Thursday, 16 March 2017

Hypermobility spectrum disorder: what it means to me.



[This interview was published yesterday by the HMSA. Copied here in full]

Our volunteer team took the opportunity to ask HMSA HSD Ambassador and Patron Hannah Ensor a few questions about how the changes to the classification of hypermobility syndromes might affect her.

What is your diagnosis?

My official diagnosis is "Joint hypermobility syndrome". (JHS)

Over the years it has flickered between JHS, HMS and EDS hypermobility type, depending on who was writing the letter, and what I'd told them.

So although EDS is listed on some letters, I've only ever been formally diagnosed with JHS.

What do you think you would be classified as under the new system? (see here for an overview of hEDS, and a basic update for clinicians)

From what I've seen, although my Beighton score is low (1/9) the system allows for 'generalised joint hypermobility' regardless of score, which is definitely true of me.

But I only have mild skin issues so I don't score enough points in that section to qualify as hEDS.

So I think I'd be generalised hypermobility syndrome (G-HSD.)

How does your hypermobility affect you?

I have generalised bendiness with instability in practically every joint - and yet my beighton score is low because my knees and elbows don't bend backwards. They bend sideways, and rotate in ways they really shouldn't.

I have POTS - quite severely.

I have reflux and mild issues with a slow gut and a bendy bladder.

And plenty of pain and fatigue (which I almost forgot to mention because it's become so normal for me.)

It affects me every day, every minute. Looking at my life objectively, there is very little I do in the same way as an 'average' young woman - I have had to adapt every aspect of my life, using a wheelchair, being conscious about posture, daily physio to enable me to function, pacing, lying down and moving about to manage POTS, avoiding heat - all sorts of things. It even affects what and when I eat and what I wear.

Does it annoy you that POTS and gastro-intestinal issues are not included in the diagnostic criteria?
It's a bit frustrating, but actually there's not yet enough information on how they are linked. Both POTS and gastro-intestinal issues can have lots of different causes so they can't be part of the diagnostic criteria until they are better understood. Thankfully the new information is very clear that doctors should be aware of, and look out for these linked conditions in someone who has an HSD or hEDS - and there's some really useful management guidance on them too. I really hope more research happens soon, but until then I think I'm in a better position for accessing help with POTS and GI issues because there is now clear, concise, up-to-date information I can refer my doctors to - regardless of whether I'm HSD or hEDS.

Do the changes scare you?

Of course. Change is scary, and as it took me 20 years to get my current diagnosis, the thought of possibly 'losing' the respect that saying 'I have EDS' can get from medical professionals is very unsettling.

What would you say to others who are in a similar position?

I've found it helps to focus on the fact that it said that HSD is not necessarily milder than hEDS - both can vary in severity. And the HMSA 'clinicians guide' (which I've heard a little about) is explicitly for both hEDS and HSD - making it clear that us HSDers can experience the same debilitating symptoms and even the same associated conditions - POTS, gastro, MCAD etc. (once it's published we'll put a link to it here.)

This has never been officially, scientifically acknowledged before, and this fills me with hope that more and more doctors will take HSD seriously. Far more seriously than 'hypermobility' ever was.

And don't forget that charities like the HMSA are on our side - helping to ensure that all of us are taken seriously. They not only support patients but educate medical professionals too. Being Patron, volunteer and HMSA HSD Ambassador, I've seen first hand how hard they are working to ensure that people with HSD are not seen as 'just a bit bendy'. I'm actually doing a talk as part of an HMSA Hypermobility Masterclass on living with HSD for medical professionals that the HMSA is helping to run in a few weeks time. I will be fighting the corner for everyone else with an HSD too.

What will you be doing next about your own diagnosis?

Carrying on as normal.

Treatment for both hEDS and HSD is symptom-based in the UK. I don't need to see someone to officially get 're-classified'. Re-classification is something that will happen along the way if I need to access services/support/advice through someone who thinks it would be helpful to re-assess me. And actually I can expect to be reclassified at various times during my life as my symptoms change - because it is a spectrum condition which can change over time.

And until I'm reassessed I will either use HSD or JHS - not assessed under the new system yet.

