Health update- Part 1 (Head and Neck)

Following on from my post yesterday I am going to do a few health updates in various parts to bring everyone up to speed. I have so many ideas of topics bubbling away in this brain of mine I really want to write about but for the purpose of fluidity they won’t really make as much sense without me touching on my current situations. The updates may not be the most riveting of reads however it paints a picture for my future posts. This will be the longest of my updates so line yourself a few expressos up and pop a pro plus whilst I try and articulate what this body of mine is up to.

Those that have followed my journey from the start will be aware one of the most disabling aspects of my health is chronic continual headaches, the term headache simply does not do these beasts justice.  Prior to 3 years ago one thing I never suffered with was headaches then in April 2015 I banged the back of my head and neck quite forcefully and the “headaches” soon commenced. We did not pursue or explore the origin of these at the time as I was also diagnosed with instability in my neck, given neck instability particularly cranio-cervical instability can cause headaches I had pinned ALL my hopes on this symptom amongst many others dissipating after fusion of my skull and neck.  When I realised the headaches were still very much alive and kicking I cannot express the utter sorrow, sadness and loss of hope I felt.  On reflection I should have been more realistic in the outcome that surgery is not a fix for everything and its primary aim was to stabilise my neck and to prevent any further damage which so far it has done.

So what are these headaches like? Here goes…….

Firstly I would like to point out the pains are there constantly ranging from a 5 where I have almost learnt to get by with it and almost call it “irritating background pain” all the way to a 9/10 whereby I am on the floor willing someone to remove my head and ended up in hospital numerous times. I genuinely cannot imagine a worse pain and anything in-between that scale. They can be extremely unpredictable and fluctuate in intensity throughout the day, they can either hit extremely hard from nowhere or build up over the course of the day but they never leave.  We’ve all suffered regular headaches in the past from one too many, too long at the computer etc just imagine that x10 all the time then that x20 numerous times a day.  This is the main symptom stopping progression of other areas of my health as I have to lay flat with ice around my head for large portions of the day. The pains feel like a heaviness at the back of my skull alongside someone taking sharp knives and stabbing it, the back of my skull can throb and often feels like someone has taken a baseball bat to it. Accompanying said knife/baseball bat comes intense throbbing/pulsating of the whole cranium which spreads into my teeth, face and eyes. When I stand or lean over it quite literally feels like my brain is going to pop out of my skull the pressure is unbelievable. Alongside this I get visual auras, blurred vision daily, loss of focus/clarity (all the time), loss of vision, greying of vision, dizziness/vertigo, poor balance, tinnitus, whooshing in my ears, stiff neck and shoulders . I can almost hear my own voice echo loud when I talk  which is beyond irritating, if someone is trying to talk to me and the television is on or its a 3 way conversation its like my brain cannot process it, its too loud for me which in reality I know it isn’t, I will have to either put my fingers in my ears or remove myself from the situation.  At the beginning of these headaches there was a postural element whereby if I lay flat they would ease somewhat but over time the postural element has subsided.  Not only are they impacting on me being able to access other aspects of my healthcare and attend appointments they are also impacting my ability to communicate with people for any period of time or be around noise.

