A Downward Spiral….

It is most definitely true what they say that the longer you leave something the harder it is to pick up and get back into.  In truth, the last five months have been and continue to be gruelling, the amount of symptoms I am enduring has made it difficult to even function on a “around the house” basis resulting in a monumental amount of neglect to my blog.

When my health becomes unmanageable and many symptoms and systems fire off simultaneously I tend to go into ‘hermit mode’. I withdraw from social contact whether that be through social media or in person. This isn’t because the desire to be around and interact with people has gone its because my physical ability to do so has diminished to such an extent I am on the whole unable.  My reserves have gone and the engine is chugging through on empty.  This is when the world of chronic illness I am in becomes extremely isolating and lonely.

In my last blog I touched on how hard it can be to manage the constant ebb and flow of symptoms, how you yourself can find it mind boggling how your capabilities from day to day are ever changing and you just don’t know whats around the corner.  It is extremely hard to ride the waves and go with the flow.  It requires an inordinate amount of mental strength and agility.  What about when there are no waves to ride?  There is just a giant sinkhole and you’re right in it. You tell yourself “things will go back to there usual level of uncertainty/symptoms in a week, next week, the week after”.  Soon a month has passed and you are still in this decline, will it ever end? Two months become five months, you are still declining. This is unchartered territory. 

You name it I can guarantee my digestive system and I have been there over the last 10 years. Gastritis, erosive gastritis, H-Pylori, Sibo, abdominal adhesions surgically cut, dysphagia, colonic inertia, heartburn, choking, acid reflux, nausea, severe gastric dysmotility, malabsorption, nutritional deficiencies, diarrhoea, gluten intolerance, extremely limited diet, severe pain, nutritional drinks and some more gross aspects we don’t need to go into further!! The truth is I am never without a stomach symptom, each day is like a lucky dip of any of the above.  As well as never being without a symptom my pain is never below a 5 in my digestive system anywhere from my oesophagus to the back door!!  When you have been in chronic pain for so long your body can learn to adjust, it almost acclimatises to it and when my pain is at a steady 5 I am much more functional.  I used to go through what I called flares whereby my pain would be anything from a 6-10 again with a multitude of the above symptoms and extreme abdominal cramping.  Like with anything I never knew when the flares would come they just came and went as they so wished.  I spent years trying to identify patterns, foods, triggers to the point it exhausted me and the truth is like anything with EDS it will just come.  I could be down and out anything from a day up to two weeks before the usual order of 5 was restored.  My body would be wiped out and I’d slowly regain strength. 

Around 10 months ago I was noticing I was needing a medication I reserved for “severe flares” more regularly to try and control the pain, it then became I was taking it daily.  It then got to the stage where it was having little to no effect at all. I switched it up with a different brand that did the same thing however still found no relief.  Soon my pain was a 8-9, I was missing valuable medical appointments and I was struggling to do the things I had worked hard to build up since my neck surgery, I felt the rug was being pulled from under me yet again. I continued to tell myself “this will ease off”. This is quite a common and a much needed pattern of thought in the chronic illness community “this pain will end” to help us get through that moment, that hour, that day. It didn’t end though. 

One afternoon in early summer I was eating my lunch and the already 8-9 pain soon became a 20, I didn’t even know pain like that existed.  My heart began to race, I started to sweat, the pain was so intense I couldn’t scream, shout or cry. I woke up on the bathroom floor, my lip bleeding and a chip in my bottom tooth.  The cramping was causing unimaginable pain.  I was sweating yet shivering, I clambered up onto the toilet and down I went again and woke up well…. in a situation shall we say !! This was familiar, I had these types of attacks before 1-2 times a year.  They are extremely frightening. The cramping becomes so severe my body basically shuts itself off almost like a system reboot and I faint.  I decided to remain on the floor as it was dangerous to be fainting from a seated position to the floor with a fused neck and I valued my teeth!!I lost all my dignity this day. Unlike previous episodes this continued for much longer and with such velocity. The next day I weighed myself and lost the predictable 5 Ibs I always did after these episodes.  The usual pattern would be id be tender and more sore than my “usual” for a week or two then I would go back to my “usual” and the weight would come back on.

  Five months down the line and the pain is still reaching a 20 daily anything from 0-5 hours after food/liquid intake and sometimes no food is required for it to occur.  Although my diet is limited to around 10 foods I have forced myself to eat knowing whats coming thereafter, which is no mean feat yet I have continued to lose weight.  My wonderful dietician came round to do a home visit a few months back,  she calculated my daily calorie intake and although its not a diet where you would put hoards of weight on it was enough to at least maintain a weight, she also included 2-3 ensure plus drinks at around 400 calories per drink yet I have continued to lose weight.  The pain has resulted in the gains I started to make 6 months after my neck surgery to be undone, preventing me from attending valuable medical appointments, become even more deconditioned and I continue to faint when the pain becomes unbearable. I feel weak with overwhelming fatigue grappling with pain so severe its wiping me out. Looking at myself so thin is not easy, I’ve invested in lots of baggy jumpers in a vein attempt to hide the weight loss.  The mix of frustration and sadness is crushing. Im eating through such pain, doing everything advised and yet I am still losing weight. My GP has written to my gastro consultant and dietician to try and bring my appointments forward she is concerned, as are we that I have now lost 20% of my body weight and the continual level of pain and fainting from the pain is serious.  I am unable to take opiate based medication or anti inflammatories as my stomach lining is like tissue paper and develops sores/bleeds on the medication even with stomach protectors creating another problem so managing pain is very difficult for me like it is for many with EDS. I am on a medication which so far isn’t working however I am working with my doctor to increase it 10 mg every two weeks, if at the maximum dosage it still isn’t managing or dialling down the pain which right now I would settle for I will then have to be weaned off it.  With EDS this could be a number of things or it may not have anything to do with my EDS, I don’t really want to speculate other than we don’t like the direction this is going right now.