And on a reassessment note, I like that this new system recognises that it is a spectrum that people can move about on. Being G-HSD doesn't mean 'definitely not hEDS' - it means 'currently not showing specific symptoms of hEDS - symptoms may emerge later so keep an eye out'. If I become less symptomatic it might change to Asymptomatic-HSD - or if I get more skin related issues it might change to hEDS. If I get less flexible with age, but haven't developed symptoms that class me as hEDS I can slide along to H-HSD (historic HSD) - whatever my symptoms are, I can have a diagnosis that accurately reflects how I am affected and flags up the potentially linked conditions to look out for. It's so different from the previous system that it's a lot to get my head around and will take me some time to get used to! (We'll link to a summary of the HSD criteria as soon as we can.)

How would you summarise how you feel about the new classification and associated information?

Well...

...a bit of "oh help, it's changing" is going on in my head....actually, maybe quite a lot!

But underneath that, having read a lot of the new medical articles in the journals, the new criteria and all the linked information that has been published along side them means that although I don't think a positive effect will be instantly visible, but I think the future is looking up.

So I am taking a deep breath, ready to ride out any initial period of uncertainty, but full of hope for the future.



Thursday, 23 February 2017

Customer consultation: Keyring card dividers and folders

For some time I've noticed that many of my customers (including me!) have quite a few stickman cards. Sometimes having lots of cards can mean that finding the right card quickly is tricky.

Over the past 5 months I've been looking into various possible solutions and have come up with the following:

A set of 4 polypropylene dividers that is the same size as a card, with a tab - so they will be durable, waterproof, wipe clean, and wont fray or disintergrate at the corners.

The sample ones I made are clear, but the actual dividers will be opaque, and in 4 different colours so it's easier to see them (blue, green, yellow and red). The dividers can also be written on with marker pen.


 And I have also found some sticky 'tabs' which can convert the cards and dividers so they fit into any standard 2 ring ringbinder, and also pocket filofaxes! (If you later want to remove the sticky tabs, and don't want sticky patches on the back of the cards, nail polish remover works well.)

So I now have my set of 20 cards plus the prototype dividers in my old pocket filofax (150 x 130 x 30mm) and I love them!
Photo of my pink pocket filofax with some cards in it, and to the left are a set of 2 tabs and a 'cuppa needed' card.

Photo showing a tab being looped onto the first ring of the pocket filofax. - one is already looped onto the 5th ring. (Make sure the tab is sticky-side up!)

A photo of the 'cuppa needed' card being placed onto the sticky tabs, and stuck down.

A photo of the finished filofax of cards and dividers.

Another photo of the finished filofax of cards and dividers, with the section of cards being flipped over.
Creating the overall effect of having a totally customisable and durable book explaining my normal, which can be used to answer 'whats wrong with you?' questions in both social and medical contexts, and as a way of clearly explaining needs and symptoms.

(Cards not included. Neither is the the file so you would need to get one from elsewhere - there are some really lovely pocket filofaxes around.)

So, would you be interested in a pack of dividers for around £3.50?

And would you be interested in a 'conversion pack' of 50 sticky tabs (enough for 4 dividers and 20 cards) and the 4 dividers for around £8?

Please let us know by commenting on our FB post, on here, or on twitter.

- We won't stock them if no-one wants them, but if lots of people want them, then we will!

Wednesday, 22 February 2017

Coping with the guilt of hidden disability

I was recently asked how I fight the guilt that comes with disability and medical conditions. The guilt from not doing tasks you feel you 'should' have, for not being able to help out or socialise with friends and family in the same way that others can. For not being able to work as much etc.

I think perhaps over time I have learnt not to fight it.

It's more that I let it fade away. I can't control how I feel - but I can control what I do with the feeling.

I recognise that yes, I feel that way sometimes. But I also don't have to believe it, or act as if it were true. And over time, although guilt still floats through my brain sometimes, it does so less and less. And affects me less and less.

You see, if I can't do something without unacceptable side-effects, then I shouldn't be doing it.

And once I've made that decision, instead of looking at what I can't do, I look at what I can do. I can't help set the tables for a party - but I can fold the napkins. I can't run around with the nephews, but I can play monopoly.