This is where is gets complicated ……

We recently went to see a neurologist who takes a particular interest in headache disorders. He was absolutely lovely asking numerous questions about my other health issues and completely dissecting the various headaches, pains and symptoms. It was kind of one of those appointments where you feel extremely understood and listened too yet come out with more questions than you went in with. He thinks there could be a few possible causes of my headaches which will involve invasive investigations. He also felt one aspect of the headache was secondary migraines which he said can occur when you have head a continual headache from a different origin for an extended period of time other types jump aboard too!!  At present we have been trying to treat the migraine aspect of it, so far to no avail.  I was placed on propranolol which is used to treat chronic migraine it is classed as a preventive medication to take continually. As it is in the beta blocker family we thought it would help my POTS also, my track record with medications is not a good one but I was willing to try anything through utter desperation. The first few days on propranolol were fine other than a bit more tiredness I had no adverse effects, I was extremely happy and hopeful this would at least start to mask some of the pains. Around day 5 I started to feel extremely weak/dizzy more so than usual and noticed my blood pressure starting to drop and heart rate was getting lower. Initially it was a welcome break to be able to stand up and not have your heart rate jump to 130 bpm from pots and continued to take the tablet as I was desperate for an ease of the head pain. Saturday night came and I was lay watching a film and from nowhere my heart started to flutter (I wasn’t watching Orlando Bloom), I went clammy and started with crushing pain in my chest although this didn’t feel like a POTS attack I suspected it was and took my heart rate to find it was 42 bpm, this continued and I tried to walk round to increase it however it would not go higher and I felt extremely faint.  After 30 minutes of this it then started to jump erratically from 42-80, 80-130 etc etc then if I lay down it would got back to the 40’s. I was being quite stubborn and hesitant not wanting to go to A&E as my life is just filled with hospital appointments however it did get a bit much and we decided to ring 111 the operator sent an ambulance which I said I did not need however once you mention chest pain and heart rates I think it is a mandatory protocol they follow.  The ambulance arrived by which time it had levelled out a bit but they said they could tell I’ve had a low heart rate as it was 59/60 when they arrived and they said usually peoples heart rates are sky high when they arrive just by the sheer fact an ambulance is there.  They did a quick ECG, blood sugar, temperature and they also noted my pale paler and clamminess.  After much persuasion I agreed to go with them to get checked out to ensure it wasn’t any kind of heart attack.  Once in A&E they did bloods and another ECG.  When doctors were in the room my heart rate was over 60 which is normal but very jumpy then once they left and I lay down quietly it would drop to the lowest 37 and my partner called them in to see and then they saw it shot within a second to 65.  I felt exhausted. Below is a picture we managed to grab of my heart rate at 39 BPM, Mo Farah has nothing on this….

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The bloods and chest x-ray came back clear and I was advised by the doctor to stop the medication immediately, I asked if I could try it one more day to see if my body just needed to adjust she could see I was desperate for my pain to ease from the headaches but she said its far too dangerous to have a heart rate of 37 and it jumping so erratically she felt if I took one the next day it would get even lower and I would be back in. My heart had stabilised somewhat and I was sent home but told to go back if it dropped again. We arrived home around 3 am and I went to lie down and it dropped to 41 I was adamant I was not going back so I walked around my house for an hour trying to get it to rise.  Sadly with it being a bank holiday I could not consult with my neurologist or GP, we have since let them know and are awaiting where we go next in terms of treatment.

The possible causes of the main headaches I will list below and can be seen in Ehlers Danlos syndrome and other connective tissue disorder types. They are notoriously hard to diagnose and many people have been diagnosed with migraine initially which was unresponsive to the treatments and after invasive investigations the source was eventually found after many years of suffering.

Theory 1-

A CSF leak. With connective tissue disorders the dura which is connective tissue that surrounds the brain and spine can be very thin/fragile and can tear either spontaneously, through injury like a whiplash or other defects within the spine. This causes a tear in the dura whereby cerebral spinal fluid which coats the brain and spinal cord which flows constantly leaks out of the tear/defect causing what they call a low pressure headache.  Alongside the headache comes other symptoms and neurological manifestations namely what I am experiencing above.  They can be notoriously hard to find if the defect is small.  They often test the pressure via a lumber puncture to see what your opening pressure is or by a bolt placed in the skull.  They sometimes do a CT myelogram which injects dye into your spinal cord that can show the dye leaking out of the defect. If the source of the leak can’t be identified sometimes they do a blind blood patch where they inject your own blood into suspect areas of the spine in the hope it forms a clot and seals the leak.  They can also move onto fibrin glue or worst case scenario surgical intervention.

Theory 2-

Blood flow issues. With EDS the blood vessels in the head and neck can be sluggish and some patients have had occluded/collapsed vessels/arteries therefore the adjacent vessels/arteries are having to do all the work of pumping the blood around the brain.  A test for this would be an angiogram to see how the vessels and arteries are functioning.

Theory 3-

CSF flow disturbance. Along my spine I have some degeneration, disk disease, congenitally fused parts, a scoliosis and an intra dural spinal cyst with noted CSF disturbance.  We are not yet sure if this cyst is just an incidental finding or if one of two things could be at play.  Either the cyst has caused a csf leak as discussed in theory 1 or if the cyst is acting as a damn and preventing the free flow of cerebral spinal fluid.  On the picture below you can see white space around the spinal cord which is CSF, in the middle is where the cyst is and you can see a marked disruption of fluid.  The picture on the right is a back view more of the bone and my scoliosis.

