Readers are advised that previous articles that were available and entitled “Hypermobility and Illness” and “JHS – an update for Clinicians” have been removed from the website. These have been replaced with an new article entitled “Hypermobility Disorders – An Update for Clinicians” that reflects changes in the terminology, the classification of Hypermobile EDS, and the most up to date literature on the subject.
The article is available on our Help and Advice page. You can go straight to it by clicking HERE .
Dr Alan Hakim. Chief Medical Advisor and Trustee, HMSA. March 16th, 2017
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?
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. So once it’s published we’ll put a link to it here [Edited to add: An update for clinicians].
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!
How would you summarise how you feel about the new classification and associated information?
…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, 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.
Please see attached document, reproduced below the link. To print please right click on the link and ‘save file as’ We will make this available in other formats as soon as possible, thanks for your continued patience.
15th March 2017
Dear Health Professional,
Your patient has brought this letter to you following changes published today, to the classification of Ehlers-Danlos syndromes and to the Joint Hypermobility syndrome diagnosis. Many of the people affected by these changes are understandably worried about the future impact of these changes on their care and treatment. The HMSA has updated our website on information which may be of help to you and your patient.
The diagnostic criteria for hypermobile Ehlers-Danlos syndrome (hEDS, previously known as Ehlers-Danlos hypermobility) have changed significantly, details can be found on our website. In addition ‘Joint Hypermobility syndrome’ is now obsolete having been replaced by the diagnostic term, Hypermobility Spectrum Disorder. You can find details of these changes, including an article called ‘Hypermobility Disorders – An update for clinicians’, on the HMSA website, hypermobility.org
The HMSA has been working with people with Hypermobility Spectrum Disorders for 25 years and is best placed to share its considerable experience and education opportunities. A few years ago, at the request of members, we agreed to bring all the Ehlers-Danlos syndromes, Marfan syndrome, Osteogenesis Imperfecta, Stickler syndrome and PXE under our umbrella to promote ‘living well’ with these conditions by providing self-management courses and information. We also offer a helpline, social media closed support groups and a patient membership.
The HMSA can offer assistance to medical, health, social and educational professionals in providing information or support to practitioners. We also offer a professional membership scheme which has proven to be very successful and run bespoke HMSA Hypermobility Masterclasses for clinicians. If you would like more information please contact us by emailing firstname.lastname@example.org
Donna Wicks (CEO)
We will be discussing the finer details and come back to you with any further comments in due course.
In the meantime, please know that the HMSA has been working with HSD for 25 years and believes we are well placed to continue to offer our experience to the HSD community. We will continue to lobby for earlier diagnosis, treatment and management.
With regard to the changes to the nosology for the EDS syndromes, the HMSA is pleased that recognition has finally been given in the influence of hypermobility.
The HMSA has been an umbrella group for many years for people with HSD, EDS, Marfan, Stickler, OI and PXE because of the lack of support for hypermobility issues.
We are very passionate about our work to assist people in living with their complex and multi-systemic conditions and in educating the professionals that members are likely to meet in their journey, through the health, social and education services.
We are already directing and assisting with a care pathway in the UK and have developed an HMSA Hypermobility Professional Masterclass, which is run regularly. Currently we are developing a new model of work in Kent, which involves building up a network of professionals, who are trained by us and our medical advisors. This is on top of us trialling a new HMSA Self-management Programme in conjunction with local services.
The services we supply enable us to support everyone because these services are devised by real representatives of people with the hypermobility disorders or syndromes. You, whether a full HMSA member or a member of our wider community, are what matters! It’s your needs we aim to meet and support.
There ‘really’ is room for everyone under our umbrella!
Hypermobility Syndromes Awareness week is drawing to a close, we’ve learned loads from you guys and hope we’ve been helpful to you!
A significant part of the HMSA’s work is in educating professionals, on the impact of living with one of the hypermobility syndromes it supports. The main reasons for this include the facts that many of our members have such poor experiences in using the services they are being referred to. A snapshot Patient Experience Survey, of HMSA members, was taken a few years back that indicated that 55% of members take 10 years plus to gain a diagnosis of Joint Hypermobility syndrome, now known as part of the Hypermobility Spectrum Disorders. (This survey was undertaken before the HMSA brought all Ehlers-Danlos Syndromes, Marfan syndrome, Pseudoxanthoma Elasticum, Osteogenesis Imperfecta and Stickler Syndromes under its umbrella.)
