Tag: CEDS

Posted in Archive, November 2022

Fridays Ambulance Call Out

Posted on by dystoniaandme

As the early light of Friday morning broke through the damp cover of night I lay in bed counting the minutes until my Drs practice opened. The pain in my lower back had been agony all night and I suspected my ongoing UTI had progressed. After a night of no sleep 8am couldn’t arrive quick enough.

By the time I arrived at my doctor’s for my 11am appointment my temp had hit 40.3c and I was in urinary retention, my heart rate and blood pressure were through the roof. I can vaguely remember being encouraged out of my wheelchair chair and onto the bed whilst they phoned an ambulance. Shivering with fever chills, and desperately tired. Three hours later I was in the back of an ambulance on my way to the hospital.

I don’t think I’ve ever seen A&E so busy. The staff were amazing and after quickly establishing my veins were playing hide and collapse they had an ultrasound out to guide them. They hooked me straight up to antibiotics for an upper kidney infection and told me urology would make me a plan.

Waiting for ward rounds

Saturday morning three burly Urology consultants appeared at the end of my bed. “What would you like us to do?” The question caught me offguard. I’m rather used to staying quiet and listening so as not insult god complexes, or putting my thoughts across in a tonned down manner. When I explained I’d be happy to be guided by them, it was really refreshing as they took the moment to explain my options. I could that day under local anesthesia have a suprapubic catheter put in: this would involve a small incision in the tummy allowing them to put the catheter through and to the bladder. Alternatively I could have an indwelling urethera catheter and go home, to come back at a later to have the suprapubic catheter put in under a general. I chose the latter option giving them a brief explanation about my Ehlers-Danlos and how due to EDS you can pump me full of local but I’ll still feel everything. They happily accepted my decision .

Back in 2019 whilst pregnant with my daughter I’d agreed to surgery where they attempted to remove a large suspicious mole from my breast without pain relief or sedation due to the pregnancy. The local failed. I felt every cut. The team did their best to distract me but the whole thing was traumatic. I made the decision at the time to not put myself back in that situation ever again. So even though the suprapubic catheter is meant to be better long term I’m happy to wait a few extra days/weeks for it if it means they can put me under.

So why has this all happened? My guess is that it’s down to the Ehlers-Danlos. My bladder has been dodgy for well over a decade, it’s just recently sped up in its decline. I’m feeling a little emotionally up and down, frustrated mainly but I think it’s healthy to acknowledge that. I know this will all feel normal soon.

*On a side note if you use catheters and have any tips on making it more comfortable please get in touch!

Posted in Archive, November 2022

First Neurology Appointment

Posted on by dystoniaandme

The nerves leading up to today’s appointment had been doubling with each sunrise. I was dreading meeting my new neurologist. After a decade under the care of Prof Wonderful, and have met more than a handful of neurologists with severe god complexes before I wasn’t feeling very optimistic. Whilst I knew that it was ridiculous to tarnish all neurologists with the same brush, I had met multiple neuro’s from this particular hospital before who had awful bedside manners. Previously they had refused to take me on as patient as they had deemed me too complicated at my first appointment. The second time they met me my symptoms were brushed under the rug with a quick “ah it’s most likely a side effect of your Ehlers Danlos” but didn’t run any tests to back this up. So I was hesitant to say the least, and that I would once again be swept under the rug.

The appointment had come rather suddenly as an opening slot had become available via their virtual neurology clinic. Whilst it meant that I still wouldn’t get my botox, it would at least mean that my foot would be in the door, my name on their patient list, which feels like a positive step forwards after several months of floating around in the neurology space waiting to be allocated to a hospital.

I was pleasantly surprised by the neurologist who met with me. He was open about his retirement plans and understood my wishes for continuity of care due to the complex nature of my health. It was agreed that I would be assigned to a different neurologist, whose specialism was Dystonia, he reassured me he would book me into the botox clinic as soon a space became available. I had expected there to be an issue when I brought up the frequency of my injections (6 weekly) as this is quite a small gap between treatments than what most prescribe. His understanding of the matter was not one I’d been met with before. Normally I have to really battle to get my Drs to understand that this treatment regime was one created out of necessity to keep me out of the hospital due to the severity of my Oromandibular Dystonia.

