“I’m heartbroken, because I love her and she’s one of my favorite singers. But also because I saw the distress in his eyes. And I know that feeling. »
This feeling, Debra Richardson has known it for more than 30 years, when she felt the first symptoms of stiff person syndrome, the disorder from which Celine Dion suffers (also called Moersh and Woltman syndrome). She received her diagnosis in 1994 and even participated, in the early 2000s, in a clinical trial of the National Institutes of Health, in the United States, to test the effect of medication.
Stiff-person syndrome is a central nervous system disorder characterized by muscle stiffness and spasms. In two-thirds of patients, antibodies are found in the blood that are directed against the part of the nervous system that allows muscle relaxation. We are therefore talking about an autoimmune disease: the body itself attacks its ability to relax.
Unlike Lou Gehrig’s disease, stiff-person syndrome is not a degenerative disease, but rather a chronic one. People who suffer from it don’t die from it either.
In general, we are talking about a condition that is quite debilitating, because it is unpredictable and uncomfortable. All of a sudden, your body contracts. It’s unpleasant, and then it prevents you from continuing.
The DD Karine Garneau, neurologist specializing in neuromuscular diseases and neurophysiology at the CHUM
Like many patients with this rare disorder, Debra Richardson had to wait years for her diagnosis. “The spasms and stiffness were getting worse and worse,” says the 65-year-old woman, joined in Florida. And the spasms are terrible: it’s like your whole body is being twisted. »
PHOTO PROVIDED BY DEBRA RICHARDSON
Debra Richardson
The stiffness is persistent, while muscle spasms are added, sometimes for no reason, sometimes because of a trivial stimulus. “I call it the princess and the pea syndrome: we are ultra-sensitive to anything, summarizes Debra Richardson, author of the blog Chronic Chaos. Even a simple noise can cause a spasm. »
A simple pause or a complete stop?
Across the Atlantic, in The Gambia, Frenchwoman Léa Jabre Fayad cried a lot as she listened to Celine Dion tell her audience about the nature of her health issues and her desire to perform again. Léa, 35, received her diagnosis in 2021 after a long journey, where she was repeatedly told that her symptoms were caused by anxiety.
PHOTO PROVIDED BY LÉA JABRE FAYAD
Léa Jabre Fayad, suffering from stiff person syndrome
“I identified with the way Celine Dion spoke,” says Léa Jabre Fayad, member of the Stiff Person Syndrome Research Foundation. She calls it a career break. I hope this will just be a break, but having lived with this disease for over six years now, there are a lot of things that I have had to stop, because my body just can’t keep up. » Running, walking long distances, dancing, going out in noisy places… « I have mourned these things. And if in two or three years, I manage to do them again, it will be wonderful,” says Léa Jabre Fayad, whose treatments are prescribed by Dr.r Scott Newsome, of Johns Hopkins Hospital, USA.
Will Celine Dion be able to sing again, go back on stage? Neurologist Karine Garneau did not comment on the specific case of Celine Dion, but she points out that the disorder has variable effects from one person to another. Some people will find relief simply by administering drugs that inhibit muscle contraction (such as Valium), while others will see their symptoms persist over time, despite the medication, she says.
If we have control over the symptoms, I think we can hope to return to activities.
The DD Karine Garneau, neurologist specializing in neuromuscular diseases and neurophysiology at the CHUM
Debra Richardson insists on the importance of having hope. She knows a man with the syndrome who ran an ultramarathon, and another who walked with a cane and was able to ski again. “And I was housebound for 10, 15 years before I could run and even be able to run a half-marathon,” says Debra Richardson, who is controlling her disorder through medication, but also through to lifestyle changes. She also learned not to stress when she has bad days. Another day will come.
“I don’t know how severe the variant Celine Dion is suffering from, but if her symptoms can be alleviated and stabilized, maybe she could do studio work with headphones, and later on video? Patience and perseverance. The first objective is to stabilize. His career can take a different turn that can be just as fulfilling. »
According to Debra Richardson and Léa Jabre Fayad, despite all her sadness, this release of Celine Dion has a part of light, because it will shine the spotlight on this disease so little known to the general public and still too little studied.
Diagnostic
Stiff person syndrome is rare. Each year, only one in a million people are diagnosed with it, which is difficult to establish, because muscle stiffness can be caused by a host of diseases. The diagnosis is based on a set of symptoms with supporting tests (presence of antibodies, impact of a drug that promotes muscle relaxation and recording of muscle activity). “When you bend your arm, the triceps will relax to allow the biceps to contract,” explains neurologist Karine Garneau. In stiff-person syndrome, when needles are inserted to record muscle activity in both agonist and antagonist muscles (such as the triceps and biceps) simultaneously, the muscles are found to contract continuously and n perform more of the relaxation that is necessary for proper functioning. »