I would love to say the summer months saw an increase in sun, sand, sea and sangria, I explored new places and made wonderful memories. In my mind I did. In essence the summer months were on the whole spent in my house, at hospital either through A&E or appointments. My dysautonomia/POTS took a big hit during the summer.  Looking back I think it was a combination of the uncharted hot weather and the major gastro flare. I was feeling a lot of palpitations for a few days, I didn’t really think much of it at first and tried to increase my fluid and salt intake which wasn’t easy with my stomach. As the days passed I felt worse, I checked my heart rate and found it to be 181 bpm, oh heck.  Often I can neglect certain aspects of my health, think it will pass and try and ignore it especially when my stomach was all consuming, it was/is taking over the show. Nick and I made the trip to A&E, once in triage and the nurse saw my heart rate at 162 (sat down) she soon bolted out of the room to get a doctor and I was taken into majors. 

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I explained about my conditions and my theory was the hot weather combined with dehydration and a gastric flare etc had amplified my POTS. Essentially my blood was pooling in the lower extremities with a weak flow to my heart causing an increase in cardiac output leading to dizziness, breathlessness, tremors, weakness and a super fast heart rate. We agreed on the first line of therapy to be an enormous bag of saline solution, they brought a huge bag and put it on the fastest rate to get it into me quickly.  I had blood tests and an ECG.

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I did numerous stand up bedside tests and the doctors could not believe what happened to my heart rate just on standing. Around half way through the bag I started to feel a little better tachycardia wise and we repeated the stand up test, my heart rate was now 140 bpm, still ridiculous but we were going in the right direction. They did notice my resting heart rate (lay flat) was low in the 40s which made it quite a dramatic jump when I stood up.  I explained I had only been in A&E a few weeks prior with a heart rate of 37.  I was placed on a beater blocker for my POTS but as I had a low resting heart rate it was bringing it down too low to worrying levels.  I have since been pulled from this medication and have beater blockers now when my heart rate goes too high and stays persistently high for a few hours I have to take one on a ‘as and when basis’. 

The last few months have felt like an endless stream of hospital appointments and tests.  It has seen us travel to various hospitals for nerve conduction studies, self-catheterisation lessons (now there is a funny story for another time), cardiac appointment, gastro appointments, copious amounts of blood tests, orthopaedic surgeons, orthotics, ENT, Opthamology and pain management.  I have also missed important MRI scans, spinal cyst appointments, hydro sessions and much more due to the severity of my pain.  I have an enormous amount of appointments ahead of me.  Although I am grateful to have the appointments I cannot tell you how much they take out of me with the travel and waiting times etc.  I can be out for days after. Sometimes I feel like I am chasing my tail on the never ending merry go round and not getting any further forward in many respects.

It feels like someone has pressed pause, its groundhog day. Im stuck in some kind of purgatory I am unable to get out of. I look enviously on at people living there lives and I feel stuck in time, not moving forward. Living with this level of pain and disability the last 5 months has been one of my biggest challenges to date. There hasn’t been a day I haven’t sobbed my heart out with grief, anger, frustration, guilt, exhaustion, boredom and above all PAIN. I am known to be very stubborn, push myself to the limits and often fight against my body and symptoms however the pain the last 5 months has brought me to my knees and I am fearful.  I am not fearful of what is causing the pain, more the thought of will this end.

“Im okay” this phrase will go down in history as “Alis most used phrase”. I find it difficult at times to articulate the pain that EDS brings to my life. The pain is widespread and differing in how it feels from burning pain, shooting pain, dull aches, pressure pain to severe cramping. How can you possibly get people to understand this when on the outside you look okay? It essentially effects everything from my head down to my ankles, muscles, nerves, soft tissue, ligaments, arteries, blood vessels, stomach, spine… yada yada. You can be hit by multiple pains within the same day at the same time, joint dislocations or subluxations, head pressure like your head will combust not to mention the symptoms POTS and dysautonomia bring. Each time you stand up you’re dizzy and almost faint, your heart rate is that of a marathon runner just from sitting to standing, your bladder decides its not working today, you have tremors so bad you keep your hands in your pocket so no-one can see, you’re so fatigued you feel like you’ve got the flu, Im not talking ‘man flu’ I’m talking fully fledged flu.  How can you get people to understand when on the whole you look okay?  You eat a biscuit and spend the next 4 hours in unbearable pain feeling like you’re dying, faint through it and then are so fatigued you have no choice but to go back to the oh so familiar place-bed.  No one knows as it happens behind closed doors. Now…. I could go on and on here, I guess what Im trying to say is it is so hard explaining to people your symptoms, that you’re not ‘better’, that not much has changed since the last time I saw you 5 months ago especially when you look ‘okay’. When people ask me how I am I find it easier to say “Im okay” I almost don’t want to appear negative, pride takes over . I am known to have a sunny disposition and I don’t want to lose that aspect of me as I’ve lost so much already so “okay” slips out. I am slowly realising it is OKAY that you’re NOT OKAY and its OKAY to say that.  I felt uncomfortable whilst writing this blog piece feeling it was negative, lacked humour and the usual cheeky joke here and there however it is a true depiction of where I am right now….

“It is perfectly okay to admit that you’re not okay”

 

 

 

Not so “Humerus”

What better way to start a day than with a 3 am blog post, I really do need to start tackling this sleep situation. It’s a shame I can’t work right now or I’d make a fortune on night shifts!!!! 

