Posted in April 2022, Archive, Wedding

Disability & Wedding Planning

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.

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, February 2022

One day, one minute, one breath.

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 March 2021

Medication Success

As I have mentioned previously following a discussion with my neurologist I tried a couple of different medications to see if we could get a better handle on my spasms. In particular the spasms around my jaw as these cause me significant issues with pain, dislocations, and make it next to impossible some days to relocate my jaw; I’ve had the joy previously of coming round from an operation to have my jaw relocated under GA, only to dislocate when I wake up due to spasms, and have my surgeon coming running over to put it back in and bandage my jaw up. Not a pleasant experience.

Trihexyphenidyl is the medication we have added into my daily regime and it has made a huge difference. Little things like I can brush my teeth now with an adult sized toothbrush rather than a children’s one are possible, and instead of dislocating my jaw by brushing my teeth, my jaw is just in a small spasm and achy. My son commented the other day on the fact that my face isn’t wonky all the time and asked if my Jaw Dr had fixed my jaw. We had a quick chat about mummy’s silly brain and moved on, but for me that showed just how amazing this medicine has been.

I saw my neurologist the other week and he has suggested increasing the dosage further as I am currently not experiencing any side effects. I’m waiting to receive a copy of his letter to my gp explaining that I have the go ahead to do this at my own pace, so we can see just what improvements we can get.

Right now, thanks to lovely female hormones, I’m sitting here feeling quite sore all over as my body goes downhill each month due to the fluctuating hormonal changes. I’ve had several subluxes today in shoulder which have in turn aggravated neck spasms. Normally I’d be quite grumpy about all of this, and yes I’m not exactly thrilled, but having the Dystonia side of things more controlled doesn’t half make coping with the EDS etc, easier. Everything just feels that little bit more manageable right now, and that’s fantastic.

Posted in Archive, March 2021

Back from Break; Update

It’s been a few weeks since my last post, as some of you will know from my Facebook page I took time away whilst my son had a major surgery. Now things are settling again the posting schedule will be returning to normal.

So what’s been happening? My neurologist and I have been trialing different medications over the last three months to try and improve my quality of life, bring my pain levels down and reduce the number of Jaw Operations I have. We tried a number of different ones before landing on trihexyphenidyl. This medicine has been life changing. It’s drastically reduced the constant jaw spasms, and whilst they are still there the severity is reduced and manageable. We’re still playing around with the dosage to see how much further we can control my spasms. It’s been amazing.

I’m still waiting for a Barrium Swallow test to confirm my chronic Aspiration and give the dietician an idea of what thickness fluids need to be to help stop this. In the meantime the speech and language therapist is checking in regularly to ensure I’m doing ok.

Currently I’m waiting to see my Gastro Dr as my GI symptoms have returned. It’s extremely painful to eat or drink anything heavier than a cup of tea. I’m pretty much living off sugary tea in the meantime to get by.

On a more positive note I’ve just signed a three ebook deal for my young adult fantasy series which is very exciting. I feel very fortunate that this is something I can do from home while the children are asleep, as given the severity of all my conditions on my body a typical job is out of the question.

Finally I want to say thank you for the support I’ve received over the last few weeks. It’s been extremely touching. Now that this post is up and you are all caught up I’ll be back to posting my usual blogs from tomorrow.

Posted in Archive, february 2021, poems

Dislocations; Smashed Avocado Toast

It’s the breath stealing, heart racing moments.

Nostrils flared, knuckles white with a fierce grip.

Head back, focused. Can’t swear.

Sausages. Bananas. Smashed Avocado on freaking toast.

Hospital? No. What can they do.

I’ll only spasm and dislocate again at one, then again at two.

Pass me Olaf, he needs his teeth done.

Sausages. Bananas. Smashed Avocado on freaking toast.

Fifth Knee dislocation of the day.

The spasms. Just. Wont. Stay. Away.

Still need to be a Floogal Rescue Machine.

Sausages. Bananas. Smashed Avocado on freaking toast.

Posted in Archive, January 2021

Disabled Parenting: A Learning Curve

Being a disabled parent is something that three years in I still have not got my head around how to nail. Though does anyone ever nail the toddler years? My children are, at the time of writing, three and 19 months old. Both children are owners of strong, hilarious personalities. Both currently are sound asleep, I know my daughter will wake up in the morning with a rendition of either Baby Shark or Let it go, and my son will wake up just before 6am, delighted that it’s early. I’ll wake up and relocate my knees.

Each day for us is always an unknown to some extent. We try to pace our days by following an activity timetable, which gets switched about at the start of each week. The timetable was introduced not only to help manage with being housebound more due to shielding, but also to encourage subtly paced activities without making it too obvious. The children, know that mummy is disabled and needs to do things differently to daddy, but I do try minimise to some extent how much of that they see.

