Stiff Person Syndrome occurs in 1 in 1 million people and has no cure.
BELFAST, ME — Imagine living with a rare neurological disorder that affects only 1 in 1 million people. It’s been a difficult road for the Belfast woman since being diagnosed with stiff-person syndrome six years ago. The progressive disease made headlines last month when legendary singer Celine Dion revealed her diagnosis.
Brenda Smith vividly remembers when her brother Alan started having painful muscle spasms.
“Your muscles are contracting all the time. It’s constant,” Smith explained.
Seven years later, Alan was diagnosed with stiff person syndrome. There is no cure for progressive autoimmune disease. The disease, which affects 1 he her in 1 million people in the United States, is not known to be inherited. But then Brenda started experiencing muscle spasms in both of her legs, which later turned out to be her SPS.
“How could we have the same autoimmune disease? It’s so rare,” exclaimed Smith.
Smith lives in the Penobscot Shores Retirement Community overlooking Belfast Bay. She uses an electric chair to get around. The biggest risk of SPS is falling. In addition to frequent spasms, patients often have stiffness and postural problems.
“Some people remain undiagnosed,” said Dr. William David, a neurologist and director of neuromuscular medicine at Massachusetts General Hospital.
Smith is one of David’s patients. David said SPS can mimic other conditions, so it can take years to get a correct diagnosis. I was. It affects the nervous system and can be detected with blood tests.Smith manages some symptoms with medication.
Little or no research is currently being done to find answers. Smith and other patients hope things will change now that Dion has announced her diagnosis.
“I thought, ‘Oh my God, finally someone with a voice who can defend this,'” Smith said.
“Fundraising and other things to support research efforts,” David said of efforts to fight the disease. there is.”
For the past few years, Smith has been receiving intravenous immunoglobulin therapy, known as IVIG, twice a month at Waldo County General Hospital. She said her treatment had improved some of her symptoms. She also does weekly physical therapy with physiotherapist Callie Lumery, who works on Smith’s tight muscles.
Smith, who once traveled the world as a river guide, is grateful to be free of debilitating symptoms. That is to write her memoirs about her travels abroad.
“I feel really blessed to be able to write,” added Smith.