Teen Becomes First in North Texas to Undergo Landmark Brain Surgery for Epilepsy

Luke Waggoner’s epileptic seizures were getting worse, striking multiple times a day sometimes in back-to-back clusters that sent him to the hospital.

The seizures caused the 13-year-old Arlington boy to jerk or jump uncontrollably. He might fall backward so forcefully that he’d bruise. Other seizures left Luke mute, confused and unresponsive.

Luke has Lennox-Gastaut syndrome, a rare type of difficult-to-control epilepsy. His seizures are medically refractory, having failed many medications and other non-pharmacological treatments. Even with five different medications taken multiple times daily, Luke was still having breakthrough seizures, the erratic misfires between neurons in his brain. These seizures often resulted in trips to the emergency room and frequent admissions to the hospital. The medications and frequent seizures also make it hard for him to think and speak clearly.

In the midst of the setbacks came a new treatment option: deep brain stimulation (DBS), a surgical therapy that utilizes electrodes, wires and a generator to modulate the brain’s abnormal electrical impulses. The movement disorders team at Cook Children’s has been utilizing DBS for dystonia since 2007 in children as young as 7 years of age. DBS was recently approved by the U.S. Food and Drug Administration (FDA) for adult patients with Lennox-Gastaut syndrome.

Luke was facing the prospect of undergoing a corpus callosotomy for his epilepsy. This irreversible neurosurgical procedure permanently severs most of the connections between the two halves of the brain to prevent drop seizures, the most dangerous and disabling seizures often seen in Lennox-Gastaut syndrome. Based on extensive experience with pediatric DBS, the movement disorders and epilepsy teams collaborated with Luke’s family about the potential to offer DBS to Luke as an alternative to callosotomy. If the DBS did not work, callosotomy remained an option.

After much discussion and planning, the decision was made to implant temporary electrodes in two sites on each side of Luke’s brain to assess the impact on his seizures and potential unwanted effects. In April 2021, Luke had the temporary leads implanted. After several days of continuous monitoring on the specialized epilepsy unit trying different stimulation settings, the sites for permanent leads were chosen in consultation with Luke and his family. A detailed proposal including the data from the trial was used to get insurance approval for the placement of DBS.

Then in a two-part landmark surgery at Cook Children’s – on July 8, when electrodes were implanted in his thalamus; and on July 14, when wires were placed through his neck to the generator in his abdomen – Luke became the first pediatric patient in North Texas to undergo DBS for intractable epilepsy since the FDA approved the treatment. He also became the first child in the United States to receive the newly approved sensing lead technology DBS system for epilepsy.

“Deciding to do the DBS and for Luke to be the first pediatric patient at Cook Children’s for epilepsy was a very difficult decision, and we did not take it lightly,” said his mom, Ami Waggoner. “We just knew we had to do something to try to help him.”

Cynthia Keator, M.D., medical director of the Epilepsy Monitoring Unit at Cook Children’s, expressed optimism about the potential to mitigate Luke’s seizures for years ahead. Other desired outcomes from the ongoing brain stimulation? Better cognitive function, fewer meds, greater independence and a more normal lifestyle.

“Our hope is that not only will this immediately start to show improvement in his seizures, but give him a chance to have a better quality of life, to be able to go back to school in person, to be able to go outside and not worry about falling down or having a seizure, and to be able to taper off of some of his medications,” Dr. Keator said.

Since 2007, the Jane and John Justin Neurosciences Center at Cook Children’s has established a record of excellence in deep brain stimulation, providing the surgical therapy to almost 150 patients with a movement disorder called dystonia, currently FDA-approved for pediatrics. Luke is a pioneer in DBS because of his underlying condition … epilepsy rather than dystonia.

Ami and Tim Waggoner said their son already has made big strides since his two DBS surgeries in July. His seizure count is down from the pre-surgery norm of five to 10 per day, she said, and the seizures that still occur aren’t the dangerous variety that requires additional “rescue” medications. Luke is able to read, play with Legos and his beloved trains, and go for short trips in the car. He started weaning off one of the drugs he takes. They are amazed at the change.

“You can just look in his eyes and see he’s more with it,’’ Ami said. “This is all really, really exciting. He knows he’s feeling better.” And from Tim: “I’m seeing more energy, fewer seizures, clearer speech and he is able to do more! It is just amazing the difference in just over a month since turning on the generator.” 

Let’s take a closer look at epilepsy facts, the precision involved in deep brain stimulation, and the route Luke took to becoming the first patient to undergo this new treatment for childhood epilepsy.

Epilepsy explained

The U.S. Centers for Disease Control and Prevention estimates that 3.4 million people nationwide have epilepsy, including 470,000 children. There is no cure and often no identifiable cause. Epilepsy is a chronic disorder that results from sudden intense bursts of electrical activity in the brain, manifesting a range of seizure types.

Someone who’s having a seizure might collapse, twitch or spasm, stiffen, blink rapidly, stare blankly, or lose consciousness depending on the type. Medication successfully controls the seizures in up to 80% of children with epilepsy.

Luke was diagnosed at age 5 with generalized epilepsy, which affects both hemispheres of his brain. Big sister Lexi didn’t know what was happening when she witnessed the first seizure.

