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”

 

 

 

Iron-brew….

I still find it hard to comprehend such rapid changes in health, a day, an hour even a minute can make in this mind boggling world of chronic illness.  Its like an endless rollercoaster with dips you don’t forsee, your capabilities change with the ebb and flow of your symptoms. Chronic illness isn’t static, one day you could be thanking the gods for a reduced symptom day thats enabled you to go for a walk, eat a biscuit, listen to music, go on an outing and the next you are in hospital seriously ill. You don’t know how long either of those situations will last for and when they will arise again.

It is just shy of two months since my last blog post, ironically my next piece I planned to write was titled “consistently inconsistent’ which pretty much sums up the last two months.  It has been such an unsettling time, I have not felt physically able to sit down to write and needed some time out. In truth I am still struggling enormously however I started my blog for a reason and that is to show the good, the bad, the ugly, the embarrassing and the difficulties people with chronic illness face.

 It feels like an enormous task to try and relay and truly depict what its like living with chronic illness.  I soon hope to write some descriptive pieces giving a true insight into day to day living as well as write about some topics I am itching to discuss.  My next few blogs will be dedicated to what has only felt/feels like a living hell the last few months. 

*I will do a series of shorter blogs over the next week or so*

Towards the end of my last blog (June) I mentioned I was heading the following week to see my orthoptist and for my iron infusion.  Sadly no update on the eye situation as I wasn’t well enough to make my appointment and 7 weeks down the line we are still waiting to be allocated another. I did however make my iron infusion which on the whole went quite smoothly. 

My lovely Mum and I arrived early to the hospital and were taken into the allocated room, it was a narrow little room with around 5 reclining chairs, some patients were already hooked up to various infusions/medications. Once we ran through the obligatory paper work the nurse attempted to put an IV in my thin spaghetti veins which were not for playing ball.  She explained they like to put it lower in the arm or wrist where you are less likely to bend as if the iron leaks it permanently stains your skin.  After a good few stabs (literally) it was in and she hooked me up to the iron which resembled treacle toffee.

 

Initially a test dose was given slowly over 45 minutes to see if any reactions occurred. During this time the nurses monitored my heart rate and blood pressure regularly and asked how I was feeling.  Apart from being in need of a cuppa I felt okay. After the test dose the pump was started at a slow rate.  The nurses continued to come round to do observations and I must say they were lovely and very thorough.  My blood pressure started to drop which they were a little concerned about however I explained it isn’t all that unusual for me so I wasn’t too concerned.  Mother hen was chief “brew fetcher” and entertainer.  We did have a few giggles regarding the reclining chair, I don’t know if it was faulty or my ineptitude in trying to manoevere it but throughout the day I nearly ended up being flipped through the ceiling!!  

As no reactions had occurred and I was feeling no more unwell than normal the nurse increased the speed of the pump around the two hour mark but said its still very slow and it’ll look like the bags not really emptying.  Note to anyone going for an infusion- do NOT sit and watch the slow drip of the iron its like watching paint dry!! I attempted to watch a film on my iPad but couldn’t get comfy with my head/neck and constantly needed to pee from all the water to help my POTS, which involved unhooking the whole shebang and dragging the infusion stand which was like a supermarket trolly with a mind of its own across the corridor to another room.

Another hour passed and the iron didn’t even look like it was going down in the bag. After more observations were taken I was good to go full speed ahead on the fastest setting, the nurse said I would now see a big difference in the speed it went in. She was not wrong the bag was going down at a much faster rate and so was I…..  nausea kicked in with a vengeance, I felt cold, headaches and generally wishy washy.  On the plus side I only had a few more hours to go and my medication worked a treat in bringing the nausea down to a more manageable level enabling me most importantly to partake in an afternoon cuppa. I know cuppas are a running theme here, you just can’t beat a good brew, I think its the Irish in me !!

