“How are you doing?”—a seemingly straightforward query that is commonly loaded for Tara Zier, a person living with Stiff Person Syndrome. Before she will even answer, people often assume she’s okay. A straightforward misdiagnosis when looking into the brunette’s warm eyes, vivid smile, and observing her tall, sinewy physique. “That feels odd due to how sick I feel,” she reveals. Not wanting to complain or be a burden, she normally responds with, “I’m hanging in there,” even when it couldn’t be farther from the reality.
From the bed to the front room and back again — the one places you’ll go all day, every single day. That is the life of somebody affected by Stiff Person Syndrome. Difficulty standing, showering, and even cooking — not to say the ever-looming fear of a muscle spasm that may harden you want a statue at a moment’s notice. That is the life that Tara Zier lives. These debilitating symptoms have slowly transformed Zier’s being. “I drag myself away from bed every morning, then arrange for my day on the couch,” she states. Before SPS, Zier was Dr. Zier, a 52-year-old dentist with a thriving practice. Karate was her hobby and she or he enjoyed traveling together with her children. “I never gave my health a second thought,” she recollects.
Then at some point, inside a month, she had lost 30 kilos. Running around from specialist to specialist, she was finally referred to a neighborhood neurologist who ran a battery of tests that eventually uncovered what was improper. She had an abnormal and elevated level of antibodies to Glutamic Acid Decarboxylase. “You’ve got Stiff Person Syndrome,” he revealed to her. “After I became sick, my whole life turned the other way up. I needed to hire someone to assist care for my kids. I want help with meals,” says Zier.
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By now you understand Stiff Person Syndrome is ultra stealthy, something you can’t see from the surface looking in, yet it tears on the physical and mental core of who someone is. “I need to desperately be myself, so I do my best to be upbeat and interesting while battling the debilitating symptoms,” says Zier, who founded The Stiff Person Syndrome Research Foundation (SPSRF), a non-profit raising awareness and funds to assist those which can be diagnosed with SPS. She explains that it’s as much a battle of the mind as it’s the body. “I feel like I’m performing all the time. It’s exhausting. I felt like I lost my identity once I needed to stop practicing dentistry.”
In December, when individuals are focused on family and holiday travel, Céline Dion revealed she was centering her health. The 54-year-old singer had been diagnosed with Stiff Person Syndrome, a rare neurological disease that leaves victims with draining and debilitating muscle spasms. The incurable disorder has caused the best-selling singer to postpone her upcoming tour to 2024 to recuperate and rest while determining the very best course of treatment. “I actually have an important team of doctors working alongside me to assist me recover,” said Dion.
Rarely ever spoken of within the news, the award-winning singer has brought awareness to a medical condition that has long gone unnoticed. Learn more about its symptoms, causes, treatments, and what it’s wish to be diagnosed below.
What’s Stiff Person Syndrome?
Medically categorized as a neuro-autoimmune disorder, Stiff Person Syndrome or SPS causes those diagnosed to have stiff muscles within the arms, torso, and legs. In response to the National Institute of Health, it may well even cause “greater sensitivity to noise, touch, and emotional distress, which may set off muscle spasms.”
What Causes Stiff-Person Syndrome?
While the reason behind SPS is unknown, its symptoms are said to be “attributable to deterioration of the parts of the nervous system that restrain our muscles. This lack of restraint results in overexcited, hyperactive muscles. Unfortunately, we don’t know why most autoimmune diseases develop, but genetics and environmental aspects each likely play a job,” in response to Dr. Basil Kahwash, a Board-Certified Immunologist at Vanderbilt University in Nashville, Tennessee. This decreased inhibition results from the disruption of some central nervous system pathways that inhibit muscle contraction. Some abnormal antibodies may impact the altered neuronal excitability.
“The motion of the abnormal antibodies is simply partially understood. It is just not known why these abnormal antibodies are produced. The conventional immune system makes antibodies which recognize and help destroy foreign bodies,” explains Zier. Although people make billions of antibodies, in SPS, a couple of antibody types are abnormal.
Since it is an autoimmune disease, antibodies are directed toward an individual’s tissues leading to the body attacking itself. She notes that more research is required to raised understand the role of the abnormal antibodies related to SPS; Since this disorder includes abnormal neuronal (nerve cells) function and abnormal antibodies, it is taken into account a neuroautoimmune disorder. It might take as much as seven years to find out an adult has SPS, and most of the time, it’s misdiagnosed as MS, Parkinson’s, or a psychological disorder, in response to Zier and the Stiff Person Syndrome Research Foundation.
Can SPS be cured? Is it treatable?
Zier maintains that while there are treatments, there isn’t any cure. While treatment primarily focuses on relief from symptoms, it is commonly not enough. Zier adds that immunotherapies involving medications and other treatments have been utilized to deal with specific autoimmune abnormalities.
Individuals with SPS have very different responses to treatments, so the patients and their physicians fastidiously try to evaluate medications. It’s unimaginable to predict which drugs might be effective for a person; nonetheless, some experts report that the sorts of SPS antibodies in an individual might influence which immunotherapies are most certainly to be effective. There’s currently no cure for SPS. Medications and other treatments may help reduce some individuals’ stiffness, muscle spasms, and pain. Unfortunately, most patients with SPS still have at the least some extent of disability.
Who’s affected by SPS?
In response to The Stiff Person Syndrome Research Foundation, SPS affects at the least 1 – 2 people in every million and is more common in women than men. It normally occurs between ages 20 – 50 and is incredibly rare in children, but The SPSRF is aware of at the least one child, I.M., who was diagnosed with SPS at 18 months old.