“He couldn't hear me. And he started walking in a circle and then he just fell over and turned blue. Seeing that freaked me out,” Lexi remembered. “The first few years were really hard for me to understand and get used to it. But now it's to the point where it's just a part of our everyday lives.”

Medications helped at first, his mom said, but the seizures started getting more dangerous and debilitating about three years ago. Ami, who is a nurse, could administer the rescue medications at home when the seizures got especially bad. But even then, about twice a month Luke required hospitalization and intravenous therapies to stop the back-to-back clusters.

Dr. Keator said electroencephalography on Luke found the two distinct patterns of brain waves indicative of Lennox-Gastaut syndrome, (slow spike-and-wave complex and generalized paroxysmal fast activity). A vagus nerve stimulator, which uses a pacemaker-like device implanted in his chest, worked for a while for Luke, but the seizures and hospital stays kept recurring.

Game-changer potential

Warren Marks, M.D., director of the Movement Disorders Program at Cook Children’s, visited Luke’s hospital room in February 2021. Dr. Marks mentioned the prospect of deep brain stimulation, which had been approved by the FDA since 2018 for adults with epilepsy. Cook Children’s anticipated that the FDA’s green light for DBS in epileptic children was on the horizon, and Luke seemed like an ideal candidate.

“Dr. Marks just really believed he could help Luke. He gave us a spark of hope,” Ami recounted. “We were on board from the beginning because of the trust I have in the physicians and Luke’s neurology team. They're just amazing. They have never, ever given up.”

Dr. Marks explained that DBS sends small electrical impulses to targeted areas of the brain to alter the abnormal movements seen in dystonia as well as tremors and Parkinson’s disease. Results were encouraging in the almost 150 dystonia patients who underwent DBS in the past 14 years at Cook Children’s. Dr. Marks thought the therapy held promise for epilepsy patients too.

Collaboration between the Cook Children’s movement disorders and epilepsy teams had already been underway to adapt technology and share expertise, Dr. Marks said. The next step was a weeklong trial in April to gather data on Luke’s tolerance for different electrical amplitudes. And the doctors needed to know exactly where to implant the DBS devices.

“We recorded and stimulated different places in the brain to decide which seemed to be beneficial, but also which didn’t cause him unwanted side effects,” Dr. Marks said. “When you stimulate the brain in these areas, sometimes you get thing that you don’t want. We were trying to find one target that would give us the best chance of success.”

Not only did the April testing phase produce essential data, but during that practice run Luke spoke more clearly and felt better than he had in years, his mom said. So DBS was scheduled for July. John Honeycutt, M.D., medical director of Neurosurgery at Cook Children’s, is the surgeon who implanted several electrodes bilaterally in the centromedian nucleus of Luke’s thalamus, the relay center for transmitting signals in the brain.

The DBS system consists of three main components:

• Leads (pronounced “leeds”) – tiny electrodes embedded deep in the brain to deliver the electricity directly to the target area. They’re held in place by caps screwed into the skull.
• Generator – a mini-computer under the skin of the chest (the abdomen, in Luke’s case). Wires run through the neck to connect leads to a generator. In some cases, the battery is rechargeable.
• Programmer – a tablet that talks to the generator, regulating the strength and frequency of electrical impulses per second. Settings can be adjusted based on the patient’s response via Bluetooth connection.

Dr. Marks described DBS as flexible, specific and less invasive than other surgical approaches. Primary candidates are the patients like Luke whose seizures originate in both halves of the brain. “This has the potential to be an absolute game-changer,” Dr. Marks said. “It’s essentially like delivering medication without all the medication side effects. That’s one way to think about this. We are directly targeting the area of interest without bathing the rest of the brain with unwanted chemicals.”

The mechanism of action of a DBS in epilepsy is not fully understood. Scientific studies have supported that certain thalamic nuclei of the brain, specifically the centromedian nuclei, are generators of the slow spike-and-wave complex and paroxysmal generalized fast activity seen in patients with Lennox-Gastaut syndrome. Studies have shown favorable seizure reduction over time possibly through modulation of network excitability through stimulation of the centromedian nucleus of the thalamus. 

Doctors will continue to monitor Luke and adjust his settings as needed. Dr. Keator hailed Luke’s patience, good humor and cooperative attitude. He considers the medical team at Cook Children’s his best friends and ploy for pranks. “He’s just a trooper, and he lets us try new things with him, which we appreciate,” Dr. Keator said. “He’s just ready to get his life going, and he’s motivated. And that makes our job a lot easier.”

Luke said having epilepsy “can be a little tough at times.” He’s glad for all the care he received at Cook Children’s and the chance to potentially pave the way for DBS in other children who have seizures. “And I hope I get better so I can go on beach vacations and go places to ride lots of trains,” he said.

His parents look forward to the possibility of Luke’s epilepsy improving to the point that he can go to school, sleep over at a friend’s house, or travel without having a seizure. They are cautiously optimistic that deep brain stimulation will provide long-term relief for Luke and other children with epilepsy. And they hold out hope that this latest twist in Luke’s journey can blaze a trail for wider options in epilepsy care.

“I prayed a lot about it. I believe a lot of things happen for a reason. I think Luke is here to show us a story, to teach us something, to show us how brave he is,” Ami said. “His attitude the whole time has been basically ‘I just want to do this to help other kids.’”