EDS sufferers are disbelieved for years. When will it end?

After the tragic news EDS campaigner and patient advociate Stephanie Aston passed away following a misdiagnosis of ill mental health, I wrote for Mamamia on the abysmal disbelief and inequality to medical care patients like Stephanie and I suffer. Hypermobile EDS is NOT rare but medics do not know about it enough leading to decades of difficulty, pain, injury, disbelief and blocks to appropriate medica care.

Read the article on Mamamia, or stay on this current webpage and scroll down for screen-reader friendly text.

By Punteha van Terheyden
Opening my Instagram last week, the first face to greet me was that of Steph Aston.

I was familiar with the New Zealander’s worthy work for better healthcare for people like me with Ehlers-Danlos Syndrome, a genetic connective tissue disorder she suffered from too.

The headline read ‘woman dies after doctors wrongly misdiagnosed her with mental health issues.’ Or something to that horrifying effect.

Some people might question how on Earth that’s possible, but as someone with EDS type 3 (that’s the hypermobile form of 13 different subtypes), it’s a frightening reality our community are at risk of.

Patients suffer dislocations, soft tissue injuries, migraines, joint pain, easy bruising, gastric symptoms, hypermobility and much more.

In an old video interview now doing the rounds on social media, Steph told in her own words how her medical issues were so complex, doctors believed she must be making it up. Many of us with EDS have had a taste of that agonising disbelief.

For years, doctors dismissed my pain and injuries. They put me on antidepressants and referred me for talking therapy. Because they couldn’t connect the dots, they thought my pain was ‘psychosomatic’ and asked about any stress I was under. As if stress could make me unable to put weight on my knee. 

How wrong they were. For years, my pelvic joints dislocated for simply standing up, or sitting cross legged. My knee ‘wore out’ from a year of driving a manual car. The tendon in my wrist bulged and needed surgical repair after I wrote a long essay in an exam. My jaw popped out of joint when I ate.

Steph Aston‘s misdiagnosis of ill mental health led to her being accused, she said in interviews before her death, of self-harm, eating disorders, faking fevers, coughing fits and fainting spells.

The reality was the anaemia she was suffering was of course making her feel extremely unwell. EDS is a complex condition and depending on the subtype, symptoms can vary wildly. It doesn’t make any of them less valid. 

Sadly, the ignorance Aston faced is common for EDS patients.

Throughout my pregnancy, I was told l needed to buck up and deal with its ‘normal’ aches and pains. I’d suffered with severe endometriosis - one of the the top 15 most painful illnesses - and I knew pain.

If I was complaining of pain it was really bad. Despite medics not believing me, i learnt months later the cartilage in my left hip had torn and partially detached, causing burning pain as it caught, pulled and ripped inside my damaged hip joint. 

Even after my daughter’s birth, my GP balked when I asked to see a specialist about my hip and laughed at me. It took two surgeries to fix the damage in my hip and seven years later, I’m still dealing with chronic inflammation of the tissues around it.

Nothing about my EDS injury was normal. Just as I know all the symptoms Steph complained of were not minor or made up. They were very likely debilitating and all-consuming for this poor woman who was failed so miserably.

And yet it is frighteningly easy for medics to pop-poo the pain and suffering of EDS patients because it is unbelievable, in their minds, that a human being could be walking around and functioning on so much pain and injury, therefore they assume we must be unhinged and lying. 

Ironically, I found out yesterday the severe  neck pain I’ve suffered since a car crashed into the back of mine a year ago, is FIVE bulged discs in my cervical spine. I cried a lot yesterday when I found out the extent of the damage I’ve been living with. No wonder it hurts to do anything like drive, cook or work. 

It’s fitting that the international symbol for our community is the zebra. It comes after the medical saying ‘when you hear hoofbeats think horses not zebras.’ Meaning, usually, it’s a common thing you’re looking for. Medics forget that sometimes, hoofbeats really are zebras. The largely invisible illness we EDSers suffer make us the medical zebras most of the time. 

By putting poor Steph Aston on a psychological hold instead of treating her symptoms, this 33-year-old was tragically condemned to death. 

