The post hits the floor with a padded thud I recognise as a book delivery. Good – I’m relieved to have an excuse to leave my desk. Writing isn’t going well this morning: a low, buzzy pressure behind my eyes, my head trying to snap backwards off my spine, blood pooling in my legs, everything in my body screaming that I should not be upright.
I was 25 when I first got the sense that my head might not be securely attached to my body. I got home from teaching my undergraduate students one day, sat down, turned my head to one side, and screamed: an indescribable, apoplectic pain shot through the base of my skull. It left me flat on my back, unable to move or hold my head up for four or five days. What I thought was a freak episode soon became regular, recurring three or four times a year, usually if I had been sitting for extended periods or walking with a laptop in my bag. I learned to normalise it. In October last year I travelled from Glasgow to Berlin, Madrid and London to promote my book Lovebug, an essay on infection and intimacy. When I got home it happened again, except this time, after a week or so on the floor, it did not get better.
Author and poet Daisy Lafarge studied at Edinburgh College of Art and completed a PhD in creative writing, geography and molecular epidemiology at Glasgow University. Her poetry collection Life Without Air (Granta, 2020) was shortlisted for the TS Eliot prize and her debut novel, Paul (Granta, 2021), won a Betty Trask award. Her latest book is Lovebug (Peninsula Press, 2023), about the poetics of infection. Her writing has appeared in the New York Times, LitHub and the White Review, among others.
When I inspect the parcel, I see that it is not book-shaped. My short-term memory is glitching; I don’t remember what I’ve ordered until the contents slide out on to my duvet: a folding walking stick. The label attached to it reads like a sick joke: “Life – we’re living it!” I tear off the plastic wrapping, assemble the stick, prop it against the wall, and cry. Like anyone raised to regard themselves as able-bodied, the thought that I might need a stick, aged 31, wasn’t just unlikely; until recently it had not even been a remote possibility in my mind. Yet if I’d been asked any time in the past five years whether it was easy for me to stand unsupported, even for a few minutes, I would have said no. In fact this recent, whiplash-like injury – which would typically be the result of major trauma – was triggered by standing in a passport queue for 40 minutes. I remember waiting in line, feeling like I was in danger, but when I asked an officer for mobility support he looked me up and down – I am 6ft tall – disbelievingly. When I couldn’t show him proof of my disability, he shrugged; I looked fine, he said, there was nothing he could do.
The accumulating tension in my muscles snapped, pulling part of my cervical spine out of alignment in the process. The usual few days of being unable to hold up my head stretched into weeks, then months, and in that foggy, indeterminate period, delirious with pain, I experienced what I can only describe as a waking amnesia. Emerging from sleep, I could not remember the life I was waking into – neither its contents, nor who I was within it. I would lie there, trying to grasp a thread that would unravel the forgetting; it felt uncannily similar to the sensation, upon waking, of trying to catch hold of a dissolving dream. Seven months later, I still feel like one of those bobble-head dolls. If I go longer than 20 minutes without leaning my head against something, the accumulating tension will start to feel dangerous: I have to act quickly to prevent another spasm, which will put me out for several days.
The arrival of the stick makes me cry, I think, not because I suddenly need it where I didn’t need it before, but because it is a very loud declaration of something I’ve spent years trying to ignore. It is a winter of reckoning. In December I finally receive a diagnosis that explains a number of chronic health issues and injuries – hypermobile Ehlers-Danlos syndrome (hEDS) – and for the first time, begin to really take on board the fact that I am not, despite appearances, able-bodied. It’s a strange, head-wrecking time of affirmation and grief. I am trying to undo years of internalised ableism – telling myself that things are in my head, or that I’m somehow making myself sick for attention, or as an elaborate form of self-sabotage.
But breaking the cycle of chronic self-doubt dislodges a backlog of grief: not only for the years in which I had less pain and more mobility, but also for the able-bodied future that I believed would be mine. I grieve my diagnosis, at the same time as feeling relieved by it. It has suddenly, painfully illuminated something at the centre of my life that has always been there; I don’t know how to rearrange myself around this glaring presence, or account for the ways it has already, without my knowledge, arranged me.