I also measure my achievements by what I found hard but managed anyway. Putting something that is challenging for me, today, on my 'To Do' list each day. Not challenging for someone else, not challenging for me on a good day, but challenging for me. Today.

 Having a schedule also helps because then I feel I have more of a purpose to my day, and it makes pacing easier too. Then instead of feeling guilty about what I can't or haven't done, I can see what I have done and celebrate it.

A win-win situation!

No guilt needed.

Tuesday, 21 February 2017

HMSA's Hypermobility Spectrum Disorders Ambassador

I'm really looking forward to getting going with my new role at the HSMA: Hypermobility Spectrum Disorder Ambassador.

Below is the article from the HMSA on my appointment (reproduced from the HMSA website, with permission.)

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HMSA Youth Patron, Hannah Ensor, is to become the HMSA’s Hypermobility Spectrum Disorder* Ambassador.

Hannah has worked closely with the Hypermobility Syndromes Association for a number of years and assists with raising the profile and awareness of Hypermobility Spectrum Disorder; the new name for hypermobility syndromes which are not covered by EDS, OI, Marfan, Stickler and PXE.

Hannah takes part in the HMSA’s self-management programmes and participates in our work with professional members as the ‘patient voice’. She is also well known for promoting the rights and needs of people with disabilities and is spending a second year as one of Britain’s 100 most influential people with a disability.

Hannah regularly fundraises for the charity and has a hugely successful business which has enjoyed global recognition, called Stickman Communications. You can view Hannah’s work on her website, stickmancommunications.co.uk

“I’m honoured to have been offered this exciting opportunity and am looking forward to helping further raise the profile of hypermobility spectrum disorders* – particularly highlighting the fact that the whole spectrum of conditions can range from mild to severe depending on how each individual is affected.

 Over the past few years, as a Patron of the HMSA, I have seen an increased number of people taking hypermobility syndromes of all types more seriously -and I’m excited by the prospect of being able to help build further on this important work, among the general public as well as in the medical and patient communities.”

*Please see our HMSA statement of position on the 2017 International Criteria for Ehlers-Danlos syndromes which explains how our use of terminology has been, is and will be changing.


Friday, 10 February 2017

"What have you done?"

I dread this question.

"What have you done?!" I risk being asked every time I leave the house, and inevitably it is followed by a outpouring of unwanted - and unwarranted - pity for the fact that I'm disabled.

Today as I was leaving my Mum's house, her neighbour arrived home. I've not actually met him before. I was using one crutch - having been able to park within 2 metres of the front door.

We said hello. And then...

"Uh oh, what have you done?" he says.

Me: (Thinks: Oh no. Another 'oh that's so terrible' pity flood to cope with.)
"Actually, it's better than my usual wheels. This is progress!"

Him: "Oh." Grin. "Enjoy it!"

I expected the 'pitiable' assumption, but what I got was the assumption that I could - and would - go and enjoy my day.

Sometimes it's lovely to be wrong.


Wednesday, 18 January 2017

Living with a variable condition - A shopping analogy.

What's it living varying fatigue and pain levels?

It's like trying to shop with a limited budget, when prices can change (usually up, sometimes down) at any time, without notice. Oh, and the budget can change without notice too.


You often have to abandon items or switch items for cheaper/lesser alternatives. The luxuries might be first to go, but even essentials can become priced out of reach. The only other option is to borrow money at extortionate levels of interest.

At that level of interest, borrowing has to be a one off thing for emergencies and special occasions. You'll go bankrupt if you keep borrowing every time the prices go up or budget goes down part way through your day.

The most important skill becomes being able to change plans without warning. Being able to adapt around whatever is happening and to prioritise so that you can a) afford your shopping and b) you keep the right items.

In the context of living with a variable chronic condition or disability, the shopping list becomes a To Do list or schedule, and the energy 'cost' of any activity will vary, as will the energy 'budget' available, due to a range of factors.

These factors will vary between people. Some of mine include: how well I've paced, hydration, temperature, what I've eaten, whether I've been able to exercise regularly recently, hormone levels, amount of sleep/insomnia, needing to do an unexpected task (anything from clearing up a spilled drink, to a journey taking longer than expected), or the body fighting an infection.