It is a fine and difficult line to tread. On one hand it is important to me that they understand that everyone is different, some people are disabled and that’s perfectly fine; however my son has a very caring nature, and does worry, so I do try to shield from him some elements that at three he doesn’t need to worry about. For example, right now due to hormones all my joints are loose, this has resulted in multiple subluxes, dislocations, general spasms and fatigue over the day. He’s aware I’m tired today, and slightly sore, but he’s also ‘tickled wrestled’ me, so I know he hasn’t picked up on much.

We made the decision quite a while ago that I would no longer cook with the oven for the family. This was due to a range of issues such as seizure, spasming with a hot pan, or dislocating. My partner does the majority of cooking, and on weeks when he is on late shifts we have carers come in to cook the tea. However I still ‘cook’ I use the phrase very loosely, things using the microwave.Today, was just one of those days that was a dropsy day. Everything I touched seemed to be destined for the floor, which is exactly where the kids porridge ended up after I picked it up to heat it up. My hand spasms were so ridiculous the food had ended up on the floor before I had processed quite what had happened. It reaffirmed to me, that whilst I order the food my place is no longer in the kitchen, and provided the kids with a good few minutes of giggling.

Learning my own hacks to make disabled parenting work for me is something that is a slow learning curve that I am just getting to grips with. For example buying a second seat belt for my wheelchair so I can strap my daughter to me when we go out for a walk. Each day is never the same as we adapt to the needs of my disobedient body and the cheeky duo. The kids never fail to amaze me with how well they cope though. I used to get in a state over the possibility of the fact they had to ‘deal’ with a disabled mum. Whereas now I am so proud of the caring nature the two of them have, along with their inquisitive minds.

Posted in Archive, January 2021

The Test Results Are In

After spending the last several months in and out of hospital, losing the sight in my eye for an extended period of time and only partially regaining it, losing all sensation in my right leg and experiencing sensory issues in my arms I was once again told it looked like I had MS. Yet the examinations didn’t agree. I was left battling for help as different hospitals and departments seemed to find it impossible to communicate with each other. Well the most recent test results are in! We finally have an answer.

If I am honest I had almost given up on a diagnosis other than unknown complex neurology condition with global sensory loss. None of my Drs were communicating with each other, no one could agree with each other and that was resulting in me receiving no treatment. It has been a period of high stress and extreme emotion.

Today I finally had my Emergency Video Consultation  with the local specialist in Neurology; this was requested back in October. Firstly they are agreed it isn’t MS which is great confirmation. What they are sure of is that is another part of my Ehlers-Danlos Syndrome. Apprerently when I’m dislocating my knees the nerves around it are being over stretched and damaged hence the loss in sensation/function. The same thing had happened to my elbows causing the sensation I was getting in my lower arms and hands. This surprised me greatly; mainly as I had in fact asked the doctors this very question when I was on the ward last year and they laughed at me for suggesting it. In regards to my eyes the nerves are not communicating with my brain effectively, but are not damaged like you get in MS.

He’s suggested we get me booked in with my EDS consultant for some advice in the meantime on how to cope with these symptoms as they can last a significant amount of time.

So whilst the EDS is generally on a slippery slope currently and it’s all about managing it, keeping on top of my pain and being proactive, I feel that overall it was a very positive chat.

Posted in Archive, August 2018

26 hours Dislocated

Late Tuesday afternoon whilst curled up on the sofa nattering away to my partner my jaw dislocated. It wasn’t a surprise. It had felt off all day, with pain radiating around the area, and visibly subluxing often so I had stuck to soft foods all day. Being me though I hadn’t considered that talking a bit less might help. I rather excel at talking. It’s quite unusual for me to not to be able to relocate my own jaw but I decided that I’d try and sleep on it and if it was still bad in the morning I’d get checked over. I can almost hear you shaking your head at me, in hindsight I agree that was a silly decision.

So yesterday morning I took myself off to the Walk-In centre where after a quick (and right) lecture on dislocated jaws being an emergency I was whisked off to my local hospital. It was my first time visiting the A&E up here since I’ve moved and I was a tad nervous. But the staff were wonderful. They were rushed off their feet, but they were so kind, it was a breath of fresh air in comparison to what I am used to.

X-rays confirmed that the right side of my jaw was fully dislocated. After using a rather unbelievable amount of tongue depressors failed to relocate it, it was decided to take me round to the resus unit where I could be sedated and they could try and manually relocate it for me. They were so full of confidence, to the point I too was full of confidence, I happily offered them my arm whilst they pushed the sedative through, I can remember giggling as it kicked in…and then I can just remember the pressure as they tried to manipulate it. Two different doctors tried three times. I screamed. My jaw failed to relocate. They were lovely though. At this point the decision was made to phone for an ambulance to take me to a different hospital to see the specialists there.

