Just Another Day – with EDS & POTS

 

Just Another Day pin

 

The world feels off kilter

I’m spinning…..round and round

Every time I lift my head

The snow  descends, engulfs me

At times it overwhelms me

And then, bang, down I fall.

 

Lucy EDS 1

 

Falling, falling, twisting down

How long it takes to reach the ground

Slow motion into blackness

Nothingness

Painless

Floating.

 

Pain, pain, pain

Why am I on the ground?

A twist here and a bend there

Limbs entangled, joints at angles

Unnatural, except for me

Stretch and snap is the norm.

 

Lucy EDS 2

Salt, water; water, salt

Compression to limbs.

Like an autumn leaf

I fall and break

My body fragile, unstable

Just another day with EDS and POTS.

POTS & Me

 

 

 

 

What are Dysautonomia and POTS?

Hannah on Sunshine and Spoons blog has written this post about Dysautonomia and POTS (postural orthostatic tachycardia syndrome).

She recently did a straw poll on a facebook group that we belong to and very few people had any idea what this medical term means.  I knew because I have it…..but had never come across the term in my own medical career.  Some of you will have read how my symptoms have been particularly bad recently, but I am now able to stand upright again!!

Please read the whole piece and share in order to help us spread awareness!

What are Dysautonomia and POTS?

What are Dysautonomia and POTS-

Did you know that October is Dysautonomia Awareness Month?

Let me back that up a little….do you know what dysautonomia is?

If you said no, you’re not alone.  Most people have never even heard of it.  I didn’t know what it was either until I was diagnosed with it.  I have Postural Orthostatic Tachycardia Syndrome or POTS which is one of the autonomic disorders that falls under the umbrella term of dysautonomia.  Other types include Neurocardiogenic Syncope or NCS and Multiple System Atrophy or MSA.

Despite the fact that many people have never heard of dysautonomia, it’s not rare.  It’s estimated that over 70 million people around the world have it in one form or another.  Often, it’s a secondary condition to another disease or disorder such as diabetes, Ehlers Danlos Syndrome, Multiple Sclerosis, Lupus, Celiac Disease, arthritis, and more   The most severe cases can result in death.

What is POTS?
Your autonomic nervous system regulates different functions throughout your body such as your heart, digestion, temperature, blood pressure, etc.  People with an autonomic disorder such as POTS have issues regulating those things.

 

To read the whole post visit Sunshine and Spoons 

You will also find great links to Hannah’s Sunshine and Spoons Shop

POTSWarrior5

 

Fainting for my Motability Chariot

Fainting in the car showroom wasn’t something that I had thought would be a part of my week!  We had a phone call to say that my Motability car would be arriving and it has felt like the whole process has happened so fast.  Not so long ago I was still convinced that I would be deemed too “fit” to qualify.Fainting for my Motability Chariot (2)

 

It is a shame really that the “passing out” wasn’t due to excitement, but rather a POTSIE dysautonomic response!  My symptoms are always worse in the morning from the time I get up to mid morning – my cardiologist said having laid down all night (sometimes) the body is then put under immense stress from gravity and “baggy” blood vessels to keep circulating oxygenated blood to the whole body on standing. So the system concentrates on the major organs in the abdomen, blood rushes down away from the head, pools in the hands & feet – and I feel extremely dizzy or faint. Yes, out cold! So a 9.30 appointment was never going to be great – but add in stormy weather, bright fluorescent lighting and a hot showroom and Bingo! you have the makings of a “dysautonomic nervous system” episode. Sounds grander than it feels!!

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Image from a range of products available at http://stickmancommunications.co.uk/

Anyway, the salesman came back to his desk to find me with my head back, shoes off, feet up, grey and clammy. Not my best look…..but precisely the reason that I have been awarded the enhanced rate benefit. Of course add in the dislocations, pain etc…..all adds up to me getting a brand new car and not being able to drive it! So disappointing. My mum and hubby are the named drivers on the inclusive insurance – no, the student engineer isn’t old enough, much to his disgust – and I I grudgingly accept that passing out behind the wheel or pulling an arm out of socket whilst turning the steering wheel, probably isn’t the safest way to get from A to B.