I met a lovely girl who arrived towards the last hour of my infusion who had POTS and was in for saline infusions to help manage her POTS.  It was nice chatting to her and sharing similar experiences of how POTS effects us and hearing how her symptoms have improved with the x2 weekly infusions that sadly she had to fight tooth and nail for.

Once my infusion had finished I had to stay a further hour to be monitored, by this time I was very much ready to go, I was worn out and my head/neck was so painful.  The hour passed with a little help from Spotify, my IV was taken out and I was good to go.  Over the weekend I felt a touch achey, usual stomach pains, nausea and generally under the weather from the infusion but nothing major. I thought I was home and dry, little did I know it was the calm before one almighty storm…….

 

Health Update- Part 3 (The Finale)

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

Gastro-

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

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

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

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

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

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

Iron Deficiencies-

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

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

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ENT-

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

Eyes-

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

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

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

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

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

 

Turners tours…….

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

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

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

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

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

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

Turner’s Tour-

Tour 1

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

Tour 2-

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

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

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

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

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

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

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

 
In other news-

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

UNADJUSTEDNONRAW_thumb_428  IMG_0251

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

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Turbulence

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

The 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.

 

A Gut Feeling

Its 1.30 am, hello ‘painsomnia’. I toy between catching up with Line of Duty or writing my next exert. I have so much I want to write about before my surgery, however monumental Line of duty is it’ll have to wait.

Considering my head is falling off and I’m like a real life rag doll, it may seem a touch unusual my next post being about my gastro intestinal system. It was one of my first symptoms and manifestions of EDS, going right back to age 11 and 21 years later nothing much has changed.

I’ve had a bad relationship with my GI system for over two decades. I have a deep destain for it and it for me. It’s relentlessly let me down. The unremitting nausea, abdominal pain that has seen me for hours on the floor wailing for it to stop, passing out from pain, hospital admissions, A&E trips, heartburn, reflux, uncontrollable bowel to now a none functional one. You name it, its happened and the worst part- it NEVER leaves. The only difference is some days/hours it’s more bearable than others. Do I know when those more bearable days/hours will be?  If only … it can strike at any given time.

Living with chronic GI issues has most definitely changed me as a person. It dominates all aspects of living almost like it’s screaming and vying for my attention all day, everyday. Some moments I can mentally switch off from those screams which I’ve learnt through years of practice and other times the screams are all consuming and there’s no getting away from them.

In the early days I tried to hide my symptoms as best I could. There were times I’d physically gag on food and struggled with swallowing. I’d roll my food around my plate mushing it up to try and make it a consistency easier to take. What I now know to be oesophageal spasms. Often putting my hand over my mouth so people couldn’t see me trying ever so hard to actually swallow.  The pains were far too often unbearable and I’d take myself off to lay down or have to avoid eating at certain times if I knew I had a work commitment or such like. Over the years I had unknowingly created my own management plan. If I knew I had a meeting at 2 pm there was no way I could have lunch before then or I would never make the meeting no matter how wobbly or hungry I felt. I was always the girl with the rumbly tummy in those meetings. Don’t you just hate when that happens?

This rigorous planning also rolled out in my personal life, if I knew I was going out I couldn’t eat for at least 4 hours before it or I definitely wouldn’t make it. Going out for meals was a thing of the past unless I wanted to roll around a restaurant floor or toilet (which happened) for all to see. The pains could last anything from a few agonising minutes into hours upon hours feeling like my bowel was being stabbed, ripped out, burnt out, often the pain that severe it would actually silence me. The worst part is not knowing again, when this will strike?, where you will be? and who will see? There’s no pattern, no trigger and often no warning.

Every aspect of living became a problem, hardwork, had to be planned and thought out. Even with the most stringent of planning there was no guarantee I’d make work, an event etc.  It was always the same flakey answer “Ill have to see how I am”. I always felt quite paranoid of what people thought, at the end of the day I had a set of symptoms but no name. Going to gigs was one of my biggest enjoyments, over time I found myself watching the person/s I went with and seeing how much they could enjoy themselves often with a tinge of sadness of why I couldn’t be like that.