Unless you’re a medical professional – and maybe even then – you might not have heard of the Ehlers-Danlos syndromes, a group of 13 genetic connective tissue disorders. Each type has distinctive features, but broadly put, EDS impairs and alters the production of collagen and other connective tissues, the “glue” holding the body together. Since connective tissue is everywhere – skin, muscles, bones, organs, tendons and ligaments – it follows that EDS can wreak havoc throughout the body. We are an inconsistent population, with symptoms that move us in and out of disability’s visible signifiers; we may appear able-bodied one day and be using mobility aids the next. We may require feeding tubes, multiple surgeries and lifelong treatments, yet never, to the casual observer, “look sick”. Where we are consistent is that we are almost always exhausted, and almost always in pain. The condition is incurable, but can be eased through lifestyle adaptation, pacing and integrative treatment. Early diagnosis is crucial to help prevent and manage the worst of the condition’s complex, degenerative, and potentially life-threatening effects.
If 31 sounds like a late age to be diagnosed with something I was born with, it’s fairly standard for EDS diagnosis rates in the UK. In Scotland, where I’ve lived since 2011, there is not a single EDS specialist; of the handful that exist in the UK, all are in England, and few are available on the NHS. Just last week, a study initiated by hEDS Start at Edinburgh University found that there is an average of 20 years between symptoms presenting and patients getting a diagnosis in Scotland, and that “a substantial proportion” had to pay for private consultations or travel to England to receive a diagnosis. The study also reported fragmented, insufficient care for those diagnosed, and the devastating impact of the condition itself and a lack of support for patients’ mental health. Often, those diagnosed in later life have spent decades going to their GP with a dizzying array of symptoms, and accrue various misdiagnoses of fibromyalgia, ME/CFS and anxiety along the way.
For many of these patients, an EDS diagnosis is when everything finally – albeit painfully – clicks into place. Yet any relief provided by diagnosis swiftly evaporates when patients realise there is little to no support or treatment available from the NHS. Many are refused diagnosis, in fact, on this basis – GPs say that there’s no use diagnosing something they can’t treat, or else that they don’t know enough about the condition to diagnose it. This outlook is so common that EDS UK offers a resource – the GP Toolkit – for patients to print out and take to their appointments.
Why is the outlook for patients with EDS in the UK so dire? The answer is complicated: a systemic lack of awareness, chronic misdiagnosis and medical misogyny, since EDS is twice as likely to affect those assigned female at birth. Hypermobility in general is also more prevalent among people with African and Asian genetic heritage. Speaking at a conference in 2014, one of the world’s leading EDS experts, Prof Rodney Grahame, claimed that: “No other condition in the history of modern medicine has been neglected in such a way as Ehlers-Danlos syndrome.” Grahame, a now-retired NHS rheumatologist, specialised in hypermobility and connective tissue disorders. He was pivotal in advocating the view that EDS is likely to be far more common than we realise, and that it is significantly under- and misdiagnosed because its signs are missed.
At medical school, GPs are taught that EDS is a rare disease, estimated to affect one in 5,000 (twice as rare, for example, as cystic fibrosis), and therefore something they are unlikely to encounter. On the other hand, they are taught that joint hypermobility is common, usually benign, and may even be advantageous for gymnasts and musicians. Many – including me – who go on to receive an EDS diagnosis in adulthood have usually been told, somewhere along the way, that our widespread hypermobility is in fact benign, a quirky gift.
I first heard the word “hypermobile” when I was 15. I had recently graduated from my Saturday job waitressing to that of receptionist at a chiropody clinic. This was good news: I was a terrible waitress. Perpetually dizzy, bumping into things, dropping things, the soles of my feet always ripped to shreds despite my “sensible” ballet flats. But my new boss noticed other bad habits – I couldn’t hold a pen properly, couldn’t sit upright unless I created a sort of vice to clamp myself between desk and chair, and I walked, to her eye, “oddly”. Soon I became her patient as well as her employee – she assessed me for hypermobility (9/9 on the Beighton score – a literal flex) and told me to consult my GP.
I was already seeing him regularly: he’d detected a heart murmur, various autonomic glitches and organ issues; I often fractured and sprained things, didn’t respond to local anaesthetic, had Raynaud’s episodes (a common circulatory problem usually provoked by cold weather) in the middle of July, an endless list of minor ailments, all of which he treated separately. When I told him I was hypermobile, he almost cartwheeled. “Wonderful!” He exclaimed. “It just means you are beautifully flexible.” He was even, I recall, “envious” of all the tricks I could undoubtedly do.