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Grey really is not my best look! Post faint…

Busman’s holiday for hubby taking a car handover and the student engineer had the bluetooth wireless phone/stereo system programmed before we had even started the engine….great except he has no understanding of why I can’t pick it up in the same “on the spectrum” way that he does.  The ride feels smoother and hopefully this will be a huge benefit for my back pain, but I haven’t been well enough to go back in it this week.  Female hormones always exacerbate my symptoms and I have had several days when I have been unable to sit upright – another post for another time!  But I’m upright today and so writing a very speedy post having missed out all week.

Car

My Chariot!

Thank you Motability for a fantastic scheme that has allowed me to have a car to house me – comfortably I hope – my wheelchair, shopping and a dog!

Rheumatology, Cardiology, Falling Down and Midodrine

Finding the time and energy to write has been a challenge recently.  I’m not sure if you would describe my worsening ailments as a flare, but my poor, body has been struggling to hold things together.0ff7167e97f625a09a50879d90d5057c

Last week I saw my GP and my cardiologist and managed to get two new referrals in the process.  The first to a new rheumatologist, recommended by the London hospital who diagnosed me, and the second to an endocrinologist to check if the cardiologist is missing anything.  Don’t you just love these multisystem chronic illnesses?!

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For the rheumy we have a family outing on the cards, as the A level student and lovely girl have also been referred.  The bookings department called last Friday and asked if we would like consecutive appointments – I bet the consultant will love it when she sees the same surname appearing three times in one morning.  “What another genetic problem?!”  I have put off having the kids diagnosed with hEDS even though I can see elements in all three of them, but it has only been recently that I learnt from the lovely online support that they might be eligible for extra help in exams.  This is down to the pain and difficulty with writing that we with bendy fingers and dodgy collagen experience.  My daughter has never been able to hold a pen properly and they both struggle to be able write as fast as their peers – their brother, the uni student, was exactly the same at school.  In fact his fingers are so flexible that you might think they were missing their bones altogether.

The schools have responded really well and thanks to some fantastic blog posts that I was able to direct them towards, have now heard of Ehlers Danlos Syndromes.  We missed the deadline with the exams boards for the A level student to receive extra time (he needs a formal consultant recommendation) but he is allowed to be in a smaller room and to have rest breaks.  Had his typing been quicker, he could have opted to use a laptop.  My daughter’s school have been fantastic!  They are keen to learn – obviously they have seen me deteriorate after repeated surgeries and progress into a wheelchair – and many teachers have been upset to learn that she has not spoken up about pain, dislocations and difficulties in games classes.  Of course she is worried that a fuss shouldn’t be made as there are other people more in need!  But in terms of her year 10 exams after Easter, she will use a laptop and take rest breaks.  When I was at school I had no idea that the pain I always had and my difficulty getting through written work was actually down to a genuine problem – in fact at the beginning of this academic year none of us dreamt that our pain would actually be considered a reason for the kids to need extra help!  Of course the new guidelines came out last week for diagnosis criteria, so I’m not sure what they might be deemed as – well I could say a few choice words that have nothing to do with EDS!

More to come about the criteria and of course how we all get on.

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The EhlersDanlos Syndromes International Classification

I have been on the waiting list for a follow up cardiology appointment since the end of last year.  Immediately after my tilt table test, I started the synthetic steroid fludrocortisone to increase my circulating fluid and raise my blood pressure…if anything it made me worse as the faints, falls and injuries have come thick and fast.  My GP tried to have an appointment fast tracked, but when this couldn’t be arranged the cardiology department told me to double the fludro dose and continue with compression tights, fluids, salt.  I have felt so unwell and so tired!  Last week the consultant started me on midodrine, which in the UK can only be prescribed by a cardiologist for extreme cases of low BP that haven’t responded to conventional treatment.  It is early days and I am only on a low dose three times a day – but I cautiously think there is a small improvement.  The last dose of the day mustn’t be too close to lying down in bed as there is a risk of increased blood pressure when flat and strokes.  One side effect I do have is a strange creeping, tingling sensation of the skin particularly my scalp – about an hour after I take it when it reaches highest blood concentration level.  So if you see me out and I’m itchy, I really don’t have nits!!13413444_143336392741362_1604407187_n