This past week has been refreshingly and unusually quiet and steady on the appointment front however high on the symptom front. The start of the week saw us travel over to Wrightington Orthopaedic hospital to see my shoulder surgeon, well a member of his team. 

I’ve been having increasing problems with my right shoulder. Any kind of movement where my arm is raised above waist height, infront of me or even doing quite simple tasks the rhomboid muscle swells so big I lose my shoulder blade to the swelling along with severe nerve pain across my upper back. The pain at times has reduced me to tears from burning/shooting nerve pain to a dull constant ache around the whole shoulder blade and upper back. Its extremely debilitating and impinging on my ability to function with the most basic of tasks.  Writing this blog for instance is undertaken in 10 minute stints, using my bed table, pillows supporting my neck/shoulders, wrist splints, neck brace and KT tape around the shoulder oh and occasionally sporting my migraine cap….its quite the look, I hope to feature in Vogue very soon!!

Around 18 months ago I had an MRI arthrogram, a test to study a joint. A needle was placed in the shoulder joint, which sat idle for a while as they injected contrast guided by an x-ray. At the time I was being tested for a lidocaine (aneasethic) allergy so any numbing agent was off the table, the doctor thought I was insane going ahead without anaesthetic but to be honest although not something “enjoyable” it wasn’t truly painful more an intense pressure within the joint. Once the contrast was injected a 45 minute MRI ensued followed by a POTs faint from laying down a while and standing up too quickly. The arthrogram revealed a rotator cuff and labral tear. At this time my main shoulder Symptoms were constant pain waking me up during the night particularly around the joint itself and at the front of the shoulder aggravated by lifting my arm above my head, reaching for things, typing, holding my phone…. basically anything involving the use of my shoulder, even pain at rest, weakness and lots of lovely cracking, my shoulder sounded like a bowl of coco pops pretty much all the time.

The shoulder is quite a complex joint, it is one of the most mobile joints in the human body and is mechanically quite poorly made, whoever designed such a joint needs sacking!! The rotator cuff is a group of four muscles that come together as tendons to form a covering around the head of the humerus. Your arm is kept in your shoulder socket by the rotator cuff so they are quite an important set of muscles. The labrum is a rim of soft tissue that makes the shoulder socket more like a cup by turning it into a deeper socket that holds to fit your humerus, your rotaor cuff then connects your humerus to your scapula. *My knowledge of the shoulder joint ends here* Basically its one big happy family of muscles, ligaments, tendons and bone that work together however with me its a seemingly disjointed, malfunctioning family that no longer communicates with each other. A labral tear can make your shoulder much less stable.

Knowing I already had the above issues we trotted off to inform the surgeon of a relatively new issue that has come about in the last 3 months with my shoulder blade. I discussed my symptoms with the surgeon and then followed an examination.  After the examination he asked me to lean against the wall with my arms in front of me and push into the wall a bit like a standing press up.  He then had me stand in front of him and raise my arms above my head (pain) you could hear and see the shoulder catching.  He then firmly held my shoulder blades and repeated the exercise with MUCH less pain around the blade. He said that as well as the rotator cuff and labral tear I have something called scapular winging.  Essentially the scapula (shoulder blade) is the largest bone of the shoulder and has the greatest number of muscles attached to it. Scapula winging can be caused by dysfunction and injury within the muscles themselves or the nerves that supply the muscles.  We discussed treatment options as he could tell I am finding the pain unbearable and it is hugely impinging the most basic of tasks on a daily basis. We discussed surgery as an option however the success rate of shoulder surgery on patients with EDS is actually pretty poor, this isn’t the first time I had heard this and the last thing I want is to be in and out of surgeries that continue to fail. We discussed pain relief as this is a challenge for me due to my stomach so my pain is rarely under control and medicated well as it causes a cascade of other symptoms. We decided to continue with the plan of hydrotherapy and get that booked asap and try and work on building and balancing the muscles to figure out if this is caused by a muscle imbalance/weakness/tear or if the nerve supplying the muscle is compressed which interestingly stems from the scalene muscles in the neck which are always swollen with me.  A follow up appointment for 3 months is planned of which the situation will be assessed again.

I left a little deflated and admittedly had a good cry when I got home.  The last few years so many aspects of my health are declining with new issues arising that we are unable to get on top of, as another issue or symptom is preventing me.  My head pain reduces my ability to function enormously and undertake physiotherapy so we are now also in a deconditioning cycle to add insult to injury.  Its very overwhelming when so many aspects of your health are going wrong, you have to try and get some semblance of order of importance as everything simply cannot be tackled at once.  When I sit and reflect on the inordinate amount of difficulties my body is facing I feel like I am sinking at times, there is no quick fixes which brings me great frustration and despair but we must add another layer to the life jacket and stay afloat as best we can although in truth I feel like I’m clinging on for dear life to an inflatable flamingo with a puncture!!

The rest of the week was spent organising my appointments over the coming months.  I have quite a schedule coming up. My appointment with the urology nurses to learn self catheterisation and self dilation arrived however had to be rearranged as it fell a few days after my iron infusion (due Friday) of which I feel terrible after and the last thing I would feel able for is a 2 hour lesson in poking various devices around!! We have booked this for July.  My Physio and I kept missing each other so I’m hoping we catch each other this week to get hydrotherapy booked, this won’t be for at least a further 3 weeks due to other appointments scheduled.

