STOCKTON SPRINGS, Maine — Jess Connor was 12 years old when she first visited the doctor complaining of headaches and blurred vision. She couldn’t have guessed that more than a decade would pass before she finally learned about the brain disorder that today, at age 30, leaves her using a walker on some of her sickest days.
Stress and even varying air pressure can worsen her symptoms, which include weakness in her hands and feet, trouble seeing, extreme fatigue and debilitating headaches.
“You can hardly breathe or open your eyes or anything,” Connor said of her headaches.
A little over four years ago, after doctors told her she had everything from fibromyalgia to anxiety, an MRI revealed the source of Connor’s pain. A condition called Chiari malformation, in which brain tissue protrudes into the spinal column, was blocking the flow of Connor’s spinal fluid. The disorder, characterized by structural defects in the cerebellum, the part of the brain responsible for balance, leads to a painful buildup of pressure inside the skull.
“A lot of doctors in Maine don’t know what Chiari malformation is,” Connor said. “They’ve never heard of it, even though it’s about as common as multiple sclerosis.”
In May, Connor, a mother of three, will undergo a risky surgery at the Chiari Institute in New York to correct the condition. Doctors will use a laser to burn off the brain tissue that’s pushing through the opening at the base of her skull, and then move the remaining tissue back into place, she said. They’ll also reposition one of her vertebra that’s dangerously tipped toward her spinal column.
“They’re basically going to cook my cerebellum,” she said.
The procedure could be deadly, Connor said, but the risk also brings greater chances of relieving her symptoms.
“I think when the surgery comes closer, I’ll be terrified,” she said. “Right now, I’ve had a really bad series of symptoms in the past few weeks.”
A group of Connor’s friends has organized a two-day benefit in March to raise funds for her treatment. Scheduled for St. Patrick’s Day weekend, the “March Madness” event in Searsport will include a polar plunge, public supper, cooking contest and children’s concert.
Connor has private insurance. The funds raised will be used to cover her $5,000 deductible and travel and lodging expenses.
Connor said she’s humbled by her friends’ generosity, but she’s also reluctant to take center stage.
“I’m a fighter, but I’m a quiet fighter,” she said. “Not ‘look at me, I’m in a wheelchair, look at me I’m using a walker.’ I don’t want people to look at me.”
Or, on the flip side, she may experience one of her good days on the weekend of the event, and attendees will wonder why she doesn’t look sicker, Connor said.
While some people with Chiari malformation don’t even know they have it, others experience a range of neurological symptoms that can be constant or intermittent. At one time, Connor said she could hear her heart pounding in her head for weeks on end.
Chiari malformation can be present at birth or appear in adulthood as the skull grows abnormally, according to Dr. Roger Kula, medical director of the Chiari Institute, which is part of the North Shore-LIJ Health System in Manhasset, N.Y. It can also be aggravated by an injury.
Connor isn’t sure if she was born with the malformation, since it took so long to diagnose, she said.
The condition is often misdiagnosed, since patients report a range of nonspecific symptoms, such as vertigo, difficulty swallowing and sleep disorders, Kula said.
“The big problem out there is a lot of these patients are thought to be psychoneurotic, problem patients no one wants anything to do with,” he said.
Experts estimate that roughly 1 in 250,000 people has the disorder, Kula said. Increased use of MRI technology has led to more cases being identified, but nailing down Chiari malformation’s prevalence is difficult given that many people don’t show any symptoms, he said.
About 80 percent of surgeries are successful in alleviating symptoms of the disorder, Kula said. He has not personally treated Connor and could not discuss her case for privacy reasons.
Connor said the shape of her malformation is slightly unusual, which makes her operation more risky. She expects she’ll need about two months to recover from the surgery.
“I am confident that I will come out not only alive but much better,” Connor said.
One positive result of her brain disorder is that Connor, who had to leave her job in medical records when her symptoms worsened, has discovered a passion for helping others affected by illness. She said she plans to return to work in nonprofit communications.
“After all these years, I’ve figured out what I want to do with the rest of my life,” she said.
The March Madness event benefiting Connor is seeking volunteers and sponsors. For information on the weekend’s lineup of events, visit the March Madness Facebook page or email firstname.lastname@example.org.