CLEVELAND — Shortly after 1 p.m. on Nov. 9, an Indianapolis teen who has both hemophilia and epilepsy was wheeled into an operating room at Cleveland Clinic. He was about to have a large chunk of his brain removed, and he couldn't have been more excited.
After years of epilepsy that couldn't be controlled by medicine, Lance Rice, 16, considered surgery to cut out the section of his brain where seizures start. He thought it might be his best hope for a life with one less medical problem.
Brain surgery is performed on 1,000 to 1,500 people with epilepsy a year in the USA, but doing the surgery on someone with hemophilia is almost unheard of. Doctors here say this is the first time they have attempted it.
Lance seemed perfectly healthy at birth, says his mother, Michelle, but when he was 5 days old, he had a brain hemorrhage and was found to have hemophilia. He was the first in his family with the bleeding disorder, caused by the lack of a protein that allows blood to clot. Unknown to Michelle, she carries the gene for hemophilia; her second child, Lucas, 11, also has it.
Lance's brain hemorrhage left him with epilepsy. At 2, he was diagnosed with hepatitis C, contracted through tainted blood products.
Over the years, he had periods where medications controlled the seizures; from ages 5 to 11, he didn't need epilepsy drugs. But when he was 11, the seizures returned.
He tried several anti-seizure medications. Some didn't work for him; others were off-limits because of side effects that could exacerbate the hepatitis C.
Lance has infusions every other day of factor VIII, the protein he needs to prevent bleeding, and he takes three epilepsy drugs, 11 pills a day. Even so, he has had seizures once or twice a week.
His mother says the unpredictable seizures changed him from "a carefree, happy-go-lucky kid" to the target of bullies so persistent that the family moved so he and his brother wouldn't have to attend high school with them. Lance grew withdrawn, depressed and hopeless, limiting his activities and circle of friends.
"I stick with the same friends as I did in elementary school," Lance said a couple of weeks before the surgery. "With new friends, I'd have to explain a lot to them, and I really wouldn't want to."
Lance, who wears baggy jeans and T-shirts over his bulky frame, keeps his brown hair short and usually under a ball cap. He's a competitor in the virtual world of online video games such as Guild Wars, and he is a big fan of Larry the Cable Guy. The day he arrived at the hospital, he got a surprise call, arranged by a friend, from Larry the Cable Guy, wishing him well and offering him backstage passes to a show in Cleveland. Lance posted the news at his website, caringbridge.org/visit/lancerice. His mother has added daily updates on the site to keep friends informed of his progress.
Surgery not for all cases
The Rice family had never heard of brain surgery for epilepsy until a few months ago, when a doctor suggested it. Lance could barely wait. "I'm more anxious for the surgery than anything else," he said. "I want to get to the surgery."
Was he afraid?
"Not really. I've had to deal with the seizures ever since I was in fifth grade. I just don't want to deal with them anymore."
He may not have to. A team of doctors here, including pediatric epileptologists, neurosurgeons and hematologists, worked together to come up with a plan. The goal: to remove areas of his brain where doctors suspect the seizures start without creating new problems, such as loss of vision, memory or the ability to move an arm or leg.
"We don't hesitate to remove even a large area (of the brain), as long as there's a safe margin between what needs to be removed and what controls function," says pediatric epileptologist Deepak Lachhwani, a neurologist who specializes in epilepsy.
Surgery is considered only for people whose epilepsy originates from a localized area of the brain that can be safely removed, says epileptologist Brien Smith of Henry Ford Hospital, an adviser to the Epilepsy Foundation. "Some people have seizures from multiple areas of the brain, some have seizures that spread so fast you can't localize them. And some have seizures from one area that, if you remove it, you have other problems, like weakness on one side," he says.
An estimated 2.7 million Americans have epilepsy, and up to two-thirds are helped by medications. About half of those with uncontrolled seizures are thought to have the form of epilepsy that could be helped by surgery and "should be evaluated," Lachhwani says. "Fewer than 20% ever are."
To find out if Lance was a good candidate, Lachhwani oversaw a series of tests that identified the damaged area as being mainly in Lance's left frontal lobe. Tests also showed that the undamaged right frontal lobe had taken over the functions of speech and memory that normally would have been controlled on the left. But to locate the exact areas of damage in order to guide the surgeon required a more precise map of the brain.
On Nov. 2, Lance underwent his first brain surgery, a nearly four-hour ordeal. Using a small pneumatic drill, neurosurgeon William Bingaman cut into the skull from above the left eyebrow to behind the left ear. He removed an oval of bone, pulled back the brain covering and placed four grids dotted with electrodes directly on the brain. Electrode wires protruded from the top of the head when the skull piece was reattached.
