Tuesday, 28 October 2014

Hypermobility type Ehlers Danlos Syndrome: Progressive or not?

I've noticed this discussion recently on social media.

The problem is that there is a difference between 'a progressive condition' and 'a condition which can cause progressive levels of disability'. With the former, deterioration is inevitable. It will happen in every case. In the latter, it will happen in some cases.

With hypermobility type Ehlers Danlos Syndromes (which many experts consider as the same as JHS and BJHS), the genetic fault that has caused the faulty collagen remains constant. It doesn't change. It doesn't progress in a medical sense of the word.

But the symptoms can get worse. The level of disability can progress.

Mine got worse. Lots worse. I deteriorated from a 20 year old with a few quirks and vulnerabilities, to a 28 year old wheelchair user, retired on permanent ill health grounds, with unstable joints throughout her body and who struggled to hold a conversation or eat.

With the benefit of hindsight and everything I have learnt since being diagnosed at 24, I can see that the reasons for my deterioration were 3 fold.

1. I was overdoing it. I was doing more than my body could cope with, and ignoring all the signs that my body wasn't coping. Because I didn't think they were real symptoms, and I didn't want to 'give in'.

2. With injuries and pains I often rested more than I should have done, causing muscle wastage - and more pain and instability. (some rest post injury is important, but I now know that keeping moving and slowly increasing use is important.)

3. I lived in positions where my ligaments were being stretched (slouching at the computer, twisting my legs into 'comfortable' positions, poor shoulder posture etc.)

So my condition progressed. I deteriorated.

But since then, I have improved - HUGELY.

No, it was not a magic cure.

But I have improved. What happened? A combination of factors.

1. I started a medication for POTS which meant my body could cope with the basics of eating and talking without throwing a compete hissy-fit.

2. I learned about managing my HMS/EDS, much of which was through the Hypermobility Syndromes Association (HMSA).

3. I crashed out. Big time. 5 weeks in hospital time. There I learned (the hard way) that when my POTS symptoms started building, I should take evasive action (cool down/get my core and leg muscles working/lie down/drink rehydration stuff) instead of waiting for symptoms to reach avalanche status and swamp me - In short I learned to pace. To put into practice what I'd learned from the HMSA.

And I built the confidence to communicate about my management choices rather than just do what I thought I was being told by the next medical professional I saw. Learning to communicate about my  choices in a way they understood and could respect, so we could work together to improve my management.

When I went home instead of trying to do as much as I could to prove I wasn't 'giving in', I started listening to my body. I learnt that I couldn't cycle for 10 minutes - it made me unable to function for days. But I could cycle for 2 minutes provided I concentrated on keeping my joints in good positions and my muscles nicely activated. 'cos that only tired me out for an hour. (By slowly building up, I now can cycle for half an hour and more!)

And I worked at living with poise. Not like I have a poker up my...spine. But upright, strong, and ready for movement. Like a gymnast about to do a run-up for the vault.

I admit I tend to go in cycles. I start committed to my exercise regime, careful with joint positions, I pace well etc - and improve. As I improve, other things start taking priority....business, housework, socialising. And I start to deteriorate again. Then I suddenly realise, feel a little foolish, and start being good again. - and as I am good, I improve. Until I get complacent and the cycle starts again.

But the important thing is that I don't go so far over my bodies limits any more. I recognise the warning signs so that when I start being good again I haven't caused new damage to my ligaments.

And at 32 I would say I am stable. Not because my condition has fundamentally changed, but because I know how to live well with it.

If we keep doing more than our bodies can cope with, we will get worse. If we consistently do less than our muscles need to in order to stay strong, we will get worse. But if you think about it, this is actually true for everyone. That is what happens to every human being who does too much or too little. It is why athletes retire young - and frequently with a long list of injuries and why people get injured doing 'unaccustomed exercise' all the time.

During those pre- and early post-diagnoses years, many bits of me have worsened - been more stretched. And I expect I'll stay stretched. But I don't expect to pick up more outside of the general deterioration that happens with life in general unless I have a bad accident or significant illness or develop another condition which makes managing my HMS/EDS even harder - and thinking about it, those types of things can happen to healthy people too and cause a deterioration of their physical condition too.