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Sedated post failed relocation attempt

When the consultant walked through the door I could have broken down; and to be fair I did about five minutes later. I’d seen him previously about 7 years ago and the visit burns in the back of my  mind as a prime example as exactly what a doctor shouldn’t be. Upon entering the room this Dr recognised me instantly. He doesn’t believe in Dystonia. He ignored the fact I have EDS and suffer with frequent jaw dislocations. Whilst I am thankful he relocated my jaw, I cannot express how belittled, put down and worthless he made me feel. Upon leaving the hospital he advised that I start on a liquid diet but gave no further advice on time frame or inteventions in the meantime.

Out of frustration with feeling like I just didn’t know what to do to help myself this morning I went to see my GP, I am lucky to have a wonderful one up here. He was quiet astonished that I had been discharged from the hospital last night as you can see my jaw subluxing still and with my history its only a matter of hours/days until it fully dislocates again. So I’ve been referred to the oral surgeon and on strict orders to maintain a liquid diet until then. Dystonia and EDS are two conditions that really work against each other so here’s hoping there’s a not too drastic treatment plan in the future.

Posted in April 2018, Archive, September

Wonky But Happy

“Hmmm that’ a nasty dislocation to have long term, take some morphine.”

“When you next see your neurologist, if I were you I would discuss having your botox more regularly. This degree of deviation, pain and dislocation on a regular basis is not good for you.”

“Wow. Ehlers-Danlos, and Dystonia. You couldn’t have asked for a worse combination of conditions there.”

“Are you sure you don’t want to go the hospital? I’m sure the A&E department will listen to you this time. I’ll even write you a note.”

These four word-for-word quotes from different health professionals give you an insight into the last week and a half of my life. My botox has worn off a couple of weeks ahead of schedule around my jaw, the rest is still working well, so overall I’m pretty happy. However this does mean I’ve been experiencing regular extreme spasms and dislocations in my jaw again, which in turn has an impact on my ability to talk, eat and drink.

Whilst my ability to communicate using British Sign Language is steadily improving, I took a trip to the doctors to get a prescription for some painkillers and muscle relaxants, as I’d like to eat, drink and talk in as little pain as possible. Whilst I have access to oramorph this is my last resort medication, and not something I am willing to take around my son unless it is an emergency. The doctor couldn’t quite believe the predicament I was in, let alone get his head around the fact that I did not fancy sitting for a couple of hours in my local A&E at a hospital that has repeatedly provided the wrong treatment despite direct instruction from my neurologist. I stated to him that as I don’t respond to local anaesthetic I would much rather take the painkillers and muscle relaxants at home and relocate my jaw myself when the spasm eased off. At this point I think he would have dragged me to the hospital if he could have.

We discussed at length (well I scribbled out for him what I was attempting to convey) my botox arrangement with my neurologist. It stunned him that I was willing to put up with these spasms for a further two and a half weeks. The moment was an odd one, with me not really in a great place with my distorted face, twisted neck and dislocated jaw to protest that actually I was doing great, but then he didn’t know me six years ago when I was bed bound, he didn’t even know me a week beforehand when my botox was working well, so I can see where his concern comes from.

At the time the above four quotes drove me nutty. But I know I’m easily wound up when in pain, so I can’t say that I am surprised. In reflection, whilst my jaw still is causing me significant pain from my current dislocation I can see my progress in pain management and self-care; which is an element I am proud to have improved on.

ips.jpg

Posted in Archive, June 2017

Oromandibular Dystonia & Communication

 

In 2012 one of the first symptoms I developed was severe Oromandibular Dystonia. This meant that my jaw, mouth and tongue go into painful, and often extreme spasms. On these occasions I struggle to speak; this can be due to several factors such as: my tongue spasming and making it impossible to talk, the jaw spasm itself, especially when dislocated, making it impossible; or it is simply too painful to do so. I often attempt to try and talk through the spasm but this can aggravate it.

Trying to communicate during these episodes is difficult, even if I manage to successfully make a noise, what I am attempting to say may not be clear. In recent weeks, since the birth of my baby, I had been trying to think of ways around this. Writing it down is one option, however, I find physically writing very painful and often dislocate when doing so. Instead my partner and I have decided to learn British Sign Language; we’re incorporating baby sign language into this too so that Stefan, when old enough, will understand as well.

We’re off to a great start and enjoying this venture. I’m finding that I feel far more settled knowing that I’ll be able to communicate clearly, even on bad days. As someone who is quite the chatterbox, this is important to me.Image result for BSL