In describing these adolescent symptoms, rehashing the rocky-road-to-diagnosis trope, I suspect I am already on the defensive. Do I mention that I started working at a young age because it’s important to the narrative, or is it because, in the same breath as describing my symptoms, I feel I have to prove that I am, and have always been, hard-working – a preemptive, kneejerk response to the stereotyping of disabled people as lazy, work-shy benefit scroungers? I’m also hesitant to dwell at length on my current symptoms, since EDS manifests so differently from person to person. Many of the health issues triggered can be treated by the NHS, albeit as isolated, rather than connected issues. Many, but not all.
Researching my neck injury, I discovered the chilling stories of those affected by craniocervical instability (CCI), a laxity of neck ligaments that creates a dangerous weakness in the area between the skull and the spine, and which affects at least one in 15 people with EDS. CCI may start out as chronic spasms and memory issues, but left untreated can result in brain damage, paralysis or death, because of the weight of the skull slowly crushing the spine. The Google results are sinister: numerous fundraising pages and media articles about women in the UK with EDS and CCI, usually in their 20s and 30s, who are forced to fundraise hundreds of thousands of pounds for life-saving surgery in the US. Despite its prevalence among the EDS community, CCI is not something the NHS even diagnoses, since it requires an upright MRI, and there are no surgeons in the UK qualified to perform the stabilising spine-fusing or brainstem decompression surgery on EDS patients.
Stories like this are among the many reasons why the EDS community is so angry, and so infuriated with the idea that we can’t be treated. We improve drastically with specialist physiotherapy, and early education in how to move safely. According to the School Toolkit resource co-developed by EDS UK, children with EDS should not be expected to do things that cause premature wear and tear to hypermobile bodies, including sitting for long periods without moving, sitting without back support, standing in queues, carrying heavy bags, contact sports and gymnastics.
In addition to improved diagnosis and treatment, we desperately need more research into EDS and hypermobility disorders. Hypermobility has a proven correlation with anxiety, and there’s an as-yet-unexplained link between EDS and neurodivergent conditions such as autism and ADHD. A study published earlier this year also showed that hypermobile people are more likely to suffer from long Covid, which deteriorates existing dysfunction of the autonomic system – “unconscious” processes such as pulse, blood pressure, bladder function and breathing. It’s now accepted that hypermobile people experience, to varying degrees, a deregulation of these processes, and exposure to Covid can turn our mild, pre-existing autonomic dysfunction into severe, long-term illness (since having Covid six times in two years, I can vouch for this).
Having already known a few people with EDS before I got my diagnosis, I knew I would mostly be left to manage the condition on my own, to devise my own timetable of pacing, exercise, rest, treatment and work; that I would have to do daily, lifelong physiotherapy to be able to spend a few hours at a desk each day without constant injury, that I would have to ride out the inevitable flares. I am lucky that, even before my diagnosis, I had already transitioned to freelance working from home. After finishing my PhD I found out the hard, hospitalised way that my body cannot handle the precarious academic job market, with its long commutes, patchy contracts and inflexible hours of sitting and standing. Many people with EDS are too sick to work altogether, and face huge obstacles in trying to explain, to the impassive benefits system, a condition that is still poorly recognised by the NHS. With so little support available, managing a complex condition becomes a full-time job in itself.
I know this in theory, but when it comes to writing, I still harbour ableist expectations of myself. I hate being too foggy or racked with pain to write or think. On these days I do admin, or try to read, or paint, which helps distract from the pain. When progress on my novel is frustratingly glacial, I remind myself that one of my favourite books in recent years is Susanna Clarke’s Piranesi, which appeared 16 years after her first, because of the author’s struggles with chronic fatigue and illness. Piranesi is not a novel about illness, but I recognise something in its lethargic, dreamlike, alternative world that exists in an uneasy parallel to reality.
Earlier this year, a friend sent me an article in a medical journal in which the author analyses the myriad symptoms of the famously sickly bed-dweller Marcel Proust, and concludes that he suffered from the vascular subtype of EDS. vEDS is the most lethal type, with a median life expectancy of 48; Proust died at 51. Whether or not this theory holds water, Proust and those with undiagnosed connective tissue disorders definitely have something in common: symptoms that are so improbably diverse that we can only be hypochondriacs, or hysterics.
My favourite part of the theory is that Proust’s staying in bed was a way of managing symptoms, which would have been exacerbated by standing; those suffering from autonomic dysfunctions such as PoTS [postural orthostatic tachycardia syndrome] will know that lying down brings relief, returning the body to homeostasis. EDS or not, Proust reminds me that the equation I sometimes make between a writing life and an able body is a false one. I remember that it’s possible to carry on, working with, rather than against the body’s limits.
