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*