Until Celine Dion revealed final December she has Stiff Person Syndrome (SPS), it’s possible you hadn’t heard of the affliction.
The 54-year-old Canadian singer introduced in an emotional Instagram video that she’d been identified with the very uncommon neurological dysfunction, which has begun to have an effect on her vocal cords and her mobility.
The incurable situation impacts only one in one million individuals, with victims experiencing intense, painful muscle contractions – so violent that they’ll dislocate joints and even break bones. A major proportion of these with the sickness in the end change into motionless and wish to make use of a wheelchair.
While it was initially known as stiff man syndrome, it’s now recognised that about twice as many ladies as males are identified with the situation – amongst them, NSW lady Rhiannon Paul.
According to docs, Rhiannon, who came upon she has the sickness simply after her thirtieth birthday, has the bones of a 65-year-old.
Given how uncommon SPS is, it took years for docs to offer her the right prognosis.
“I had been to six different doctors and they all just said, ‘Well, it’s stress, we’ll just keeping upping [your] medication,” Rhiannon tells The Project’s Sarah Harris in a phase on tonight’s present.
Eventually, she known as her husband, former Wallabies participant Jeremy, and instructed him: “‘I’m just going to walk into hospital because I don’t know what this is, and I can’t manage it.’
“And then it was five hours later, I was bed-bound and couldn’t walk,” Rhiannon stated.
“When she was first in hospital, her legs were sort of going like this,” Jeremy instructed Harris, of the way in which his spouse’s limbs would spasm.
“And then all of a sudden, in the first couple of months, her body was like almost snapping in half. Like, her feet were like bent, like almost snapping and [she’d be] screaming in pain.”
For Rhiannon, “the muscle rigidness and stiffness is felt from my trunk down, and afterwards it feels like I’ve run a marathon”.
There have been occasions the situation has left her unable to stroll for weeks, counting on Jeremy to dress and bathe her.
Asked by Harris if she will be able to get away from bed on her personal most days, Rhiannon stated “probably 50 per cent of the time is good for me”.
“It’s obviously difficult when you don’t know from day to day if you’re going to get up and walk or not,” she stated.
“If I’m waking up and I can put my feet on the ground, that’s fantastic.”
Professor of diabetes and autoimmunity on the Queen Mary University of London, Richard David Leslie, defined in a December piece for The Conversation, there isn’t a treatment for SPS.
“Treatment is to limit pain, relax the contracting muscles and suppress the aggressive auto-immune response,” he stated.
“The latter can be done with drugs that dampen the immune response, including intravenous infusions or by filtering the blood to remove the harmful GAD antibodies.
“Botox can also be used to reduce muscle spasms, especially when diazepam (an anti-anxiety drug) has failed to relax the muscles.
“Other treatments include stem cell therapy, but there is no definitive treatment plan and each case must be managed individually to find the best approach.”
In Rhiannon’s case, what makes it extra distinctive is that she and Jeremy expect their first youngster collectively, due in May.
“We were told there were only seven women prior to that that had ever given birth with Rhiannon’s condition,” Jeremy stated.
“And you know, to think that we’re going to be the eighth in the world, it’s a miracle. It really is.”