 

Saturday Submissions: “Dysautonomia for Dummies”- With Evie from The Zebra Mom

Dysautonomia for Dummies from the fantastic Zebra Mom

Irish Dysautonomia Awareness

I’m Evie and I come from Cork, Ireland. I’m a 29-year-old mother of two baby zebras. Alex is 7 and Olivia is almost 2. I am diagnosed with Hypermobile Ehlers Danlos Syndrome (hEDS), Orthostatic Intolerance and Vasovagal Syncope. I first heard of EDS after interviewing a young woman with EDS for the paper I used to work for. Something about this woman’s story stirred something inside me and I became passionate about raising awareness of the condition. A year later I was diagnosed with EDS. When I’m not blogging, looking after my two children or lying in bed ill, I help my husband run our wedding videography business and co-host a radio show on Saturday evenings from 7pm (Irish time) on www.clonlineradio.com.

evie blog

I write about Ehlers Danlos Syndrome an awful lot and with where I am guest posting today, I decided to focus on Dysautonomia. I recently wrote A Simple…

View original post 1,780 more words

Tilt table, echo, cardiology & probably #POTS..Y!

So yesterday saw me back in our local cardiology department to undergo investigations for my funny turns & faints – the symptoms of a malfunctioning nervous system, common with EDS.  I was inexplicably nervous – particularly when I think about some of the major operations I have had over the years.  Maybe it was the thought of having my symptoms induced or worrying that the tests might be negative and I might have to start convincing everyone that I’m not imagining my symptoms.

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Anyway we arrived at lunchtime, me having starved for the obligatory number of hours, and the first test was an echo ultrasound of my heart.  The first thing to establish was whether my scs would interfere with the scan as it did with the 12 lead ECG on my last visit.  I perhaps should have been more concerned about my joints as I managed to pop my shoulder out whilst lying on my side, scaring the young sonographer silly as it literally “popped”.  Not a good start before the tilt table as a sling was hung from the very same shoulder to support some of the machinery!  The ladies performing the test were most concerned about my pain and my ability to stand still for long enough – I was instructed not to be brave.  At this point we didn’t know if I would be able to keep my stimulator on or whether it would interfere with the heart trace.  Happily there was no interference, so at least I would be able to keep my leg pain under control!

The first part of the test is easy – provided lying flat isn’t an issue (I managed) – lying on the table and being monitored for about 10 minutes.  The next stage would normally be to be tilted up to standing – yes I was strapped on – and monitored for a further 20 minutes prior to GTN spray being put under the tongue, ahead of the final monitoring after the blood vessels had dilated.  So great care was taken to elevate me gently to avoid jolting my back….and within seconds my vision was going, my blood pressure dropped, my pulse jumped and I started to heave!  With this heaving apparently my BP dropped too low to measure and the student thought I was about to throw up over her.  The next thing I was aware of was being flat and being told that this was the quickest and most dramatic positive result they had ever had!  The same thing happened when I was slowly sat up 5 minutes later, so the test needed to go no further.

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Not looking quite my best today! The ECG is attached to one pad on the chest wall and via a lead to another pad below the left armpit. The monitor can be unclipped to shower.

I was sent home attached to a 7 day ECG monitor which I need to activate every time I have palpitations, sweats or dizzy spells and am due back to see the cardiologist on Thursday presumably to talk POTS (postural orthostotic tachycardia syndrome). This morning I woke with the headache from hell and have been so tired, and I’m also feeling slightly paranoid about when I am pressing the heart trace button on my new piece of equipment -did I really feel something??  For the next few days I will be filling in a data diary and be even more wired than usual – with electronic gadgets that is!

 

Cardiology, POTS & Some Enchanted Entertainment

This last week has been a challenge, as I’ve really tried to carry on as a normal mum during the school holidays – and have used far too many spoons. So much for pacing myself.  Those of you who follow my facebook page will already know that I went for afternoon tea with my mum and some friends at the very posh Cannizaro Park, Wimbledon.  We were c
elebrating the 80th birthday of a family friend and I was delighted to be able to enjoy the afternoon – and to be able to wear a dress that I haven’t been able to get in to for a while.

