Health Update- Part 3 (The Finale)

Congratulations…..get your cap and gowns as you’ve all graduated from the “Ali’s crash course of health issues”. This will be my last health update post and the aim from now on will be to update weekly/fortnightly on what is happening, how I’m feeling and run alongside that some hot topics I am itching to write about. Grab yourself a celebratory tipple for the final instalment. 

Gastro-

My gastro system was one of the first issues I had going back 10 plus years and we went round a merry go round of IBS, Crohns disease and many more illnesses over the course of that time having more investigations than I care to think about. You can read more about this in one of my earlier blogs “A gut feeling”.  Once I was referred to a neuro-gastroenterologist I started to have the right investigations and gradually the pieces of the puzzle started to fit together. Many people with EDS have gastric dysmotility this can be in the form of too fast or too slow transit or it can swing between the two. Both conditions share some similar symptoms but there are marked differences between them. One thing they both have in common is how debilitating they can be for the patient. 

A few years back I had exploratory surgery for a mystery “lump” during the surgery adhesions were found which are bands of tissue that were wrapped around my lower bowel. I remember the relief thinking we had finally found the cause, this tissue was cut and removed however I was told the possibility of this tissue growing back was high and in fact the more times you go in and cut the more scar tissue forms and the worse the problem becomes, great stuff!! Shortly ensued a colonoscopy and barium studies.

 My most recent “bowel” related test was something called a tracer study under nuclear medicine. I had to take a set of pills over the course of a few days, each pill dissolved releasing various shapes into my digestive tract which are highlighted on an x-ray. On day 7 I was scanned with the aim of the game being 80% of the markers to have packed up and gone down south, I’m clearly a Northern lass through and through as you will see!! The follow up with my gastro came who said the good news was the markers weren’t stopping at a particular junction which meant there was no need to remove a particular section of my bowel that had nerve death. I thought I was home and dry I turned to my mum relieved about this however I soon noticed the expression on his face and he explained that the entire bowel seemed to not be as it should even parts of my small intestine and it was a worry that this related to the nerves and muscles ability to propel contents along. He said if this was a case of ‘simple constipation’ the transit time is usually normal and most markers would have left with only a few residual in the sigmoid colon.  Below are x-rays of my small and large intestine, the images were clearer on my consultants computer whereby he could fade some of the white out for clarity particularly at the bottom of picture 1.  Off memory 59 remained from 72.

As you can see there is quite the shape game going on here and unfortunately they are dotted throughout which signifies an issue with the nerves and muscles throughout the large bowel and a portion of the small bowel. This is called colonic Inertia. The pain this causes at times is unmanageable, all of our systems propel food in what should be a nice rhythmic manner however mine is out of sync, completely un-coordinated and poor functioning which leads to such extreme cramping at times I have passed out not long after eating. The pain can fluctuate throughout the day but never leaves.  My diet is extremely limited and standard laxatives just sit in my bowel causing further cramping instead of doing the job they are aimed to do.  To try and relieve the pain of the cramping I am unable to take opiates as they feed into the problem further as they slow the bowel down and they don’t stay down long due to inflammation of my stomach lining. I was assigned a bowel function nurse and currently experimenting as and when with a medication called linaclotide, enemas, self bowel massage (funnily enough I can’t find a beauty establishment that lists this in there treatments) and some home remedies. There have been a fair few trips to hospital over the years with suspected bowel blockages.

A radioactive egg and jam sandwich came next, yes you hear me correct!! It was certainly not gourmet. This test was to study the motility of my upper stomach.  I explained to my consultant some days I feel my food is sat in my stomach for hours and I can only manage a few bites through nausea and feeling full and other days it feels its tipping out of my stomach too quickly and I get shaky, nauseas, low blood sugar and some other symptoms I will kindly leave out. Once I managed to get the sandwich down which is no mean feat I can assure you I was scanned at regular intervals. The radioactive substance within the sandwich is highlighted so they can trace its location in your system. On this day the result showed rapid gastric emptying, essentially my food is tipping out of my upper stomach quicker than it should leading to nausea, sweating, cramping, low blood sugar and not really feeling you have eaten shortly after a meal. I know there are times when my stomach is extremely slow and this would be slow gastric emptying of which I feel sick and I can’t actually get any food down at all of which I have nutritional drinks as a back up. It basically swings between the two but on this day it showed rapid.  As you can imagine its quite hard to treat a stomach thats tipping too quickly and a bowel and small intestine that are on a permanent vacation as the two contradict each other.

