Mum-of-one Karen Long, 53, got her correct diagnosis in 2015 after years of tests and being misdiagnosed with anxiety and stress. She is speaking out to raise awareness of the condition
One mum is speaking out on her debilitating condition after it was misdiagnosed for years as “anxiety and stress“.
Karen Long, 53, suffers from Stiff Person Syndrome (SPS). The condition is currently making headlines after Canadian singer Celine Dion shared a heartbreaking video of her suffering an intense seizure from it.
Karen, from Romsey in Hampshire, can relate all too well to this, as she’s had to drastically adjust her life due to living with SPS.
For Karen, the condition causes her to freeze on the spot and then fall over when she is startled. The illness made it impossible for her to continue her successful career as a software engineer, has destroyed all the cartilage in her knees from falls, and often leaves her body battered and bruised. On top of this, muscle spasms and limbs locking into place can cause excruciating pain for hours at a time.
Recalling an early episode, the mum-of-one said: “I remember being in Malta and my late husband, James, walked off. And I said ‘Come back! ‘ And he asked why and I said ‘I can’t walk’. And then we had a few times when he would go to go across the road, and I couldn’t. He was like ‘What are you doing? ‘ And I would be like ‘I can’t’… Your mind cannot control the muscles.”
Karen was first diagnosed with the condition when she was 45 but this was after years of doctors failing to understand what was wrong. She admits that the tears she shed when she was first told she had the incurable disease were not for the reason many might think.
“Most neurologists have only seen a couple of people in their lives because it is one in a million,” she recalls of the moment she was given a diagnosis. “He said, ‘You’ve got something called Stiff Person Syndrome’ and he said it’s incurable and so I was a bit in shock and then I burst into tears.
“He was like ‘It’s okay, it’s okay. We can do stuff.’ But I was like ‘Actually I’m just relieved.’ Because prior to all of this, I was having MRI scans on my back, I was being told I just had anxiety and stress and all along it was the build-up of SPS.”
Stiff Person Syndrome, or SPS, is a neurological condition where the body begins to attack its own glutamic acid decarboxylase (GAD) proteins. This can cause muscles to tighten to an unbearable degree, prompt limbs to lock in place, and provoke uncontrollable muscle spasms.
Complications can particularly arise if muscles seize around major organs like the heart and lungs. The cause of the condition is unknown, and there is currently no cure.
Karen says she is grateful to Celine Dion for speaking out about having the condition as it has raised awareness, but she fears other sufferers could be misdiagnosed like she was. “When it first happened, it felt like a trapped nerve and it wouldn’t release. And that’s what it does.”
“It just gets tighter and tighter and tighter… It does often get mistaken for fibromyalgia (a different medical condition that causes pain throughout the body),” Karen shared, reminiscing about how her earliest symptoms were misdiagnosed.
“My back was seizing, and I was finding it hard to walk or do things. And that’s when they said it was anxiety and stress and they put me on antidepressants thinking that would make things fine. But it didn’t.”
Today, Karen relies on a mobility walker and faces particular challenges when walking in open spaces lacking railings or surfaces for support. It wasn’t until others pointed out her overly cautious movements that she realised she had subconsciously altered her habits.
Recalling an early episode, she said, “I remember being in Malta and my late husband, James, walked off. And I said ‘Come back!’ And he asked why and I said ‘I can’t walk’. And then we had a few times when he would go to go across the road, and I couldn’t. He was like ‘What are you doing?’ And I would be like ‘I can’t’… Your mind cannot control the muscles.”
Now 53, the proud mum-of-one was first diagnosed with the condition when she was 45 – but this was after years of doctors failing to understand what was wrong. She admits that the tears she shed when she was first told she had the incurable disease were not for the reason many might think.
“Most neurologists have only seen a couple of people in their lives because it is one in a million,” she recalls of the moment she was given a diagnosis. “He said, ‘You’ve got something called Stiff Person Syndrome’ and he said it’s incurable and so I was a bit in shock and then I burst into tears.
“He was like ‘It’s okay, it’s okay. We can do stuff.’ But I was like ‘Actually I’m just relieved.’ Because prior to all of this, I was having MRI scans on my back, I was being told I just had anxiety and stress and all along it was the build-up of SPS.”
Stiff Person Syndrome, or SPS, is a neurological condition where the body begins to attack its own glutamic acid decarboxylase (GAD) proteins. This can cause muscles to tighten to an unbearable degree, prompt limbs to lock in place, and provoke uncontrollable muscle spasms. Complications can particularly arise if muscles seize around major organs like the heart and lungs. The cause of the condition is unknown, and there is currently no cure. Karen says she is grateful to Celine Dion for speaking out about having the condition as it has raised awareness, but she fears other sufferers could be misdiagnosed like she was.
“When it first happened, it felt like a trapped nerve and it wouldn’t release. And that’s what it does. It just gets tighter and tighter and tighter… It does often get mistaken for fibromyalgia (a different medical condition that causes pain throughout the body),” Karen explained, going on to recall how her earliest symptoms were wrongly diagnosed. “My back was seizing, and I was finding it hard to walk or do things. And that’s when they said it was anxiety and stress and they put me on antidepressants thinking that would make things fine. But it didn’t.”
