|Living with rare disorder a life-long struggle|
|Written by Scott Graves, Pilot staff writer|
|October 04, 2013 10:39 pm|
Victoria Gobron bombed Facebook. She emailed friends. She called radio stations and newspapers — anything to get the word out and raise funds for Chiari malformation research.
For all her work, a benefit walk at the Port of Brookings Harbor last Saturday garnered her $18.
“The rain just ruined it for us,” she said.
But it wasn’t all for naught. The Brookings woman found four other people from the area who suffer from Chiari malformation, a neurological disorder where the lower part of the brain descends into the spinal cord, compressing nerves and forcing cerebral spinal fluid — the thick protective fluid that bathes the brain and spinal cord — into areas it’s not supposed to go.
“It gets misdiagnosed so many times, it’s unbelievable,” Gobron said. “People are dying from this.”
The disease is named after Dr. Hans Chiari, an Austrian pathologist who classified the four malformation types in 1891. Roughly 300,000 people are diagnosed with Chiari in the United States, with females three time more likely to have the congenital disease. If left untreated, it can lead to paralysis.
It is a disorder caused when the skull is too small or poorly shaped. As the body grows, the lower part of the brain, which controls balance and coordination, is forced out of the only orbit in the skull — where the head and spine meet — and crowd the spinal coard.
The condition puts pressure on both the brain and spine and disrupts the normal flow of the fluid that bathe them, resulting in an array of symptoms — poor coordination, neck pain, muscle weakness and numbness among them — common to many other maladies, and therefore, often misdiagnosed.
In about 80 percent of cases, surgery can relieve the pressure and prevent further progression.
A second type of Chiari is usually seen in infants born with a type of spina bifida, and involves more of the brain than its more common malformation and whose symptoms are often worse and occur earlier.
Two other variations are rarely survivable.
Years of symptoms
Gobron said she experienced her first seizure when she was younger than 3. The emergency room doctors told her mother the little girl was allergic to milk and pickles.
But she kept falling off her bike, blacking out, dislocating shoulders — a common symptom. She’s 39, and other than a diagnosis and treatment, still suffers from symptoms — and post-traumatic stress disorder from all her visits to the doctor.
It took 37 years and innumerable tests before Gobron was correctly diagnosed, and then only because an eye doctor noticed something awry: the blood vessels in her eye were “undistinguished”: they couldn’t be seen clearly.
Gobron took that information to her neurologist. She had surgery in September 2010 to put in a shunt to drain the fluid; an MRI led to a second surgery involved removing a part of the rear of her skull to relieve pressure on the brain.
Gobron had an MRI done before. The results should have been a red flag, she said.
“That’s the problem I have — and a lot of Chiarians have,” Gobron said. “I thought I was going insane. I had seizures on and off my whole life, a lot of late-term miscarriages, and they couldn’t figure out why. I have so many intense headaches, and they told me I was suffering from migraines — for 15 years. Come to find out. … All that’s part of Chiari.”
But the doctors didn’t see it.
“Pain is not a reaction to nothing,” she said. “Pain is a reaction to something wrong. There are so many errors in my papers, it is ridiculous. One said I denied ever having had seizures. Some are outright wrong.”
Passing it on
Gobron worries about her eldest daughter, Cecily, and her youngest, Nicole, who is beginning to show some symptoms of the disease.
Yet, despite the challenges brought on by the disease, the weather that squelched her fundraiser last weekend, Gobron continues to advocate on behalf of awareness. And now she wants to write a book about the frustration many Chiarians face.
“There are several amazing people I want to interview,” she said, adding that she met a woman in the area who wasn’t diagnosed until she was in her 50s. “Now she has a buildup of water around her neck and spine; she can hear it as she moves. I’m trying to get these people’s stories; I’m so interested in hearing them.”
The International Chiari Association, a California non-profit public benefit organization, was founded in 2011 by Pete Dal Bellow, who went eight years before getting an accurate diagnosis for his condition.
The mission of the organization is to educate the public about the malformation and “bring awareness and research” to this little-known disorder.
Local sponsors include Dr. Janine Jason, Gold Beach Refrigeration and Heating, McKay’s Market in Gold Beach, Grocery Outlet, By May Hand, Stylin’ Salon, Applegate River Ranch House, St. Timothy’s Episcopal Church, Brookings Tax, Port of Brookings Harbor and Pete Dal Bellow of ICA.
Half of contributions go to Chiari research, 25 percent to an assistance fund, 20 percent to upgrade the website and 5 percent to help with miscellaneous expenses.
To contribute to Gobron’s book, contact her at 541-813-1744. Donations can be made through www.ChiariAssociation.org.