Celine Dion Documentary Sheds Light on Rare Disease

In a new documentary, “I am: Celine Dion,” the famous singer tells her story of battling a rare neurological disease, stiff person syndrome, and replacing fear with hope.

The documentary, which premiered last week and is now available on Amazon Prime, was directed by Irene Taylor, an Oscar-nominated and award-winning director and producer.

In 2022, famous Canadian singer Dion announced the diagnosis of stiff person syndrome (SPS), a rare autoimmune neurological disorder that affects only about one or two people in a million.

The condition is characterized by muscle stiffening, which can eventually remain constant and can make it hard to function. SPS may also cause episodes of violent muscle spasms, lasting from a few seconds to a few hours.

The deeply intimate documentary reveals Dion’s health struggles, which started 17 years ago, with voice spasms among the first symptoms. The loss of the ability to sing is especially painful for Dion, who describes her voice as the conductor of her life.

Although the movie depicts the worst periods in the disease progression, with Dion being unable to move or talk, the documentary is much more than about stiff person syndrome. It is also the celebration of Dion’s life and career and the perfect example of not allowing the disease to take over her life.

After several years of being out of the public eye, Dion appeared at the movie premiere in New York City, where she praised her neurologist, Dr. Amanda Piquet, who helped establish her diagnosis and “replaced fear with hope.”

Taylor, the filmmaker, said Dion asked her only one thing — to make a movie where only she talks about herself, rather than everybody else talking about her. This meant spending more time with Dion than if other people were interviewed.

The film became intimate not only because she gave me so much of herself but because she had so much to say. Someone suffering is going to have a different experience than a doctor or someone researching the cause of that suffering. Since the film is not about SPS specifically, I allowed the story to rise out of Celine's narrow — and fascinating — perspective.

Taylor

Dion is a success story

Marinos Dalakas, M.D., a neurology specialist, says about Dion's story, “In my long career of treating hundreds of patients with stiff person syndrome, I have seen improvements but to a variable degree, from mild to impressive. When a patient goes back to almost normalcy, I consider it a success story.”

However, Dalakas says that even with success stories, there is a possibility of a relapse. SPS, like any autoimmune neurological disease, may regress again and require re-treatment.

What are the early signs of stiff person syndrome?

In the movie, Dion describes her first symptoms of SPS as voice spasms and her voice going up, leaving her unable to sing. The singer speaks of the darkest period when she had difficulty walking and was losing balance, along with experiencing a lot of pain.

The symptoms of SPS can include the following, according to the Stiff Person Syndrome Research Foundation:

• Muscle rigidity, stiffness, and painful spasms in muscles of the trunk, including the back and limbs.
• Changes to posture, such as exaggerated sway back posture or a hunched posture in the upper spine.
• The stiffness, along with rigidity, can contribute to an unsteady gait. This may cause difficulty walking.
• Significant emotional depression and anxiety due to living with physical symptoms.
• More rare symptoms like shortness of breath or a sense of requiring more work to breathe, as well as spasms of the face or a change in appetite.

How is stiff person syndrome treated?

The documentary shows Dion working with her sports medicine therapist and multiple bottles of pills but doesn’t go too deep into the details of the treatments she receives.

Dalakas says there are two types of treatments for SPS. Antispasmodics are used to manage symptoms as they provide immediate effect, while immunotherapies change the course of the disease.

Dalakas conducted a study evaluating the effectiveness of intravenous immunoglobulin, which he says is the most effective therapy for SPS. It was found to help 80% of the patients and is usually the first treatment given.

If its benefits decline or are insufficient, patients may be prescribed the anti-B cell agent Rituximab, which is effective in nearly 40% of the patients.

Taylor says endurance is the most important thing she learned from Dion, who spent decades performing around the world and had suffered physically for 17 years at the beginning of filming.

Taylor tells Healthnews, "And yet, here she is today — still focused, still determined, funny, and, most of all, aspirational. I have no doubt Celine will use her talents — and her life's hardest lessons — to help others and help herself."

The movie leaves little doubt that we will see Dion performing on the largest stages again.

Dion says in the documentary, “If I can’t walk, I will crawl. But I won’t stop.”