‘My rare condition means I can’t cross the road in case I turn into a human statue’

When Celine Dion revealed to her fans that she was suffering from Stiff Person Syndrome (SPS), it was probably the first time many had heard of it. But for one-in-a-million Karen Long, she is all too familiar with the debilitating symptoms, having lived with SPS for the past 12 years.

The 52-year-old from Southampton can no longer cross the road over fears she could turn into a human statue – as her excruciating spasms cause her muscles to lock into place, causing her to fall over.

Her condition often leaves her with bruises stretching from her knees to her ankles and she has been left unable to walk while out shopping and has had to ask strangers to walk her back to her car.

Mum-of-one Karen says she is now mainly housebound, with no quality of life – but is thankful for the incredible awareness that has come with Canadian star Celine speaking out, with many people joining the SPS charity support group, which Karen is an admin of, in the past few days.

“I have the heightened stimulus – so if someone makes you jump, you go into a spasm mode, well, I live in that,” Karen says, explaining her every day to the Mirror.

“If I was outside and someone beeped their horn, I would fall.

“With SPS, you fall like a statue, you have no chance and your body freezes, you go down flat.

“I can’t cross an open space so I can’t cross the road.

“If I need to go anywhere, I will go the longest way around so I’m next to a wall because physically you go to step and the antibodies in your body say no.”

The rare disorder, which affects around one-in-a-million people, is a rare neurological disorder with features of an autoimmune disease.

For those affected, which is predominantly women aged over 30, it sees a part of their own bodies as alien and begins to destroy it.

Twelve years ago, Karen started to experience back pain and began walking hunched over and her walking became robotic.

It wasn’t until five years later that she was diagnosed with SPS – which came about by chance.

Her husband died in 2013, which was a massive shock to Karen.

Three years later when she was moving house with her new partner, she had a particularly bad episode, which she thought was a trapped nerve.

Looking back, she thinks the stress of the move combined with the loss of her husband might have triggered her SPS – a condition believed to be hereditary, yet no genetic defect has been identified to cause it.

Karen was in excruciating pain around her truncal area and was rushed to A&E.

She was placed in the gynaecology department for tests but it just so happens that a neurologist was doing a round on the ward with his students and came across Karen.

She was recommended for electromyography (EMG) – a diagnostic procedure to assess the health of muscles and the nerve cells that control them.

“It tested how many antibodies I had and mine was absolutely off the charts,” Karen remembers.

“They hear how your muscles react and your brain should be able to control them, but I had no control.

“My diagnosis was pure chance, I was so lucky”.

While there is no cure, Karen takes daily muscle relaxer medication and has Intravenous immune globulin (IVIG) treatment on the NHS every four weeks, where someone else’s antibodies are put into her body.

Just like Celine, who announced she is unable to play planned shows in the UK and mainland Europe next year, SPS affects Karen’s daily life.

Simple tasks that people take for granted like putting on socks, shoes and even cooking are severely affected.

Karen could be sitting watching TV on the sofa and suddenly not be able to get back up.

But she has gotten to know the feeling of when an episode is about to strike.

“Even my door buzzer being pressed gives my startle reflex a hit and I have a spasm,” Karen continues.

“My anxiety is now too much for me to go into town.

“I often book to go places and I would say 99 per cent of the time I cancel.

“If it was spontaneous I could possibly go. But if I’m told to go somewhere three days in advance, if you have to think about it, you can’t do it.”

Since her 18-year-old son left for university in September, Karen has been living alone in a ground-floor flat and is deemed unfit to work.

She does beauty blogging to pass the time and is open about her invisible illness online.

It has massively affected her relationship with her partner though, who has been with her since her diagnosis and goes to consultations with her and takes her to hospital appointments.

While he is supportive, her condition means they’re unable to do much.

“If he doesn’t hear from me by 10am each morning, he thinks ‘has she had a fall?'” Karen begins.

“We don’t have a life. Sometimes we go out for lunch, very rarely, but it’s massively stressful walking out of the house but I try.”

She adds: “This is the thing with an invisible illness. People see me online when I have my makeup on, they don’t think there’s anything wrong with me. What they don’t see is that it’s a front.”

More recently, Karen has experienced spasms in her chest, which has seen paramedics called out around six times over concerns she was having a heart attack.

It’s a progressive disease with no cure, so Karen could end up in a wheelchair or even bedbound.

But she tries all she can to still be a regular mum to her son, who is studying at Brighton University, and tries not to let the disease define her.

“One day trying to be normal wipes you out,” she says.

“As a mum, you just want to be mum, and be a fun mum. I’ve always tried my hardest to do as much as I can whilst I can.”

The awareness of a public figure like Celine Dion has drawn to the condition in speaking about her struggles has been incredible for the SPS community.

Karen hopes strangers will be more understanding of those with invisible disabilities and that further research can find a cure.

“My heart goes out to her [Celine] because I know how horrible it is however the number of messages of support that I have received… all of a sudden the awareness has risen.

“I have a bag made that says ‘SPS warrior’, I’ve always been open about it but this has raised so much awareness.

“It takes a long time to diagnose and the hardest part is that it cannot be cured. She [Celine] has this for life like the rest of us.

“It’s something that I don’t have a choice with but I’m trying my best to make the most of the life I can possibly live – I don’t live, I exist.

“It’s never going to be normal life but I will always try my best to see the positives and not just be a victim.

“I’m not going to allow the illness to define me.”

For more information on SPS and the help available, you can head to the support group Stiff Person Syndrome UK and Ireland Support Group and Charity here.