Since surgery I have had problems with my swallow, my food is sticking and I can either get it down with fluids or its back up we go. I had a barium swallow which showed some pooling of barium but we have not yet bottomed why this is happening daily. Thankfully I am not aspirating my food which is the real danger however none the less its extremely unpleasant and can be frightening. Due to NHS cutbacks my consultant has had one of his two clinics taken off him so he now has to cram two clinics in one so I don’t get to see him until October (insert frustrated face).

Iron Deficiencies-

I have difficulties with malabsorption of nutrients combined with a very limited diet I often don’t get enough of what I require. I had visited my GP some time ago reporting I felt much more tired than usual, breathless and pale. She ran some bloods which came back as my ferritin (iron stores) were extremely low. I have been here before and recognised the feelings. I have tried many iron supplements over the years and they do not raise my levels, they cause a lot of side effects and are now a contraindication due to my bowel difficulties. My ferritin has dropped further since the initial blood test and an iron infusion (cosmofer) has been arranged for late next week which will take around 6 hours if all goes well, ill get some movies downloaded for that one!!  Ive had one before many years ago and I do tend to feel quite unwell during and a few days after them but when this lifts I feel I have much more energy. Essentially I’m running on fumes at the moment so hopefully topping the tank up will make a positive difference.

Years ago I was placed on B12 injections after reporting the same symptoms as above in addition pins and needles and numbness. My B12 was tested and was rock bottom, I got quite unwell at the time as it was not reported properly by the hospital initially so it went untreated. I was commenced on B12 injections and have them 3 monthly.  As part of a new initiative my GPs practice are training patients or partner/family to do the injections. My partner and I went down for the training with the lovely nurse.  I didn’t think I would have a problem doing this injection myself and drew the fluid up, changed the needles and when it came to putting it in at a 90 degree angle (all the way in I might add) I just couldn’t push it in. I was surprised as I am not in the slightest fearful of needles.  My lovely partner stepped in and was shown how to give it in my arm. The injection is uncomfortable as it has to penetrate the muscle and not hit the tissue so it is a very deep injection and the substance itself stings but it is more than manageable and usually only lasts for a few minutes. My partner did a sterling Job and said he could not have done it to himself either but I have to be nice all the time now as he has the needles 🙂 The lovely nurse said we are welcome to come back anytime for a refresher which we probably will do, I will give it another go myself as I don’t like to be defeated!! Below is the sharps bin, B12 and needles, to be fair you can see why I struggled pushing that needle all the way in myself. My arms are so thin I’m surprised it didn’t come out the other side!!

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ENT-

I see my ENT consultant in a month to discuss setting  a date for my deviated septum surgery. I was on the fence whether to have this surgery or not, to be honest it would be nice to get my right nostril out of retirement and to alleviate the chronic sinusitis it causes as its no fun really. Ive recently been having problems with my ears which will be added to the list to discuss during the appointment.

Eyes-

I have Posterior vitreous detachment of both eyes meaning the membrane has separated from the retina. Its quite a common condition but usually in those over 65 not 33.  On my left eye the membrane has fully come away and on the right its hanging on for dear life and half pulled away causing floaters, flashes of light and sometimes it feels like I am looking through a cobweb, its extremely annoying however not painful.  I have to be careful for any sudden changes and have to be checked out by the eye hospital due to the possibility of retinal detachment which is a serious condition.

My vision is at times blurred, double but more so a loss of focus and clarity.  I am under vision therapy at present trying to get the brain and eye to communicate better however we are not sure if this is related to my head/brain and CSF problems which can cause similar symptoms so it feels a bit of a guessing game at the moment as to what is actually going on.  It is like this with many aspects of my health right now and many conditions mimic each other symptom wise so to pin down the origin is proving a challenge, a frustrating and tedious change at that.

I have dry eyes which are extremely annoying!!  They itch, burn, sting and feel like the gritters have been doing a day shift pretty much 24/7. I use eye drops recommended from my eye doctor about x8 a day and use warm heated pads on my eyes to add moisture to them.

There are MANY other things going on with my health, only yesterday I was told I had yet another issue which I will talk about in my next post. I just wanted to get the bulk of my current situations up to date for fluidity and now I am good to go.  As you can see there is an awful lot going with my body but just how does it feel physically, mentally and emotionally, just what goes on at the appointments, how much advocating has to take place, what mistakes are made, is there joined up care, how much support is available for those living with a chronic illness, what judgments are made about those living with invisible illness….these are just some of the topics I will be writing about over the coming weeks. 

