Cook Children’s Patient Becomes First in North Texas Implanted With Smart Device to Control Seizures
Cook Children’s Medical Center is the first pediatric hospital in the DFW Metroplex to implant a potentially life-changing treatment device in a patient suffering from epilepsy. On Wednesday, Nov. 4, 13-year-old Wyatt Keele of Lavaca, Ark., underwent the surgical procedure to implant the NeuroPace Responsive Nerve Stimulator (RNS) System—a smart device designed to monitor, target and interrupt unusual electrical activity in the brain that causes seizures.
“The impact of this treatment device can be pretty dramatic for the lives of the people who are candidates for it,” said Daniel Hansen, M.D., a neurosurgeon at Cook Children’s Jane and John Justin Neurosciences Center. “These are children who, five years ago, we would have told them there’s really nothing else we have to offer and you’re going to have to continue with medications for the rest of your life and manage it as best you can.”
The RNS System is a small, titanium neurostimulator about one-third the size of a credit card. During implantation, surgeons remove a portion of the skull in the same shape and size as the device and place the stimulator flush with the skull. It sits virtually undetected under the skin. Tiny wires, or leads, connected to the device are placed into the area of the patient’s brain shown to be the origin of their seizures. The RNS System monitors electrical activity in the brain and, when it detects unusual seizure-inducing electrical patterns, delivers an impulse, or stimulation, to disrupt the oncoming seizure. The technology is similar to that of a pacemaker that monitors and stimulates abnormal heart rhythms.
While approved by the FDA for use in adults, RNS can be used in select pediatric cases where all other treatments have been exhausted and where surgical removal of the area of the brain where seizures originate would cause significant permanent defects. Unlike anti-seizure medication, which can cause dizziness, drowsiness, depression or confusion, the RNS System does not cause chronic side effects. Patients do not feel the impulses it delivers.
“There are lots of kids who have responses to medicine or who are candidates for more aggressive surgeries, but there’s a good percentage of kids who we tell there’s really nothing left we can do,” Dr. Hansen said. “Now we have the ability to offer families another option and data shows, over time, you actually have a good chance of getting impressive seizure control.
A Glimmer of Hope
Wyatt Keele and his family had little hope left for relief from his lifetime of debilitating seizures. They tried everything from a ketogenic diet to multiple medications to thermal ablations of brain tissue to removing the portion of the brain where Wyatt’s seizures first originated. None of the interventions gave him significant, long-term seizure reduction or control.
“We were out of options,” Jennifer Keele, Wyatt’s mother, said. “There's no more medications for us. There's no more surgeries as far as taking out more of his brain. We were just kind of stuck.”
In February, doctors at Cook Children’s Medical Center told the Keele’s about the RNS device and its potential as a new treatment option for Wyatt. During the following months, physicians spent time mapping out the electrical activity in Wyatt’s brain using previous brain scans and electroencephalograms (EEGs) in order to pinpoint seizure location and determine potential placement of the device’s leads. By July, the Keele’s were convinced this was the right next step for Wyatt.
“My husband and I had already said that we weren't going to put Wyatt through anymore surgeries,” Keele said. “But after talking to his physicians we started researching and saw that this is a big deal and it has helped so many adults. The success rate is so high that we just couldn’t say no.”
It was the hope they longed for. The potential for Wyatt to be independent, attend school without worry, take part in activities with other kids and do all of the things his older siblings get to do without suffering from daily or weekly seizures.
“We do hope that the RNS is able to, over time, significantly control Wyatt’s epilepsy,” said Cynthia Keator, M.D, Wyatt’s epileptologist and medical director of the epilepsy monitoring unit at Cook Children’s Jane and John Justin Neurosciences Center. “We’re not sure if he’ll be able to fully come off of seizure medications, but we do hope that it'll decrease his medication burden while also decreasing the seizure burden and restoring some of his quality of life.”
The RNS device will continuously monitor and collect data related to Wyatt’s brain and seizure activity and, as it learns more and more, will adjust and improve its seizure-preventing interventions. In other words, the more data points the device collects about the electrical patterns in Wyatt’s brain, the smarter it will get in knowing when to send an impulse to interrupt a seizure, potentially improving Wyatt’s outcomes year after year.
Clinical studies of the effectiveness of the RNS System demonstrated a continuous improvement in seizure reduction over time. According to NeuroPace, at 9 years post implantation, patients experienced a median of 75 percent reduction in seizures and 28 percent experienced seizure-free periods equal to or greater than six months. Quality of life also improved, with patients reporting a rebound in cognitive function and physical and mental health.
The device will be teaching Wyatt’s doctors along the way, too. Data collected by the RNS System can be downloaded wirelessly, providing physicians with important feedback on the intensity, duration and location of seizures. This type of long-term monitoring can help physicians better pinpoint seizure activity origin and guide them in treatment decisions and options as the child grows.
“I think what will be most interesting is what data these devices provide us,” Dr. Keator said. It's another advancement in how we understand epilepsy networks and allows us to continue to find the best ways to ideally help these patients and hopefully find a cure for them.”
Dr. Hansen believes the lessons learned from RNS data collection has the potential to help more than just the device recipients.
“This really does give us the opportunity to capture that long-term data that we never had before, which I think will also be hugely impactful in the overall research of epilepsy,” Dr. Hansen said. “So all of these children and adults who have the device implanted are really creating a unique research population that we never had before. And they all understand that they're getting to contribute something to our understanding of epilepsy and how that electrical activity propagates throughout the brain and that what we find out may help someone else, too.”
One in 26 people in the United States will be diagnosed with epilepsy in their lifetime, according to the Epilepsy Foundation. For Wyatt, that diagnosis came at just 10-months old.
“Neither my husband or I had ever dealt with anybody that's had epilepsy before,” Keele said. “So this was a big learning experience for both of us. In the very beginning, we were scared to death all the time because of what we didn’t know and all the scary things that you hear about epilepsy.”
It’s a disease wrapped in a lot of stigma and misunderstanding, Keele said, and their family—Wyatt especially—has experienced the resulting isolation. But they remain guardedly optimistic about the future.
“As parents we try very hard to make sure Wyatt doesn’t feel like he is different,” Keele said. “We feel that is so important because he always says he just wants to be normal. Unfortunately, this is our normal, but we try to tell him God made him this way because he’s one tough kid and can handle anything thrown at him. We are excited because this could be life-changing for him.”
A seizure can happen to anyone, anywhere at anytime, so it’s important to know the signs and symptoms and how to help when you witness someone having a seizure. To learn more about epilepsy and available treatments, visit Conditions Treated: Epilepsy at cookchildrens.org. For a consultation or referral, call 682-885-2500.