What does the change in diagnostic criteria mean for the person living with a hypermobility condition?

Theoretically, the change in the criteria has affected some patients to the point that they no longer meet the criteria for Hypermobile EDS. This has been concerning for a lot of people with hypermobility conditions, and understandably so. They have just had the diagnosis they have been working with for X number of years “taken away from them”, if you will. While the Ehlers-Danlos Society has tried to placate concerned patients by saying their diagnosis won’t be “taken away from them”, in the grand scheme of things, we believe this goes against the whole objective of narrowing down the diagnostic criteria in the first place.

The Consortium has declared that we need to reduce the heterogeneity within the hEDS diagnostic category, and that can only happen if those who are already diagnosed, as well as those diagnosed in the future, are in fact given the correct diagnosis. The Ehlers-Danlos Society (2017) have since stated: “If someone was diagnosed with hEDS before the 2017 criteria, there’s no cause to seek a new diagnosis unless they decide to participate in new research or need to be reassessed for some other reason”. So essentially, if you’re going to participate in research, then you will need to be reassessed before entering any research project. That is at least reassuring that any research done will be done so on the correct population groups.

Typically, those who don’t meet the criteria for hEDS anymore, meet the criteria for one of the Hypermobility Spectrum Disorder subtypes, but this also needs to be assessed. Also bear in the mind that there is the possibility that some people who have been in the hEDS category for some time, may in the future have their diagnosis changed to something new, if the genetic studies & genome sequencing that can now take place, discovers an entirely new category. As I said in my talk at the opening of Genetic Awareness Week 2016 – we live in an exciting time; we are in an era when the genetic cause of our conditions may soon become know. And, that is what we have to hold onto: Hope.

Our approach at Hypermobility Connect is to try to encourage people to see a change of diagnosis in an alternate way. This change in criteria means that people have now received a far more accurate diagnosis. They are still living with a disorder that sits on the Hypermobility Spectrum, which can be just as disabling, painful, and frustrating, it’s just no longer considered to be Hypermobile EDS at this point in time.

In a lot of ways, this change in diagnosis means nothing other than a label change. The management of hEDS and G-HSD is essentially the same. Management is largely symptom and prevention-focused in both conditions.

What we need to be aware of is the idea of “downgrading” which seems prevalent in the EDS/Hypermobility Community. There is a belief that hEDS is “worse” than HSD. And because patients have often struggled to have their condition recognised and their pain validated, many (not all!) have a tendency to want the diagnosis that “sounds the worst” and therefore attracts more empathy & sympathy from others, especially medical professionals. They feel that after years of being ignored, a label like Ehlers-Danlos Syndrome may actually get them the help they need. This is where education has to come in to play, for both professionals & patients. A correct diagnosis is always better than the most severe sounding, but we have a long way to go in helping people to see it this way. It’s a big change.

We know that Hypermobility Connect needs to play a part in educating health professionals so that they recognise both hEDS and Hypermobility Spectrum Disorders for what they are – often disabling, painful and fatigue-causing conditions. We know that the Quality of Life of people with rare conditions like the Ehlers-Danlos Syndromes is much higher than the general population, and even those with more commonly identified conditions like heart disease, diabetes & arthritis (Oregon State University, 2017). We believe with education of both professionals and patients we can help to increase the QoL of people living with hypermobility conditions of all kinds. Just as hypermobility doesn’t discriminate, nor do we – we aim to educate professionals and patients about all the hypermobility-causing conditions, including Loeys-Dietz Syndrome, Marfan Syndrome, Osteogenesis Imperfecta and the other even less known conditions.