After I.M. had reached the milestone of crawling in her journey of growing up, she was suddenly unable to proceed a short while thereafter. Her parents were alarmed and immediately took the young child to a bevy of specialists — her mother wanted a neurology consultation immediately and was finally in a position to secure one. At the moment, the doctors ran every test they might consider and determined that the infant had Stiff Person Syndrome inside two months of examination at a hospital in Windfall, Rhode Island. This was later confirmed by neurologists at Boston Children’s Hospital.
Does the COVID Vaccine cause SPS?
Currently, there isn’t any established link between COVID-19 vaccines (or any vaccine) and increased risk of SPS, states Dr. Kahwash. “We don’t know why SPS develops in some individuals. SPS was first discovered within the Nineteen Fifties and has remained very rare since then. At present, tens of millions of Americans have received a COVID-19 vaccine, and up to now, there’s no sign that the population rate of SPS is increasing.”
What’s it like living with SPS?
Zier suffers from SPS and states that it has affected every aspect of her day by day life. “I now take care of fatigue, muscle rigidity, and pain on a day by day basis. My sleep is compromised. My back muscles feel like they’re in a vice. I actually have to plan all the things right down to once I’m going to shower attributable to limited energy. I endure physical therapy three days per week. It’s difficult and adds to the fatigue and pain. Nevertheless it’s value it because I’m getting stronger. For me, this illness is invisible, which has its unique set of challenges.”
People assume she feels tremendous due to her outward appearance, but battling the debilitating symptoms can sometimes feel like a performance.
“Everyone’s experience with this condition is different,” tells Zier. It’s a spectrum that ranges in severity. “Some have muscle spasms so severe they break bones, dislocate joints, and lead to life-threatening respiratory problems. Most have problems with mobility and require assistance like walkers and wheelchairs. Some individuals are bedridden.” A significant challenge with SPS is serious injury from a startling or unexpected body spasm. Those with SPS can fear going outside or turn into uncomfortable with it, and this may result in isolation, depression, and anxiety — an unlucky side effect of medication and treatment, in response to Zier.
Lea Jabre, 35, together with her partner.
Courtesy of Lea Jabre
Lea Jabre, a 35-year-old woman who serves on the Patient Advisory Board of the Stiff Person Foundation, feels every aspect of her life has modified. “As my symptoms have progressed, they usually have developed quite rapidly within the last three years, I feel my life has been placed on hold. I’m at an age where I believed I might either be a mum or starting a family or traveling the world with my husband to find latest and exciting places.” SPS has taken over her life — she cannot walk with no cane or for long distances, she will’t exercise, and she or he can’t be in loud places or around vivid lights. An excessive amount of stimulation gets her stiff or triggers spasms for her. Lea feels that her body has complete control over her life.
For young I.M., it’s a traumatizing experience when she is overstimulated by sound or motion, especially as a toddler who cannot articulate her feelings with words. Her pain or comfort level is expressed as any child’s is, but due to her diagnosis, it becomes evident when it stems from SPS. The psychological piece is just not even a consideration with tiny children because it is with adults. Navigating the varsity system has proven difficult as they’d never heard of this diagnosis. I.M.’s parents needed to get her an IEP (Individualized Education Program) to proceed receiving the mandatory support in her preschool classroom, on top of ongoing therapies.
Dr. Kahwash breaks it down even further, explaining that “most individuals know that it’s essential take into consideration moving a muscle before you possibly can accomplish that, and that requires a nervous system. Not everyone knows that your nervous system also holds your muscles back from moving on their very own if you don’t want them to be moving.” He paints an image of what this kind of spasm can feel like for somebody living with Stiff Person Syndrome: “Imagine contracting one in all your muscles and holding it that way — that is what occurs when an SPS patient loses the power to calm down their muscles. This lack of rest control can be what causes muscle spasms.”
Courtesy of Lea Jabre
Each Lea and Tara feel strongly in regards to the public, and even those that practice medicine, acknowledging their day by day difficulties – Jabre even wept the day she received her diagnosis. “After five years, greater than 20 doctors, and being always called crazy, the day a neurologist finally acknowledged something was improper with me, I cried tears of joy. Little did I understand how difficult of a diagnosis it will prove to be.” She hopes that the medical community may have access to raised research so we will have higher treatments to eventually discover a cure. To this point, the muse has donated $97,500 to Johns Hopkins Stiff Person Syndrome Center for research on Biomarkers to raised understand the disease and develop more targeted treatments.
Céline Dion’s announcement feels monumental for Lea and Tara, who see her courage as a approach to open doors to more awareness in regards to the disease, hopefully resulting in much-needed research in the long term. As for what Zier hopes will change for individuals who suffer from SPS, she leaves us with this final thought. “This disease is just not incurable. It hasn’t been cured yet. That’s why we want research.”
The Stiff Person Syndrome Research Foundation is propelling forward with a plan of action, launching the first-ever patient contact registry and natural history study in 2023 to present researchers a big pool of patients to check, which is critical for driving research forward.
Receiving this diagnosis is beyond words — it may well be scary, difficult, and feel such as you’re left at midnight. Zier still doesn’t let this get her down. Actually, her pain has transformed into motivation to raised the lives of those in her shoes. “As soon as the decision [with her doctor] ended, I used to be on a mission to search out out what the disease was, who were the very best doctors to treat it, and if anyone had gone into remission. And now, five years later, I’m on a mission to cure it.”
Danielle James is the Digital Beauty Director of ELLE.com. Previously, she was the Fashion and Beauty Director of HelloBeautiful.com and MadameNoire.com. She’s bylined for The Cut, InStyle, Allure, Business of Fashion, Nylon, Essence, Good Housekeeping, The Grio, and Huffington Post. Danielle enjoys sailing, thrifting, Japanese whiskey, Naomi Campbell’s runway walk, and Rihanna within the comment section.