*I receive many e-mails from my blog from patients and families which is lovely, if there are any particular topics you would like me to write about or hear more about please get in touch either via the comments section or you can e-mail me through the contact link via the menu*

 

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

Turners tours…….

Firstly I must apologise for not updating my blog sooner, since being back home we’ve been on tour…..of local hospitals *sighs*.

So……how am I feeling post fusion wise??? Well in terms of the surgery recovery I am doing very well, I think. I seem to have healed beautifully, the scar is very tidy and my undercut is starting to grow out to a more disheveled/shabby kind of look. I am in the process of weaning myself out of the brace and really just listening to my body during this process. The fact I can now carry the weight of my own head is simply amazing, I will never take that for granted. I’d say I manage about an hour before the real spasms kick in of which I either put the brace back on for a rest or lay flat and alternate heat and ice. Today I actually had my first physiotherapy appointment since surgery, it was nice to see my physiotherapist again, she is lovely. We are taking things very slowly and starting off with some light isometric exercises which strengthen the neck muscles, I will also be doing some extremely light core strength exercises. My body has become very deconditioned over the last few years and my muscle tone and control is poor but with EDS its always a case of starting right from the beginning and working our way up at a slow pace so for the next month it will be light isometric exercises and some stomach in/bottom squeezes lay flat. And on top of that some light daily massage (sadly the NHS don’t provide a masseuse) to loosen up the trigger points as my neck and shoulders are very tight since fusion partly due to the magnitude of the surgery and partly because of the limited movement I have now.

In ten days I will be having my post op three month x-ray to ensure nothing has worked loose and to see if the bone has started to grow. I’m quite nervous for this actually as the aim is for my own bone to grow over time for the surgery to be a success.

So many people have been asking what improvements I have noticed since surgery, this can be quite a tricky question to answer one being as it’s still relatively early days and also I have multiple chronic conditions that all feed into each other and give off an array of symptoms and fluctuate in nature daily. So far the very fact I can hold my head up independently again without a brace or holding it myself with my hands is AMAZING. I have noticed an improvement with my vertigo, tinnitus, neck pain and bladder issues which is very positive. I’d say the biggest blow so far is that the debilitating headaches I’ve had for 2.5 years daily still remain as well as talking aggravating the headaches further and for anyone that knows me personally knows how much I LOVE to talk!!! This has been one of the hardest elements for me, being muted 80% of the day just to try and keep my headaches at a medium/low level. I also struggle with sounds around me it’s like sensory overload (very bizarre). Myself and Nick are off to London in the New year to see a leading professor who specialises in headache disorders particularly in EDS so I am very much looking forward to meeting this expert. Headaches and EDS are very complex as there can be so many different causes from migraine, blood vessel problems all the way through to high/low brain pressure and anything in-between. I shall keep you all updated how that appointment goes. Before I banged my head 2.5 years ago and this cascade of symptoms came about I had never suffered from headaches in my life and they are so very hard to articulate as they are not like your average headache, or hangover headache (I wish) the pain moves all around my head and face, changing in intensity going from severe pulsating pain to knife like pain to pressure. Very odd. Very unpleasant. Very debilitating. This is the symptom that has stopped me in my tracks the past few years.

Now…..the rest of my body!! I have completed a diagram labelling a body of the various organs/joints affected for a clinician and thought I would share it on my blog. Labelling the body doesn’t really give insight into what it’s like living with each and every symptom but I thought it was useful none the less. *Get your magnifying glass out or use our zoom*

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Since being back my parents and I have toured the hospitals catching up with all my consultants. My parents…. literally the best humans on earth.

Turner’s Tour-

Tour 1

Myself and Nick went to see the pain team. We spoke with a nice lady and discussed my conditions and the impact they have on my life. As well as controlling my pain one of my big issues is coming to terms with my conditions and how my life isn’t what I expected. There are many aspects of this for me personally that I struggle with and I’ve decided I’m going to open up more in my next blog post about this topic I’ve quietly struggled with the last few years.

