Living with EDS (Hypermobile EDS)

A note on the reclassification of hypermobility type

There has recently been a re-classification of hypermobility so that there is now a spectrum of disorders, ranging from ‘simple hypermobility’ which does not present with symptoms through to hEDS. If you were diagnosed prior to the re-classification, as I was, you keep that diagnosis. Whether I would be diagnosed now with hEDS or with one of the conditions in between I don’t know, but to keep it simple and inclusive to all with hypermobility I will mostly refer to ‘hypermobility disorder’ in this post. The Ehlers-Danlos Support UK website has more resources on the new classifications.

1: Doctors don’t look for zebra

It was 2014 when hypermobility syndrome was first raised as a possibility, and 2016 when I became an official medical zebra. I was a zebra for years before, my whole life probably, but didn’t know it. Perhaps the reason I went for so long being a horse not a zebra was that most doctors aren’t taught to look for our stripes . . .

Confusing you? Well, the zebra is the symbol of EDS. The reason it is the symbol, as my hypermobility physiotherapist explained to me, is that doctors are taught the phrase ‘when you hear hooves, think horses not zebras’ during their training. The term ‘zebra’ is used to reference a rare disease or condition. Doctors are taught to think of common conditions for a set of symptoms, ‘horses’ and not ‘zebra’, so that people aren’t misdiagnosed with a rare condition.

Of course, this works fine for the majority of people – the horses of the population – but is far from ideal for those of us with rare conditions such as EDS – the zebras of the population. Given the focus on horses, it is unsurprising that it often takes zebras many years to have their conditions diagnosed, and often see a large number of doctors before they get to the right specialist. Of course, this means delayed testing, and treatment if a condition is diagnosed.

2: Being a zebra means being invisible

Well, not literally, but being a zebra often means living with an invisible condition. Nobody can see joint pain, tachycardia, bladder issues or any of the other typical symptoms that come along with hypermobility disorder.

One of the issues a lot of people with hypermobility discuss is the difficulty of prolonged standing, for example. For some, using a wheelchair is the best or necessary option.

Others, such as myself, can stand but find it painful to do so if it is for a long period of time. But you can't see the pain in my hips, although you may notice me shifting my weight around from foot to foot. For those traveling in London the 'please offer me a seat' badge is a good initiative that you may find helpful.

3: hEDS often runs in families

Hypermobility EDS is a genetic connective tissue disorder. As EDS UK describes, hEDS can be ‘inherited from a parent with the same faulty gene, alternatively someone can be born with a new mutation so that the condition is occurring in their family for the first time’.

I was born into a family with a pre-disposition to hypermobility, yet neither I nor any of my affected family members knew anything about this until I was diagnosed about five years ago. That may sound strange – how can you not know that you are hypermobile? Well, we did know, we just didn’t know that it could be a medical issue that caused symptoms and required a degree of treatment.

For as long as I can remember, my mum, myself and my sister have been ‘flexible’. We can all touch our toes with ease and my sister can do party tricks with her arms - tricks that are actually far more impressive than mine. In an age where being flexible is sought-after and yoga poses and stretches are practiced to increase flexibility, it seemed for a long time that we had actually won that particular health lottery.

Neither my mum or my sister are symptomatic with their hypermobility, aside from having a few more headaches than is probably the norm. It was only when I went through the medical journey of being diagnosed with POTS / dysautonomia, fibromyalgia and chronic migraine (and later MCAS) that hypermobility EDS was suggested by a doctor due to my symptoms, and hypermobility came to be understood by my family as a medical issue.

4: Hypermobility isn’t just about being bendy

While the Beighton test, a test that is typically used to contribute to the diagnosis of hypermobility, involves rating the hypermobility of the joints, the condition can go well beyond this. Collagen is found throughout the body, and is sometimes described as the ‘glue’ that holds us together. If collagen is weaker than it should be, ‘the tissues in the body will be fragile, which can make ligaments and joints loose and stretchy’ (NHS).

Beyond the joints, other areas of the body can be affected by the change in collagen and 'stretchy' tissues. As the NHS notes, people with hEDS can have problems with their bladder, and it is believed that my bladder issues (interstitial cystitis / bladder pain syndrome) is a consequence, at least in part, of my hypermobility.

Fatigue, of which I suffer daily, is a common problem with hypermobility (The Ehlers-Danlos Society).