The survey also showed that the members who were able to see professionals who were ‘hypermobility experts’ or involved in ‘hypermobility services’ had much better experiences. This included physiotherapy, rheumatology etc.
It was also clear via our work at that time, and in discussions with professionals, that most professionals did not receive any training on hypermobility syndrome when they were qualifying. Some professionals received training post-grad but this was limited. Of those who did, training usually looked at the ‘normality of hypermobility’ and not the ‘syndrome’ aspect that many experience.
The HMSA commenced working closely with the services allocated to hypermobility (‘hypermobility clinics’) to feedback the experiences of patients using them in 2008. This was received very positively by the service professionals and of course, an element of this work continues today.
We now work very differently and in recognition that not all members can travel hundreds of miles to see a recognised expert in hypermobility or attend a clinic. Additionally, it is in fact, the responsibility of all geographic areas to supply’ fit for purpose’ services, which meet the needs of the people in their catchment area. Some of our work has been delayed by the changes to the NHS commissioning and provision of services but we are now starting to work more closely on a commissioning level.
The HMSA feels it is important to ask service providers to take responsibility for the education of professionals working in services where they are likely to meet people with a hypermobility associated condition. This in reality is ALL services; including neurology, orthopaedic, gynaecology, gastroenterology, maxofacial etc. The more obvious ones include; physio, pain management, OT, rheumatology, genetics etc.
To assist with this the HMSA can now set up a HMSA Professional Masterclass in areas where we are asked. The funding of these needs to come from the service providers, or the delegates themselves, as we do not have ‘spare’ funding but this has not been a barrier in our experience.
To complement all of this the HMSA is also currently trialling a new HMSA model in Kent. This model includes the HMSA educating professionals, and is backed up by the work that the HMSA does with their patients. We have been able to run several Masterclasses to enable the education of the professionals in that area. This work has also allowed us to work on building a ‘care network’, which our Chief Medical Advisor, Dr Alan Hakim, explained to the professionals during his presentations. Part of the HMSA Masterclasses include, telling professionals what the HMSA has to offer for their patients. For example; most of our information is free of charge on our website, and the Helpline and open Facebook pages are there to support everyone. We also offer more individualised services and a membership scheme, which their patients can also access.
The HMSA has exciting additional plans which will directly assist Kent patients with a hypermobility syndrome. These will be announced as soon as our plans have been finalised! In addition, there is a lot of ‘behind the scenes’ work going on to use what has been learnt from Kent to help people throughout the UK and benefiting our global members too.
Once we have finished assisting Kent and reviewed the outcomes, we will be looking at running other programmes throughout the UK. The programmes are not quick fixes, they take a lot of organisation and professionals need to be able to free up time from seeing patients, to actually go and attend the Masterclasses. Obviously, people with a hypermobility associated syndrome will instantly benefit from seeing professionals who have had training. But it is probably more realistic to see this as a long term model, with several smaller goals achieved along the way.
A new snapshot survey will be undertaken in the next few months to assist us with setting objectives for the next few years. So please keep a look out for it to be announced in the e-news and in closed FB groups.
What we need to do now is to build up a database of professionals and services recommended to the HMSA because of a ‘positive experience’ by users. So if you have had a good experience in attending any of the services, regardless of department, then please do let us know. We are also very interested in hearing what GPs are known to be supportive and who understand about the conditions we support.
You can send us the details to email@example.com or use our social media*
We will collate this information;
- To give to people who need a GP or other professional or service in a specific area (something we are asked on a daily basis)**
- To give feedback to services
- To target for HMSA Professional Masterclasses
*Please note; any negative comments naming professionals directly on our social media will be removed. This isn’t because we don’t care. We do! But to be fair, any professional who is named as providing a poor service should be able to offer a defence, rather than being found automatically guilty! We hope that you will support this.
** Please note; With NHS services it is often the department that your GP needs to send a referral to and not a named professional.