You know how hard it can be when people use a different name for the same thing? Imagine how difficult that is when there are interchangeable names for your medical condition! Something I’ve been thinking about a lot is how the terms Ehlers-Dalos syndrome (EDS) & joint hypermobility syndrome (JHS) are used in this way. It’s time for me to share my thoughts on the EDS vs. JHS debate, as I’ve not seen some of my musings being talked about before.
*Please Note: The new nosology, released in 2017 has identified some of the points below as issues and has put things in place to tackle them. You can read more about this on The Ehlers-Danlos Society website. As of the new nosology, the term joint hypermobility syndrome has been replaced with hypermobility spectrum disorder (HSD) and the criteria for hypermobile Ehlers-Danlos syndrome has been tightened.
I am a volunteer with the Hypermobility Syndrome Association. You can find out more about the work of the Hypermobility Syndrome Association & the conditions they support at hypermobility.org.
It’s worth starting this by talking about the different names that have been used recently to describe these conditions.
Hypermobile Ehlers-Dalos syndrome: Previously known as Ehlers-Dalos hypermobility type & Ehlers-Dalos syndrome type 3.
Joint hypermobility syndrome: Previously known as benign joint hypermobility syndrome.
Hypermobility syndrome: A name that can be used for both EDS & JHS. This term is now used as an umbrella term for a number of conditions that have hypermobility as a main factor. These include conditions like EDS, JHS, Marfan syndrome & Osteogenesis Imperfecta (brittle bone disease).
There are a number of different types of EDS, which you can read more about on my Ehlers-Dalos syndrome page. When we are talking about EDS vs. JHS, we are only referring to Hypermobile Ehlers-Dalos syndrome (hEDS). Classical, Vascular and all the other types of EDS, are not comparable to JHS because they have additional (more sinister) features that are not found in JHS.
Something important to note before reading my thoughts on EDS vs. JHS is that JHS can be genetic, or can be caused by other factors. These factors can include the shape of the bones that make up the joints, deconditioning, poor proprioception. However, it’s worth noting that deconditioning & poor proprioception can be an effect of JHS. So in this post, we’re going to refer to genetic JHS & non-genetic JHS to distinguish between the two & allow me to make my point more effectively.
EDS is a genetic condition. The gene involved hasn’t yet been identified, so it’s totally possible that genetic JHS & hEDS are the same thing. Until this gene is found, we won’t know for sure either way. The non-genetic type of JHS can’t be the same as hEDS (as this is genetic).
hEDS & JHS are considered clinically indistinguishable. You will often find that people who have internal co-morbid (often found together) conditions (like PoTS or gastroparesis) or suffer with severe dislocations or subuxations (partial dislocations), will receive a hEDS diagnosis. While those who’s main feature is joint pain will receive a JHS. In practice though, this often doesn’t happen. It very much depends on the clinician you see.
Ok, so now we’ve discussed the basics, here’s my take of the EDS vs. JHS debate.
So my thinking with the EDS vs. JHS debate is that EDS & JHS may be the same thing when talking about the genetic form of JHS. They can’t be the same when talking about the non-genetic form of JHS, because EDS is genetic. So EDS & non-genetic JHS are not the same thing, but EDS & genetic JHS may well be. I think a lot of the time when people say that EDS & JHS are the same thing, they forget that some people have non-genetic JHS.
So where does that leave those of us with one of these diagnosis? Thankfully, the management options for EDS & JHS are the same, whether the JHS is genetic or not. Unfortunately, having so many names that are often used interchangeable can mean that it is hard to be taken seriously.
When I was diagnosed, the consultant used hEDS & JHS in my appointment interchangeably. However, on her clinic letter, she used the outdated term ‘Hypermobility syndrome’. This has lead to other doctors dismissing my hEDS & not taking my ‘hypermobility syndrome’ diagnosis seriously, even though the three names are often used interchangeably.
Another impact the EDS vs. JHS debate has had is on statistics. Until recently, hEDS was thought of as a rare condition, when in fact it was just rarely diagnosed. Imagine what would happen to the hEDS statistics if you add all the people who have diagnosis of genetic JHS or hypermobility syndrome!?! I know this isn’t possible until it has been established for certain whether hEDS & genetic JHS are the same thing, but there is the potential for the hEDS statistics to rise massively & if we are using the terms interchangeably, this needs reflecting in some way by the statistics of the condition.
On top of all the confusion & recent changes it the terminology used, there is another on the horizon. This change is currently being discussed & a decision is expected in the first half of next year. We won’t know whether this will address the EDS vs. JHS debate or not.
So how do I feel when doctors use EDS & JHS interchangeably?
I’m ok with it, though I know not everyone feels the same. As hEDS & JHS receive the same management & are widely used to be the same thing, for me there really is no difference as to which a doctor uses. If I’m honest, I’m still at the stage where I’m relieved that I’m not being told that I’m imagining my symptoms! As there is currently no way to know whether they are the same of different, using either to describe me could be entirely accurate. So as long as the doctors I see for my co-morbid conditions acknowledge that I have HEDS/JHS & I get to access the treatment I need, I don’t see it as a problem.
What’s your take on the EDS vs. JHS debate? Were you aware that JHS can be caused by non-genetic factors?
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yep my letter is benign hyper mobility syndrome. I have taken to calling it EDS-HT, because that is the clinical name and seems to be what everyone is thinking now.
