April and Scott Robertson were spent. For three and a half months, they had watched doctors try medication after medication in a futile effort to stop the seizures – more than 50 per day – their infant son Wesley had experienced since birth. The drugs would halt the seizures temporarily in the hospital, but once they got home, the episodes would resume.
Doctors have traditionally relied on medication to treat epilepsy, viewing surgery as a last resort. But now there is strong evidence that the longer children endure seizures, the more likely they are to experience developmental delays or even regress. Epilepsy specialists are urging parents and pediatricians not to wait for children to fail on multiple medications before being evaluated for surgery.
“Often they don’t get referred to comprehensive epilepsy surgery centers, and they just kind of suffer and struggle for many, many years, and their development gets delayed. Then it’s not repairable after a certain point,” Shah said.
Half of brain disabled
Wesley’s seizures had started a mere 12 hours after his parents had brought him home in June.
“Initially, it just looked like a really bad hiccup,” April said. “But he looked kind of lethargic, his arms were twitching, so we’re thinking something is wrong.”
When the seizures continued for more than 30 minutes, they took the boy to an emergency room.
Wesley spent the next three months in and out of the hospital before Shah got involved and transferred him to Children’s Memorial Hermann. The MRI had shown that Wesley had two malformations on the right side of his brain, clusters of neurons that weren’t supposed to be there. Those neurons were causing electrical discharges that led to seizures, not just in those spots, but throughout the right side of his brain.
“Often the malformed brain can cause the brain around it to act bad too, like a bad kid in a playground,” Shah said. “Every other kid in the playground is going to act poorly until you take out the bad players.”
But the MRI showed no seizures in the left side of Wesley’s brain, allowing Shah to take out the malformation and sever the right side of the brain from the left. Five days after he was first called in, Shah operated on Wesley’s brain in a marathon eight-hour surgery known as a hemispherectomy, in which the half of the brain experiencing seizures is disabled. Surgeons formerly removed that half of the brain, but over time they learned that patients have fewer complications if the disabled half is left in place.
Children can function with only half a brain because the remaining side takes over many of the functions of the half that was removed. The earlier the surgery is done, the more likely it is that the child will be able to compensate for the loss of one side of the brain.
‘Vastly underutilized’
But such surgeries are rarely done in children as young as Wesley. Shah had never done the procedure in a child that young.
Doctors told the Robertsons that Wesley might struggle to use his left arm and leg. But within days, relying solely on the left side of the brain, he was kicking both legs and waving both arms with equal gusto.
Surgeons must balance the benefits of operating as early as possible to avoid developmental delays against the risks of operating on a child so young.
“There aren’t a lot of centers in the world that could do this kind of a procedure in an infant three months old,” Shah said. “But there’s a big difference if you can do it before they’re 1 or even six months, versus if you wait till they’re 7 or 8.”
Shah felt confident that the operation would reduce Wesley’s seizures, but he wasn’t sure it would eliminate them.
“There are a lot of different epilepsy surgeries you can do, and the vast majority of them have a 70 to 90 percent success rate if you choose the right patient,” he said.”
Wesley exceeded all expectations. He has not had a seizure since his operation. He spent five days recovering in the intensive care unit after the surgery, then went home two days later. His parents had to retrain their brains as well, to avoid constantly expecting seizures.
“He had a certain sound, a whine he’d make before it started, and a certain way he would shake his head before it really kicked in,” Scott said. “Any little noise he made, you jump.”
Doctors don’t expect Wesley to have any lasting deficits from the epilepsy or the operation. He’ll remain on medications for six months, but if all goes well, he will not have to take any drugs after that.
Wesley’s case is unique because of his young age at the time of surgery, but Shah said the outcomes are typical. About two-thirds of children having seizures respond to epilepsy medication, seeing a marked reduction or elimination of their seizures. But in the remaining third of patients, surgery might be the best option.
“Surgery is vastly underutilized,” said Dr. Anne Berg, a professor of pediatric neurology at the Northwestern University Feinberg School of Medicine. “We’re really trying to get people to think of it not as a treatment of last resort, but maybe what should be done right from the get-go.”
Part of the problem, Berg said, is that pediatricians don’t always recognize the symptoms of epilepsy, particularly in very young children. And when they do, they often discount the seriousness of having seizures or believe that nothing can be done.
“That is inexcusable, unconscionable,” she said.
‘From hopeless to this’
Berg was the lead author of a study published in the journal Pediatrics in 2004 that evaluated children with epilepsy every year from the time of diagnosis until they reached school age. The researchers found that children who continued to have seizures fell further and further behind in their development. Some lost skills they had previously gained.
Other studies have shown that the later children are diagnosed with epilepsy the worse they do. And children who do have surgery, often see a rebound in the development.
“That’s been seen in some of the most desperately ill children,” Berg said.
Overall mortality for children with epilepsy is double that of the general population, and the rate of sudden unexpected death with epilepsy is higher than the general rate of sudden infant death syndrome.
Before the surgery, the Robertsons thought Wesley would face a lifelong disability, or worse.
If doctors couldn’t get the seizures under control, “We thought eventually he would die,” April said. Now, other than a long, thin scar on his head, Wesley looks and acts like a healthy boy. He lacks some of the strength he would normally have at five months, and may have to work to regain that. But it’s a far cry from where the family thought they would be.
“We went from hopeless to this,” Scott said. “And I when I say hopeless, that’s exactly what I mean. We were sitting there in the hospital with no hope.”
Source: Houston Chronicle By By M. Hawryluk