Add to the unbearable pain unremitting nausea, a massively underestimated symptom. We’ve all had it either the precursor to a stomach bug, eaten a dodgy takeaway, drank a little too much its a nasty symptom with many cross over causes for me now the one common denominator being it’s always there, to differing degrees. Then we add in the generics- heartburn, reflux, diarrhoea, constipation, oesophageal spasms, and what a cocktail of treats that is.  My very own  gastric-mojito!!

I have had to change my diet dramatically over the years due to increasing difficulty with breaking down and digesting foods. My diet now consists of around ten foods on repeat, all gluten and soya free. My mouth waters at the prospect of steak, chunky chips and peppercorn sauce 😉 and there again comes the tug of war between mind and body.  The thing is I LOVE food, always have, sadly that feeling isn’t reciprocated.  One of the hardest aspects to begin with was how alone and different I felt. I couldn’t take part in what are probably the two most popular and social aspects of living food and drink. As hard as it is not being able to eat the foods I love and more annoyingly so that my limited diet still doesn’t take my symptoms away I do still hold onto the fact at present I can eat. Many EDSers have sadly gone into intestinal failure and are unable to manage solid food what so ever.  I know this is a possibility for my future given recent events but I don’t dwell, why worry about something that hasn’t happened yet?

Aged 23 me and my GI system had fallen out on a whole other level. I unknowingly jumped aboard what would be the bumpiest, unsettling and frustrating of journeys.

A seven year uphill hike trying to navigate the health care system began. It opened my eyes……

Like anything in life there is good, bad and indifferent and the same goes for healthcare professionals. Before I go on I would like to point out that I am a huge advocate of our National Health Service which is clearly being run into the ground (I’ll save the politics for another day). Being so heavily entrenched in it for so many years it was impossible not to see the chaos and dysfunction deeply embedded within it.

I stopped counting at 60 the amount of GI investigations I’ve had. Multiple cameras, catheters and balloons (that was an interesting day) in every orifice, MRI’s, CT scans, drank litres upon litres of barium, bowel prep, pill cameras, hydrogen breath tests, tracer studies, infusions, motility tests and so on.  For those reading that have gastro issues of any nature and have undergone investigations you will know how unpleasant gastro testing is in particular.  Your dignity is well and truly left at the door as you pop that hospital gown on and head into yet another invasive procedure/investigation.

Ironically the aforementioned tests were never the most painful aspect, it was the attitudes of GI consultants in the early days before I had a solid diagnosis that was the most damaging of all.

I had mentioned that I was experiencing pins and needles in my extremities,  feeling breathless and unwell . He looked at myself and my Mum with a puzzled expression and said my blood results were clear, absolutely nothing wrong with me. I left the appointment in floods of tears, this wasn’t the first time I hadn’t felt believed and I knew it wouldn’t be the last.  My legs were becoming increasingly numb and weak yet I walked back to my dads car hanging my head with distain, all these symptoms very real and happening and yet again not being believed. That weekend I collapsed at home, my legs were numb, lips grey and I felt extremely confused. I was rushed to hospital to find out the bloods I had were NOT normal and that I had dangerously low B12 on which treatment commenced.  At this stage we made a complaint which we had never done before only to receive a back dated letter from the consultant reporting that my bloods were not normal and I need to commence on intramuscular injections of B12 with immediate urgency.  The sheer cheek, back dating a letter to cover ones back.  Id of had much more respect with an admission of a mistake and a quick apology. Done. Did that ever happen …..nooooo the complaint seemed to disappear into the ether.

I can fully accept mistakes, we all make them.  Crikey Ive made a lot in my time.  Often I received clinic letters addressed to a different name, clinic letters that bared no resemblance to what the conversation entailed, inaccurate information which I can accept, to a certain extent. An overflowing NHS system mistakes will happen but when its your life and wellbeing at play its very worrying.

