Tag: classical ehlers danlos syndrome

Posted in Archive, March 2023

Check Engine Light

Posted on by dystoniaandme

It’s been a bit of a rollercoaster of emotions these last few weeks. That’s to be expected I suppose. Gastroparesis and its impact on my Dystonia and other conditions has been yet another life pausing event. Another painful event. Another force outside my control. There’s been a lot of tears in frustration, upset, anger and confusion at just how to manage this new hurdle. I don’t doubt that I can and will adapt. It’s the grief cycle again.

It’s got to the point where the doctors have finally agreed to have the district nurses come to my home three times a day to administer antisickness injections. These are the only antiemetics that I have found of any help. But in typical misbehaving form that my body truly excels in, this has not gone smoothly. I expected some bruising, but I’m now looking a tad black and blue, and bleeding a silly amount each time. It’s worth it to be able to keep my Dystonia and heart medication down though.

I’ve found the pain from the Gastroparesis, on top of my Dystonia has left me exhausted physically and emotionally.I do my best every day. Some days my best may look like nothing. I like to call that energy saving mode. I’m in that mode a lot lately as I’m acknowledging what my body needs. This is hard. Sounds silly I know. But life doesn’t exist for us to function this way. It’s geared up so that the expectation is you fall down, you brush yourself off and carry on. I need to stay down a bit longer.

The best way to describe what I’m trying to say is this. You wouldn’t set out on a long trip with a car that had a check engine light flashing, four flats, a drop of petrol and no oil. You wouldn’t get anywhere, you’d destroy your car. My body is the same thing, it’s out of fuel, every light is flashing and tires are shredded. I need to spend some time working out how to patch me up and that’s ok. It’s allowed. It’s important to remind ourselves of that in a world that is fast paced.

Posted in Archive, February 2023

Hide & Seek…

Posted on by dystoniaandme

What? You found me already?! Dang you’re good. Though I suppose being back here in my second home isn’t really the best hiding place. But hey I had you for a minute there 😉

All jokes aside, I was admitted last Friday back to my local hospital. It wasn’t a surprise. I’d been going down hill quickly since my last discharge; unable to keep down food, meds or fluids for more than a few minutes at a time. I was feeling incredibly unwell.

My local practice has an amazing Advanced Nurse Practitioner (ANP) who takes fantastic care of me. She started doing regular tests to monitor me. My renal function was dropping fairly dramatically despite valiant attempts to sort the issue nothing helped so off to the hospital I was sent and have remained.

So far I have remained my usual medical mystery self. A Zebra to the core. Tolerating anything orally for generally mere minutes at best. Whilst urology have ruled things out on there side, my symptoms remain just as severe. Referrals have been made to the inpatient dietitian and gastro team so here’s hoping they’ll turn up at some point and they might have some fresh ideas..

Posted in Archive, January 2023

Dysfunctionally Me – A Reintroduction

Posted on by dystoniaandme

I’ve had some new followers to this blog and it’s accompanying Facebook page recently, some of whom have been curious as to what on earth is wrong with me; after all the name implies Dystonia but a multitude of conditions get discussed. It’s a good question. So I thought I’d take a moment to write a short post reintroducing myself to you all.

I started this blog back in September 2012 after my life felt like it had been turned upside down. I’d been training as a midwife at Anglia Ruskin University Chelmsford and felt as if I was living my calling. However I was plagued by ailing health that got more and more severe.

I had to go on medical leave at the end of my first year. Despite my determination I would never return to midwifery, which to this day still breaks my heart. My body was no longer my own. I was wracked with spasms, needing a wheelchair to get about, I couldn’t even feed myself. It took months going from doctor to doctor, hospital to hospital, ambulance after ambulance until we finally met my old neurologist who finally shed some light on what was happening to me.

Initially I was diagnosed with functional Dystonia. A diagnosis that I fought against. I’d been physically abused as a teen by my father and this trauma led doctors down the functional route. Five years later my diagnosis would be changed to Generalised Dystonia. Upon my diagnosis I felt so lost. I’d moved back home, had no independence, lost my career and no one really seemed to know what Dystonia was. I made it my goal to educate myself as much as possible. So I set up this blog and it’s Facebook page as a way of connecting with others who were going through similar experiences, and as a way of raising awareness of the condition. Advocacy has become a great passion of mine.