We had had a brief chat over where we felt my treatment needed to be going moving forwards as whilst the majority of my body is fairly well controlled my jaw is feeling much like a losing battle. I still have a lot of baby teeth as I don’t have the adult teeth for them in my mouth, which at almost 30 I’m pretty happy their still in place! However several are now painful and wobbly due to the battering they have received from my tremors, spasms, and dislocations. We know from previous experiences there is no point in wiring the jaw shut as the spasms are strong enough to break the wires, but further intervention in some form is needed. We briefly discussed today playing around further with the EMG machine to see if that can pinpoint better the areas needed to be injected and some surgeries we can try. These though will obviously be needed to be discussed again in greater detail face to-face.

Overall it was a very positive appointment. I’m still devastated not to be under my old neurologist, he really helped me not only accept my conditions but also reign myself back in when I needed it. He helped me to adapt to my new life and for that, I will be forever grateful.

Posted in Archive, July, July 2022

Urology Pick Me Up

Posted on by dystoniaandme

Yesterday I attended my first appointment with my urology consultant. I had no idea what to expect. Urology issues are common in my family but I’ve never pushed to have my issues investigated. I had a scan once in 2012 and was told oh it’s just an overactive bladder just try to go to the loo less and you’ll be fine. I was training to be a midwife, I was queen of going to the loo less.

I hadn’t realised just how nervous I was. I’ve been coping with on/off incontinence issues for a longtime now, it can take me twenty minutes to void my bladder and it’s never fully empty. Throw in my prolapse on top and things are just not great in the pelvic region. When he asked me to explain what was wrong the words just tumbled out at top speed, I was vaguely aware of my hands nervously shaking. He stopped me a few times, got me to take a breath, reassured me there was no rush he had time to listen to me and that I could explain in as much detail as I could.

He was eccentric in his mannerisms, but put me completely at ease. After my neuro left this was the pick me up I needed. Before I left his clinic I had appointments in my hand to come back for further testing, instructions for at home testing and a date to review the results. The NHS at its finest.

Posted in Archive, June 2022

Neurological Comfort Blanket

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*Professor Wonderful has been my neurologist for a little over a decade. When I first became ill at 19 my symptoms broke me. I went from being a confident but accident prone 19 year old, who loved every second of her degree to a spasming, wheelchair bound young adult whose carefully planned out career was slipping through her misshapen fingers. The day my mum collected me from uni I cried the whole way home. I was on sick leave but I knew I wasn’t coming back. There was a twisting pit in my stomach that knew it.

Over the next few months we would clasp at every hope offered that I would get better. Meanwhile I continued to deteriorate. Every road we took was slightly different but each one mentioned Prof Wonderful name as the expert in my symptoms. My mum being the force of love that she is found his contact details and emailed. A few weeks later we sat in his office.

After months of seeing consultant after consultant, each previous appointment more crushing than the last I didn’t want to get my hopes up. I was used to being told I could stop my eyes from spasming if I wanted to, being told by multiple consultants that my symptoms were the result of the abuse I’d gone through in my teens, or being left with the results failed operations (a mouthful of broken wires ) with the spasms broke the wires being used to try to control and he ghosted me rather than fix the mess. My expectations were low. Yet my preconceived judgement vanished when he shook my hand and immediately noticed my hypermobility, he referred me to a specialist that day which resulted in my EDS diagnosis. I can remember crying in the lift after the appointment. They were tears of hope.

He did so much more than provide injections. He listened. When I got ahead of myself when my symptoms improved and thought I could go back to midwifery he gently disagreed. He was right. When I’ve needed my team’s to communicate he has fought to ensure they all do to help ensure I’m getting the right care. When he left his post at the hospital I was first under him at I was reassigned to a different consultant. He refused to give me injections at my usual frequency or dose. I went downhill fast, so contacted my original neuro who immediately had me transferred back to his care.

When he informed me on Tuesday he was leaving and no longer would have a patient facing role it was all I could do not to be cry like the last time. I knew it would happen one day. I’d just hoped it would be a long way off. After all these years he is essentially a comfort blanket of sorts, a safe place amongst the god complexes and arrogance that I’ve come against time and time again. I’m nervous about this new chapter. Here we go.

Posted in April 2022, Archive, Wedding

Disability & Wedding Planning

Posted on by dystoniaandme

Our wedding date is set for the end of next year and we couldn’t be more excited. The kids ask ‘how much longer” almost every day, and are very much looking forward to playing their parts on our special day. We have got well and truly stuck in to the planning and booking of our venues.

