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 body's 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 :)