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For Celine Dion, her heart will always go on. The show, however, must pause.
The three-octave soprano and “Titanic” Oscar winner for best song revealed Thursday in a widely-shared, emotional Instagram video that she has “stiff-person syndrome,” or SPS, a condition that has necessitated a continued break before her busy 2023 touring schedule even kicks off.
“Recently, I’ve been diagnosed with a very rare neurological disorder called the stiff-person syndrome, which affects something like one in a million people,” Dion says in the post.
“While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having,” she continued. “Unfortunately, these spasms affect every aspect of my daily life, sometimes causing difficulties when I walk and not allowing me to use my vocal chords to sing the way I’m used to.”
Her revelation has grabbed many people’s attention, spurring more than 200,000 Google searches for “stiff-person syndrome” in the 24 hours since her announcement.
So here’s what you need to know about what stiff-person syndrome is, what symptoms to watch out for, and how it’s treated.
What exactly is ‘stiff-person syndrome’?
Stiff-person syndrome, or SPS, is a rare neurological disorder with some features of an autoimmune disease, according to the National Institutes of Health.
SPS affects women two to three times more than men, NIH says.
“It hurts me to tell you today that this means I won’t be ready to restart my tour in Europe in February,” Dion said, while choking up. “I have a great team of doctors working alongside me to help me get better and my precious children, who are supporting me and giving me hope.”
Dion said that figuring out just what exactly was impacting her health this year took some time. She had canceled North American tour dates in January and European dates in April due to health issues. Now, getting better is her No. 1 priority.
“Stiff-person syndrome affects women two to three times more than men.”
“I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again, but I have to admit it’s been a struggle,” she said. “All I know is singing. It’s what I’ve done all my life and it’s what I love to do the most.”
Dion did share rescheduled dates for her European tour on her Instagram stories. Those will kick off March 6, 2024, in Prague and wrap up April 21-22, 2024, in London.
What are the symptoms of ‘stiff-person syndrome’?
SPS is characterized by fluctuating muscle rigidity in the trunk and limbs, the NIH says. Symptoms include muscle spasms, hyper-rigidity, debilitating pain and chronic anxiety. And episodes can be the result of heightened sensitivity to noise, touch and emotional distress, which can set off muscle spasms.
Abnormal postures, with sufferers often hunched over and stiffened, are characteristic of the disorder. In fact, people with the disease, which has no known cure, can be so disabled they are unable to walk or move — while falls, and the risk of secondary injuries as a result, are also likely, NIH said.
“ SPS episodes can be the result of heightened sensitivity to noise, touch and emotional distress, which can set off muscle spasms. ”
NIH also said twice as many women as men are afflicted with SPS. It’s unclear what causes it, although NIH notes it “is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia.”
What is the treatment for stiff-person syndrome, and how much does it cost?
There are two main treatments for SPS: GABAergic (gamma-aminobutyric acid) therapy and immunotherapy.
Treatment with Intravenous Immunoglobulin (IVIG), a therapy for patients with antibody deficiencies, has been approved for SPS in recent years, and there’s a self-infused application that some patients use instead, the NIH detailed. Most major insurance carriers will cover treatment with a professional diagnosis.
Anti-anxiety drugs, muscle relaxants, anti-convulsants and pain relievers are often used to improve the symptoms of SPS, but they will not cure the disorder.
Treatment also calls for care from several disciplines. Johns Hopkins University hospitals, for instance, draws together a team of experts in neurology, immunology, ophthalmology, neuromuscular disease and other specialties at its Stiff-Person Syndrome Center. The Stiff-Person Syndrome Research Center also provides more information and answers treatment questions.
Other treatments, such as acupuncture, aqua therapy and cognitive behavioral therapy are used to address chronic pain and other symptoms that can greatly reduce a patient’s quality of life, the Hopkins experts say.
Specific data on the expenses associated with SPS were not immediately available. Total spending on autoimmune disorder treatments in 2021 totaled $127 billion and are projected to rise to $149 billion by next year, according to Statista, using a report from pharmaceutical concern IQVIA. In comparison, costs stood at $49 billion just six years earlier.
Meanwhile, the annual cost for neurological conditions in the U.S. was $655 billion in 2020, including direct medical and non-medical costs and indirect costs from lost productivity and uncompensated caregiving hours, according to the nonprofit Partnership to Fight Chronic Disease (PFCD).
Does stiff-person syndrome get worse?
SPS is a very serious, progressive condition. Over time, stiff-person syndrome can lead to an altered posture. Severe cases can limit your ability to walk or move.
But there are also degrees of severity. Some people with this disorder need ongoing treatment for years to manage symptoms and maintain quality of life. At its most severe, SPS leads to muscle apnea that impacts organs.
What is the life expectancy for stiff-person syndrome?
By some measures, the life expectancy for people with SPS ranges from 6 to 28 years from the onset of the condition.
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