Survivor works to raise awareness of rare disease affecting the brain
Tara MacInnes was enjoying a barbecue dinner with her parents near their San Jose, Calif., home in 2003 when the high school junior suddenly threw her fork across the restaurant table.
The 16-year-old was not prone to causing commotions in restaurants.
MacInnes heard her mother tell her to pick up the fork, but after losing control of her right hand, numbness quickly spread down the right side of her body. Even more unnerving, her mouth and tongue would not obey her brain’s commands.
“I knew something was drastically wrong,” MacInnes said. “I just couldn’t verbalize it.”
After 10 minutes, MacInnes felt better and the family went home for the night.
Jill and Campbell MacInnes had grown cautiously used to their only child’s debilitating migraines followed by facial numbness and tingly sensations Tara had experienced since early childhood.
But this seemingly random episode did not fit her usual monthly pattern of a morning migraine followed by a brief “episode” by early afternoon, Tara MacInnes said. The day after the restaurant episode, her parents brought her to the pediatric neurologist she had been seeing since the third grade.
The physician told Tara MacInnes her brain had suffered irreversible damage from multiple strokes, most likely before she turned 3.
“It was a huge shock to my family,” said MacInnes, who was a double varsity athlete on her high school’s water polo and swim teams and had a 3.5 grade point average.
Her diagnosis as a bilateral stroke patient led to even more disturbing news more than half a year later.
MacInnes would have to undergo two separate brain surgeries to save her life after being diagnosed with a relatively unknown and rare disorder called Moyamoya disease.
“I had never heard of it before,” MacInnes said.
Not many people have.
MacInnes, 25 and a graduate of Our Lady of the Lake College, hopes to raise awareness in the Baton Rouge area about Moyamoya disease — a potentially fatal disorder that even some physicians aren’t familiar with.
“We each try to educate one person a day about Moyamoya,” MacInnes said, speaking of her parents who still live in California.
Often misdiagnosed as multiple sclerosis, blood vessel inflammation or simply migraines, many physicians try to fix the symptoms of the disease — strokes, seizures and headaches — instead of determining why the symptoms are occurring, said Dr. Gary Steinberg, chief of neurosurgery at Stanford University Medical Center and neurosurgeon at the Stanford Moyamoya Center in Stanford, Calif.
For still unknown reasons, the disorder causes the neck’s carotid arteries to narrow over time until little or no blood flow can reach the brain, Tara MacInnes said.
The results include headaches, difficulty speaking, facial and extremity numbness, and ultimately, a life-changing or fatal stroke, she said.
The disease is called moyamoya, or “puff of smoke” in Japanese, because the tangle of new blood vessels created by the body in an attempt to get blood to the brain resemble smoke on a medical test called a cerebral angiogram.
MacInnes said the incidence rate of the disease in the U.S. is between one in 1 million to 2 million, but could be as frequent as one in 250,000 because it is so underdiagnosed.
The disease has no cure, but brain surgery to reroute blood flow to the brain has proven very successful, Steinberg said.
Since 1991, Steinberg has performed more than 1,000 bypass surgeries — the kind Tara MacInnes would need, one operation on each side of her brain to transport blood to her brain via different vessels, Steinberg said.
In what is a called a direct bypass graft, Steinberg would sew a branch of a main scalp artery to a branch of an artery on the brain’s surface to improve blood flow.
Steinberg scheduled the first surgery on the right side of her brain for just three weeks later.
From prep time to the surgery’s end, MacInnes’ procedure lasted eight hours. She stayed in the hospital at Stanford University Medical Center for three nights and then went home.
Just one week later, Steinberg operated on the left side of her brain to perform the same procedure.
It was good doctors moved so fast to operate.
Within six months of her surgeries, tests revealed her carotid arteries had totally closed, MacInnes said.
MacInnes wants more physicians to be able to recognize the symptoms of Moyamoya disease, provide the diagnosis and then arrange for surgeries like her own direct bypass graft before the arteries completely close up and cause a massive stroke.
“The natural history of these patients if they’re not treated with surgery is very, very poor,” Steinberg said.
Sixty-five percent of Moyamoya patients who do not undergo surgery will go on to have a major stroke, he said. That percentage can be reduced to just 5 percent if the patient undergoes surgery, Steinberg said.
Steinberg said he believes there is a hereditary and genetic link to the disease that causes Moyamoya disease to affect more women than men and those with Down syndrome or sickle cell disease.
Although the number of Moyamoya disease patients Steinberg sees each year has jumped from one or two in the 1990s to more than 100 bypass surgeries each year, Steinberg said he doesn’t believe the prevalence of the disease has changed.
“But, I think it’s been underdiagnosed, particularly in North America,” Steinberg said, adding the disease is more common and recognized in Japan, where the disorder received its name.
Serving as a living, breathing survivor of the disease, Tara MacInnes and her mother, Jill, began volunteering at Stanford University Medical Center and providing support to families of Moyamoya disease patients.
Together, the pair has sat through 40 Moyamoya disease patient surgeries, Tara MacInnes said.
Steinberg even hired Jill MacInnes as an administrative associate and she still works for him today.
Although MacInnes could no longer serve as goalie on her water polo team after her surgeries, she jumped back into the water to raise awareness about Moyamoya disease.
MacInnes swam across the San Francisco Bay from Alcatraz Island to San Francisco and also swam the length of the 8,890-foot-long Golden Gate Bridge across the same body of water.
It was after MacInnes had swam the length of the bridge when she first met Prairieville residents Margaret and Annamarie Cardello who had arrived at Stanford University Medical Center for a checkup for Annamarie Cardello.
Annamarie Cardello, who has Down syndrome, eventually underwent two bypass surgeries for Moyamoya disease.
A ministroke at age 15 had led her mother to online message boards at the Stanford Moyamoya Center’s website, where users of the message board urged Margaret Cardello not to listen to a doctor who had advised delaying treatment.
“Since the surgeries, she’s done remarkably well,” said Margaret Cardello, who works in admissions at Our Lady of the Lake College and suggested Tara MacInnes enroll there.
Margaret Cardello described meeting and getting to know Tara MacInnes, who underwent the same surgery as Annamarie, as a blessing for the family.
“I love her,” said Annamarie Cardello, 20 and a graduate of Woodlawn High School.
The Cardellos are part of Tara MacInnes’ Moyamoya disease support group in Baton Rouge.
They meet regularly and Tara MacInnes stays in touch with another woman with Moyamoya disease in Breaux Bridge.
Tara MacInnes also talks about the disease frequently to strangers and hopes one day the disease will be featured on the National Council Licensure Examination for Registered Nurses to familiarize nursing students with Moyamoya disease.
While her surgeries haven’t cured Moyamoya disease, MacInnes has lived without symptoms of the disease for eight years.
She takes aspirin daily to keep her arteries open, drinks plenty of water and drinks alcohol in moderation.
“I’m expected to live a full life at this point,” she said.