I received a letter from my neurologist regarding what happened with my little ticker whilst on the propranolol. He wrote asking me to start back on the propranolol this time instead of 80 mg slow release start on 10 mg normal release x3 a day building the dose up every few weeks however before each dose increment increase get an ECG to check on the old ticker then if the same symptoms happen again he will get a 24 hour ECG arranged to monitor what my heart rate is doing on the medication.  He has also booked me in for an up to date brain MRI with contrast so I am just awaiting that appointment.  I was a little disheartened regarding the medication as not only do I not want a repeat performance of the 80mg saga I also don’t want to push through side effects for weeks/months to then reach the higher dosage levels which are required to medicate the problem for said saga to occur again. We can but try, perhaps gradually increasing the dosage at a steady rate will give my body ample time to adjust to it. Who knows….

This week we are off to see my lovely orthoptist, to see how I am progressing with my vision therapy.  This lady would brighten up anyones day, she is such a jolly and fun lady whilst being super at her job. Friday is iron infusion day.  Im a little apprehensive about this if I’m honest. Ive only had one or two before many years ago and this one is at a different hospital so I’m unsure if you are provided with a bed or if its a chair as Im still struggling to be upright for long periods and the infusion takes around 6/7 hours.  I have a little anxiety about potential reactions, CosmoFer infusions can cause quite serious  reactions in people however off memory its done in an extremely controlled manner with a test dose given at a slow rate then observation over an hour of your blood pressure, heart rate and any symptoms you start to have if no serious reaction has occurred they will start you on the full infusions building the speed up over the day whilst regularly checking your SATS. I didn’t use to have these anxieties about “serious reactions” until I reacted at the dentist many years ago to the anaesthetic, I could not breath, developed a swollen neck with a rash, my fingers started to turn white and an ambulance was called.  Needless to say it has most definitely left a mark on me and has produced an anxiety that was never there before of “breathing difficulty” type reactions. I am much better now at managing this anxiety and rationalising the situation, not giving into it and avoiding treatments because of it, well……… perhaps the dentist a little!! That might be due to the huge bill that follows each visit!! Going off my previous infusions I have felt quite unwell during them with nausea, stomach cramps and shivering and for a few days after them with what feels like a dose of flu, headaches, fatigue, nausea and stomach cramping.  This is quite common after this type of infusion though and I’m just trying to look past all that and fast forward to two weeks where hopefully I will feel an increase in my energy levels, reduction in fatigue and breathlessness which is only a POSITIVE. Sometimes we just have to push through tests and treatments for the greater good.

As I’m not feeling my best, this week I plan to rest up and reserve my limited energy for the appointments, you’d be surprised just how fatigued I get after hospital appointments.  Us chronically ill folk can often miss important appointments which are designed to help us because we can be too unwell to attend, oh the irony!! Which leads me onto my next blog post I’m working on “Consistently inconsistent”……

Thank you for reading and I hope you all have a lovely and positive start to your week wherever you may be.

 

Health Update- Part 2 (Orthopaedic, Urology & dermatology)

Im back again…. with another health update.  I am conscious these health updates are quite clinical and factual and you may feel like you’re reading a medical encyclopaedia or studying for your finals.  The truth is when writing about my various health conditions I find it much easier to write in this tone and almost detach myself from the situation and be quite factual in my writing.  These conditions are having an enormous impact on my life and those closest to me with much emotional upset and turmoil however I feel I want to write separately about that soon.  I am not being very descriptive about how some of these symptoms actually physically feel like day in, day out and again I intend to do posts talking about this aspect.  I have so many ideas simmering away for future blogs from emotional well-being, the massive impact this is having on us, the health care system and much more which I plan to run alongside weekly blog posts.  As mentioned yesterday I aim for the updates to be completed by weekend so I can get stuck in.

With Ehlers danlos syndrome being a connective tissue disorder it means our collagen, the most abundant protein within the body is weak, fragile, stretchy and overall not made very well.  Collagen is found throughout the body providing support to skin, tendons, ligaments, blood vessels, bones and internal organs.  Essentially the glue that holds our bodies together is of poor quality.  As you can see by the role connective tissue plays within the body this can give rise to a whole host of multi-systemic symptoms throughout the body.  One area largely effected are the joints.

A large portion of my joints have fully dislocated over the years whereby there is a full separation of the bone from the joint (ouch) these include my right shoulder, fingers on both hands, right wrist, both knees and ankles.  I also get what are called subluxations which is a sort of full dislocation “tease” otherwise known as a partial dislocation or significant structural displacement of the bone. I get subluxations of the above mentioned joints as well as my neck, left shoulder, hips and jaw.  Personally I am more plagued by chronic subluxations of said joints numerous times a day.  With the subluxations being daily the pain which accompanies them and that they leave behind is chronic in nature. That isn’t to say I don’t get full dislocations as I do however some joints are affected more than others. My worst areas for full dislocations are my knees, right wrist and fingers on my right hand. My right knee (the devil) is a daily occurrence and on average wrist and fingers weekly depending on what I am doing.  My knees have dislocated whilst turning over in bed which believe me is more efficient in waking you up than any alarm clock going !!  At present my main struggles relating to my knees are firstly navigating the stairs in my house, going down them isn’t as bad as going up. Im either having to bottom shuffle up, which to be fair the old girl could do with a work out or if able take the stairs one at a time. Secondly getting in and out of the bath, I’m still working on my partner getting a walk in Jacuzzi bath however don’t seem to be making much progress!!

When a joint fully dislocates it is painful not to mention looks damn right weird.  I have had so many dislocations, like many people with EDS we learn to relocate the joint ourselves or we would never be out of A&E (ER), due to the nature of our stretchy collagen some joints believe it or not can be quite easy to relocate/pop back in.  There have been times that I have been unable to relocate the joint namely if my left knee dislocates inwards instead of outwards which thankfully isn’t a regular occurrence or when I have thought I had relocated a joint only for it to repeatedly dislocate and find out via x ray it wasn’t quite in.  When a joint dislocates it isn’t just left at that it can cause damage to the surrounding ligaments, tendons, muscles and nerves thus feeding in to the cycle of further dislocations and chronic pain.