 

Theory 4- 

High intracranial pressure.  Many people with EDS, although this condition is by no means exclusive to EDS can have pressure issues within the brain. This theory is that the pressure in my brain is high which causes severe headaches, nausea, visual disturbances, tinnitus and much more.  To diagnose this would be an ICP bolt placed within the head to test the pressures. The bolt tends to be more accurate than a lumber puncture as it will give you continual readings over however many days you are in hospital with it in and the specialist can have you moving, bending and see what happens to the pressure in your brain.  It is also deemed safer for connective tissue disorders as a lumber puncture can infact cause a CSF leak (theory 1) by tearing the dura so you may have had high pressure however from the investigation it has caused a leak and now you’re suffering low pressure.

You can see from the various theories the symptoms are all quite similar which makes it extremely hard to distinguish which course of action is best to take so at present we are at a cross roads.  We have to weigh up risk vs benefits however at the same time I can no longer live with the level of head pain and the symptoms that accompany it.

Now….if you have got this far thank you so much as I know that was a long one.  Id say step it down a notch and make yourself a cuppa for the next part.

Neck-

Since being fused the muscles in my neck are extremely tight,painful and go into spasms which isn’t easy to live with daily but at the same time this is the nature of the beast of neck fusion with limited neck movement. Biofreeze, heat and ice are my best friends. A few months ago we had a bit of a scare whereby the pain increased and was “different” my neck also started to click and almost catch.  It was frightening. I contacted my surgeon who advised an earlier than expected CT scan to ensure the hardware hadn’t moved and the bone was still where it should be.  We went for a CT  scan, once in receipt of the images we uploaded them onto the computer and panic set in, my fusion appeared bent and warped.  Note to self Ali there is a reason radiologists have years of training you are NOT a radiologist. We sent them to my neurosurgeon in Washington who immediately reviewed them and was happy the hardware was still in place and the rib graft had not slipped.  Then ensued 100,000 questions from me!!  He explained depending on the angle of images taken will depend on how it looks, essentially from certain angles/views it will appear bent and misshapen however it isn’t.  He said there is some element of ensuring the hardware fits your anatomy during surgery so rods may have to be moved so no two fused people will have the same images.  We were so relieved by this. We also got a report from the radiologist who did the scan with words of the same effect. Below are a handful of the hundreds of images taken, to be fair you can see by picture one why we were concerned.

 

 

Over the course of the next week I will be doing other health updates as there is an awful lot going on and aim to be all caught up and blogging order to be restored within the week (or so) as I really want to get stuck into writing about some particular subject areas.  I predict around 4 more combined updates still to come.

Thank you for taking the time to read and follow my journey, it means an awful lot to me.

Much love

Ali xxx

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

Homeward bound.

We had only one hour to pack before our taxi was arriving to take us to Dulles airport. In theory I thought I would have a spare empty case on the return trip as I travelled with a case of food however this soon filled with pillows, neck braces and medical supplies I had accumulated whilst in Washington.  Why couldn’t it of been handbags, clothes and perfume!!!

The taxi soon arrived, I was still trying to peel my support stockings on.  These are fantastic for any long haul flight but especially for those with POTS who have difficulty getting there blood to pump back up to the brain.  I did look like I had mannequin legs though.  I felt quite wobbly walking out to the taxi, everything was so rushed I think adrenaline was getting me through. We said a teary goodbye to my parents, I really did find this upsetting as I wanted to end the journey how we started it, together. The journey wasn’t too bad to the airport. As we changed flights so quickly we didn’t book special assistance which proved difficult in the airport getting my wheel chair, myself and all our bags through check in however we managed. Once checked in we went through security, what an episode that was!!! When you’re in a wheel chair you are pushed through then your wheel chair is searched, I was searched and neck brace swabbed it took around 10-15 minutes!! I must have a suspicious look about me !! We then headed up to the lounge where we waited to be called for the flight. I rang my brother and sister in-law to explain what was happening. I sat back on a chair with a cup of tea, exhausted and struggling with my vision which I had/have since surgery.

