Doing Ourselves A Disservice?


Most people with Ehlers-Danlos Syndrome (EDS)/Hypermobility Spectrum Disorder (HSD) have told medical professionals ‘I have a rare connective tissue disorder’. The EDS world adopted the zebra as its mascot due to a saying we’re told medical students hear while studying (‘If you hear hooves, think horses, not zebras’). 

Horses are ten a penny.  Zebras are not. In other words, look for common conditions, not rare ones, and EDS is rare, right? Not according to one internationally respected EDS specialist and his fellow authors. And, if this invisible illness isn’t rare, the question is are we doing ourselves a disservice when we say it is?

Written in 2014 by a group of known experts including Professors Grahame and Aziz, and Dr Hakim, the study The 2016 paper called A Prospective Evaluation of Undiagnosed Joint Hypermobility Syndrome in Patients With Gastrointestinal Symptoms, (Grahame, Hakim, Aziz, et al.) opens with a short paragraph introducing Ehlers-Danlos. In it is:
 ‘The Joint Hypermobility Syndrome (JHS*) is a common connective tissue disorder
characterized by joint hyperflexibility, Dysautonomia and chronic pain.'
[*see new diagnostic criteria 2017 for HSD details]
It's important to keep in mind that when asked about the two terms, Professor Grahame said EDS and Joint Hypermobility Syndrome (JHS) are the same thing, and that the JHS term will one day cease to be used. In the study, the experts use the two terms interchangeably. [UPDATE: NEW CRITERIA INTRODUCED MARCH 2017 - NEW CATEGORY CALLED HYPERMOBILITY SPECTRUM DISORDERS (HSD]

In Europe, states a medical condition must affect no more than 1 in 10,000 people to be classified as rare. However, EURORDIS,   the 'Big Daddy' of rare disease this side of the pond, says no more than 1:2000 is the ceiling.  Down Under, The Australian Therapeutic Goods Authority use the same figure. In America, the National Institute of Health (NIH) say the limit sits at no more than 200,000 people in the entire US population (of 318.9 million) being affected, while it’s 50,000 in the UK. That’s confusingly variable.
In the 1990s, it was said that 1:10,000 people would have EDS. Today, the figures have changed significantly. The EDS Society in the USA say as many as 1 in 2500 people will have some form of the condition, though some subtypes (like the Kyphoscoliotic Type, where your chances of having it are CURRENTLY hundreds of millions to one - #ysu thinks it's rare now due to few recorded cases being identified, more must be out there) are extraordinarily rare – or rarely diagnosed and documented. 

This side of the Atlantic, EDSUK say ‘The exact incidence is not known, but is estimated at 1 in 5000, however, it may be more common’. 

Whichever way you look at it, 1:2500 or 1:5000 does not satisfy the necessary scarcity of cases for an illness to be classified as rare. And the varying statistics mean someone is wrong about how many of us have it...

What's the harm?

Improved awareness has increased diagnosis rates, and as more medical papers are published about EDS, the statistics will continue to change and it will become more and more common, just as it has done over the last couple of decades. So, what’s the harm in using the word ‘rare’ when it comes to Medicine?

Picture the scene…You have Ehlers-Danlos/Hypermobility Spectrum Disorder (EDS/HSD). You've seen doctor after doctor and have a string of unpleasant experiences behind you. You walk in to the office of a new physician and say ‘I have a rare connective tissue disorder called Ehlers-Danlos'. Before anything else happens, a few things already have

Unless professionals are confident in their knowledge of EDS/HSD, and if they're not of humble character, using ‘rare’ immediately puts a medical team on uncertain ground and under pressure to perform. This is fine if they’re open to a quick Google (which is all it takes) and to reading, but it can be disastrously bad if they’re running on insecurity and ego instead. Medics who feel their expertise is inadequate or under question often refuse to listen, research and provide what’s needed. In this circumstance, EDS/HSD patients are all too often invalidated and dismissed as exaggerating their symptoms at best, or a fabricated Illness case at worst. Either way, the medicine field marks them as liars, and their care suffers or even grounds to a halt.

When conditions are uncommon, expectations are lower. The patient can’t expect staff to know how to treat and manage them, or even how to carry out the correct investigations. As soon as medical teams hear ‘rare’, they’re aware that the usual investigative and treatment protocols are probably out of the window (not to mention the budget) and adaptions need to be made. NHS Health Trusts in the UK and insurance companies around the world argue over agreeing to fund appointments and treatments. Why?  Because in their eyes, rare = unknown, unresearched, unproven - and pricey.
Conversely, with common conditions, expectations regarding tests and treatment is pretty standard – staff are confident with their competence, standard procedures exist, and how patients’ bodies will respond is predictable. There's a firm footing from which to work. 

When something is common and understood, the attending medical team had better get on top of things as a failure to do so would be unacceptable. 

Language is powerful
Using one word instead 
of  another can  improve 
life for those with 
EDS/HSD.  It’s time to stop calling them rare.
With a rare condition, failures become 'understandable' because, to the uneducated, the illness is not understood.  Tell a medical professional Ehlers-Danlos/HSD are common, and you put yourself in a position where their lack of knowledge becomes a gap in knowledge, and official guidelines and laws exist stating that medics must fill them.  

Language is a powerful thing. Using one word instead of another can vastly impact situations and outcomes that could drastically improve life for those suffering with EDS/HSD. 

It’s time to leave out the adjective and stop calling Ehlers-Danlos/HSD rare. 

Written by #ysuWords.
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