I find the chronic pain of frequent subluxations almost worse than a full dislocation. When a joint fully dislocates the pain is acute, I maybe known to shout out the odd naughty word, however mentally you know that level of pain will inevitably fade until the next full dislocation whereas the chronic joint pain I find much more exhausting and problematic. I have a lot of aids and braces to hand which I use as and when needed.  I alternate heat and ice daily and myself and bio freeze are very good friends.  I will only use a brace if a full and particularly nasty dislocation has occurred or a joint is going through a phase of persistently dislocating.  The braces rest and immobilise the joint which in turn gives a good level of pain relief and time for healing. I am however very mindful if the braces are on for too long the muscles can atrophy quite quickly which ultimately feeds into the problem further.  As we rely on our muscles to hold and support our joints as many of us have ligament tears, damage, long stretchy ligaments that are unable to to do the job they were created for it is vital to keep the muscles as strong as possible (easier said than done).

I recently saw my wonderful physio, I absolutely adore this lady.  She has just the right amount of care, compassion, understanding and empathy whilst telling you just what needs to be done. I told her about my ever increasing problems with my shoulder, limited range of motion and that around my shoulder blade keeps swelling when trying to do certain basic tasks using the right arm/shoulder and that the pain has me either in tears or screaming when its nerve type pain. When you have so many pains you learn to differentiate between muscle and nerve pain quite well.  She suspects a rhomboid strain or even tear as well as the rotator cuff and labral tear I have. My shoulder blades are also misaligned. We arranged for me to go back to my shoulder surgeon for review which is coming up very soon. She brought another physio in who looked at my shoulders and both were concerned about marked muscle wastage around the shoulders, shoulder blades, arms and upper back which a few consultants have noted recently also. I explained when I try and do even the most basic of the exercises the shoulder blade pain flares so severely I am left having to lay with ice on it to bring the swelling down as the blade disappears through the swelling. After a discussion it was felt I would benefit from hydrotherapy as the water will not only support my joints but the heat will also ease my pain whilst we start to mobilise the joints and build up the muscle in surrounding areas.  The muscles are not only wasted, whats left are imbalanced and weak so there is very little to support me right now.  Sadly this isn’t just exclusive to my right shoulder this is going on throughout my body and my goodness the pain!!

We have to be cautious getting in the pool with my POTS but as long as I’m well hydrated I’m hoping I will be fine.  They have had a few POTS fainters in the pool before but we can only try I say and I’m all for it. Best get myself a decent cozzy!! Ill be starting this as soon as I have had my upcoming iron infusion. The physios taped my shoulder and gave me two very basic exercises to do which although uncomfortable are bearable and don’t seem to be swelling the area up. We had another little discussion about pacing and listening to my pain instead of fighting against it (Don’t seem to be catching on with this very well) often letting stubbornness and sheer frustration take over the show.

I hobbled off to see an orthopaedic surgeon about my knees who examined both knees.  Based on my MRI scans a number of years ago I know the ligaments are not in great shape and the grooves of my knees are shallow allowing the knees to slip out quite freely.  In fact on the MRI it was noted both knees were subluxed in neutral lay down!! The consultant sent me for an X-ray and an up to date MRI of both knees and I see him in clinic in August for the results.  He discussed creating synthetic ligaments as its no good using my own tissue and pinning the knee caps in place however I firmly want, when I am more able to get some physio underway before taking surgical routes.  I will however see what he says in August.  I was also sent to podiatry to have some insoles ordered which will try and align my knees better.

Next came the multi-skeletal clinic and the topic of conversation was my wrists.  For many years my hands have been going numb, tingle with pins and needles and change colour particularly if the wrists and elbows are bent.  My GP had already sent me for an ultrasound of both wrists to rule out anything structural so this was step 2. The specialist took a detailed history and began to examine my wrists and elbows.  She put my wrists and elbows in various positions and pressed certain areas which brought on the symptoms of pins and needles and numbness quite quickly.  She explained she was putting pressure on the nerves which is what happens in people with carpel tunnel syndrome and ulnar nerve entrapment when the wrists and elbows are bent. The longer the nerve was pressed my hand changed colour, I explained to her I could never hold a cup of tea for more that 2 minutes before my hand would go numb, change colour and id drop the mug – my poor carpet and what a waste of brew.  She briefly explained the surgery which involves releasing the pressure off the nerve and said as it is happening in both my wrists it would make sense to have my dominant wrist done first.  She has referred me on for nerve conduction studies and said once in receipt of the report a referral will then be made to consult with an orthopaedic surgeon.

Urology-

For as long as I can remember I have had problems with my bladder, for a long time it was put down to “irritable bladder” that absolutely annoying phrase used by some doctors, usually when they aren’t quite sure whats going on and don’t wish to pursue with testing.  I persevered with it for many years until a few years ago it became much more troublesome. When you have so many health problems you sort of create a priority list in your head of level of importance.  Almost like a mini risk assessment of how much is this impacting my life through pain, discomfort etc and this id say was around the middle of my list.  Before my fusion surgery I saw a fabulously thorough NHS lead urology consultant who did not feel this was “irritable bladder” by how I was describing the symptoms. I won’t be shy…. I have a constant pressure feeling I explained to him in “that region” and a consent feeling I need to pee, I will pee and have to almost push it out and I never feel like I am finished which results in a large portion of my day sat trying to piddle, I think a reason why there should be some kind of entertainment system in my bathroom to join the walk in jacuzzi bath (I can but dream).  He knew all about EDS and how the bladder can be lax, nerves not necessarily functioning correctly and much more. By the symptoms I was describing he suspected a stricture. He ordered urodynamics testing which I must admit I did not find pleasant at all involving a catheter inserted up both ends shall we say whilst you pee, stop, pee etc during this pressures are taken and how much fluid you retain is detailed. The results came back as normal filling capacity with high pressure, slow flow and some retention. Below is some of the tracking results, not that I have a clue what it means.