Our names were called and Nick wheeled me to the aircraft. Not long after boarding a lovely member of staff came over and said he would be looking after us on the flight and should we need anything at all just to ask. I was a little teary on take off as I wanted my parents to be with me and didn’t like the thought of them back in Washington, it didn’t feel right we weren’t altogether. I remember looking out of the window listening to the same song I did when we took off from Dublin five weeks before not believing this journey was coming to an end. It felt surreal. Muscle spasms and headaches prevented me from sleeping during the flight and due to numerous food intolerances and gastro issues I didn’t eat on the flight. I watched Nick as he tucked into food I could only eat in my dreams now. I tried to get as comfortable as possible by making a little pillow den and lay flat to take the pressure off my head and neck.

 

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We soon landed in Dublin, the flight was just over an hour faster on the return leg due to wind direction. As always the care with Aer Lingus was outstanding, I couldn’t recommend them enough. It was around 4.30 am (U.K. time) I was absolutely exhausted. My vision was making it difficult to navigate through the airport to the arrivals gate. Waiting at arrivals was our friend Rich who very kindly arranged again for us to have a few hours at his friends flat just around the corner from the airport for me to be able to get some breakfast and lay flat before our Dublin-Manchester flight.

Back at Rich’s friends I attempted to eat some cereal which was a sloppy affair, I couldn’t open my mouth that wide and food was sticking in my throat. I actually felt very nauseas so gave up the cereal war and tried to rest my body. The muscle spasms had totally kicked in by this stage and came with no warning, some so strong they through my jaw out and visually made my face jerk. Absolutely no fun at all. Nick was walking around the room trying to stay awake, we hadn’t slept In 24 hours. I do okay without sleep as I’m used to it but Nick is definitely a man that needs his sleep 🙂

A few hours had passed and it was time to head back to the airport for our connecting flight. We had already checked our bags in as we came off the Washington flight so it was just a case of going through security (again). We said our goodbyes to Rich and headed through security. Once through we headed towards the gate whereby they were boarding people onto the bus. As I was in my wheel Chair I got priority boarding on the front where I had a little giggle with the driver. As we arrived, the plane was the same size as last time – a coach on wheels !! The flight back was bumpy and both myself and Nick felt a little queasy. Once we descended into Manchester I had a few little butterflies, home we were finally home. I made it.

The journey back home was rough I felt very sick in the taxi after all the travel and dizzy but when we pulled up outside our home I cried. I cannot put into words the sheer relief I felt that I had made it and I was finally home. I had missed our home so very much. Nick opened the door and my little man Colin the cat came downstairs and was greeted by a weepy mummy giving him lots of cuddles. He had been well looked after by Nicks sister Michelle, so much so I don’t think he missed myself or Nick a jot!!! I feel the need to share a little picture of my Colin, he does not always look as angelic as this!!

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It was unbelievably nice to be home and true to form the first thing I did was have a good old cuppa and then plough through my NHS letters.

We haven’t managed to catch up with all our friends since returning as things haven’t been easy at all. So what’s happened since returning to the U.K. and what does it really feel like to have rods and screws in your head and neck?

A Diary of HDU, time lapses and all !!!

Ideally I would have liked to have given a full and in-depth account of my time on the HDU however my memory does not serve me well at all. I think the combination of pain medications I was on had an effect on my memory as I can only remember snippets of each day. Nick stayed every evening and my parents everyday, well so they tell me!! I was that gone at times I wouldn’t know who was sat beside me. If you’re reading guys……I know you were always there!!!

Wednesday 13th September-

I was transferred to the High dependency unit around 10 pm, apparently I had somewhat of a comfortable night, waking on average every 15 minutes and pressing the pain pump. I vaguely remember the pain waking me and with being in HDU the room often being busy with staff putting things in my IV’s, but no real specifics the first night.

Thursday 14th September-

I recollect the pain being pretty intense and repeatedly saying “if I knew it was going to be this painful I wouldn’t have had it done” now I was still off my rocker on the medication but the pain was very real. The staff increased the dosage in my pain pump as I had asked for a smaller dose to start with due to my gastro issues. I was wheeled for a routine x-ray early doors and I really do remember giving off about the pain as we went over the bumps of the elevator in the bed and when asked to lean forward for a board to be placed behind my head I quite literally couldn’t move my head it felt as though I had a dumbbell in the back and staff had to pull me forward to place the board. I had little interest in eating or drinking anything and continued to suck on ice chips. I was struggling to swallow quite severely which was quite frightening. Dr Sandhu was called and he immediately attended, I vaguely remember seeing him and him ordering an emergency CT just to ensure the fusion was in place as sometimes if fused too far forward this can impinge swallowing. I have zero recollection of the CT scan. The swallowing team came and assessed my swallow, again I have no recollection of this. I was placed on puréed food to be reviewed the following day. I didn’t eat anything, I had no appetite and wasn’t really with it half the time. I vaguely remember trying some cranberry juice and apple sauce and having a serious discussion about how apple sauce should be consumed with pork and not on its own out of a tub!!! Sponge swabs and ice chips it was. Dr Sandhu was more than happy with the CT scan and the positioning of the hardware, the swallowing was put down to swelling and muscle spasms. I am still having issues with my swallow now so were monitoring it and at times the spasms are knocking my jaw out.

