A DC mom has shared her hellish ordeal fighting a one-in-a-million condition that turns her into a human statue.
Tara Zier, 53, is raising awareness about ‘stiff person syndrome’ so that others don’t need to live in limbo for years as she did.
She began experiencing bouts of excruciating back pain, difficulty walking, and fatigue in 2015 after the death of her ex-husband.
For three years, Ms Zier was misdiagnosed by a range of specialists who could not pinpoint the exact cause of her debilitating symptoms.
Tara Zier sought answers about her worsening back and neck pain for three years before receiving a diagnosis of stiff person syndrome
Ms Zier’s spasms became severe and debilitating that she soon was no longer able to walk her dog or work in her dentistry practice. Experts suggest stress, such as the sudden loss of a partner, can magnify symptoms
Finally, in 2017, a neurologist ordered a barrage of blood tests that confirmed she had the extremely rare stiff person syndrome that affects fewer than 5,000 Americans.
The rare condition made headlines last year when Canadian superstar Celine Dion revealed in an emotional post to fans that her SPS diagnosis and painful symptoms had led her to cancel upcoming tour dates.
The exact cause of the neurological condition has eluded researchers, but they suspect it is an autoimmune disorder in which the body’s own immune system attacks and kills healthy cells.
Diagnosing the problem can be extremely difficult and the severity of symptoms varies by person.
The most common symptoms are muscle stiffening in the torso and limbs along with episodes of violent muscle spasms. Those spasms can be so intense they can cause bones to splinter and break.
They can be brought on by stress or external stimuli, such as a knock on the door or a loud shout, as well as physical touch and changes in temperature.
The muscle spasms and rigidity can turn people into ‘human statues,’ able to be knocked over like a trail of dominos.
The spasms can be so severe they cause people to fall over or lead to difficulty walking and, over time, even greater disability. It is often misdiagnosed as Parkinson’s disease, multiple sclerosis and fibromyalgia.
A major risk factor for stiff person syndrome, which affects one or two in a million people, is being a woman. Women are twice as likely to experience the disorder. It sometimes goes hand-in-hand with autoimmune disorders such as vitiligo and type 1 diabetes.
In Ms Zier’s case, the condition caused debilitating back and neck pain that stripped her of the ability to work at her dentistry practice and engage in activities she loves including karate.
She also experienced regular panic attacks, which landed her in the emergency department more than once.
The stress of losing her ex-husband and children’s father likely exacerbated her SPS symptoms, which can be worsened by stress.
Ms Zier told Insider that she was operating at about five percent. She had to renovate her house so that she could live exclusively on the first floor, having lost the ability to climb the stairs. She had to hire a caregiver to help her as well as her children and lost her ability to drive and walk the dog.
She said in 2019: ‘It felt like a deep spinal pain… I backed off from kickboxing and martial arts, but I was still short of breath and just didn’t feel great.’
She tried for years to find a treatment that worked for her condition, which doctors still had not diagnosed. She sought the insight of an endocrinologist, psychiatrist, rheumatologist, a general cardiologist and an electrophysiology cardiologist. Still, her symptoms persisted, disrupting daily life.
People with SPS are more likely to suffer from anxiety and depression. Scientists believe this is because patients have lower levels of the neurotransmitter GABA, which regulates anxiety.
Many people with SPS make antibodies against an enzyme called glutamic acid decarboxylase (GAD), which plays a role in making GABA.
Drugs that enhance GABA neurotransmission, such as diazepam, vigabatrin, and baclofen, provide some relief of clinical symptoms, but there is no cure for SPS.
There are three types of the syndrome.
Classical person man syndrome is characterized by rigidity and spasms around the back and stomach, and occasionally thighs and neck. It can cause back curvature over time.
Stiff limb syndrome causes spasms especially affecting the legs and feet, occasionally causing them to become fixed in place. Hands can also be affected.
And jerking stiff person syndrome is the rarest, most aggressive form, which includes symptoms from both the others, and also affects the head and eyes.
Since receiving a diagnosis of SPS following a battery of tests, Ms Zier founded the Stiff Person Syndrom Research Foundation to bring patients together and fundraise for a cure.
She said she is now operating at 60 percent, considerably better than before getting answers about her condition. She can drive and walk her dog again, but can no longer practice as a dentist.
Even standing for a shower is painful, she said.
Ms Zier is doing better now. Despite the condition’s rarity, she is not alone in her frustrating search for answers. A former real estate agent named Gay was finally diagnosed with the condition by doctors at the renowned Johns Hopkins University hospital system in Baltimore, Maryland.
Gay began experiencing a combination of pain and weakness in her legs, along with muscle stiffness that she describes as excruciating in 2004. By 2015, she needed crutches, and was close to becoming wheelchair-bound.
Symptoms of the condition can come on gradually as they did for Gay, often appearing between the ages of 30 and 60.