It is more, as a friend 'Flick' said to me "relapsing/remitting (flare/rehabilitate), with some cumulative tissue damage. Un-managed it mimics progressive."

My body will never be one that can cope with 'average'. It never has been. Finding the right balance and maintaining it will always be harder for me than the average bod, but it is doable.
And I am extremely grateful to the HMSA, the hypermobility clinic at UCH London, and my cardiologist for helping me learn to manage my condition, so that I can respect and understand it instead of fear it.

To all you who have not yet been able to find the right balance, don't give up. It is hard work, but also very, very rewarding.

(Images are extracts from the book "You know you have HMS or EDS when..." by Hannah Ensor.)

Sunday, 26 October 2014

ASC - creating a keyring card pack

As some of you will be aware Stickman Communications (R) has a number of cards which are relevant to people on the autistic spectrum. Some are specific, like the one about difficulty making eye contact. Others are more general, like the card saying "Thank you".

I am in the process of putting a set of cards together as a 'starter' pack of the cards which are relevant to most people with ASC, which will then be sold at a discount (compared to buying the cards individually). This pack will go on sale on Tuesday 28th October (the delivery date for the new designs). The aim is to have a simple 'starter' set of the key cards that people can customise by adding other cards relevant to them.

The 'traffic lights' card set is already available at a discount, and so I'm not planning to include them in the ASC starter set. (But I'd be interested to know your thoughts on this.)

So far, my ASC starter pack list has the following cards:

But which cards do you think belong in the ASC starter pack? There are many more cards in my shop, and the ones I think may be relevant to ASC are here. (The full keyring card range can be seen here (Scroll down the page).)

If you'd like to leave your feedback in the comments, on my facebook page, or by email, I would be most grateful. I won't be able to do exactly what everyone says because everyone will have different opinions, but at least I will be able to get a better idea of what most people will find useful, and therefore end up with a good selection.

Also let me know if you think there are products on my site which are not keyring cards which would be useful to include in the pack.

(and if you have ideas for cards that I haven't yet made, feel free to make suggestions. I can't always make the cards suggested, and sometimes it takes a while, but I do my best.)

Tuesday, 14 October 2014

Type 1 Diabetes: Let me manage my condition

I've spent the past few days working on some type 1 diabetes cards, at the request of a group of parents of children with the condition.

I knew little about the condition, but as I read through the parents requests I realised that actually, the condition might be different, but the communication problems were the same. Assumptions and disbelief. And well intentioned things which REALLY don't help - even trying to force me to do something which I know I can't. These are barriers that I have encountered - and ones which I know the stickman keyring cards can help break down.

The scenarios described, like people not believing that a child with diabetes could enjoy the food at a birthday party, and simply taking their plate from them. Or assuming that they caused their condition through an unhealthy lifestyle etc were not ones which I had experienced personally, but they resonated with my experiences - and next thing I know I've finished the drafts in 2 days rather than the week I had planned!

So here are the drafts:
The text for the first one isn't easy to read in this image, so I've copied it here:
"I have Type 1 Diabetes, a life-long condition where my body can’t regulate my blood glucose levels. It is not caused by diet or lifestyle, but an autoimmune reaction destroying my insulin-producing cells. I don’t have to follow a special diet, but I do have to take varying amounts of insulin. Treating episodes of high or low blood sugar immediately is really important for my long term health so I may need to check blood glucose, inject insulin or eat a sugary snack. Please don’t ask me to leave, but let me treat my condition wherever I am. Any delay will make things worse. I know what to do. It is my normal." 
(each card is 110x80mm, laminated, durable plastic, and come on a keyring.)

As I once heard someone comment, the keyring cards aren't for people who can't speak, so much as for people who won't listen.

A way to state requirements with confidence - and in a way which people accept far more easily than when spoken, and are equally effective with both adults and children. I'm still not sure why. Perhaps it is because they are professionally printed and official looking. But I wonder if Terry Pratchett was right when he wrote "Laughter helps things slide into the thinking."