Friday 15th September –

Again a total blur, it’s a good job I’m not writing a novel about this hospital stay with the amount of memory lapses and gaps but I have to be truthful. My parents said as soon I pressed the pain pump within minutes I would be asleep. I do remember discussing with the nurse my love for cups of tea, it was even on my notes which I thought was lovely and very personal. Happy Friday people!!!

Saturday 16th September-

Another hazy day bar one significant event that got my attention. The catheter had been removed of which I have zero memory of, it then got to the 5th hour and i hadn’t managed a wee. My wonderful nurse Brian hadn’t long been on shift and asked me if I was Urinating okay since catheter removal. I informed him I hadn’t been, he came over and my stomach was quite distended. Then came quite a frightening rush of people and a portable scanner which showed I had over a litre of urine in my bladder. Before I knew it my bed was being tilted back and I was being told the catheter needed to go back in. I was shouting for my mum, who had gone back to the rental at this point as I felt a little scared as I knew that often if the bladder doesn’t work it can be an indication of tethered cord which can become more evident after fusion surgery so I was concerned this was at play and id lost function of my bladder. The catheter was placed back in which was eventful as I have a stricture in my urethra so you can imagine how delightful it was!! Soon the bag was filling up and the pain and swelling in my tummy eased. The nurse said there was a strong possibility this happened because I was still on the PCA pump which can effect the bladders functioning whilst on it and the plan was to stop the PCA and then remove the catheter tomorrow.

Sunday 17th September-

I remember being a little more alert on this day and took the brace off for the first time and got nick to take a picture so I could assess the undercut and rib situation !!!

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I had a new nurse called Holly who was wonderful, literally lovely. I was sat in the same position for so many days as I couldn’t bare to lay down flat with the pain in the back of my head I’d developed a sore on my coccyx and it was so painful, Holly disappeared and came back with an inflatable waffle type cushion which she placed underneath me which took the pressure off my coccyx. I was removed off the pain pump and told I would be going onto oral meds with the view of taking the catheter out to see if I could pee myself. I was concerned about oral meds due to my gastro issues so we agreed I would have the stronger meds through my IV in my wrist to bypass my stomach. The catheter was removed again and it was a wait and see if I could pee. I felt under pee pressure!!! I remember my first pee on the wonderful commode, who knew how monumental peeing in a bucket on wheels would be!! My bladder worked yipeeee. To make my day even better the nurse brought in a sandwich bag of Yorkshire tea bags that the nurse on the previous night shift had brought in after our discussion about my love for tea. He had wanted to bring me a little bit of home, I almost cried, such a lovely gesture, what a guy.

Monday 18th September-

I could definitely tell the difference being off the pain pump, it attacked the pain at speed and in short bursts so you knew you had 10 minutes before you could press it again. Now I was on IV meds i could definitely notice the pain level increase. I managed to waddle out of the bed to the recliner chair and remember my head feeling so heavy I just wanted it resting against something at all times. Late morning we were told I would be discharged today on the premise the physio was happy I could navigate around. She came round to do her assessment, once she saw me attempt to walk and once she knew I hadn’t properly walked since before surgery she got me a walker. I really struggled to walk, I was very weak, my legs like jelly and they felt like they would buckle from under me. I walked like Bambi I couldn’t keep my head straight as my head felt unbelievably heavy. No one could have prepared me for that first walk, I cried and felt quite worried that my head would always feel this weight as I felt I couldn’t carry it. It was like learning to walk again and I didn’t feel I was going to manage it. Next were the stairs, I had to prove I could navigate steps before I was discharged. The physio supported me in side stepping the stairs as I couldn’t look down at them and told me to feel for each step whilst having someone in front and behind me for support. I then had to do a lap of the ward. Heres me after completing to ward lap-

 

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The physio cleared me on the proviso I had a walker to take home with me and a commode, yayyy always wanted my very own en suite !! We then sat for a couple of hours waiting for discharge papers, medications, the equipment and spoke to our lovely international liaison officer. I felt quite nervous leaving the hospital, a sort of insecure feeling. A wheel chair was brought up for me and we said goodbye, i remember this picture below being taken as once I left the hospital and went outside I couldn’t see properly, it was like my eyes wouldn’t adjust to the light.

