Guy’s Journey : From the Torment of Epilepsy, a Young Boy Is Given a Better Future Through Rare, Delicate Surgery

For Rae Gabelich of Long Beach, the ordeal’s hardest moment came when she had to hand over her 6-year-old son to the doctors. “I mean, what are you supposed to say when they tell you they want to remove half your child’s brain?” she asks.

Gabelich, 42, said yes. Now she’s thankful that she did. “He’s doing great,” she says of Guy, her blond, cherubic son who likes playing with electric trains, building block towers and watching a videotape of the Walt Disney classic “Lady and the Tramp.”

Four months ago, Guy couldn’t do any of that. Racked by incessant epileptic seizures and in an almost constant state of agitation, he had to be watched constantly to prevent his injuring himself.

Then came the surgery, during which doctors probed deeply into his brain, tied off countless blood vessels and removed about half the organ. Guy’s symptoms disappeared. And now, his mother says, grinning from ear to ear, “He’s a miracle!”

He was the subject of a rare, radical operation--a hemispherectomy--that offers new hope to certain kinds of young epileptic sufferers. For the right child, doctors say, the surgery can provide the possibility of something previously considered unattainable for those with severe epilepsy: an independent life.

Scary, but a Godsend

“It definitely fits into the ‘very big deal’ category,” said Dr. Donald Shields, a pediatric neurologist at the UCLA Center for the Health Sciences, where a handful of the operations, including Guy’s, have been performed since 1986. “It’s very scary. It’s not something you would do for very many patients, but where it’s appropriate it’s like a godsend.”

Guy’s journey to the operating table began on the day of his birth, when, during a long, difficult labor, the flow of oxygen to his brain was diminished. That caused serious damage to the brain’s left hemisphere. As a result, doctors say, his motor functions, particularly his ability to walk and speak, were impaired. He began having occasional epileptic seizures when he was 4 months old.

For a time, though, his condition seemed controllable. By the time Guy was 2 years old, he had learned to walk with a special brace. Though speaking was difficult for him, he was making slow, painful progress. His seizures were tempered by various medications prescribed by neurologists.

But gradually his situation worsened. His tolerance to drugs diminished.

“He was like a zombie,” Rae Gabelich recalls. “He would lie on a pillow and not be able to move.”

The frequency of the seizures increased. And more and more of them resulted in a temporary but complete loss of body control, putting him at risk of grave physical injury.

By 1987, his mother said, Guy was averaging 40 seizures a day. He even went once into “status epilecticus,” a three-hour period of non-stop seizures that his mother later learned could have been fatal. When doctors put him on a new medication to prevent a recurrence, she said, the results were disastrous.

“He became like an animal,” she said. “There wasn’t five minutes out of any waking hour that he didn’t scream at the top of his lungs.” Once he even bit her hard on the hand and wouldn’t let go. “It was probably the first time I understood the possibility of child abuse,” she recalled. “My nerves were frazzled.”

That same day she called her doctor. “We need help,” she recalls telling him. “This isn’t living.”

The theory behind hemispherectomies is simple, UCLA’s Shields said. In cases where epileptic seizures can be traced to disorders exclusive to one side of the brain, that side is removed, allowing the remaining, healthy hemisphere to take over its function.

While the procedure can have marked results, it is not even considered, he noted, until all other options--including medications--have been tried and found wanting. Even then, the brain of a patient undergoing the operation must pass two thresholds: First, the dysfunction must be limited exclusively to one side of the brain; second, the remaining side must be absolutely, unquestionably healthy.

“If the children are carefully selected, they seem to do very well” through the procedure, said Dr. James Cereghino, chief of the epilepsy branch of the National Institutes of Health in Bethesda, Md., and editor of Epilepsia, a bimonthly international journal on the disease. While the operation is generally accepted by doctors, he said, it is little known in the lay community.

It is not new, Shields said. First performed in South Africa in the 1940s, the operation fell out of favor a decade later when doctors discovered that many of its subjects died within a few years due to excess bleeding in their craniums.

Only after physicians began installing special shunts to divert the excess blood from the head to the abdomen, a procedure that appears to have solved the problem, did the operation experience a revival in the early 1980s.

Since then, Shields said, about 70 hemispherectomies--all of them on children who are considered better able to adapt to life with half a brain--have been performed at a handful of institutions nationwide. At UCLA, the only institution in California to offer the delicate surgery, 11 procedures, all successful, have been performed in the last three years, Shields said.

A careful series of tests last year determined that Guy Gabelich was a prime candidate. So last October, after a great deal of soul-searching, Rae--the widow of race car driver Gary Gabelich, a one-time world land speed record holder who died in a 1984 motorcycle accident--took her son to the hospital to undergo the nine-hour procedure, which doctors say has a 3%-4% mortality rate.

What finally swayed her, she said, was the realization that without the operation, her son would never have the chance to live independently.

Guy spent 18 days in the hospital, 11 of them in intensive care. Like others who have undergone the surgery, his recovery was painfully slow.

Skills, Behavior Improve

But when it was all over, his mother says, the difference was dramatic. Guy has not had a single seizure since; his speaking and cognitive skills, as well as behavior, also have improved markedly, side effects that are not uncommon, Shields says.

Shields theorizes that the dysfunctional part of the brain has a negative influence in many areas including behavior, and, “when you remove the noxious influence, the normal brain takes over and does what it would have been doing without the noxious influence.”

While a normal person would lose abilities through removal of a brain hemisphere, Shields said, the only loss when the hemisphere is already dysfunctional is its negative influence.

“You’re not taking out something that the patient is using,” he says. “You’re taking out something that is causing epilepsy. Nobody would say that Guy doesn’t still have problems, but he’s a lot better off than he was.”

That seemed evident during a recent visit to his sprawling country-like home in north Long Beach, where Guy’s pace, previously frenetic, now resembles that of most other children.

Some Personality Changes

Rae also has noticed personality changes in her son since the operation: Sounds that once went unnoticed now seem to bother Guy; he now shuns some people he once liked.

But mostly, she says, his changes have been positive.

His speech is still pained and slow. But his vocabulary has increased. And though Guy--who spends part of his day in a special-education program in the Long Beach schools and part of the day at home under close care--still has trouble focusing on tasks, his ability to learn new things has greatly improved.

The medical experts caution that Guy always will be mentally impaired, though they hope now that he at least has the possibility of someday being able to support and care for himself.

That prospect combined with his current progress has had a liberating effect on Rae’s life as well as her son’s.

“I’m no longer carrying around a cloud of gloom all the time,” she says. “I feel like I’m back in the real world.”