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 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!!

IMG_1506

 

ENT-

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

Eyes-

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

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

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

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

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

 

Health Update- Part 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

 

Turners tours…….

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

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

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

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

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

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

Turner’s Tour-

Tour 1

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

Tour 2-

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

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

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

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

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

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

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

 
In other news-

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

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

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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.