Karen, who is from Romsey in Hampshire, now walks with a mobility walker and found it particularly difficult to walk in open spaces where there were no railings or surfaces for support.
It was when other people noted that she was moving around with extra caution that Karen realised she had started changing her habits subconsciously. She recalls that her current partner, Andy, who she met through a dating site several years after her husband passed away, noted some unusual behaviours.
“Andy couldn’t understand why I couldn’t go up the steps (to my house) and it was because there was no banister. Or I would walk around the back of the car and the front of the car to get into the car. And he used to look and go, ‘Why did you do that?’ And I would ask ‘Do what?’ If I had to walk across the field at the summer fete, I would walk all the way around the edge. I started to believe that I was nuts. And it turns out it was because it’s a massive fear of open spaces.”
Karen ended up being diagnosed with SPS by chance. After suffering an excruciating spasm attack that affected the lower part of her body, she was admitted to a gynaecological ward. “Because the spasms were all from the waist down, they assumed it must be lady issues. And it wasn’t,” she recalls.
“A neurologist was doing rounds and asked what was happening and then said, ‘I’d like you to come and see me’,” she recounts. He submitted her for a GAD test; a blood test that counts antibodies, and was alarmed when the results came in. “Most people have a GAD count, which is how many antibodies per milligram of blood, of eight or nine. My count is in excess of 80,000.”
Karen was then sent for an Electromyography evaluation to see how her muscles reacted to stimulation by having needles inserted into her leg and back muscles. “I had no control over those muscles whatsoever and that’s how I got my diagnosis,” she said.
To manage the symptoms, she undergoes intravenous immunoglobulin (IVIG) every four weeks. This is when immunoglobulin, or plasma, from blood donors in intravenously injected into her bloodstream. The body then attacks these foreign antibodies, taking attention away from her body. The infusion takes roughly four hours and isn’t without risks.
“Having IVIG is more of a risk than the disease itself. It can cause you a stroke, chemical meningitis, or anaphylactic shock,” she explains. “And then the day after, you get flu symptoms, a migraine, all sorts. I’ve listed all of those for years and they are all the same. So I go ‘Oh yeah. The usual chest pains.’ I can check them off. After three days, normally, I start to feel the benefit and become more mobile. The pain is less.”
Karen, who is now a make-up and beauty blogger and can be found on Instagram and TikTok under the handle aggsy1211, hopes that having SPS in the headlines will help others who are yet to be given a correct diagnosis. “People are questioning the disease. Which is great,” she said. “If it means that just one person finds out they’ve got it, it means they can get the right medication to help them.”
Featuring: Karen Long
When: 09 Jul 2024
Credit: Cover Images
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She recollects how her current partner, Andy, whom she met on a dating site after the passing of her husband, noticed some peculiar behaviours. “Andy couldn’t understand why I couldn’t go up the steps (to my house) and it was because there was no banister. Or I would walk around the back of the car and the front of the car to get into the car.
“And he used to look and go, ‘Why did you do that? ‘ And I would ask ‘Do what? ‘ If I had to walk across the field at the summer fete, I would walk all the way around the edge. I started to believe that I was nuts. And it turns out it was because it’s a massive fear of open spaces.”
Karen’s diagnosis of Stiff Person Syndrome (SPS) came about by sheer chance. After experiencing a severe spasm attack that targeted her lower body, she found herself in a gynaecological ward. “Because the spasms were all from the waist down, they assumed it must be lady issues. And it wasn’t,” she said.
During a routine check, a neurologist noticed her condition. Karen said: “A neurologist was doing rounds and asked what was happening and then said, ‘I’d like you to come and see me’. Following his advice, Karen underwent a GAD testa blood test measuring antibodies and the results were startling.
“Most people have a GAD count, which is how many antibodies per milligram of blood, of eight or nine. My count is in excess of 80,000.”
Subsequently, Karen was subjected to an Electromyography test to assess muscle response to electrical stimulation, involving needles being inserted into her leg and back muscles.
“I had no control over those muscles whatsoever and that’s how I got my diagnosis,” she explained. To keep her symptoms at bay, Karen receives intravenous immunoglobulin (IVIG) treatments every four weeks, where plasma from donors is injected into her bloodstream.
This process, which takes about four hours, tricks her body into attacking these foreign antibodies instead of its own tissues, although it comes with certain risks.
“Having IVIG is more of a risk than the disease itself. It can cause you a stroke, chemical meningitis, or anaphylactic shock,” she outlines. “And then the day after, you get flu symptoms, a migraine, all sorts. I’ve listed all of those for years and they are all the same. So I go ‘Oh yeah. The usual chest pains.’ I can check them off. After three days, normally, I start to feel the benefit and become more mobile. The pain is less.”
Now a makeup and beauty blogger, Karen – known as @aggsy1211 on Instagram and TikTok – hopes that raising awareness of SPS will assist those still seeking a correct diagnosis. “People are questioning the disease. Which is great,” she expressed.
“If it means that just one person finds out they’ve got it, it means they can get the right medication to help them.”