Over the years more conditions have been diagnosed, such as Classical Ehlers-Danlos Syndrome, Postural Orthostatic Tachycardias Syndrome, Lyme Disease Osteoarthritis and more. I’m surprised I don’t shake with the amount of medication I take. Learning how to manage all these conditions and live a full life has been hard and is still something I am trying to learn to balance. The symptoms and pain I experience in a day change hour to hour, I dislocate multiple times a day, have vocal tics, spasm, etc. It can be a minefield to manage. I’m very lucky that I now have a career as an author with books 7,8 & 9 being published this year, which gives me the freedom to write around my health and my family.

This site has become a diary of sorts. Where I can let out the awful days, whether it’s a physically bad day or an emotional bad one. Or celebrate the triumph’s with you all, which is something I try to do often. It’s a way of processing my conditions in a healthy manner while also raising awareness and supporting others. I find when I need to reflect on how far I’ve come in learning to manage my conditions through medication/physio/wheelchair/splints etc reading back through my past posts is a great aid. I can easily reflect on my progress, and see the milestones that I thought I’d never meet; such as graduation (I studied publishing instead) having children, getting my independence back through my electric wheelchair.

I’ve had this blog for coming up to eleven years now. While I hope I don’t need to add anymore conditions to it, I don’t plan on stopping posting anytime soon. I’m so grateful for the readers and friends I have made through here and for their support. Thank you.

Posted in Archive, January 2018

You’ll learn to deal with the pain

Posted on by dystoniaandme

It’s been a long day. I arrived at the walk-in unit before 8 this morning in agony and with an obvious infection in my incision site.A half hour later I was on my way to hospital being sent straight back to the surgical assessment unit, ive since bounced around the wards for a few hours as they are full to the brim.

My incision site has been leaking pus and the pain I have internally can only be described as a scaping and burning sensation. It’s been leaving me in tears unable to move. The member of staff I saw was lovely. Quickly established that I needed my suprapubic catheter changed to a new one due to the infection and did so. Despite the morphine I struggled with the pain.
The reg came to check and make a plan “once the infections gone you’ll learn to adjust to the pain”. A sentence that seems utterly barbaric to utter to a woman shaking in pain, clearly unwell, and whose just expressed the severity of the pain she’s in.

I count my lucky stars for the team member who was in charge of looking after me today. Who could see how much I was struggling and did everything she could for me.

I’m now waiting for a bed on the ward where we can get antibiotics started and try to get my pain under control.

Posted in Archive, January 2023

Surgery 1 Done

Posted on by dystoniaandme

Well that’s the first surgery of the year done and dusted. After much anxiety over preop appointments falling through, fear of it being cancelled due to arriving on the ward and finding out that surprise surprise I had a temperature, and the team not being overly sure over the best way to handle my misbehaving jaw – to now be recovering on the ward is such a relief.

There had been some back and forth over how they were going to carry out the surgery due to my jaw having the potential to spasm and dislocate which could impact their management. My last surgery went fine until waking up when it dislocated and I needed to be sedated to have it relocated. A situation we all wanted to avoid repeating. One option presented was to have a spinal anaesthesia and be awake. I declined this as my neurology team have always stressed that they don’t want anything invasive being done to my spine due to all my health complications. This disappointed the team here and we chatted about this a few times, as understandably to them this was the safest way to proceed, however I chose to go ahead with the general as my neurologist was always very clear on the matter.

It went really well which is fantastic. They’ve inserted a suprapubic catheter; which is a catheter that is inserted through an incision in my lower abdomen and into a small incision in the bladder. I’ve had bladder issues for about eleven years now but over the last year these got severe. I reached the point in the summer where my bladder just stopped working and I was unable to void anymore. We tried intermittent catheterising but due to my tremors and spasms in my hands along with frequent dislocations in my fingers this was impossible on some days. Over the last few months I’ve ended up in hospital on multiple occasions with UTIs, kidney infections and retention.

When my consultant originally announced this next step I felt quite down. It seemed like such a spiral in health and I was overwhelmed. However I’ve had time to come around to it all and the relief I now feel having had the operation is significant. This should hopefully mean I start feeling much better, infections should stop being a regular occurrence and life should return to its usual level of uniqueness. So I’m embracing my new addition with a happy heart. Now to recover and get well enough for surgery no2 on my jaw.

Posted in Archive, December 2022

Snowballs and Spasms

Posted on by dystoniaandme

I have loved snow for as long as I can remember. I’m a winter girl. It lights a spark in me like nothing else. My eyes are forever scanning the grey winter skyline in search of signs of an incoming flurry.

It doesn’t matter that the freezing bite in the air adds another layer of pain to my joints, or the kiss of the snow brings on more spasms. All of that is worth the delight in hearing the crunch of the snow, the fits of laughter of my children as they attempt to stay upright (whilst their dad dramatically falls in the snow), watching as they all run ahead in a cascade of ice, snowballs , giggles and shivers. It brings me such happiness.