Tour 2-

I have seen my GP a few times to catch up with her post surgery and to discuss my worsening POTS symptoms as I’m not due to see my cardiologist until next year. It’s quite common for POTs to go crazy after major surgery and inactivity so I’m not surprised. My heart rate is spiking quite high but at present it’s coming back down on its own within a reasonable time frame of which I am thankful as there have been times when it’s landed me in A&E when it refuses to go below 150. My other issues are the inability to stand for longer than 10 minutes without intense blood pooling in my legs and low blood pressure. I am soon to start a medication to increase my blood pressure and widen the blood vessels, this tablet doesn’t come without side effects and possible complications so I will need to check in with my GP regularly for my blood pressure to be taken as it can cause hypertension and effect my heart rate. I also had some bloods taken and my 3 monthly b12 jab. I’m so fortunate to have a lovely practice with lovely doctors, nurses and staff.

Tour 3– Ear, nose and throat appointment. We discussed the surgery I require for a severely deviated septum which I’ve had for a long time however it has got progressively worse to the point my right nostril is now out of order. Given I will need to be intubated and require a bit of extension of my neck we agreed to catch up again in January to book a date for surgery hopefully middle of next year as I need a break but it maybe sooner.

Tour 4- The dietician came to my house, so I call this half a tour. She was extremely pleased with the weight I have gained but understands how it can fluctuate with my gastro symptoms. Although my diet only consists of around 10 foods she was happy I’m getting what I need to sustain myself and recommend i take some vitamins also. We have a plan of nutritional drinks that are easier to digest for when my weight dips. I have put back on the stone I lost after surgery which is fantastic news.

Tour 5– I saw my gastro as I am struggling immensely as always with my stomach/bowel. My gastro system has plagued me for around 10 years and I suffer so much because of it. Firstly we addressed how my food has been sticking since surgery and he was a little
Concerned if it was nerve related. He booked me in for a barium swallow. I have also had intractable heartburn/reflux/chest/back pain the last 6 weeks which isn’t responding to potent acid reducing drugs. My gastro wanted to do an endoscopy however I am unable to tolerate them without being put totally to sleep which he felt so soon after surgery wasn’t advisable but we are going to discuss this further as the symptoms are debilitating. Way back I had a study called a sitzmark study which involved swallowing various pills over a few days, each pill contained different shapes and then being x-rayed to see where the shapes were. The majority of the shapes are meant to have left the body however with me 90% remained, I’ll never forget when he turned the screen round to show mum and I and my little intestine and colon was just swimming with little triangles, circles and rectangles, I didn’t know if it was the shape game perfection from the 80’s I was looking at!!!! This shows a condition called colonic inertia, basically the nerves and muscles in my bowel don’t fire off like they should it’s a step up from good old constipation and the worst thing for it is high fibre foods as they cause unbearable cramping, broccoli is the devil!! We discussed how I seem to fluctuate between dumping syndrome (classic name) whereby I eat and within 30 minutes I am sweating, pass out and find myself on the thrown (too much information?) and within an hour or so have low blood sugar like I haven’t eaten in days or extremely slow stomach transit whereby my food sits in my stomach for hours and hours causing indigestion/ nausea and bloating, couple that with my snail like colon and it’s HELL. The nerves in my stomach just misfire and the muscles are uncoordinated so I often pass out with the level of cramping and pain. I am being sent for a gastric emptying study in the new year to chow on a radioactive egg and jam butty to see the speed food is leaving my stomach.

Tour 6- Barium swallow day. I went and swallowed some chalky paint which tasted of refreshers and stood on some kind of contraption whilst x-rays were taken to assess my swallow. Sadly due to fusion I couldn’t complete the final part to look for reflux and hernias as it involves getting my head into some rather funky positions I couldn’t do. The final part of the test with me being gluten free involved me eating a mince pie top I brought with me coated in barium (how to kill a good mince pie right there) !! I chewed it to death to get it down, more than I would normally as I wasn’t allowed water to send it on it’s way and didn’t want to choke.

Tour 7- caught up with my lovely physio who has put me on a very light post fusion programme.

We have speech and language, pain clinic x2 and x-rays before Turners tours finally finishes 28th December for the year before resuming in January which is booking up fast, magical mystery tour has nothing on us.

 
In other news-

During all of the appointments and symptoms I have tried to get back to making the odd item. It’s very important for me to achieve something with my day for my pride, my mind and my self worth. Although now everything has to be adapted and done in stages (gone are the days of spending 10 hours behind a sewing machine) everything, due to pain has to be undertaken in 20 minute stints. I’m still trying to learn this pacing business. However I am super proud of the few little things I’ve made and achieved, it really is the little things at the moment that keep you going.

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I thought I would finish off with a quote that I read daily at present through the trials of recovery and EDS.  I think its ideal for anyone who might be feeling overwhelmed right now, for whatever reason.

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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”