Stiff Person Syndrome: Terrible rare illness taking toll on Sheffield former motorsport star Ian Rawlins

And for former Sheffield motorsport hero Ian Rawlins, it has devastated his life, leaving him with violent spasms and excruciating pain that leaves him unable to be touched.

He has been struck down by Stiff Person Syndrome – an illness which affects only around one in a million, and which few people have heard of.

Ian, from Longley, Sheffield, a former pupil at Herries School was a successful motorcycle trials rider, who appeared on the BBC show Kick Start. He is now home from the Hallamshire Hospital until his next treatment for what he describes as an excruciatingly painful condition. He and wife Andrea made a lot of adaptations to their current home, in Wombwell, installing a stairlift, wet room and closomat toilet – but everyday is still exceedingly challenging for him. On a good day, he can get about with walking aids but there are a lot of falls. He is having a wheelchair assessment.

Andrea said: “People simply aren't aware that the condition exists. Yet it completely and utterly alters your life. At the moment there is hope for SPS sufferers with the stem cell transplants but there needs to be far more research and definitely a dire need for support for people with this rare condition. Sufferers shouldn't be written off simply because it's so rare.”

“When I first met Ian, he used to compete every week in motorcycle trials. He competed not only in and around Sheffield but also throughout the world, USA, Canada, Europe, New Zealand.

“He was what is classed in the sport as a British Expert and he was also the Yorkshire Champion. He was absolutely dedicated to the sport. Now he cannot so much as watch it. The world's top trial riders will be coming to Sheffield arena in January 2023 and he is too ill to even sit in the crowd to watch them.

“He has stiff person syndrome and I can honestly say I think it's the most horrifying, terrifying cruel disease I've ever had the misfortune to witness first hand.

“Stiff person syndrome is a rare, one in a million disease. When you're diagnosed, there is no leaflet or pamphlet to give you any kind of information about the disease. Nothing at all. You're on your own.

In the early stages it was painful but endurable stiffness in the joints. As it progressed it became a nightmare with spasms anywhere in the body. Andrea says in some patients, the spasms are so violent that it has been known to snap their femur.

But Andrea said the most terrifying thing was that not only is it excruciatingly painful but the person experiencing the spasms must not be touched. Touching can accelerate the spasms, as can sudden noises. “You can't comfort a person with this condition who is trapped in a living nightmare whilst they're being dragged to hell and back because the lightest of touches can stimulate such aggressive spasms it can become life threatening,” she said.

With no known cure, the best hope is a stem cell transplant but because the disease is so rare there, Andrea says there is little research funding. She believes if he had Multiple Sclerosis, he would at this point have been offered a stem cell transplant.

Andrea said she found even getting hold of medication to be a battle, with doctors knowing little about the condition. Even though Ian can't even move during these spasms, he was once told to crush and grind his medication if he is struggling to swallow it.

She said: “When we explained he cannot grind up his tablets, there simply is not time, never mind the fact he could not swallow them, we were told instead to insert them into his rectum. It's like banging your head against a brick wall. He cannot be touched. Even if he could be touched, I couldn't physically handle him to carry out this manoeuvre.”

“To date, my husband has had immunosuppressive medications that suppress his immune system. He's had plasmapheresis. Plasmapheresis is where you have a hickman line put into the jugular vein so blood can be withdrawn from a large vein. A machine separates the plasma from the red and white blood cells and these are then returned to the person with a plasma replacement fluid. Over the past year he's spent around eight weeks having this done.”

Until a few days ago, he was in hospital having an intravenous antibody treatment, but had suffered attacks of the syndrome which had lasted for several hours. On occasions he has had three in a day, making it difficult to administer his medication.

Andrea said: “There doesn't appear to be any kind of equality in healthcare when it comes to rare diseases.

“People with SPS feel they are abandoned. Trapped by a terrifying disease in a horrifying condition. Unable to help themselves but unable to access help from others.”

NHS England has been approached for comment.

University College London, which describes the condition on its website as characterized by persistent spasms involving multiple muscles, primarily in the lower limbs and trunk, list a number of support groups on its website, including Neurosupport; and Stiff Person Syndrome Support Group and Charity for the UK and Ireland, which says on its website: Many neurologists and GPs are still unaware of the condition. In most cases, the first symptoms are insidious and victims are often initially misdiagnosed with anxiety or depression.”