Our little exploration may have exhausted me, but it was worth every single moment.

Posted in Archive, November 2022

Return to the Hospital

Posted on by dystoniaandme

Well I’m back. In the hospital that is. After a short escape and my catheter spectacularly failing I was instructed back to return to the hospital. I was ushered back to the ward and greeted by baymates from the previous day. The nursing staff quickly whipped out the original catheter and placed a larger one in.

Whilst the original one was by no means pleasant, I’d got through the insertion with barely a grimace having been used to catheterising myself daily anyway. This second one was something else. It took several painful attempts to insert, immediately I found myself experiencing a burning sensation that left me in tears and trying not to move so as not to aggravate the sensation. The staff reassured me it should pass.

This morning, after no sleep thanks to pain, the consultant ordered medicine to ease the bladder spasms, assured me it would help and then I’d be discharged. It took 12 hours for the medicine to come to the ward, I’ve only just been given it. I have spent the day begging for relief. The bladder spasms I could deal with, the bypass round the catheter I could deal with, the burning sensation in the urethera? That I couldn’t cope with. The searing relentless sensation frankly overwhelming. After a day being inconsolable the nurses removed it. The relief was immediate. I’ve no idea why it happened when the first one was ok. They are now monitoring to make sure I don’t go back into retention and can cope with intermittent catheterising in the meantime.

The indwelling catheter was meant to be a solution until they could do the op to fit the suprapubic catheter. Hopefully I’ll be able to manage without it until then.

Diagram of Suprapubic catheter
Posted in Archive, November 2022

Motherhood and Pacing

Posted on by dystoniaandme

Being a mum is without a doubt one of the best, and most challenging, joys in my life. Whilst there is an element of predictability, there is also the beauty of their ever growing imagination. We never quite know what they will ask next or what game they’ll invent. It really is a blessing. Hearing my youngest call out in her sleep the other night “Sing for your treasure pirates!” is a memory I will treasure with a chuckle.

Last week my son and I found ourselves in the local children’s ward again. Luckily this time our stay was short. He had clung to me like a young monkey. The way children do when they’re feeling awful. He’s five now, and as much as I love holding him, especially as he’s usually so full of beans and always on the go, 13 hours like that took a physical toll. I knew better, but when our children are ill pacing goes out the window. It’s a week on and I’m only just starting to feel myself again.

We had found ourselves in hospital without much warning having been sent straight there by the GP. Normally I go armed with extra clothes, extra meds, books, etc. This time I just had my handbag. I will now always carry an extra day’s worth of meds with me.In the days after his discharge the fatigue was at levels it hasn’t been for awhile. My partner found me taking a break whilst getting ready for church on Sunday morning. I felt ridiculous but at the same time knew I needed to listen to my body

It’s a new balance I’m needing to learn to walk. In reflection I can see ways I could have helped limit the physical impact. For example I could have explained my conditions and asked if I could have had a small blanket to fold up to cushion behind my back where it was spasming etc. Hopefully this will help a tad next time.

You’ll be glad to know that although still on antibiotics my eldest is back to his bouncing about cheeky self.

Posted in Archive, November 2022

Declining Health

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“It’s your choice. Do you want to keep trying to cope with the pain at home, or I can send you up to the hospital?” I sat opposite my doctor, wanting to explode. Not at them. My doctor has gone all out over the last month trying to help me. I’m on week four/five of an infection which has in turn irritated my usual spasms, my pelvic prolapse which has been a problem for 3 years (and I’m still waiting to see the specialist) is causing its own issues, and I now have to self-catheterize daily. Hospital admission is tempting, but the local hospital isn’t that great with rare conditions, and someone needs to be here for the kids. I decided to remain home, where I know my meds wouldn’t be messed with and I could use heat therapy and a TENS machine too.

Week four going into week five of antibiotics

It’s something that I’m struggling with quite a lot at the moment, the sensation that my body is deteriorating at a rapid rate again. It goes through phases of this, its part and parcel of having multiple chronic conditions. Knowing that doesn’t make it any easier though. I recently made the decision to start having therapy again; struggling to accept new declines in my health is something I’m making sure to discuss.

My decline in health at the moment is mainly due to my Ehlers-Danlos, however my Dystonia is impacting this too. Ever tried using a catheter with hands that tremor? It’s by no means easy! I’ve not got a Botox date yet with the new hospital but I’m hoping they maybe willing to try injecting my hands so as to make this whole process a bit easier.

Posted in Archive, November 2022

First Neurology Appointment

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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.