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 1 (Head and Neck)

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

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

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

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

This is where is gets complicated ……

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

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

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

Theory 1-

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

Theory 2-

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

Theory 3-

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

 

Theory 4- 

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

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

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

Neck-

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

 

 

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

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

Much love

Ali xxx

Turners tours…….

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

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

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

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

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

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

Turner’s Tour-

Tour 1

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

Tour 2-

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

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

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

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

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

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

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

 
In other news-

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

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

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A Racing Heart

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

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

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

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

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Reading taken a matter of seconds after standing up.

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

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

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

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

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

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

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

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