ANYONE MEETING KATE Arnott O’Flynn and hearing her feisty tones and quick wit would be hard pressed to imagine her bedridden and, in her own words, “very, very ill”.
Yet Kate, who is now in her fifties, suddenly developed scleroderma at the age of 46. The illness “came in like the March wind”, hardening her body, swelling her joints, seizing her lungs and rendering her unable to move in a few short months.
“It was very aggressive. My hands ended up like two tree stumps, useless, hard and sore. I couldn’t do anything for myself. I couldn’t get out of bed, dress myself or shower. My husband did it for me. It changes your life, there’s no two ways about it.”
Systemic scleroderma is unpredictable, irreversible, progressive – and currently incurable. This chronic auto-immune condition is considered the deadliest of all connective tissue diseases. The word scleroderma comes from the Greek words skleros, meaning hard, and derma, meaning skin, simply because the most visible symptom is hard skin all over the body.
But scleroderma is more sinister than just hard skin. It develops when the body produces too much collagen and hardens, which then damages not only the skin, but also the vascular system and internal organs, all at random. It is estimated that 1 in 10,000 people, mainly women, develop this rare but difficult disease. Yet many people, including patients who are being diagnosed, have never even heard of it.
“I said ‘what?! How do you spell that? Is it curable?,” remembers Frances Harney, who runs a drama school in Portlaoise. Frances, who is in her fifties, was dealt a heavy blow when she first heard the word, having just been given the all-clear from cancer. “If my particular cancer was put in front of me and scleroderma was put in front of me, I’d pick cancer,” she says. “It may seem strange, but I’m better from cancer, I’m not going to get better from scleroderma.”
Attacking from all sides
One of the more challenging characteristics of scleroderma is that fact that it can affects each person completely differently. It can develop slowly or attack very much at random, so in its early stages, specialists agree that it can be difficult to diagnose.
“For me it was my lungs and this awful joint pain,” explains Kate. “I couldn’t move, I was like the marble lady, everything goes hard.”
It’s also a disease where you’re cold all the time. I’m convinced I became a serious tea drinker just to keep myself warm by holding a cup!
“You have sweats, you have reduced brain function, you have disturbed sleep, you have difficulties swallowing and you develop this painful red rash just under your skin,” added Frances, who used to run at competitive level and now still mourns the loss of her favourite activity every day.
It’s not yet really understood why scleroderma affects more women than men, or indeed what triggers it in the first place. It’s thought to be caused by a complex mix of genetic and environmental factors.
One of the most common symptoms of scleroderma is a painful circulation problem called Raynaud’s phenomenon which constricts the blood vessels in the extremities. It’s estimated that around 10 per cent of the Irish population is affected by Raynaud’s. In most cases, Raynaud’s is painful but not life-threatening. However for 2 per cent of Raynaud’s sufferers, it can be a secondary symptom of something more serious like scleroderma.
Prognosis
Scleroderma is treatable but not curable. Treatment involves a close relationship with a rheumatologist who will help the sufferer manage the various symptoms through moisturising, controlling reflux, physiotherapy, sometimes even throat widening or extra oxygen, in addition to prescribing disease modifying therapies which include chemotherapy drugs. The aim is to prevent the disease from progressing, as damage caused by scleroderma is considered irreversible.
Prognosis is in itself a difficult topic to address given that in some cases, depending on what internal organs are affected and how, scleroderma can kill. But doctors feel that patients that are seen within the first couple of years have a better chance of modifying the progression of the disease, which is why early diagnosis is important.
“They’ll never give you a time, as everyone is different,” says Kate.
My husband asked our specialist about my own prognosis and it’s very hard, you know… because I’ve known a few people who had it that died.
But I’ve had it 8 years full blown now and I’m doing pretty good. You can’t live your life in a shell and at the end of the day I’m as well that I can be. I was that really ill person, but I’m proof that you can come back.
Today is World Scleroderma Day, which aims to raise awareness of the condition for both sufferers and the medical community.
Monica Heck is a freelance journalist, marketing consultant and copywriter based in Dublin. To hear an extract of her radio documentary on scleroderma, visit this link.
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