Playing together at Walton Gardens

This is where my arsenal of equipment that holds my body together will come in handy. We will be splinting up my knees and ankles in the hope that I can hobble down the aisle on my walking sticks. I’ve currently got a Pinterest board dedicated to walking sticks in different shades of white/ivory/champagne so that once I’ve brought my dress I can match them. However if I have to roll down the aisle that’s fine too (it’s what I do every Sunday anyway 🤣 during mass), so I can always spruce my chair up with flowers.

Example of different wedding canes

One of the big factors for us is getting me through the day without a trip to A&E or an ambulance having to be called. Sounds simple really, doesn’t it? Yet it’s a very real possibility. I tire very quickly these days and my body goes downhill when that happens, so utilising my aids and working breaks into the day/sitting down frequently will be important. Having these planned in advance seems best for not running out of spoons* too quickly.

Spoon theory explantation

I’ve spoken to our photographer about my disabilities and he’s had experience with people with similar issues. Hes happy to listen and go off what I’m saying. If I’m doing well then fab, not feeling so hot then that’s ok too we can rejig positions. I was quite nervous about this conversation so this was a big relief that he was cool with it.

I’m ever so slightly taller than my partner which I am over the moon about as it removes all temptation to break out the heels I hung up years ago. I used to love love love a chunky wedge heel. But it’s just asking for trouble. So sensible flat shoes it is with good ankle support it is – to be honest I’d be quite happy barefoot but I think the church and the hotel wouldn’t be as thrilled. I’ll be gradually breaking these in as part of my EDS means my skin breaks super easily. It takes me months to adjust to shoes even when they’re a perfect fit without my feet bleeding.

An old photo of us because im always just taking photos of the kids and the garden 🤣

Posted in Archive, march 2022

Reflecting on Side Effects

Posted on by dystoniaandme

I’ve not had to resort to Lorazapam this week for managing my jaw spasms/dislocations (yet). So far my usual meds and my Dr ordered bandage support, are doing the job along with Damon relocating it when needed. I hadn’t realised just how much the Lorazapam had been affecting me until it started to work its way out my system.

Now this isn’t a surprise. Clonzepam and Diazepam are listed as allergies for me as they cause psychotic reactions when I take them. It seem to be a family of meds I don’t get along with but unfortunately need at some point now and then unless we find a better alternative to turn to. This time it was like someone had extinguished all hope. Even though my Dystonia is well controlled these days, the fact that my Ehlers-Danlos is getting worse seemed unmanageable. Crushing. Uncontrollable.

Now that it’s out of my system I can see how much of an affect it was having. Yes my EDS is on a downwards spiral at the moment, but we’re adapting and I am blessed with a supportive Fiancé and family who are helping me. My life is very much one full of hope and love. It’s helpful that I have my blog to turn to read to myself on bad days.

Botox is on Friday which I’m much looking forward to. I cannot wait to take these bandages off.

Posted in Archive, February 2022

One day, one minute, one breath.

Posted on by dystoniaandme

Each passing minute, moment, breath, feels like a drawn out hour at this time of writing. By Botox was due at the end of January. It was scheduled for April. Thank Lord it was moved to mid March. My medication routine had kept me healthier for longer than I had dared hoped for, but now my little Dystonia alien is kicking my ass with vengeance.

My jaw has been dislocated for a full 24 hours straight now. It has been coming previously on and off before that. The aches in the joint and the building spasms told me all I needed to know. I’d adjusted my food intake to make sure I wasn’t aggreviating it, I was regularly applying heat packs, and doing basic physio stretches. On Tuesdays I sneezed and dislocated the jaw, and it came out again hours later while I was enjoy a nice brew.

I’m extremely lucky that while I was pregnant with Evie at around 9 weeks I got admitted to the gyny ward due to suspect Hyperemesis Gravidarum and kidney infection. The maxfax team came and taught my fiancé how to relocate my jaw to save me living in the A&E department trying to educate the staff. Them taking the time to teach Damon had a life changing impact as it’s dramatically cut down the amount of time I spend in A&E have it manipulated back in place.

The shear strenth of the spasms shock and terrify me. Despite muscle relaxants Dame has struggled to relocate my jaw today. Normally this would mean I need to take a trip up the hospital before it becomes worse. But honestly I don’t want to go. I know the drill, they’try twice, when it fails they send me by ambulance to Aintrree, the med students try and fail, I get scheduled for surgery, and then Dr. Godcomplex cancel the surgery because he doesn’t believe in Dystonia or EDS.