Her wrong diagnosis of factetious disorder - a newer term for mental illnesses like Munchausen - meant Aston was wrongly and fatally pigeon-holed as a person seeking attention for made up medical issues. She was placed on an involuntary psychological hold instead of being treated.

The details of this tragic case are hard to hear as a fellow EDS suffer, but sadly, not a shock.

This month, the U.K. EDS charity released a survey of its members, revealing patients in the U.K. with EDS take an average of 20 years to be diagnosed. The figures do not fair better worldwide, with EDS patients left floundering for medical support for decades. Aston was a vocal campaigner for patients in New Zealand, co-founding EDS advocacy groups. 

A quick look in any EDS support group on Facebook will show posts from hundreds if not thousands of sufferers worldwide, battling, fighting and failing to get medical help. From rheumatologists in the UK who don’t ‘believe’ in EDS, specialist in the USA taking away pain relief prescriptions, to doctors in New Zealand labelling EDS patients as mentally ill, it’s a shocking failure in care worldwide. 

It’s no wonder EDS has been labelled the most ignored and misunderstood illness in the world. 

And the ignorance of medics on this condition means patients are at real risk of misdiagnosis, injury, harm and sadly even death.

The details of Aston’s death have not much been detailed but it is believed her anaemia was not taken seriously and her transfusions stopped. Despite begging for them to be reinstated, they were not. 

Aston’s death was announced on social media to the horror of not only the EDS community, but society at large. 

My EDS took 20 odd years to be diagnosed and it has been a disabling, horrible battle. If I’d been diagnosed in my teens or 20s, I would have strength trained and preserved some function and mobility. Now, I am trapped in a broken body that only breaks further when I try to do anything. Not even trying to get better, but just surviving within the confines of my very small world. 

New Zealander Aston's case is a stark reminder why the EDS community is globally campaigning so hard for quicker diagnosis and better care. 

Currently the UK and US don’t have diagnostic pathways in place, letting patients slip through the net for decades. And even if we’re lucky enough to get a diagnosis, there is no care plan in place to help us control pain, injury and improve mobility. I know of patients who meet a nice doctor with great hope, only to have them deny their symptoms, medically gaslight them and strip them of any emotional fortitude they have.

So for Aston to have been wrongly labelled with having fictitious disorder, must have been so frightening. Immoral to the point of inhumane, actually. 

We are a community of disabled people whose largely inadequate healthcare is in contravention of one of the most basic human rights: access to adequate healthcare.

To stop a case like tragic Aston’s happening again, a number of things must change quickly.

First, medics needs to understand and accept all types of EDS are real - including type 3, the hypermobile type. It’s the only type which has not yet got a genetic test. Yet, we know it’s genetic as it moves through families, generation after generation. Just because the exact gene has not been identified, it does not mean our illness is any less real.

The second is accepting chronically ill patients function on a high level of pain daily that most people would end up crazed with. But we cannot go around screaming and crying all the time, because, well, this is our life.

Minute by minute we endure dislocations and subluxations, injured tendons and ligaments. Yes a dislocated elbow would send a person screeching to the ground, but for an EDS Zebra? It’s the sort of thing that might easily happen because we reached for something too fast, or in my case, rolled the dice for a board game.

The third is a set clinical pathway for diagnosis, treatment and management. Depending on the type of EDS, this might include regular blood tests and scans to monitor organs at risk of damage like the heart, specialised and expert physio with teams who fully understand we can’t do normal physio like everyone else. The physio itself can easily damage our tissues. 

Whatever the management, it has to come holistically, with teams who all understand EDS. A generic referral or care pathway does not work.

We need dedicated teams, funding, pain relief and importantly: research.

Research to understand our pain, injuries, the genes causing all the various types of illness and above all: ACCESS. Access to the right healthcare exactly when we need it. We do not need to be passed from pillar to post, from ignorant GP to ignorant rheumatologist or cardiologist and then back round to ignorant physio.

For all the advocacy Aston did with the EDS groups she stood with, now we have to advocate for her. Justice for the neglect she suffered and renewed action in her name. I can only hope the people who loved and mourn her can find some peace, though under the tragic circumstances Steph, that must be very hard to do. And who could blame them?

ENDS