I was advised I would need a rigid cystoscopy under general anaesthetic and urethral dilation and was advised to start self-catherterising at least x1 a day and self dilate my own urethra (fun, fun,fun). Given we were shortly heading for surgery I did not do any of the above as the main focus was getting to Washington. Since our return I have been back to urology and the pain remains, I am currently awaiting an appointment with the urology nurses to learn the process and I’m awaiting an appointment with an anaesthetist to try and get a date booked in for the rigid cystoscopy to open the urethra and have a peek inside my bladder. The consultant did say this is something I may need to have numerous times over the course of time as the urethra can narrow again and again.  Sadly the urology services are so busy I am still awaiting my appointments.

Dermatology-

I have a few skin “things” going on and after showing my GP she sent me onto  dermatology for review. After explaining the symptoms and showing the dermatologist I was diagnosed with a few different conditions. Firstly Erthema ab igne, this is quite a common condition seen a lot in the elderly. Essentially my skin when exposed to heat sources such as heat pads, hot water bottles, if I sit next to the fire, rest a brew on my lap will burn, mark, scar and go extremely red initially followed by brown pigmentation  which can either last months or scar. There is no cure for this or magic potion other than removing the use of heat sources however like the consultant said is not easy to do when you have a chronic pain condition. There are laser options to try to make it look aesthetically better.  I am trying to experiment with different heat sources to find one that does not cause such significant marking. I am also finding aloe vera gel quite useful and bio oil. Secondly is a condition called Livedo reticularis which involves a disturbance of blood flow to the skin causing slow blood flow and reduced oxygen to the skin which results in what I have crassly nicknamed “the dead body” or “corn beef legs”.  Alongside this I also like many with POTS have blood pooling, if I stand for not much longer than a minute without moving my blood will drop to my feet and legs, they go very cold/sting/burn if I was to stay in this position I would eventually faint unless I moved around again to get the blood pumping back up to my head. Whilst in the hospital the other day a mild version occurred whilst stood in the queue, I moved one leg and foot purposely for comparison. I’ll put the picture below however with a WARNING as if you’re anything like me I HATE feet and I certainly don’t want to be putting anyone off there dinner!! This would be a mild version of blood pooling for me however I do not want to lose readers by showing the real deal.  Needless to say fake tan is my ultimate best friend for my legs however I often feel I am just replacing one problem with another as I am very challenged when it comes to the application of it, basically I’ve swapped red/purple blotchy legs for orange legs instead !!

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What a way to finish this blog with a picture of my purple foot, how lucky you all are!! Ill be back soon with my final 2 health updates.  I hope you’re all having the best possible day you can.

Ali xxx

 

The weeks following surgery

I have no recollection of getting back to the rental. I’d say my first memory would be laying in bed with ice packs on the back of my head watching friends. A very productive recovery in my eyes, there is something so comforting about friends you almost feel a part of it when watching or perhaps binge watching 10 years worth in two weeks does that to you !!
I recall utter distress trying to get myself out of bed, my head felt so unbelievably heavy to lift I had to hold the back and brace myself counting 1..2…3…whilst pushing myself forward. No one can prepare you for this weighty feeling and the fear your head is going to be that heavy forever plagued me quite often. It was such an unnatural and uncomfortable feeling.
I was unable to do much but rest in bed with many an ice pack, hot water bottle and Netflix. I had my own en suite, a lovely comode in the corner of my bedroom so getting to the toilet was easier . Not long after being back at the rental we had to return to the hospital as I was having trouble swallowing (still am) and we also noticed spots on the back of my throat. I am unable to remember the Journey back but remember sitting in my wheelchair in the waiting room fighting away the tears as it was so painful to just keep my head upright and that was in the brace. I felt truly dreadful. My mum came in with me and we saw two of Dr Sandhu’s nurses who checked me over and said I had a throat infection and prescribed some anti-biotics, I burst into tears in the room I just felt so unwell and in so much pain. I wasn’t taking the pain killers I was meant to take as my gastro issues were so bad I knew the tablets would make it even worse so I was only taking muscle relaxers and paracetamol. Both nurses said there was no wonder I was mentally and physically drained on no pain relief the first week of leaving hospital but I was adamant I could get through, not out of heroism, simply to try and avoid any further gastro flare ups which can be equally as debilitating as fusion pain. Everything with my health is a fine balancing act.

A few days after being home I attempted a lap of the landing, this picture taken below shows me walking with the frame, I have absolutely no memory of doing this walk. I’ve heard it was a big improvement from how I walked in hospital.

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This picture was taken a few days later walking without the frame of which I do remember. I recollect my head feeling so heavy, like it didn’t know where it was meant to be in space knocking my balance off but it was a big achievement to walk unaided.

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I remember these little walks taking so much out of me that I had to get back into bed. Bathing was equally as exhausting despite the fact my mum had to help me bathe. I couldn’t have asked for better carers my Mum, Nick and Dad all looked after me so well. My mum was never out of her apron with the kangaroo pouch as she nick-named it every-time she came upstairs there would be a snack, an ice pack or something stored in it for me.