That’s really interesting. I find doctors take me more seriously when I say HEDS (they’ve changed the name again!). The thing that really surprised me was that my gastro used JHS rather than EDS. As I understand it, you’re more likely to have EDS used if you also have internal, organ interplay. So I was expecting her to use EDS. Xx
Honestly, I’m not too knowledgeable on the subject but I think what you said could apply to a wider range of conditions in general. At the end of the day, like you said, as long as your condition is managed in the right way by the doctors, it’s all that matters, really. <3 :)
Kay
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Yes, I completely agree! ME/CFS is one that springs to mind. There’s also at least one other name for CRPS too. Mears-Irlen Syndrome has a number of different names too – Scotopic Sensitivity syndrome & pattern glare. It makes it very difficult when talking to medical professionals, as they all have their own preference with the name they use. Some can get very funny if you use a different name. Out of EDS/JHS/hypermobility syndrome, I use EDS. I’ve had problems being taken seriously when I’ve used hypermobility syndrome, though I don’t know why as it’s acknowledged to be the same thing! Xx
From what I’ve heard from others with EDS or JHS here in the Netherlands, EDS is taken more seriously. You will get a mobility aid easier, or an allowance because of the inability to work. It shouldn’t be that way, but apparently it is. To me it’s the same, but it would be a lot easier if it was just one name.
From my own experience & those of friends, it’s very similar in the UK too. Though I do get more doctors not believing me when I use EDS, I do get taken more seriously (once I convince the disbelievers that the diagnosis is not wrong!). It really shouldn’t be this way. I really hope that any future name changes address the HEDS/JHS issue so that it’s easier to work with medical professionals. Xx
It’s so difficult when the terms are used to mean the same thing, when you pointed out a genetic type is different and often comes with other issues like GP and POTS.. Personally from my POV I prefer medical terminology to be used correctly as it can interfere with access to medical treatment xx
I’m the same, I prefer using medical terminology too. It’s hard though with EDS?JHS, as both are considered medical terminology. I like to use drugs chemical names too, as I find using brand names can cause confusion. Saying that though, I’ve had a few medical professionals who don’t know the chemical names (worrying!) but prefer the brand names. This makes no sense to me, as there are often more than one brand producing the same drug. Maybe it’s just paramedics, who deal with people who prefer brand names. It certainly doesn’t help to get the best care. Xx
My daughter has HEDS too, so for me it’s important to stress the genetic factors and for her to get the best treatment from the start, which is something that still isn’t happening for me tbh x
I completely agree! One of the big differences between genetic & non-genetic JHS, is that genetic carries the possibility for all sorts of other issues of the connective tissue, such as gastro issues, heart problems & bowl & bladder difficulties. If people don’t understand that, it becomes a lot harder to get other symptoms diagnosed & the correct help for them. I’m so sorry that you still aren’t getting the best treatment. This is such a common thing for use unfortunately. Hopefully the new names will reflect that genetic JHS & HEDS are the same (or at least clarify why they are different!) so that we are all working from one name to make treatment & management more successful. Xx
Given the paucity of literature on ANYTHING hypermobility-related, I’m curious about what (if any) you’ve found on non-genetic JHS? We haven’t found the gene(s) for EDS-HT yet, so it seems tough to definitively rule out seemingly non-genetic JHS as simply a different variant, possibly with a different inheritance pattern. If it is a different variant, that could also explain the different propensity toward comorbidities like POTS and gastroparesis: a different mechanism for hypermobility
That said, there is certainly benign hypermobility (such as exhibited by gymnasts and some dancers, not to be confused with Benign Hypermobility Syndrome, the worst name for EDS-HT EVER) and what I’ll call “post-gymnast syndrome,” where those people have orthopedic issues later in life secondary to their careers in hypermobile sports and activities. But it’s difficult to say that even this is non-genetic, as the capacity for that level of acquired flexibility has genetic factors itself. Any degree of multi-joint hypermobility causing enough problems to merit a JHS diagnosis seems to suggest an underlying structural difference in the connective tissue, and that structural makeup points to either a genetic or epigenetic phenomenon.
Thoughts?
Before I started volunteering with the HMSA, I thought that EDS & JHS were genetic. While I haven’t been able to find any literature, it is currently the opinion of the medical board of the charity that non-genetic JHS is a ‘thing’. We as volunteers have members asking us about the causes of JHS because they have been told that theirs is not genetic in nature by their consultants. We are encouraged to explain the other factors that can contribute to JHS.
I believe that there is currently no literature on non-genetic JHS because it’s a new theory. Certainly the HMSA don’t publish information until it has been through the rigorous Information Standards checks, which can take a number of years.
As you say & I think I touched on too, until the HEDS gene is identified, we won’t know if HEDS & JHS are the same thing. If they aren’t, it will then take researchers to find the JHS gene to determine if all JHS is genetic or if there is also a non-genetic variant. As you say, genetics affects structure so it may well be that genetics are responsible for the shape of joints, one of the possible causes of the suspected non-genetic JHS.
One possibility, assuming JHS is purely genetic, is that it has occurred as a sporadic genetic mutation in those who don’t have a family history of JHS.
As we have benign hypermobility, I think it’s feasible that there is a non-genetic form of JHS. Along the same lines of ‘post-gymnast syndrome’ but where normal life, rather than careers in hypermobile activities, trigger symptoms. It could be that these people’s bodies have a lower tolerance level to activity. If this is the case & this is just one of many possibilities, then the reason for a lower tolerance level would need to be investigated. But again, the reason could be genetic. I think everyone is going to go round in circles with EDS/JHS/non-genetic JHS until the genetic mutation is discovered. Xx