And while discussing the cards with one of the parents I realised that one particular aspect of their treatment was very familiar. For several months (or possibly years, I can't remember!) I had to inject myself 3 times a day. And sometimes I really wanted to say "actually, I need it NOW and don't have then energy to waste getting to somewhere 'private' just to make you feel 'better' about a young person needing injected medication!" - so it became a more general 'injections' card.

So if you have any feedback, suggested improvements or errors spotted, please let me know by this Thursday - as I will be sending them off to the printers then :)

Saturday, 27 September 2014

Bruised Knees

I have bruised knees.

Both knees.

Many bruises.

They kinda hurt. but they make me smile.

Because every time I feel the ouch I remember Tuesday night. 'Dugout Dance' had been closed for the summer, and Tuesday evening was my first session back.

I decided to dance not in my wheels, but from sitting on the floor. That way, when I fall I don't have far to go, so I give all my muscles a good workout and make full use of my flexibility - an asset when dancing, even if it has stolen my ability to walk properly.

It is really hard to explain just how alive it makes me feel to dance 'floor based'. It means that my Postural orthostatic tachycardia Syndrome doesn't complain too much - because I move at my own speed, can switch to lying down whenever I decide to, and there is no need to attempt standing poses or ones with my arms above my head (Both huge triggers for my POTS). The result is bruised knees and 2 hours of exercise at whatever intensity I feel able to do, and where I feel truly beautiful and at one with my body. A moment where there is nowhere else I'd rather be.

So I feel the bruises and remember the beauty.

And and all is right with the world.

Saturday, 20 September 2014

To ask, or not to ask, that is the question.

Last Saturday there was an article in The Guardian by Robert Hoge called
Don'ts' aren't helping: here's five things you can say to someone with a disability
It makes some important points - not least that many people who aren't used to disability are so
terrified of saying the wrong thing that they are almost unable to relate to people with disabilities, and this does need to change.
Description: A scared looking stickman in a cloud of politically correct angst sat next to a stickman in a wheelchair saying Hi. Caption reads "Differently Normal, not the PC police".

But I'd have to agree with Mik Scarlet's response that actually, no. Some of those are not things people should be encouraged to ask.

Mik is totally right where, when addressing the 'tell me about your disability' question, he  states
"...for every disabled person who is in a place where they can answer this question there are just as many who are not. "
There are times when asking this question is cruel and extremely upsetting. Don't ask a random stranger this, just don't.

Even so, Robert makes this excellent point:
"If we want to bash through barriers confronting people with a disability, we need the community to have a better understanding of us. This probably means some uncomfortable conversations – for others and for us. Focusing on what people shouldn’t say isn’t the way to build good relationships. If we can face living our lives in these bodies, we can face some difficult exchanges along the way."
But this doesn't need to be said to the non-disabled public. It needs to be said to us people with disabilities, so we can support each other in finding our own ways to open up the lines of communication, and find ways to let people understand our world. It is why I started Stickman Communciations - communicating about disability in ways that both disabled and non-disabled can relate to.

So: What should be said to the general public? (Other than Rob's much needed observation that it is OK to say hello.) To me, it is simple.

Forget about disability.

There are various phrases which are routinely used for 'random stranger' conversations.

1. "Lovely/Awful weather isn't it!"
2. (In a shopping centre, to someone with many shopping bags) "Managed to find some good stuff then?"
3. (On public transport) "Looks like the train/bus is late again/on time for once."
4. "Having a good day?"

The reply to these is usually
Chatty, encouraging more conversation - in which case carry on chatting. And over time chat might contain more slightly personal questions (about work, life, health, family etc.). But not overly-personal (i.e. toilet habits, sexual issues etc.)
Blunt, annoyed, or they turn away from you. In which case, don't attempt to carry on chatting cos they aren't in the mood.

And if we put the disability issue back in? It's exactly the same. 

(No. it isn't OK for a random stranger to walk up and ask me how I use the loo. Yes, this really happens.)

As to when is it OK to ask about disability...well, it is a personal matter. Some parts of a disability might fall into the 'slightly personal' category, some the 'overly-personal'. So, if you've got chatting to someone and would like to ask about their disability, the best option is to say:
"May I ask you about your disability?" - and then respect whatever answer you are given, whether a yes, or a no.

