In heart-rending interviews this week, singer Celine Dion shared insights into her life with the rare health condition, Stiff-Person Syndrome, or SPS. But what exactly is SPS, and how do sufferers such as Dion best cope with it?
As the name indicates, SPS is predominantly characterized by a recurring stiffness of the muscles and uncontrollable, often painful, muscle spasms, which can lead to other problems such as trouble walking, falls, and a gradual curving of the lower back.
A one in a million condition
The stiffness and other symptoms of SPS usually progressively worsen over time, but episodes can come and go in spurts or be sparked by specific triggers, such as the cold, stress, or even touch. There are different forms of SPS, depending on the body parts affected (some, for instance, only experience stiffness along their limbs) and whether certain additional symptoms occur. SPS is thought to affect about one in every million people, though some cases may be misdiagnosed as other chronic neurological disorders such as multiple sclerosis or Parkinson’s disease.
There is still much we don’t know about the exact causes of SPS, but it appears to be an autoimmune condition (when the body’s immune system mistakenly attacks its own healthy cells and tissues). Researchers have found that SPS patients tend to have antibodies to an enzyme known as glutamic acid decarboxylase (GAD). GAD is an important ingredient to the body’s production of the neurotransmitter gamma-aminobutyric acid, or GABA.
GABA is often the body’s stop sign, helping slow or block specific nerve signals to keep the brain’s traffic flowing smoothly. It’s theorized that the disruption of the GABA pathway, instigated by the destruction of GAD, can throw this traffic off-kilter, causing these nerves to become overactive. But not everyone with SPS has anti-GAD antibodies and some have antibodies to other chemicals in the body, so there are lingering questions about the mechanisms behind the disorder.
‘Like somebody is strangling you’
Regardless of how exactly it happens, SPS can be a debilitating ailment. In her newest interview with NBC, aired Tuesday, Celine Dion discussed how it has changed her functioning on most every level.
“It’s like somebody is strangling you. It’s like somebody is pushing your larynx/pharynx,” Dion told NBC’s Hota Kotb.
Dion first announced that she had been diagnosed with SPS in December 2022, following delays and later cancellations of her concert performances that began the year before. But it’s likely that she began to show signs of SPS as early as 2008, which predominantly affects the singer’s upper body muscles.
As her symptoms worsened and prior to her diagnosis, Dion was reportedly prescribed a litany of medications to manage her spasms, including the sedative diazepam (better known as Valium). Her attempts to push through the illness and perform as usual led to her taking ever-increasing doses of Valium, eventually reaching a potentially life-threatening 90 milligrams a day (for context, the typical max dosage for spasms is 40 milligrams).
Determined Dion
“I did not understand that I could have gone to bed and stopped breathing. And you learn–you learn through your mistakes,” Dion told People in another interview released Tuesday. Even these dosages stopped working and by the time of the covid-19 pandemic began in 2020, Dion decided to wean herself off Valium, which unfortunately led to a worsening of her symptoms at first.
The discovery of her SPS does seem to have helped Dion better adjust to her situation. And while there is no cure for the condition yet, she is determined to make the most of it. She’s currently being treated with a combination of medications, immune therapy, physical rehab, and vocal therapy, according to People. And she has said that she is planning to return to the stage and sing once more for her fans. In her NBC interview, she can be heard using her distinctively powerful singing voice.
“I’m going to go back on stage, even if I have to crawl. Even if I have to talk with my hands. I will,” she told Kotb.