After a while I started to venture downstairs navigating the stairs with the help of my Dad.  We set up camp on a chair in the doorway so I could feel the sun on my face and fresh air. I only managed short stints but it was lovely, each day we increased the time I was able to tolerate holding my head upright for. I made it into a mini challenge everyday. We then began doing walks with either my Dad or Nick across the street with the walker and then with my cane and holding onto them until eventually I walked to the local park (5 mins away) and was able to sit on the bench in the sun, granted only for 10 minutes as the pain was too great, I felt very unsteady and shaken but it was still an achievement none the less. This picture of the sun peeping through the trees was taken the first time I made it to the park.

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Frustration with myself soon reared its head of how slow I was at doing menial things. I knocked many a cup of tea off my side table as I couldn’t turn my head to pick it up. I couldn’t open my mouth enough to fit a tooth brush In to begin with due to muscle tightness and spasm. I wasn’t allowed to bend down to pick things up and all my movements were slow and unnatural not to mention painful. I desperately sought the fluidity of my movement again. It became apparent that when coming to the end of a drink I could not tip my head back to get the last part of the drink so in came the straws. This was all going to take some getting used too and I hadn’t even got into first gear experiencing my new neck mobility and range of motion (still haven’t).

Two weeks had passed, it was time for my follow up with Dr Sandhu back at the hospital. Dr Sandhu took my steristrips off and was happy with how the wound was looking both on the neck and where the rib was removed.

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We discussed some issues I was having with my vision and swallow and then went over some general aftercare for the next 3 months of which time I’ll require a scan to see how the bone is fusing.  Dr Sandhu pulled up some images of the hardware and was extremely happy with the positioning.  He answered my many questions regarding healing, muscles, pain, movement, feeling the bolts in the back of my head and much more, Im sure he thought he was on question time!!! He asked for us to arrange another appointment with him before we head back to the U.K. but so far he was very happy with the progress I was making and how well I was managing the pain.

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A day or so after my follow up I started to develop severe POTS symptoms, my
Blood pressure plummeted and heart rate high. My gastro system flared to an unmanageable degree and i was in a great amount of pain eating the smallest of meals. I wasn’t coping well at all, it was far too much hitting me at once. Over the course of days it became increasingly difficult to stand without feeling like I was going to pass out, my heart would pound and my legs were like jelly.  I increased fluids and salt intake which made little difference. We began talking about the possibility of coming home a week early and getting back to the U.K. as medical bills could have escalated if I was to be admitted which we felt was pretty  imminent.  Our friend liased with our airline and managed to get the flights changed for myself and Nick but sadly not my parents. We had 1 hour to pack our bags before we had to leave for the airport.  This was extremely emotional knowing I was leaving my parents behind as we had started this journey together and I wanted to finish that way. I hadn’t been upright for any more than an hour since fusion surgery, this was not to be an easy journey ……

*For those reading my blog awaiting fusion surgery I will soon add a piece of what I found helpful before surgery and during my recovery updating as time goes on in my recovery *

The day of my neurosurgery

After a restless evening of sleep I awoke, it was D-Day. It felt very surreal that everything we had worked for and focused on for the last 18 months was finally here. My family didn’t sleep too well either, it was around 4 am when the house started to bustle with noise from people having breakfast, showers and packing etc. I had attended my pre-op the previous day, being an international patient the majority of testing was done back in the UK and sent over so it was just a case of testing my blood type and running through some final health questions and informing me of what to expect on the day. We were up super early as my surgery had been brought forward from 12 pm to 8 am which suited me as it meant less time without food and fluids, two aspects that can play into a POTS flare for me.

Our taxi arrived at 5.50 am, it was still dark outside and the street quiet. The hospital was only a 5 minute care ride away from our rental which was ideal for Nick and my parents who would be travelling back and forth for the time I would be in hospital. We arrived at the surgery entrance and checked in. It was quite a large room sporting the obligatory fish tank for relaxation. Given it was so early the room was full of patients awaiting various surgeries that day. I was given my wrist band and then informed the surgery schedule had changed again and my surgery would now be 10 am and would head down to the holding ward at 8 am, we had 2 hours to kill and I couldn’t even have a cup of tea!! We sat at the far end of the room, we were all quite quiet, I could tell everyone was nervous, myself included. I felt a little weepy so attempted to distract myself with my music which as mentioned in previous blogs has become somewhat a saviour to me over recent years. I then became quite restless and fidgety and went to sit outside the hospital on the bench and tried to focus myself and my mind on what was ahead. I was very conscious not to become too panic stricken at this juncture and worked on the relaxation techniques I had been practising during the lead up to surgery. Dad came out shortly after and sat with me with the sweetest smelling coffee, I don’t even drink coffee now due to my various issues but when I smell it I can almost taste it. I proposed my dad and I pull up a taxi and make a run for it, as tempted as we were as no parent wants to see there child go through such a major neurosurgery we shed a little tear and decided doing a Houdini act was not one of my better ideas. We went back inside to wait, a nurse came over and explained that during my surgery my family would be in the relatives room and able to track the progress of my surgery with a unique ID number I was issued on screen and a staff member would come to give regular updates. Before I knew it another nurse approached saying the holding ward was ready for me, I had decided to take my mum to sit with me for the 2 hours before surgery. I gave both my dad and Nick a hug and told them I loved them. They both wished me luck and told me how brave I was. I could see they were both holding back the tears, as was I.