A new pair of eyes, it gave me hope. This time after years they would get to the bottom (no pun intended) of what was happening. When I was first investigated after explaining my symptoms to gastro No 2 his view was set well and truly on Crohn’s disease, everything fitted in he said. Chronic diarrhea, weight loss, joint pains, mouth ulcers, B12 deficiency “TEXTBOOK CASE” , he said. After many investigations no inflamation was found and the tide rapidly turned.  I couldn’t believe the shift.  Suddenly it became about my mental health, am I stressed? depressed about anything?…..”only my stomach ” was my answer to that.  Here we go again. I was then inadvertently accused of being anorexic, my eating habits questioned and weight loss. I could not believe this switch it was like talking to a different person.  Granted Crohn’s disease may not be the cause but something had to be and to jump to psychosomatic causes was rather damaging.  Around this time I had infusions as my iron was dropping and I was not absorbing the tablets, id even had surgery and adhesions were found wrapped around my bowel, gastritis had been found x3 times and various bacterias yet no pieces were ever put together I was only told “If we keep testing we are inevitably going to come across things”.  Id absolutely had enough, the appointments ended up causing anxiety in the lead up.  Knowing you were about to enter the lions judgement den, made to feel like the symptoms were in your head over and over yet I couldn’t give up, I knew how I felt, I knew this wasn’t right, I would NOT give up.  

Sadly I know of many more stories like my own and not just exclusive to EDS.  I am sure some people reading this post can relate. It seems over the course of time we’re going to healthcare professionals for help and being greeted with such questions as “What do you think it is?’, “Are you depressed or stressed?” it seems to be the first port of call which is costing lives.

Not feeling believed by the very people I was turning too for help put a question in my mind as to whether other people believed me.  Do people think this is in my head?  Over the course of years it was genuinely very hard to keep plugging on often hearing comments “Have they not found out what it is yet?”. “They need to sort you out”,” They need to move quicker”. Sadly the health service doesn’t have the fluidity it once did.  Things are missed, not joined up, mistakes happen, books need to be cleared ready for the next cohort of patients who have been waiting over 6 months for an appointment. Almost like the conveyor belt on The generation game.

Fast forward to gastro number 3- The Messiah. I am extremely fortunate to finally be under an excellent consultant.  He specialises in neuro-gastroenterology and is the most understanding and supportive consultant to date. Over recent years through having the correct tests its been proven I don’t absorb my nutrients adequately so no matter how much of the 10 foods I eat I cant gain weight like other people and struggle maintaining it.  I have been diagnosed with gastric dysmotility which sadly has progressed in recent months resulting in many A&E visits and further tests. Essentially the nerves in my bowel are not firing properly nor are the muscles. What should be a smooth rhythmic action is that of a disorganised, uncoordinated and beyond sluggish.  This explains why loading up on laxatives didn’t even work as its the nerves themselves. This causes a great amount of pain as food can back up and the fear with EDS is that obstruction or perforation of the bowel can occur as our tissue is weak.  I will soon be meeting with my bowel function nurse to discuss a neuromodular medication which they want me to try which acts directly on stimulating the nervous system alongside another medication.  I have this same dysmotility throughout my digestive system causing the above mentioned symptoms but much worse now in the bowel. Its essentially a failing colon.

It transpires that gastro-intestinal manifestations in EDS are rife.  Connective tissue is throughout our digestive system and is essential to the passive mechanical movements needed to complete digestion.  Connective tissue is also present around the nerves of the digestive system.  With our connective tissue being weak, fragile and abnormally produced this can create a variety of problems anywhere in the digestive system.

To read more about the gastrointestinal manifestations in EDS, please click the link below.

Gastrointestinal manifestations of EDS-

Now if you’ve got to the end of this extremely long blog post, THANK YOU and go and have a refreshment 🙂 This was always going to be quite a lengthy post which I found quite hard condensing (believe it or not). All my posts won’t require an interval and loo break, I promise.

Much Love

Ali xxx

Next Stop- EDS Diagnosis Day.