Over the next four days in the hospital's pediatric epilepsy monitoring unit, the electrode wires were hooked to monitors, and Lance was taken off his anti-seizure medications. Lachhwani wanted him to have three or four seizures while being monitored to pinpoint where the electrical storm begins and how it spreads. The day after all medications were withdrawn, Lance had seven seizures.
The next day, doctors sent small electrical charges to the grid electrodes. Lance — now back on anti-seizure drugs — reported the physical effect of each stimulation.
The fact that Lance had no developmental delays during childhood, despite the extent of damage, attests to the ability of young brains to compensate, Lachhwani says. When the left frontal lobe was scarred by bleeding in his brain, his right frontal lobe took over functions normally handled by the damaged area. Removing the damaged area was not expected to cause any disability because "that part of the brain is not being used anyway," he says.
On Nov. 9, the day of the second surgery, Lance lay on a gurney with his head shaved and wrapped in gauze, cheerfully bantering with family members, doctors and nurses. Asked how he felt, Lance put his thumbs up and grinned. His father accompanied him to the operating room, and as the surgical team administered anesthesia, he held onto his son's hand. He gave him a quick kiss on the cheek and left the operating room with tears in his eyes.
Bingaman came into the room at 1:45 p.m. and, assisted by neurology fellow Mohamad Khaled, removed the stitches from Lance's head. He examined X-rays and pointed out areas of the left hemisphere that appeared smaller than the right. That was Bingaman's target. "The seizures start at the front of the frontal lobe," he said.
Repeating the procedure used a week earlier, Bingaman pulled back the scalp and the tissue under it and removed the screws that held the piece of skull in place, handing it to a nurse, who set the shell-like bone aside on sterile material.
Bingaman removed the grids and used a tool that both cauterizes and irrigates the region to cut out a piece of the frontal lobe, a pinkish-gray, rubbery piece about 4 inches by 3 inches around and about an inch thick.
"Some of these blood vessels supply parts of the brain that are healthy, so we want to protect those," he said. "We know where our motor area is. We'll be well away from that."
He then moved to the temporal lobe, taking out two smaller pieces: the amygdala, an almond-sized organ that helps process emotional responses, and the hippocampus, slightly larger, which stores new information and plays a role in spatial orientation. In Lance, these functions had moved to the undamaged right side of his brain. In all, Bingaman removed three-fourths of the frontal lobe and half of the temporal — about a fifth of the brain.
While still in the operating room, Lance was awakened and asked to move fingers and toes. He then was wheeled to the pediatric intensive-care unit, where family members, who had been waiting with anxiety and nervous humor, were able to see for themselves as he opened his eyes and smiled groggily.
"Hey, dude," said his mother. Lance raised a thumb.
No seizures so far
Bingaman does 250 epilepsy surgeries a year, about a third of them in children. But Lance's hemophilia was a concern. "Does the hemophilia worry me? Yes," Bingaman said before the surgery. He did 55 brain grid implants last year, he said, but "Lance was the first hemophiliac I have done this procedure to, and there are no other cases involving hemophilia and subdural grid implantations reported in the literature anywhere."
Yet hematologist Kate Gowans, who monitored Lance's blood levels during his stay, says with continual infusion of factor VIII, "the risks for this surgery are the same as for any other kid."
Because the right hemisphere of his brain is undamaged, Lance is not expected to have any physical effects from the surgery. But neither Bingaman nor Lachhwani offers more than cautious optimism that his seizures will stop permanently, and it may take a couple of years to find out.
Lance will be home-schooled for the rest of the calendar year. He plans to go back to high school in January. He'll see his local doctors in six weeks and return to Cleveland Clinic in six months, then annually for the next two years. He'll stay on three anti-seizure drugs for six months. If there are still no seizures, he'll stop them one at a time.
Doctors believe that, at the least, the surgery will provide significant improvement and the chance to control seizures with a single drug. For Lance, that's enough, though his family hopes for a cure.
"This kid keeps stepping up to the plate and taking what's delivered to him," says Michelle Rice. "I can't believe God would present this opportunity to him and not have it be a positive thing."
Lance and his family made it to Larry the Cable Guy's performance in Cleveland on Nov. 17, the day he was released from the hospital. By last week, he was back home in Indianapolis and feeling well enough to make updates to his website and visit with friends who stop by after school. He has had no seizures but is still a bit sluggish and headachy. And except for a slightly tilted "Elvis smile," caused by nerves that still remain a little numb, Michelle Rice says he's "doing really well."
Lance has no regrets. "I haven't had any seizures since the day before the second surgery," he says. "I guess so far I'm on a good streak."