What a strange avenue for hope: Coils delicately wrapped around a nerve in the thin neck of 11-year-old Eric Zgoda might be the unlikely conduit by which his persistent seizures finally stop.
Eric has epilepsy. His first noticeable seizure occurred on Jan. 13, 1993, at 3 a.m., and since that early morning episode in his Arlington Heights home, each day holds the threat of another seizure–or a debilitating string of them. At his worst point, Eric had 10 days straight with seizures. At his best, he lived 12 seizure-free days.
In the first new approach to treating epilepsy in the last 100 years, an implant likened to a pacemaker for the brain is providing hope. Eric is one of just 13 patients in the Chicago area to receive an implant to help control their seizures since the device was approved for general public use. The U.S. Food and Drug Administration granted approval last summer after years of clinical trials, some at Rush-Presbyterian-St.Luke’s Medical Center.
It is part of a growing attention to a disease that has been one of mankind’s most mysterious and burdensome afflictions. Along with the introduction of the implant, a new drug on the market seeks to control seizures in a unique way, and there is revived interest in dietary treatments. Most significantly, geneticists have identified two human genes that cause a specific type of epilepsy. Each new gene discovery will help in the development of new and better drugs, says Dr. Jeffrey L. Noebels, an expert in epilepsy at Baylor College of Medicine in Houston.
As for Eric, like all epilepsy sufferers, he has abnormal brain wave activity that precipitate his seizures. The goal of the new device is to interrupt that activity with electrical impulses.
The device was implanted in Eric in early December at Rush and turned on Christmas week. But it will be months–as the current is slowly turned up and Eric’s body adjusts–before the family knows if the device can improve Eric’s life.
It works like this: The coils will carry bursts of electricity into Eric’s brain in intervals–30 seconds on, five minute off. The coils connect to a generating device roughly the size of a stopwatch that is implanted under the skin of Eric’s chest.
The coils wrap around the vagus nerve, one of 12 cranial nerves and the leading information provider to the brain from the heart, lungs, blood vessels and gastrointestinal tract.
For some reason that scientists can’t fully explain, electrical impulses carried through the vagus nerve into the brain help to disrupt seizure activity.
Eric would have gotten the device sooner if his mother, Ann, had had her way. Ann, 43, was so frantic to find relief for her son that in 1995, after reading a request for patients for the implant’s clinical trials, she lied on the application, saying Eric was 12 when he was just 8. He was turned down not due to his age, but because he had undergone brain surgery the year before; some of the clinical trials did not accept patients who previously had surgery.
Now that the device is commercially available, the University of Illinois at Chicago also offers the procedure.
While Eric waits to find out whether the device works for him, he lives as normally as possible but needs constant supervision, since he often loses consciousness during his seizures.
Quick with a quip or a high-five, he attends public school but studies some subjects one-on-one with a special-education teacher. Once a week, an “inclusion facilitator” takes Eric shopping to develop his real-world skills. He bowls on Tuesdays and is a Boy Scout, but he no longer plays his favorite sport, soccer; his old trophies still line his bedroom dresser.
As for the device, “We don’t know how (it works), we have strong suspicions, but we don’t know how,” said Dr. Ruzica K. Ristanovic, the associate professor of neurology at Rush who oversaw the clinical trials.
According to the Epilepsy Foundation of Greater Chicago, about 1 percent of the population, or roughly 2.5 million Americans, suffers from epilepsy. Of that figure, about 30 percent are under age 18.
Until recently, seizures have carried such a social stigma that some people have tried to hide their disorder.
In 1993, on his way to a public appearance, then-Cook County Board President Richard Phelan had a mild seizure, apparently triggered by a high fever. He had not disclosed on his driver’s license applications that he suffered from seizures and voluntarily suspended his driving privileges while his neurologist monitored his medication. Soon after he entered the Democratic campaign for governor, Phelan applied for and received reinstatement of his driving privileges because his doctor attested that his seizures were being controlled.
People with epilepsy have traditionally had two options to control their seizures: brain surgery or medications. The stimulator offers a new choice, and is geared toward those, like Eric, for whom medications have proved to be of little help and surgery was not successful.
“I told him the device would make the seizures be quiet and go to sleep,” said Ann Zgoda, as she sat alongside her son in their home.
“Stupid seizures,” responded Eric, “get out of my brain and go to a different brain.” He touched his blond head lightly with one hand and grinned at his mother.
Doctors don’t know what causes Eric’s seizures. And in about 60 percent of all epilepsy cases, no specific cause is found; such cases are usually detected early in life, often in people younger than 15 years old. But older children and adults are more likely to get epilepsy from head injuries such as from auto accidents or strokes.
While roughly two-thirds of patients can control their seizures through medication, the rest are looking for other solutions, noted Ristanovic.
Surgery to remove the part of the brain where seizures originate is extremely helpful in certain patients. But in Eric’s case, doctors in 1994 tried to remove the part of Eric’s brain they felt was the focus of his seizures, but it turned out that the seizure activity had “mirrored itself,” meaning that other parts of his brain had taken over the activity.
After a 15-year period in which no new drugs went on the market, there have been several new medications since 1993.
One of the newest, called Gabitril, takes an innovative approach in that it targets a specific chemical in the brain to control seizures; most drugs act non-specifically on multiple places in the brain, says Jim Fischer, associate professor of neurology and pharmacy practice at the University of Illinois at Chicago.
But while new medications have rolled out in recent years, there has never been a device that sought to control seizures. The idea for the implant came from neurophysiologist Jacob Zabara, who in the 1980s was at Temple University in Philadelphia and realized from past laboratory studies that electrical stimulation of the vagus nerve altered brain wave activity. He decided to apply the technique as a therapy for epilepsy.
Since 1988, the device has been implanted in approximately 1,000 patients worldwide; because the therapy is new, testing was extensive. After three months, about 30 percent of those patients saw the number of seizures they had reduced by half, according to Cyberonics, Inc., the Texas-based company that manufactures the device, called the NeuroCybernetic Prosthesis System.
Depending on the type of seizure he is having, Eric might lose consciousness and fall to the ground, his body stiffening and jerking. After such a seizure, called tonic-clonic (or in the past, grand mal), Eric is unaware of having had a seizure but feels tired and dazed.
If patients feel seizures coming on, they can pass a magnet over the generator in their chest to activate an electrical impulse that may stop the seizure from progressing. But for some, including Eric, there are no sensations that hint when a seizure is coming.
The device so far is seen not as a substitute for surgery or medications, but as an additional tool comparable to a new drug. But while the effectiveness of drugs often diminishes with time, the stimulator actually has shown more success with prolonged use.
The strength of the electrical current running through Eric’s vagus nerve will gradually be increased over the next several weeks to give his body time to adjust to the stimulation. His mother says he seems more lively and energetic, but he has also had seizures since it was implanted, including one on Christmas morning.
It will take months to determine if the implant will help Eric, said Dr. Donna Bergen, assistant chairman of the department of neurological sciences at Rush.
“I try to keep the hope realistic by reminding the family of the odds of success and the odds of failure and try and keep their feet on the ground,” Bergen said. “It’s impossible to keep people from being bitterly disappointed if this doesn’t meet their expectations. If this doesn’t work, it’s going to be a great crash for them.”