Bearing in mind that the 'right items' will also vary (sometimes personal hygiene might be the most important, other times socializing or doing something that helps mental health, other times physical health or exercise might take priority) this process is pretty exhausting and turns a simple shopping trip into an epic lateral thinking, problem solving challenge. It's not just the very limited energy levels that are the problem. It's the variability that makes life a constant balancing act of decisions, and makes every day a step into the unknown.

The overall effect means starting the day with a plan, in the certain knowledge that the plan will need to change. Perhaps cancelling activities, or tweaking activities to reduce their cost (like eating smaller meals - because digesting takes me a lot of energy, or using dry shampoo and baby wipes instead of a shower, or lying down for a conversation.)

These changes of plan are the only way forward - the alternative is to run off adrenaline and then crash out. This might look fine from the outside because the crash tends to happen behind closed doors, when I've relaxed, so others often don't realise the toll it's taken. Crashing out takes far longer to recover from. The more I push, the bigger the crashes and longer the recovery. Trying to keep pushing through my fatigue hospitalized me for 5 weeks. Totally energy bankruptcy. It's taken years to get back to close to where I was pre-crash. I really don't want to repeat that!

But sometimes things can't be abandoned - perhaps I'm out and can't go straight home, or it's an emergency, or a special occasion. Then I need to do what I can to reduce debt. For me this will include: rehydration salts, lying down whenever I can (park benches, quiet corners of shopping malls, the floor of the train, the back seat of a car) eating little and often, accepting help, periods of zoning out and not talking to anyone, and generally pacing. And then taking a day or more afterwards trying to spend as little energy as possible/spend energy on things that will improve long term energy like nutrition,exercise, and pacing. Then the 'spare' budget at the end of the day can go towards paying off the debt - i.e. getting myself to a point I am less symptomatic and more able to function.

At times I've had to reassess and rearrange my whole lifestyle (career, exercise, social life, diet etc) to get things back within budget. It felt horrible, like a failure, but it turned out to be the best thing I could have done, and something I now see as one of my most precious achievements - why? because by having a more flexible lifestyle I've been able to live mostly within budget, and that has triggered a slow increase in my budget over time. Things I thought I'd given up for ever are now becoming possible again.

I rarely (if ever) get my budgeting spot on, but getting it 'more or less right-ish' most of the time, and taking the time to recharge properly if I've gone totally over budget has made a huge difference to my overall energy levels and ability to do things.

The irony is, that the process of making sensible decisions so that I stay within energy budget also takes energy! Thankfully, in my experience, the energy saved far outweighs the energy it costs - and it's got easier over time, although I don't think it will ever stop being a challenge.

A worthwhile and rewarding challenge, but a challenge nonetheless.

Thursday, 12 January 2017

I can get into my house!

As some of you will know, I will be moving house in a few months, from a ground floor flat to a bungalow - which we are currently in the process of completely renovating.

Up 'til now work has been of the 'destructive' or background variety. Starting with removing carpets smelling of cat wee and wallpaper, on to removing fixtures and the odd wall, progressing through chiseling holes in walls for new cables and pipes. Then building the odd new internal wall and putting in plumbing.

And now we are at the exciting stage when new stuff is going in, holes are being filled and a home is just starting to emerge from the building site. Still got weeks and weeks of work, but I had to post today because...the new doors are in!

I've been a bit too flollopy to leave my flat today, so I can't see them, so my builder sent me photos (because he's awesome)
 

Look carefully.

Can you see it?

Can you see the bit that's exciting?

LOW THRESHOLD!

The prospect of not having to step over a huge lip to get into the house, and of being able to wheel in and out once we've sorted ramps is just marvelous.

I will be able to get into and out of every single door in my home! EVERY SINGLE ONE!

Sorry if I seem over excited - especially as I haven't even seen them for real yet, but the prospect of being able wheel out of any door I choose - front, kitchen or rear patio, is really rather marvelous.

I haven't been able to do that since starting using a wheelchair 10 years ago. Many wheelchair users never have this luxury. I feel like I've won the lottery.

Obviously I've known I'd ordered level access - but actually seeing these photos made me realise that it is actually happening.

I will be able to get into and out of any door of my home, safely and easily.

Bliss.

Words can't say just how cool this is. So I shall leave it to your imagination.