So to those all too frequent swoony moments (no hunks involved sadly) and one story that File 24-08-2016, 17 01 22I put on facebook.  The weather has been beautiful, but has left me with massive dizzy spells and faints – I even managed a fantastic one during a short dog
walk the other day! I knew I felt rough, so Duncan called home for a teenager to come and help get me along the road. By the time Olly came wandering along I was just coming round on the pavement….what do you think he did? Straight to the dog to check he was ok!! Forget the blasted dog, what about your mother sprawled on the pavement?? Dunc says I’m not allowed out again…

 

 

 

This week has included a hospital trip to see a new consultant in a different speciality – cardiology.  The neurologist had recommended that I be seen as she felt, along with me, that the faints are not epilepsy related but probably a dysautonomia condition.  It probably wasn’t a bad thing that it was a warm day and as I wasn’t seen until late afternoon, I was feeling pretty rough.  I was lightheaded and massively fatigued.  Twelve lead ECG first with a new cardiographer and then the usual wait in a corridor.  My blood pressure was taken on a dynamap(always think a proper sphyg better) in the corridor and, not surprisingly, was elevated – well for me anyway, as my BP is normally on the low side.  The cardiologist was great.  He seemed to have a sound understanding of Ehlers Danlos, wanted to know about childhood issues related to it, when & where I diagnosed (University College Hospital, London) and what heart investigations I had already had…..answer: none since childhood.  He seemed slightly surprised, but went through some of the common issues of leaky valves, postural issues, thyroid levels, palpitations and then took a lying animages (3)d standing BP.  Whilst there was no significant difference with this (and I thought he nay think I’m making it up), he could see that I couldn’t stand up straight – it feels like I’m being pushed backwards, although I’m told I fall forwards. Weird! Anyway, he believes me enough that he has ordered a tilt table test (exactly as it sounds, but the patient is left lying flat for considerably longer than an outpatients lying/standing BP and injected with nitrates etc to observe reaction when tilted to angles which will induce symptoms. Can’t wait!), bloods and a 5 day heart trace.  He mentioned postural orthostotic tachycardia syndrome (POTS) and that postural circulatory issues are a symptom of EDS – which I knew, but had been concerned that he might not recognise!  More news as it unfolds.

 

The light entertainment has been enjoyable but tiring.  I have been writing this post for days now, but the fatigue and brain fog have taken over!  Duncan had bought tickets for my mum and I to visit Cadogan Hall in London for the concert “An Enchanted Evening” – with Lesley Garrett, Ruthie Henshall, Gary Wilmott and Michael Xavier (I have copied my short review to the books & reviews page). download (2) Public transport is a nightmare for me these days and I can’t manage the journey home after a show, so Duncan drove us up to Sloane Square where we managed to get the one and only disabled bay.  This is where I feel so guilty as my husband came to dinner with us – really could have done with a wheelchair to get to the restaurant- and then took himself off for the couple of hours that we were being enchanted!  Our darling eldest son was also in London for the evening and eventually called his father to invite him to join his group for drinks – because he felt guilty.  Too late, student engineer – Dad was already headed down the Kings Road for the Curzon and a performance of the new David Brent film. Of course Duncan spent many years working on THAT Slough trading estate! Office fans will understand.  Without him I just would not have been able to do this night out, and for that I am so grateful.

We had a lovely evening as a family with our dear friends Evi & David over the weekend too – they have been a major support to us in so many ways for all the family, and this is ongoing.  Evi and I are just massively relieved that David & Duncan have found each other – kindred spirits in their love of Prog Rock music, meaning that we don’t have to listen any more (sorry, boys!).  Then, bam!, it hit me.  The fatigue, the pain – everywhere, the swelling of my feet and hands…..here comes another PJ day!  I’m so sorry, I can’t come out today….

 

Compassionate Advocacy

Before I write my own piece this week, I want to share this video link with you.  I am aware that some people won’t want to watch and will dismiss it as sentimental.  But I view it as a celebration of 2 remarkable young women – and as we are bombarded with news daily of the harm that we are doing to each other, it is wonderful to remember the good that still exists in abundance.

Thanks to Hospitalprincessblog (https://hospitalprincess.com/) for sharing and Dysotonomia International