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.