In a moving video posted to Instagram, Canadian singer Céline Dion, 54, shared that she was recently diagnosed with an extremely rare neurological disorder called Stiff Person Syndrome, or SPS, and has been quietly dealing with the incurable disease for a long time. . weather.
“As you know, I have always been an open book and I was not ready to say anything before. But now I’m ready,” Dion said in the video posted on Dec. 8. “I have been dealing with health issues for a long time and it has been very difficult for me to face these challenges and talk about everything. I’ve been going through
People with SPS experience muscle stiffness and repeated episodes of painful spasms that often worsen the stiffness in their muscles, according to the National Organization for Rare Disorders. The exact cause of the disease is unknown, but it is believed to be the consequence of a faulty autoimmune response in the brain and spinal cord.
Only about 1 in a million people worldwide have the disease, but that may be an underestimate of the actual cases.
Dion said her muscle spasms affect “every aspect of [her] daily life”, sometimes causing difficulty walking or using your vocal cords; they are the reason he delayed reopening and canceled some shows at his Las Vegas residency last year and canceled the remaining shows on his North American tour.
Now, Dion said she will not be able to continue her European tour in February, which had already been rescheduled for April. The tour was originally scheduled for May to September of this year.
“I have no choice but to focus on my health right now, and I am hopeful that I am on the road to recovery,” Dion said. “This is my focus and I’m doing everything I can to recover.”
SPS cannot be cured, but a variety of treatments can help alleviate some symptoms.
“I always give 100% when I do my shows, but my condition doesn’t allow me to give you that now,” Dion said. “I am working hard with my sports medicine therapist every day to get my strength back and my ability to perform again. But I have to admit, it’s been a struggle.”
“All I know is singing, it’s what I’ve done my whole life,” Dion continued, “and it’s my favorite thing to do.”
Tara Zier, founder and CEO of the Stiff Person Syndrome Research Foundation, which also has SPS, told BuzzFeed News that she reached out to Dion on Instagram a month ago “to offer resources because her symptoms sounded so familiar.”
Dion’s official diagnosis and announcement brought Zier to tears, she said: “It’s a mixture of shock and really deep empathy. Hearing her talk about being supportive of her children, empathizing with how lonely it can feel and how hard it is when you can’t participate in what you love to do really resonates with me.”
Zier said Dion’s hope means everything. “When you are faced with an incurable disease, you can feel hopeless. But Céline will give hope to thousands of patients. Maybe now people take the disease seriously.”
She partly blames the disease’s “ridiculous name” for the “agonizing” amount of time it takes to get an SPS diagnosis — about seven years on average, Zier said. “People are not just rigid. It affects the vocal cords. Breathing. Walking. Go out in public. It is impacting Céline’s life and every patient suffering from SPS can relate to that.”
Because of the various symptoms that accompany this disease, people are often misdiagnosed with multiple sclerosis, Parkinson’s disease, fibromyalgia, anxiety or a psychosomatic disorder — in other words, “psychologically unwell,” Zier said.
But Zier is confident that people will start to take the disease more seriously because of Dion’s decision to be open about her diagnosis.
“Céline’s SPS diagnosis is going to wake people up. Today,” Zier said. “She is already using her beautiful voice to shed light on a devastating disease that is often misdiagnosed.”
What are the symptoms of stiff person syndrome?
The severity and progression of SPS vary from person to person. Researchers consider the disease to be on a spectrum, sometimes involving just one area of the body such as the torso or more of a widespread problem affecting the brain and spinal cord.
Symptoms usually develop over months or years, beginning as painful aches and stiffness in the legs or lower back that comes and goes, until finally the pain becomes permanent and spreads to other parts of the body. Body. Some people may develop a stooped or arched posture once the disease affects the spine.
Muscle spasms can occur randomly or after a variety of triggers, including loud or unexpected noises like car horns, minor physical contact like a tap on the shoulder, cold temperatures, stress, emotional distress, and more.
These spasms can be severe, causing some people to fall and seriously injure themselves, or develop severe anxiety that prevents them from leaving their homes. They can occur anywhere on the body, most often in the legs, but spasms in the abdominal muscles, for example, can fool people into thinking their stomach is full and lead to unwanted weight loss. Similarly, chest spasms may require some people to seek emergency respiratory assistance to breathe.
The spasms tend to last several minutes, but can last for hours. As a result, some people with SPS need to use canes, walkers, or wheelchairs.
Most of the time, there are no other neurological symptoms.
What Causes Stiff Person Syndrome?
There are still many unknowns about what causes SPS, but researchers believe it has something to do with the immune system attacking healthy tissue for unknown reasons.
Most people with SPS lack gamma-aminobutyric acid, or GABA, a neurotransmitter that helps control muscle movement. So some experts speculate that the immune system attacks certain nerve cells involved in GABA production.
Who is most likely to develop stiff person syndrome?
There are no clear risk factors that make someone more likely to develop SPS, but twice as many women as men are diagnosed with the condition, according to the National Institutes of Health.
Symptoms usually become more apparent for people between the ages of 30 and 60, but children and older adults have been diagnosed with SPS.
SPS also appears to be associated with other autoimmune disorders, the most common being type 1 diabetes. Some people with the disease may also develop thyroiditis (inflammation of the thyroid), vitiligo (irregular loss of skin color), pernicious anemia, and epilepsy.
What is the treatment for stiff person syndrome?
SPS can be diagnosed with a blood test, an EMG test that measures electrical activity in the muscles, and a lumbar puncture, a procedure in which a doctor uses a needle to collect fluid from your spinal canal.
Treatment for SPS is based on a person’s specific symptoms and can include a variety of approaches, such as medication and physical therapy.
Anti-seizure medications such as vigabatrin, valproate, pregabalin, and gabapentin, as well as other anti-anxiety medications and muscle relaxants, have been shown to help relieve some symptoms.
Intravenous immunoglobulin, or IVIG, has also shown promise in clinical studies for the treatment of muscle stiffness and sensitivity to stimuli such as noise and stress. However, IVIG has been associated with an increased risk of stroke and heart attack.
If people stop taking their medications suddenly, severe and life-threatening muscle spasms can occur.
Dion did not share details about her treatment plan, but said her doctors and family are by her side.
“I want to thank you very much for your encouraging wishes of love and support on my social media. This means a lot to me,” Dion said in the video, as she held back tears. “Take care. Be well. I love you very much and I really hope to see you again very soon.”