While the recent diagnosis has been devastating for Celine Dion, for sufferers of SPS it has shone a light on this rare but horrible condition.
Affecting one in a million people, the condition is not very well understood but is characterised by fluctuating muscle rigidity in the trunk and limbs and a heightened sensitivity to stimuli such as noise, touch and emotional distress, which can set off muscle spasms, according to the National Institute for Neurological Disorders. People can be too scared to leave the house because of noise sensitivity, have frequent falls or abnormal posture. They live in chronic pain, with little recourse to effective treatment.
Liz Blows runs the Stiff Man Syndrome Support Group, for those suffering with the syndrome and their families, and is based in East Yorkshire in the UK. It has 959 members from all over the world, but predominantly in the UK. Most of her day is spent on the phone, FaceTiming those who have been recently diagnosed or who call for advice as their condition progresses.
A sufferer herself, it took seven years for Blows, now 70, to get diagnosed. “I was going to the doctors every week with different things each time, for example, one week my left leg wouldn’t go where I wanted it to go.” A former nurse, she knew things were getting worse when taking her children to school became difficult.
“I’d take my son Chris into the playground and start holding his hand. I’d be OK to get to the classroom, but coming back out, it just seemed as if the playground was about 1,000 times bigger than it really was. I’d have to find someone I knew – one of the mums or dads – and walk with them. It is such a cruel condition.”
Finally, on one visit to the doctors, her left leg “jumped”. “You must have seen that,” I said, and he sent me to see a neurologist. “It was 1997,” she says. “The neurological specialist I was referred to said, “This looks like stiff-man syndrome, but don’t worry, it won’t be that, it’s far too rare and I’ve never seen a case of it.”
She then spent a week in hospital where they ruled out other conditions, during which time he concluded that it was in fact the diagnosis he had dreaded. At the time, little was known about SPS, despite it first presenting itself in the United States in 1956, and the prognosis was grim. “He said, ‘Your condition will get worse until you’re in spasm all the time, when you’ll have to stay in bed, and then you’ll die.’”
Treatment is still varied and aimed at slowing down the progression of the condition rather than curing it, with those with SPS learning to live with the symptoms, which vary greatly from person to person. Drugs only go so far. Following her diagnosis, the next day, Blows and her husband David, who worked for the Press Association at the time, went to the library to find out what they could about SPS and found that the first line of treatment was Diazepam, a version of Valium, from the benzodiazepine family, which acts as an anxiolytic, commonly used for seizures, muscle spasm and anxiety.
“We suggested this to the specialist who said, ‘no’ and it was only when I pushed that he gave me some, allowing me to improve over the next few months to the point where I could at least shop, cook and wash up for everyone over Christmas – and there were nine of us. I couldn’t do that before the diazepam.
“My specialist has since tried me on several different medications, and I know that some others benefit from Intravenous Immunoglobulin Therapy [a mixture of antibodies used to treat several health conditions], but the only one that works for me is diazepam.”
What is her quality of life like 25 years after first being diagnosed with SPS? “I wouldn’t say it’s good, but you have to carry on. My husband has five slipped discs, so between us, I like to say that if something is on the floor, I can pick it up whereas he can’t; but if it’s high up, I can’t reach it, so he has to do it.” But while she puts on a brave face, Blows is fully aware of the reality of the condition: “You don’t die of it, you die from complications that arise from it. And to be honest, it’s horrid. It really is horrid. For the majority of people with it who are bedbound, it’s just a really horrible way to live.”
What advice would she have for Celine Dion? “I have no advice,” she says, “because I would want to wait for her to tell me how she is dealing with it. I know she is very famous, but I’d want to know who is helping her, and I’d want to listen to what she had to say. That’s all you can do.”
