep up with my minds desires.  With that comes a minefield of emotions whereby these emotions can and do detonate at any given moment.  The unprectitblity of symptoms and capabilities adds further explosive devices to the field and before you know it you’re gently tip toeing through each day hoping a device doesn’t set off.  Essentially each day is dangerous, unpredictable and exhausting.

Through blogging I would like to address the emotional turbulence of  living with a variety of chronic illnesses.  How I feel, how I manage these feelings or don’t in some cases, what are these emotions and what triggers them?

The journey to diagnosis was far from smooth. It was 10 years of constantly being let down, judgements, deflation, anger, upset, fear and exhaustion. There were many times over the years i felt like giving up, at times i lost hope.  I knew something wasn’t right, I knew my body did not function like that of the majority. We spent 7 years intensively going round in circles within the medical world. Sometimes my hopes were raised when something was found “This is it, Im going to get better” only for treatment to fail or have no effect on symptoms and back to the drawing board I went.

When you don’t have a name for your symptoms it makes it even harder for people to understand and to some extent believe.  I found myself keeping a lot of my symptoms to myself, I became a master in disguising my symptoms.  Socialising became increasingly difficult.  I could make a plan with the best intentions but have to cancel due to my health.  I often felt people didn’t understand or thought I was picking and choosing what i wanted to go too.  I began to stop socialising as it was easier than constantly feeling like you are letting people down.

Work became increasingly difficult and I found myself adapting my days, eating certain things at certain times of the day in order to try and reduce symptoms, napping in my car and shorter days. Every single part of my life had to be planned with military Precision, yet I still didn’t know why. I really gripped onto my job for as long as possible but in truth I struggled for a lot longer than I let on.  I loved my job, I didn’t want to lose it.

I hope that blogging will give me the platform to be able to reflect on the past from constant testing, people not believing me, trying to act normal and keep up with others, the loss of my job and at times what feels like the loss of my identity.

From diagnosis to beyond!!  I will never forget ‘diagnosis day’ from the trip, to the consultation itself to all the emotions that came instantly and thereafter.  The diagnosis finally gave me validation and the jigsaw was finally put together. Once diagnosed with EDS in some respects medical doors opened for me and in other respects it became quite apparent of the limited knowledge within the medical community of such a complex condition.  Another battle then began.

I would like my blog to be a true reflection on what living with EDS is like day to day, my experiences of testing, appointments, medical professionals and things I have learnt along the way. I would like to go back to diagnosis day and certain tests with the main aim of helping others who are facing similar.

The hammering as I like to call it!! Shortly after my diagnosis of EDS came what felt like an endless stream of further diagnosis’s within a short period of time.  I remember at the time thinking ‘how unlucky can one person be’ spending 30 years with symptoms and no diagnosis and then they don’t stop coming.

The diagnosis of CCI and AAI was my biggest blow to date.  I really did struggle and still do accepting this.  Having a potentially life threatening/altering condition gives you a constant unease.  Not just an unease of symptoms but an unease of ‘am i going to wake up in the morning’, ‘ am I going to die at 32’, ‘will I be paralysed’, all very real feelings.  A further blow came when it became apparent how the operation was not carried out in the UK on EDS patients.  The only one positive that came from this is that it opened a door whereby I have met THEE most amazing people suffering with the same conditions from all over the world.  Now that I am forever grateful for.

I hope my blog reflects what living with CCI and AAI first hand is like.  I would like to post about the current situation in the UK on this surgery and the position patients are left in.  I would like to discuss the importance of having a strong network of people that really do ‘get it’.

In October 2016 fundraising began.  I personally had a mental struggle with exposing my story and vulnerability.  It did not sit well with me to begin with and I felt very anxious at the time of launch.  The response has been phenomenal, breath taking and overwhelming.  So many people came forward from friends, family, old school friends, past work colleagues and total strangers and have organised the most amazing events, offered support and love.  I will never forget this for as long as I live and has been a huge driving force in keeping me moving forward.

I would like to blog about the daunting prospect of fundraising, everything it entails behind the scenes, media and the power of people.

Rods and bolts in the neck you say???  All being well May 2017 will see me and my family take the journey over to Barcelona to undertake the biggest surgery of my life.  I will be having my skull fused to the top two vertebrae in my neck.  This is a high risk operation given the junction of where the surgery takes place and the spinal cord and nerves involved.  The operation will be tough, the recovery long yet necessary to save my life and ultimately eventually give me some quality of life back.  I will be away from home for 5-6 weeks before its safe to return to the UK where recovery will continue.

Documenting this journey is very important to me as a reflection of hopeful progress.  I would like my blog to discuss in more detail what the surgery entails and my trip to Barcelona for consultation. After surgery my partner will take over the blog  for a time documenting first hand the experience.  I hope to blog thereafter.

In essence my blog will be a little bit of passed, present and future sprinkled in for good measure.  I really do hope you enjoy my blog and walk alongside me on this journey.

Much Love

Ali xxx