Cerebral palsy: can the incurable be made less unbearable?
Cerebral palsy: can the incurable be made less unbearable?
Jean Binda’s birth was that one in 500: somehow, the umbilical cord wrapped around the child’s neck, choking him, cutting off the oxygen supply to his brain. Thirteen years later, Jean remains confined to a grey metal wheelchair. Until recently his movements were jerky, his speech full of grunts, his IQ perfectly normal. Jean, like some 50,000 other Canadians, is a victim of cerebral palsy, literally imprisoned in his own muscle-spastic body. This July the Ottawa youth was one of the first Canadians to undergo a surgical operation that promises degrees of improvement for this otherwise incurable disease. Says Dr. Enrique Ventureyra, one of two Ottawa neurosurgeons at the Children’s Hospital of Eastern Ontario who performed the surgery: “If there is any hope at this time, this operation is the only hope we have.” But that hope may be delayed for other cerebral palsy victims. For one thing, doctors have criticized the technique, and, for another, the Ontario Hospital Insurance Plan refuses to pay for it.
In the three-hour surgical procedure, which involved implanting a brain stimulator, a pair of miniature electrodes was attached to the upper surface of the cerebellum, located at the back and base of the skull. Then wires leading from the electrodes were run under the skin of the neck to a tiny receiver, about the size of a wristwatch, implanted in the chest. After the operation, an external transmitter, or “neurostimulator,” with an antenna, was taped on the chest over the implanted receiver. It is powered by a nine-volt battery. Known as a cerebellar pacemaker, it feeds electrical impulses to the brain on a sevenminutes-on, seven-minutes-off cycle. The effect: the pacemaker relaxes the muscles and reduces the involuntary contractions. “We don’t want to create any false hopes,” says Dr. Leslie Ivan, the other neurosurgeon at Jean’s operation. “This is no cure. What can be expected is some functional improvement.”
A week after surgery, Jean was propelling himself around his hospital room and out into the corridor, a feat he had never managed before. He was able to wipe his face with a washcloth, a task that had always been done by others. His parents, though ecstatic, were leery of jumping to false conclusions. They noticed his voice was clearer, the words came out louder and easier when he talked excitedly about his favorite sport, hockey. His movements were more exact, his coordination better controlled. “For him, these are big achievements,” says his father, Robert.
The young boy’s progress is expected to improve steadily for the next six months, after which it will level off. Because the implanted parts don’t move, the equipment should not wear out and the operation need not be repeated.
The cerebellar pacemaker is not a new idea. Eighty years ago experiments with animals showed that electrical stimulation of the brain would reduce muscle spasms. But it was not until five years ago that Dr. Irving Cooper, a New York neurosurgeon, was able to devise the cerebellar pacemaker. To date, Cooper has performed more than 300 operations with the pacemaker, and concludes that 78% of his cerebral palsy patients improved significantly. More specifically, if cerebral palsy is divided into four stages of disability, each patient who receives an implant progresses by one stage. A small step to a casual observer, but a giant leap toward self-reliance for the cerebral palsy victim.
Though Cooper’s cerebellar pacemaker surgery has been performed in centres across the United States and more recently at the Hospital for Sick Children in Toronto, some doctors remain skeptical of the results. They are cautious of encouraging false hopes of miracle cures. Dr. Robert Lee, professor of neurology at the University of Calgary, echoes the sentiments of other colleagues when he says that Cooper “has a tendency to claim re-
sults that don’t stand up to careful scientific scrutiny.”
Says Dr. Robert Cullen, director of pediatric neurology at Variety Children’s Hospital in Miami, Florida: “The problem is, you can’t say this is black and this is white. We’re talking about degrees of improvement. It’s not like measuring a cup of coffee.”
But Cooper, who has fought for acceptance in the past and come out on top, shrugs off such criticism. He is convinced he has, at last, the means of quantitatively measuring improvement after the operation, thanks to Dr. Adrian Upton, a neurologist from McMaster University in Hamilton, Ontario. Upton has devised a method of using specialized computers to electrically measure changes in the brain. When he tested it on 30 of Cooper’s patients, including a Hamilton man, Upton says he found “repeatable, objective changes when the stimulator was on.” Cooper, with Upton’s assistance, is now working with the International Cerebral Palsy Association in New York to determine more precise scientific results. He is also trying to determine which cerebral palsy victim is best suited to the operation. “If done on all victims of cerebral palsy, very few would be helped,” says Cooper.
For, as with cancer, there are many types of cerebral palsy. The two Ottawa neurosurgeons. like Cooper, will be working in the coming months to determine which types can best be helped by the pacemaker. But, because this work is still considered experimental, the Ontario public health insurance plan will not cover the $2,300 cost of the operation. This means that private funds must be sought and many operations are delayed for lack of funds. In the United States (where the same operation can cost up to $8,000) law suits have been filed against private insurance companies. They also refuse to pay up, claiming the operation is still in the experimental stages.
The experiment is ending for Jean Binda, who may have been disappointed when there were no overnight miracles with the pacemaker. Only with patience, over the next six months, will he notice gradual improvement. But he is willing to wait—as a sticker on the side of his wheelchairsays, “Don’t be a fool. Stay cool.” For patients who come after him, the cerebellar pacemaker could have even more dramatic results. Says Cooper: “Our machine is just like the plane that Lindbergh flew over the Atlantic—we’re right at the very beginning.” JULIANNE LABRECHE
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