But can you offer help? - again, take out the disability. Do they look like they are struggling? Yes? - offer help. No? - Don't offer help.

And as to telling a person with a disability that they are inspiring....guess what.....take out your perception of disability, and then consider whether the facts that you know give rise to the inspiring or not.
For example:
I get on a train, and wheel myself expertly into the wheelchair space. A gentleman leans over and says 'That is really inspiring'. No. It isn't. It is a basic skill that is well within my physical ability, and no more notable than an average non-disabled person sitting in their seat without squashing their neighbour. Unfortunately all this comment told me was that his expectations of my were almost non-existent, so his praise was, at best, extremely patronising.
Another example:
I get on a train. I chat with the chaps opposite. During the conversation my wrist dislocates and one of them helps me relocate it. I then manage to choke spectacularly and can't speak. I roll my eyes, and signal "I'm OK" - and use my keyring cards to explain that 'My ability to speak coherently is temporarily missing, please try later.' My part of the conversation switches to hand gestures. As they help me off the train at my destination, one of them says "You are inspirational."
Fair enough. They had seen me fall apart and deal with it calmly. They had seen my body do things which I hate it doing, and stay positive.

So before you tell someone that they are inspiring, just ask yourself "do I know from what I have seen and heard, that they have pushed their limits in an exceptional and inspiring manner, or am I making assumptions?" - and when you have your answer, you will know whether it is appropriate to say it or not.

[Edited 22/09/2014 to add: Perhaps the reason there are few 'what to say to someone with a disability' lists because they would be so long the brain would melt half-way through. Topics from space travel, to 'Lost' via whether Elvis really died, and where do odd socks really go?]

Monday, 11 August 2014

The best of conversations

When fatigue is playing up, conversations decrease. Word finding isn't easy, sentences don't happen and word order becomes increasingly creative.

On Saturday I was facebook chatting with a Carla. Who was also a fatigue zombie.

Minions to the rescue!!

What happened next was one of the best conversations I have ever had.

Thursday, 7 August 2014

It's been busy!

I know my blog has been quiet.

July's offline stuff stole all my energy -it's been quite a month!

New products, attending Warrington Disability Awareness Day, more new products, helping with the Hypermobility Syndrome Association's residential conference and.....new lycra.


Yes, lycra. from www.dmorthotics.com

You see, some of the way my POTS and EDS have affected me include: lots of blood pooling in veins causing reduction of blood supply to the brain, very poor proprioception and lots of unstable joints. To help manage the blood pooling I've been wearing standard compression stockings plus sports compression wear plus abdominal binder for years, and been on various meds (including Octreotide - an injection every 3 weeks in the butt). They have helped but not enough. So I grabbed the chance to try a custom made lycra outfit (socks, leggings, T-shirt) with extra compression in relevant places.

I wore it on the Saturday of the HMSA conference. Yes, this is me:

How awesome is that!?!

Funnily enough, this was me the next day:

(My wheelchair is out of shot. Or possibly being sat on my the photographer. I can't remember. I didn't mind what went on around me so long as I was left in peace.)

It's not a cure, but it is a really useful management tool - even if I currently have no idea what my capabilities are and consequently am totally failing on the pacing front. Moments of 'LOOK I can have a conversation standing up! Isn't this amazing!!' followed by flat on the floor moments where words are not a viable prospect at all. But balance will come with time and experience.

The next few months will be trial and error, discovering whether can I wear it everyday without trashing my skin, without injuring too many joints while putting it on, and possibly hardest of all, learning where my limits are and what I need to do to manage my conditions as best as I can.

This will definitely have to include not sitting upright and relaxed for more than 30 minutes without doing something to get the blood moving (says me after 2 days of going 'whoop I'm alive', doing an hours work on the computer, then crashing for the rest of the day. I try to be sensible, but sometimes I end up learning the hard way!)

So it's no cure, but it is an exciting new tool which should improve my condition management and help me maximise my abilities.

And as I am now off all my POTS control medications.....my butt cheeks are most pleased not to have an elephant sized needle attacking them every 3 weeks!

[Edited to add: this is also a result of 8 years of physiotherapy, pilates, adn working really hard on posture and joint control ;) ]