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I cannot remember the journey back to the rental or going into the house!!

 

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. 

Meeting my neurosurgeon for the first time. 

We had only been in Washington a few days when it was time to meet my neurosurgeon, Dr Sandhu for the first time. I was still jet lagged and struggling with adjusting to the time difference but tried to just roll with it and sleep whenever I could really. 

It was Wednesday 6th September, We all took a taxi down to Medstar Georgetown University Hospital. My first thoughts as we approached the hospital were that of the sheer size with different entrance numbers depending on your purpose for visiting. The site was huge with many campus’s with it being a teaching hospital. 


*A very small segment of the hospital* 

We went up to level 7 of entrance 1 (physicians offices) and checked in and waited to be called. Whilst waiting I had numerous forms to fill out, my vertigo was still bad from the journey so i felt like the paper was moving up and down which made for interesting handwriting!!

Initially I was called by the nurse who was lovely and took my blood pressure, weight, height and asked me some general health questions whilst documenting them on the computer. 


Not too long after I met the man himself. It was lovely to finally meet the man who would soon be performing such a major surgery. I maybe biased but I can quite honestly say with confidence that Dr Sandhu is one of the nicest consultants I have ever met, he is extremely polite, non-assuming, very calming and down to earth. Although the topic of conversation was serious it was an absolute pleasure to talk with him. I had my usual 101 (22) questions all written down and prepared and he was more than happy to answer each and every one. 

We discussed the surgery in more detail, I am very much the type of person that needs to know the details. Dr Sandhu initially pulled my imaging up on the computer and explained how C1 and C2 are dislocating and over rotating and how this compromises the vertebral artery and how my clivo-axial angle was acute causing compression of the brainstem which meant I warranted a skull fixation -C1-C2. Dr Sandhu discussed the method which was his own he had designed. The hardware really is like mechano only much more expensive than at Toys ‘R Us and can be adapted and tweaked for individual anatomy. The most common are T- bars or two rods at the back of the skull but quite often surgeons will design there own method which they have found works well and will use that. Dr Sandhu has designed the ‘grappling hooks’ method which essentially was like two bolts either side of the skull, less hardware but felt they fixated to what is very thin bone in that area well. Dr Sandhu proceeded to show me on his phone the method on an open skull during a surgery, I am a little geek at times so i was fascinated by this and not at all squeamish . Shame I didn’t show such enthusiasm back in the day in biology and I might have passed the exam!! We discussed length of surgery, the potential for ‘surprises’ during surgery especially with having EDS and the risk of excess bleeding due to fragile tissue. I had many questions about other possible complications afterwards like developing tethered cord and instability in other areas of my spine. The truth is this is very much a possibility and all I can do is try and keep those areas as strong as I possibly can and keep an eye on it with symptom tracking and scans. Dr Sandhu explained he would take rib from the back and place this over the fusion with the main aim for this to take and my own bone to grow which is then a successful fusion as you need your own bone to grow as you can’t rely solely on the hardware. We discussed a little about what would happen the morning of surgery and how I would need to arrive 2 hours before and I would be taken to a ward which is almost like a holding area where all patients imminently awaiting surgery go. Here I will be marked out by Dr Sandhu, meet the anaesthetist, possible student doctors and have IV’s placed. I discussed some of my fears regarding my stomach with Dr Sandhu as my gastro system is so shocking, he was extremely reassuring that we would find a combination of medications that would work for my stomach and use maximum anti-nausea medications to try and combat sickness. He was extremely well versed in EDS patients and POTS so knew all the things that could potentially arise. I would say I was in over an hour asking various questions and airing my worries and anxieties, never feeling rushed and always feeling understood. 
At the end of the consult we shook hands again and I said I would see him on Wednesday, Dr Sandhu assured me everything would be okay. I trusted him entirely. 
Seven days to go …….

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”