What it’s like to live with stiff-person syndrome

It’s been more than three months since Canadian singer Celine Dion announced that she was diagnosed with Stiff Person Syndrome (SPS).

Dion, 54, posted on Instagram at the time saying he has to cancel the rest of his world tour because of SPSa rare neurological condition that has no cure.

In the emotional video, Dion explains how the muscle spasms from SPS affect “every aspect” of his life. and interferes with its performance.

Since the announcement, Tara Zier, founder of the Stiff Person Syndrome Research Foundation, said public understanding of the condition has increased, leading people with SPS to hope that more research and funding will follow.

“It was a scary feeling that she has this disease because I know what it’s like to go through the early stages of a diagnosis, but I’m so thankful for the awareness,” Zier told CTVNews.ca. “The disease has this name that just doesn’t do it justice.”

SPS is described US National Institute of Neurological Disorders and Stroke as a disorder with “features” of an autoimmune disorder. It affects the central nervous system and causes a person to be highly sensitive to noise, touch and emotional distress.

Zier and other SPS patients say there’s more to the disease than stiff muscles; it comes with constant “debilitating” pain and fatigue.

“I’ve talked to a lot of patients … and they’re suffering, and it’s crushing to me,” Zier said. “I was a dentist for 20 years. I can’t do it anymore. And I know that other people are disabled, some are bedridden, some are in wheelchairs, many can no longer work. Celine has a hard time singing.”

March 15 is International SPS Awareness Day, and Zier says Dion’s openness with her diagnosis has created more empathy for the often-invisible disease.

“For him to actually come out and say he has it, he’s so likable and trustworthy, it almost gives it validity,” Zier said. “Now people are actually taking it seriously.”

Hours after the video was posted, Zier said his foundation, the only one in the world, has been inundated with comments and requests to help explain what SPS is. He also said there had been an “increase” in some donations.

The SPS Foundation was created after Zier was diagnosed with the disease in 2017. The foundation focuses on raising awareness and funding for research, as well as supporting others with SPS.

“Anything that happens like his (Dion’s) announcement helps support our mission. We want to be able to use this in any way we can to achieve better treatments and cures,” he said.

It took Zier three years to get the diagnosis, a timeline he said is fast compared to other SPS patients. During that three-year journey, Zier said, he was “misnamed, misdiagnosed (and) mismanaged.”

After being diagnosed, Zier discovered one of the reasons there was little research on SPS.

“It was probably my third visit with my neurologist (at Johns Hopkins Hospital) where he told me that they had recently applied for a grant to study Stiff Man Syndrome (National Institutes of Health) and they had been denied the grant because there was . “There is not enough research to support a grant,” Zier said. “So it just completely blew my mind.”

Zier hopes that increased awareness of SPS in light of Dion’s announcement will lead to more funding for research.

WHAT IS IT LIKE TO LIVE WITH SPS?

Like Zier, Leah Jabr had a difficult journey before being diagnosed with SPS.

Before his health problems, Jabre loved to dance and go to parties. He enjoyed electronic dance music and going to raves, but now with SPS he has increased noise and light sensitivity and is unable to enjoy the things he loves.

Jabre, 36, started having problems with his digestive system in 2017, something he now knows is related to SPS. Doctors ruled him out because he has gastritis, an inflammation of the stomach lining, and told him to take antacids.

But severe stomach problems continued.

After more doctor’s appointments, Jabre was diagnosed with gastroparesis, a condition that slows the movement of the stomach’s digestive system, which he says is related to the nervous system.

“I kept meeting doctors who kept telling me: “You’re panicking, you’re just stressed, and you know it fits your diagnosis… It’s all in your head, there’s nothing wrong with you,” Jabr said in an interview with CTVNews.ca. interview

Jabre said he had to fight to continue in the eyes of doctors, convinced he had been misdiagnosed. The symptoms progressed to severe back pain and a “strange pain,” which he later determined was caused by muscle spasms.

“SPS kind of comes and goes, it’s not permanent as long as the symptoms stay decent,” Jabre said. “I had periods of remission, so it went up and down.”

Doctors encouraged Jabre to exercise to reduce symptoms. By the end of 2020, he was running, doing high-intensity workouts, and felt that exercise was helping.

“I pretty much had everything under control, or so I thought, because everyone kept telling me that nothing happened to me,” he said.

In December 2020, Jabre moved with her husband to The Gambia, a small country off the west coast of Africa. By then, his back had started to hurt again, but he was sure the pain would go away.

“January 2021 to May 2021 were the worst months of my life because strange things started happening to me,” Jabre said. “I started cramping, I didn’t understand it. I started having spasms, my legs would move on their own, my hands would shake on their own, my knees would shake on their own.”

One day, Jabre went to the bathroom and said he couldn’t turn his back to get the toilet paper.

“That was the first symptom that made me think something was wrong,” he said.

The hardness reached the point that Jabre could not hug her husband. Her spasms became more frequent as her body writhed in pain.

His fitness trainer suggested he take a step back from training, which Jabr says made matters worse. From there she started limping and couldn’t go outside because they lived on the second floor and she couldn’t manage the stairs.

“It kind of snowballed really quickly,” Jabre said.

“There are no words to describe the pain I was in,” she said. “People often say stiffness is like cramps. It’s not like cramps in any way. It’s a billion times more painful than a cramp because your joint completely shuts down and you can’t move it.”

Severe symptoms led Jabre to fly to Lebanon, where his family is based. He was seeing a doctor who later referred him to a neurologist.

After explaining her medical history to the neurologist, the doctor ran tests to rule out all other conditions. Jabr then got a second opinion at Johns Hopkins, where his SPS diagnosis was confirmed.

“I was really happy because I was saying: “Wow, finally someone admits I have something,” Jabre said.

Aside from pain pills and muscle relaxants with CBD oil, treatments for SPS are minimal. Jabre was started on intravenous immunoglobulin, which is donated plasma, with the goal of boosting his immune system, but he did not respond to the treatment.

Doctors then gave him plasmapheresis, when a person’s blood plasma is exchanged to remove antibodies. Jabre says the treatment gives him relief, but he will never let him go back to his old life.

“This disease is not a sprint, it’s a marathon,” he said. “If I decide it’s a sprint, that’s the end of me. So I have to really take it day by day. It is hard: It is hard”:

SPS changed Jabre’s life. This has forced her to stay away from her husband in Gambia and to abandon some of her passions. But despite the illness, Jabre remains positive and uses her voice and story to raise awareness.

“We have to get involved because nothing will happen if we don’t do anything. And then the hope that yes, after Celine Dion comes out, something will move and something will happen,” he said. “It is my belief that as a patient, I have a responsibility, that if I want to heal, I have to work for it.”