The holding area was essentially a ward with numerous bays and beds. This was the area where patients waited right before surgery and met with there surgeons, had IV lines put in etc. My allocated nurse was called Jackie and I could not have asked for a nicer nurse, so warm, bubbly and kind I could see why she worked with patients who were imminently heading into theatre she had just the perfect ammount of humour and warmth to add ease and calm to the proceedings. Jackie asked me a handful of health questions, did some final checks and explained Dr Sandhu and the anaesthetist would be round before my surgery. Jackie handed me a lovely gown, socks and hat to change into. Once changed another nurse came and placed an IV, I have notoriously rubbish tiny veins that often collapse so it was placed near the wrist which was a little uncomfortable but nothing major. Due to my POTS they placed a fall risk band on me and an allergy band for my allergies. Whilst waiting I started to cry, it was strange as I wouldn’t say I was in extreme panic mode which I thought would happen but I just felt overly emotional and frightened. Mum cried too and we gave each other a hug and again I asked if we could just leave and escape !! No-one was taking me up on this great escape !! I spoke to Mum about a lot of my worries and concerns, there is nothing like a mothers love and comfort no matter how old you are.

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Dr Sandhu then appeared from around the curtain and shook both mine and my mums hand. He had just come out of a lower neck fusion surgery and I told him to have a rest before he started work on me to ensure his hands were nice and steady, we joked. Dr Sandhu explained the surgery again and asked if I had any questions. My main question for Dr Sandhu was the repositioning of my skull. Dr Sandhu then took my brace off and made a few markings on my neck and said he would see me soon and everything would be okay. I found him very calming and instantly felt more at ease. Next came the anaesthetist who was quite frankly amazing, we discussed my issues with anaesthetic and vomiting, my pots and how my heart rate may become erratic and blood pressure low and she instantly put my mind at rest. I told her my fear of being awake during surgery which stemmed from a previous experience whereby after my stomach surgery I was In recovery with the intubation tube still down my throat, my brain had woken up however my body hadn’t and I could not move an inch with no means to communicate as I felt like I was choking on the tube. The anaesthetist explained that with the nature of my surgery not only will she be next to me the whole time monitoring me but I will also be plugged into a navigation system that also does her job. She went and got me a scopolamine patch for behind my ear used to treat post surgery nausea and informed me she had just got out of a surgery whereby the patient had POTS. I could not have asked for a nicer anaesthetist. She asked if I wanted a pre- med before going into theatre as most people do to settle the nerves and so you’re not really aware of heading in, ever the control freak I declined and said id rather enter awake as I don’t like the whole semi conscious state situation. We filled in some more forms together whilst some students came and observed me and did a neuro exam. With the hospital being a University hospital it is full of students training and eager to learn. The two students would be in my surgery observing. They were both keen to talk about EDS and the instability in my neck.

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It was then time to head down to theatre, I gave mum a hug and got myself properly into the bed. I was wheeled to the theatre door and at this point I said goodbye to mum, we told each other we loved each other and Mum had to take my glasses off me at this point which I didn’t like as I really can’t see too much without them. I was as calm as I could have been really, in fact I surprised myself at how calm I became. I think this also made it easier for my mum when she left to go and sit with my dad and Nick.

As the double doors opened into theatre although without my glasses my vision wasn’t the best I felt like I was in a real life episode of ER. The room was quite big, sterile smelling and I remember huge spotlights on the ceiling, big monitors and a bed quite high up in the middle of the room. The anaesthetist I had met came over to me and comforted me and another came over and explained some medicine would be going through the IV which may burn a little after they had run some saline through. I was asked to hold an oxygen mask over my mouth and nose and breath into it. I remember seeing a nurse at the bottom of the bed and thinking please wait until I’m out before you put the catheter in!!!! The last thing I remember was saying “please look after me”.

Unbeknown to me as I was out cold in theatre my parents and Nick were in the relatives room. It came up on screen that the first incision was made at 12.10 pm which meant I was being prepped whilst asleep for just short of two hours before any surgery began. This was to set my head in the right position to be fused, set me up on the neuro navigation system, position my head In the clamps and much more. A staff member came out to inform my family all was going to plan in theatre. My family just waited in the room throughout the duration of the surgery and as it was heading into the 5th hour they were wanting to hear I was out as they knew the expected time of the surgery was 4-5 hours and low and behold Dr Sandhu was stood before them in his scrubs and shook everyones hands reporting to my family all went as well as it could have gone. He informed them I was in recovery being woken up and a nurse would come for them when I was ready.

Now for me recovery was a blur. I remember briefly seeing the international liaison officer who came to check on me. Apparently Nick came down first to see me of which I cannot remember, he gave me ice chips and wet sponges and apparently I was giving off about a picnic blanket !!! My dad then came, again I don’t remember and then my Mum. Apparently Mum stayed with me the longest to try and get some medication down me as I couldn’t swallow but I have no recollection of this either. I am not used to medications so I think the cocktail I was on were really taking effect. I do remember complaining of a lot of pain and I couldn’t move my head an inch and a nurse explained I had a pain pump and when the light was on green to press it. After a good few hours in recovery I was transferred to the high dependancy unit, again I do not remember this or anyone being with me or this photo being taken below. I do not even remember any feelings of relief or elation that the surgery was over. Apparently at times I would mumble and then I would drift off again.

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Then came five days on HDU………..

My escape before surgery 

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

Manchester to Washington 

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

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

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




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

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

 

Turbulence

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

A Racing Heart

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

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

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

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

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Reading taken a matter of seconds after standing up.

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

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

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

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

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

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

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

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

The day I was diagnosed with EDS.

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

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

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

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

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

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

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

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

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

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

Beighton Scale

 

Image 1- Elbows bend backwards more than 10   degrees.

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

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

Image 4- Knees bend backwards more than 10 degrees.

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

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

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

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

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

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

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