If you’ve made it this far through my foggy ramblings! Congrats 👏

Left on Sunday where spasms were ok. Right is today

Posted in Archive, COVID-19, January 2022

Covid Round Two

Posted on by dystoniaandme

Whenever I get ill with something like a cold/your more normal bugs, or as I prefer to call it muggle sick, my body always protests. Sunday I was feeling off and presumed I was having an ocular migrane, I get them often so didn’t really dwell on it. Alarm bells started going yesterday when I woke up with a temperature, sore throat, ringing ears and eye pain still. The lateral flow was negative so chalked it all up to an awful cold. After all I’d had covid a few months back and I am fully vaccinated.

It was a bad night of neck, jaw and back spasms. Upon opening my eyes this morning all I wanted to do was put pressure on them both. I recognized the pain. I had the same issue for almost six weeks following contracting covid last autumn. Sure enough this mornings lateral flows came back positive straight away. Which explains why my spasms have been playing up. My body is doing its usual protest, a way of saying it’s overwhelmed.

I’m feeling pretty run down but greatful to be vaccinatinated.

Meme depicting the a squishy monster covid monster clinging to a cartoon person whose trying to escape
Posted in Archive, January 2022, September

Day to Day Management

Posted on by dystoniaandme

No day is ever the same when living with chronic illness. The routine may be vaguely the same but each day revolves around adapting to what symptoms are presenting that day and the severity of them in the moment. What may be rather bad in the morning may be insignificant in comparison to another symptom by midday

A good example of this is today. Sundays are always our family rest days. We go to church, sometimes have family to us but generally speaking we are at home together. Now I was already shattered after a bad night sleep with nerve pain in lower right leg and lower back pain. However upon getting up my neck spasm started pulling my head down towards my shoulder. It’s a particularly nasty spasm that’s hard to break. I have a percriped Aspen collar for when my neck does which I alternate with wearing a TENS unit and a heat pack.

Picture of me ready for church wearing my Aspen collar to been help support and straighten my neck

I’ve not had to wear this collar in a while. The overly nervous me did my best to disguise it with a scarf as we sent out for church. It deffinently took some getting used to wearing it out and about and learning to ignore the second glances once again. But it’s worth to help ease off the painful spasms somewhat.

Off to church. Scarf ‘hiding’ my neck support

My Botox appointment is extremely late this time round having being schedule for almost six months instead of three. Whilst I’m hoping for a cancilation to come up, I am in the mean time going to ask my general practitioner to allow me to my Trihexyphenidyl untill I’ve had my injections

Posted in Archive, November 2021

Learning To Cope With A Prolapse

Posted on by dystoniaandme

I woke up the other week with a pain that felt slightly familiar but I couldn’t quite put my finger on it. I lay in bed gently testing my muscles and noting what was spasming or dislocated. After all it’s not unusual for me to wake up with a dislocation here and a spasm there. Satisfied that no major joints were out of place I got up. Then it hit me.

The pain in my vaginal area brought tears to my eyes. I immediately clamped my thighs together. I can only describe it as feeling like my uterus was plummeting out of me. A quick check with a mirror confirmed what my nerves were telling me. Prolapse. Again. This time far worse.

Ive been back and forth to the drs multiple times since that morning. Honestly the difference between seeing a male Dr and female Dr is gobsmacking. When I saw the female practioner I got asked how I’m managing, offered pain relief, and my concerns listened to. When I spoke to the male Dr he dismissed my pain levels telling me that he couldn’t understand it as a prolapse shouldn’t hurt – never mind the fact I also had an Infection in my uterus at the same time. He gaslighted me completely.

I’ve been researching like a madwoman since I started this journey in desperation to make sure I am referred to the right department as quickly as possible. 38 weeks is the current wait time to our hospitals urogynyolgical clinic. 38 weeks. I can’t wrap my head around that figure. Meanwhile I’m sitting here with my prolapse literally outside of my body causing a significant amount of pain.

Ive been pretty upset, a lot of tears have been shed. My mental health has not been great and I’ve not really got my head round this yet. I know that the prolapse will be due to my EDS. I can accept that. What I’m finding hard is the fact I can barely do more than a few minutes movement before needing a long rest due to pain.

Im not sure how long this journey will be but I’ll keep it documented. I’m sure this reads as a ramble but it helps me to get it all written down.