Skin issues can also take a long time to heal. Injections, surgical procedures or just a cut from picking up something sharp by mistake - they will all leave a wound that will take a long time to heal, and maybe even a scar that will take years to fade. Hypermobility disorder often brings with it more fragile skin that takes longer to heal. I'm still waiting for some scars to fade from injections in my abdomen that took place about 8 months ago . . . For more information on the symptoms of hypermobility have a look at EDS UK website, which has a lot of helpful resources.

5: You may need a LOT of local anaesthetic when you have a dental (or other) procedure

Many years ago I had to have a tooth removed. A fairly common procedure. Except the local anaesthetic did nothing. The dentist kept giving me more and more injections to numb the area, but I could feel everything. She looked more and more perplexed, and then weirdly suspicious.

Little did I know, back then, that I had hEDS and an insufficient response to local anaesthetic. It took 9 injections to numb my gum! The reason for this poor response to local (not general) anaesthetic is not known, but a study found that nearly 60% of individuals with hypermobility had this experience.

6: Symptoms can wax and wane

Painful joints is one of the common symptoms of living with EDS (NHS UK), and is the reason I feel like I have the hips of someone at least twice my age. In fact, my mum has no hip pain at all, while I often hobble around after exercise or sometimes just a simple walk saying ‘owwww’ quite a lot. Symptoms aren’t static though, they wax and wane. One day is pretty fine and I can go for a run (on a good day), the next, for no apparent reason, I just want to curl up with a hot water bottle and watch a lot of rubbish TV.

7: hEDS, POTS and MCAS are often associated conditions

The conditions hEDS, postural orthostatic tachycardia syndrome (POTS) and mast cell activation syndrome (MCAS) are associated conditions, and so there is a suggested link between them. Over the years I have been diagnosed with both these conditions, and then 'un-diagnosed' with POTS, although I still am diagnosed with autonomic dysfunction / inappropriate sinus tachycardia. Living with these conditions together is tough, as I have written about before in the post living with MCAS.

8: Exercise and physiotherapy are key treatments

Symptoms of EDS can be helped with exercise and physiotherapy. Last year I attended a talk given by Jason Parry, a physiotherapist that specialises in hypermobility, which was incredibly useful.

I found it to be really encouraging to hear his positive stance on exercise and the benefits it can bring.

I was very fortunate to see a (different) physiotherapist on a private basis some years ago who was very well-versed on the condition, and actually does a lot of talks for EDS UK. We put together a programme of exercise that would help with the joint symptoms while also taking account of my POTS symptoms.

Initially I used a self-seated pedal exerciser at home (sort of like using an exercise bike but you sit on your own chair), gradually building up time and resistance. This got my body used to moving in a safe and low intensity way, without standing. Then I moved on to walking more, and adding in light resistance moves as well. This programme was personal to me, but I would highly recommend trying to see a physiotherapist, although as point 10 sets out, this can be difficult . . .

9: Exercise gains are hard won, and easily lost

So, you have started exercising and feel that it is going well, but the 'gains' (especially in terms of muscle strength) seem to be minor or even non-existent. Then you have a flare-up, don't exercise for a week or two, and the little gain you did make vanishes into thin air . . .

As Jason Parry spoke of at the talk on hypermobility and exercise I attended, it can take a person with hypermobility twice as long, or even more, to gain muscle strength than someone without the condition. Frustrating as can be, I know. And then any gains are lost quickly. As he said, hypermobility exercise is a long-game - you need patience!

10: It can be challenging to access treatment

It is the case, as with many rare conditions, that getting diagnosed and accessing treatment can be, let's say, a challenge. While there are some amazing primary care doctors out there that will pick up on symptoms and make the necessary referrals to a specialist, this isn't the experience of all patients.

Awareness of rare conditions is lacking, amongst doctors as well as the general population. I was actually referred for assessment by my cardiologist years ago, but am currently in the process of trying to access physiotherapy again, the referral for which has already been rejected twice. I wish I could offer some advice on this other than try to be your own advocate and access resources such as those offered by organisations such as EDS UK to take to your doctor.

As EDS UK helpfully describes, no two zebras have the same stripes. We are all different in our symptoms, types and experiences. What are your experiences of living as a zebra with hypermobility disorder or hEDS? Comment below with anything I have missed!

Helpful resources

Websites:

Ehlers-Danlos Support UK

POTS UK

Mast Cell Action

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