Holly Hoben flies down the street on her bike, into the sunset and away from her mother’s call to come inside. Reckless as any 10-year-old, even brain surgery couldn’t keep Holly down for long.
“She wanted to play softball, but then she decided she’d rather do drama class,” said Holly’s mother, Hillary Hoben. “That was a relief to me.”
In the spring of 2004, Holly was diagnosed with a rare brain disease called “Moyamoya.” She received emergency surgery in May 2004 and now seems to lead the life of a normal fifth-grader — though with a few restrictions.
“She can’t play soccer because something might hit her in the head, and she can’t jump on a trampoline,” Hillary Hoben said. “We have to be very careful to keep a water bottle with her at all times because she can’t get dehydrated.”
According to the National Institute of Neurological Disorders and Stroke, Moyamoya is a condition that causes the narrowing of blood vessels to the brain. Researchers do not know what causes Moyamoya.
Dr. Patricia Aronin, Holly’s pediatric neurosurgeon in Austin, said the name Moyamoya comes from the Japanese word for “puff of smoke.” Because the main arteries to the brain are blocked, the smaller blood vessels are forced to transport more blood. The overworking vessels appear cloudy on scans of the brain.
Holly’s symptoms began in 2003, when she experienced transient ischemic attacks, or mini-strokes, on her left side. The attacks often occur as warning signs before a full stroke.
“I could feel a tingling in my arm,” Holly said. “It didn’t hurt too much, but it made it harder to do stuff.”
Other Moyamoya symptoms include disturbed consciousness, speech deficits, sensory and cognitive impairments, involuntary movements, and vision problems.
Various physicians tested Holly for epilepsy and multiple sclerosis, but were unable to find a clear reason for the tingling. Hillary Hoben, a former emergency medical technician, said she insisted that doctors be absolutely sure before treating her daughter for any medical condition.
“One of the doctors told me that something was wrong on Holly’s (magnetic resonance image), and she wanted to start her on seizure medicine,” Hillary Hoben said. “I didn’t buy it. I didn’t want them putting her on medicine if they weren’t sure that she needed it.”
After nine months of doctors visits, screening and testing, Holly was diagnosed with Moyamoya disease in April 2004.
The family lived in California at the time and was able to get Holly an appointment with Dr. Gary Steinberg, a professor and neurology specialist at Stanford Hospital.
“They told me she needed surgery in a month to prevent a stroke,” Hillary Hoben said. “The artery on the right side of her brain was 99.9 percent blocked, and the artery on the left was about 20 to 25 percent blocked.”
In a procedure called revascularization, surgeons drilled a hole on each side of her skull and reconnected the arteries directly to her brain during two separate surgeries. The surgeries resulted in instant blood flow to the brain.
“Before the surgery, she would have up to 20 (transient ischemic attacks) every day,” Hillary Hoben said. “Last week she had the first one in six months.”
Linda Hoben, Holly’s grandmother, said the family was blessed with friends and neighbors who watched out for them during the surgery. They also were fortunate that Holly was able to be seen by an expert on the condition.
“Dr. Steinberg is a lifesaver,” she said. “He saved a lot of lives, because we would have gone insane if anything happened to her.”
Steinberg said he has performed 158 revascularization surgeries on Moyamoya patients, and he thinks Holly’s prognosis is good.
“Her postoperative studies look very good,” he said. “Holly’s arterial blocks will not improve with surgery, but we have bypassed the blocks with a new collateral blood supply.”
Last year, Holly moved from California to New Braunfels with her mother, grandmother and 13-year-old sister ,Hailey. Holly is now a fifth-grade honor roll student at Canyon Intermediate School.
Holly has returned to her energetic self over the last year. The only visible reminders of the surgery are the holes that can be felt through her skin.
“I have a hole on each side of my head,” Holly said. “If I tap on one side, I can feel it on the other.”
Hillary Hoben said the faculty and staff at her daughter’s school have been very supportive and protective. Linda and Hillary Hoben have gone to the schools to speak with Holly’s teachers and classmates about Moyamoya.
Holly and her family also have found support through a Web site for others with the condition, www.Moyamoya.com.
Aronin said Holly should now have a typical life expectancy and will probably be able to have children. She said that when a patient receives surgery before they experience a stroke, the chances of long-term damage are much lower.
“Moyamoya is one of the few identifiable causes of stroke in childhood,” she said. “The damage from transient ischemic attacks is reversible, unlike a full stroke.”
Hillary Hoben is glad she didn’t stop searching for the truth about her daughter’s health. If left untreated, Moyamoya can lead to death.
“I had so many people tell me that I was just worrying so much, but I knew from the beginning that something was wrong,” she said. “I just never dreamed it would be something like this.”
