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What it’s like to live with stiff-person syndrome, a rare neurological condition with no cure

By Natasha O’Neill Writer

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    Toronto (CTV Network) — It’s been more than three months since Canadian singer Celine Dion announced she was diagnosed with stiff-person syndrome (SPS).

Dion, 54, posted to Instagram at the time, saying she needed to cancel the rest of her world tour due to SPS, a rare neurological condition that has no cure.

In the emotional video, Dion explains how the muscle spasms from SPS impact “every aspect” of her life and hinder her ability to perform.

Since the announcement, Tara Zier, the founder of the Stiff Person Syndrome Research Foundation, said the public’s understanding of the condition has increased, resulting in hope for people dealing with SPS that more research and funding will follow.

“It was this horrible feeling that she has this disease – because I know what that means to go through the initial stages of getting diagnosed – but so grateful for the awareness,” Zier told in an interview. “The disease has this name that just doesn’t do it justice.”

SPS is described by the U.S. National Institute of Neurological Disorders and Stroke as a disorder with “features” of an autoimmune disorder. It impacts the central nervous system and causes a person to have heightened sensitivity to noise, touch and emotional distress.

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

“I’ve talked to numerous patients… and they’re suffering, and it crushes me,” Zier said. “I was a dentist for 20 years. I can’t practice anymore. And I know that other people are disabled, some are bedridden, some are wheelchair bound, many can’t work anymore; Celine is having difficulty with singing.”

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

“For her to actually come out and say that she has it — she’s so loved and credible — it almost gives it validity,” Zier said. “Now, people actually take it seriously.”

Hours after the video was posted, Zier said her foundation, the only one in the world, was flooded with requests to comment and help explain what SPS is. She also said there was an “uptick” in some donations coming in.

The SPS Foundation was created after Zier’s own diagnosis of the disease in 2017. The foundation focuses on raising awareness and funding for research, and supporting others who have SPS.

“Anything that happens, like her (Dion’s) announcement, that helps support our mission. We want to be able to leverage that any way we can, so that we can get to better treatments and a cure,” she said.

It took Zier three years to be diagnosed, a timeline she said is quick compared to other SPS patients’. During that three-year journey, Zier said, she was “mislabelled, misdiagnosed (and) mismanaged.”

After being diagnosed, Zier discovered one of the reasons why little research on SPS exists.

“It was probably my third visit with my neurologist (at Johns Hopkins Hospital) where he told me they had recently applied for a grant to research stiff-person syndrome at (National Institutes of Health), and they were denied the grant because there was not enough research to support getting the grant,” Zier said. “So that just completely blew my mind.”

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

WHAT IT’S LIKE LIVING WITH SPS Like Zier, Lea Jabre had a difficult journey before being diagnosed with SPS.

Before her health issues, Jabre loved to dance and go to parties. She enjoyed electronic dance music and going to raves, but now with SPS she has heightened noise and light sensitivity and is unable to enjoy the things she loves.

Jabre, 36, started having issues with her digestive system in 2017, something she now knows is related to SPS. She was dismissed by doctors as having gastritis – inflammation of the stomach lining – and told to take antacid medications.

But the severe stomach issues persisted.

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

“I kept seeing doctors who kept telling me, ‘You’re panicked, you’re just stressed and you know it fits with your diagnosis…It’s all in your head, nothing’s wrong with you,'” Jabre told in an interview.

Jabre said she had to fight to keep being seen by doctors, convinced she was not being diagnosed properly. The symptoms transitioned into severe back pain and “weird pain,” which she later found out was caused by muscle spasms.

“SPS kind of comes and goes, it’s not constant, until the symptoms properly stay,” Jabre said. “I had periods of remission, so it went up and down.”

Doctors encouraged Jabre to exercise to decrease symptoms. By the end of 2020, she was running, doing high-intensity workouts, and felt like exercise was helping.

“I had more or less everything under control, or I thought so, because everyone kept telling me that nothing was wrong with me,” she said.

In December 2020, Jabre moved with her husband to Gambia, a small country off Africa’s west coast. At this time, her back was starting to hurt again, but she was convinced the pain would pass.

“January 2021 to May 2021 were the worst months of my life because I started having weird things happening,” Jabre said. “I started twitching – I didn’t understand it – I started having spasms, my feet would move by themselves, my hands would shake by themselves, my knees would shake by themselves.”

One day Jabre went to the bathroom and said she couldn’t rotate her back to grab the toilet paper.

“That was the first symptom that pushed me to think that something was wrong,” she said.

The stiffness progressed to a point where Jabre was unable to give her husband a hug. Her spams became more frequent as her body became ridged with pain.

Her fitness coach suggested she take a step away from exercising, which Jabre says made things worse. From there she started limping and was unable to go outside because they lived on the second floor and she couldn’t manage the stairs.

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

“There are no words to describe the amount of pain I was in,” she said. “People often say that stiffness resembles cramps. It does not resemble cramps whatsoever. It’s a billion times more painful than a cramp, because your articulation locks completely, and you can’t move it.”

The severe symptoms resulted in Jabre flying back to Lebanon, where her family is. She was seeing a doctor who later referred her to a neurologist.

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

“I was really happy because I was like, ‘Wow, finally someone is acknowledging that I have something,'” Jabre said.

Other than CBD oil for pain and muscle relaxants pills, the treatments for SPS are minimal. Jabre started intravenous immune globulin, which is donated plasma meant to boost her immune system, but she was not responding to the treatment.

Doctors then gave her plasmapheresis, which is when the plasma in a person’s blood is changed to remove antibodies. Jabre says the treatment gives her relief but will never allow her to go back to her old life.

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

SPS has changed Jabre’s life. It has forced her to stay apart from her husband in Gambia and to let go of some of her passions. But despite the disease, Jabre is staying positive and using her voice and story to raise awareness.

“We need to get involved because nothing’s going to happen if we don’t do anything. And then the hope that yes, with Celine Dion coming out, something’s going to move and something’s going to happen,” she said. “It’s my belief that as a patient, I have a responsibility that if I want to cure, then I need to work for it.”

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