Drug-Resistant Seizures: Derek’s Story
Thirteen-year-old Derek Ahlstedt just wants to do what other teens his age do—play baseball with his friends, go to scout camp, and one day, drive a car.
But a lifetime of epileptic seizures put Derek’s dreams on hold, until now.
The seizure roller coaster
Early on, Derek seemed like a normal, healthy baby. He hit all his developmental milestones right on target and was progressing well.
But at about 18 months old, his parents, Sarah and Mike Ahlstedt, Topeka, Kansas, noticed some unusual tremors as their son woke up from a nap one day.
“I remember there was a lot of body trembling and Derek didn’t seem quite right,” Sarah described. “Mike and I weren’t sure what to think.”
A couple of weeks later, Derek had two seizures in one day. Though the first didn’t seem too serious, the second one was.
“We were in Kansas City and I was carrying Derek into a mall,” Mike said. “He literally had a seizure in my arms as we were walking in.”
Sarah immediately called Derek’s pediatrician in Topeka. He suggested they take him to the closest emergency room.
“We did, and they transferred Derek to the Children’s Mercy Kansas City Adele Hall Campus,” Sarah said.
After several tests and a few days in the hospital, Derek was released to go home on an anti-seizure medication.
That first medication worked, and the Ahlstedts breathed a sigh of relief. But 18 months later, Derek had another seizure, and another, and another.
“Since then, it seems like we’ve been on a roller coaster of medications and seizures,” Sarah said. “Derek has been on six or seven different medications and combinations of medications over the years. Sometimes he’ll be seizure-free for a few weeks, and sometimes for a few months. They’ve been difficult to manage.”
Unfortunately, Derek’s experience is typical for patients whose seizures don’t respond well to medications. About 35 percent of patients with epilepsy continue to have seizures, despite treatment with multiple medications.
These patients also may suffer from cognitive dysfunction, depression, accidents and medication side effects. Mike said the seizures and medications have taken a toll on Derek cognitively.
“When you would ask Derek a question, he was very slow to respond. We’ve noticed it, his teachers mentioned it and even the doctors at Children’s Mercy saw it,” he said.
Life with seizures
The impact on the family’s life hasn’t been easy either. “We are used to Derek’s seizures,” Sarah said, “and we haven’t let them stop him from taking part in the things he enjoys.”
Derek has taken swimming lessons, has gone to scout camp, and has played baseball and basketball, but it’s always been under the watchful eye of his parents, a trusted friend or a relative.
Activities that are common for other kids, like sleepovers, have required extra caution. “We had to be sure the parents of his friends understood how important it was for Derek to take his medications on time and to not stay up late, or it could trigger a seizure.”
While other parents have been supportive and helpful, Mike and Sarah naturally have worried when Derek was away. “There was always the concern that he might have a seizure, and that someone else wouldn’t know what to do,” Sarah said.
Derek’s younger brother, Jason, has worried so much about his brother that he sleeps in the same room.
“Jason has his own room,” Mike said, “but he was concerned that if Derek had a seizure at night, no one would hear him. Little brother definitely looks out for big brother.”
Monitoring Derek’s seizures
Over the years, Derek has had a couple of stays in the Epilepsy Monitoring Unit (EMU) at Children’s Mercy Hospital Kansas.
The EMU is one important aspect of the Comprehensive Epilepsy Center at Children’s Mercy, which has been recognized by the National Association of Epilepsy Centers as a Level 4 Center, the highest level in epileptic care.
While there, a multidisciplinary team of providers continuously monitors seizure activity and works with the patient and family to better manage the child’s seizure disorder. These specialists include pediatric epileptologists, neuropsychologists, advanced practice registered nurses (APRNs), electroencephalogram (EEG) and radiologic technologists, nutrition and dietary specialists and social workers.
The center’s EEG lab performs tests to evaluate and record brain wave patterns to detect potential problems, and is certified by the American Board of Registration of Electroencephalographic and Evoked Potential Technologists, or ABRET.
This means the Children’s Mercy technologists and facilities meet the highest standards for providing excellent quality EEGs, the test used to monitor and evaluate brain activity. Fewer than 10% of labs across the country achieve this certification.
After Derek’s EMU stay in June 2019, there was some promising news. Ara Hall, MD, pediatric neurologist, reviewed Derek’s EEG results, telling the family that Derek could be a good candidate for surgery.
“Dr. Hall said the EEG indicated that most of Derek’s seizure activity was centered in the right temporal region of his brain,” Sarah explained. “Because the activity was focused, she thought surgery might be an option now.”
New technology, New hope
For patients like Derek whose seizures don’t respond well to medications, surgery to remove the area of the brain responsible for the seizures can, in most cases, successfully and safely stop or reduce the number and severity of seizures. Surgery was the Ahlstedt family’s best hope for an effective treatment.
“We tried not to get too far ahead of ourselves,” Sarah said.
The next step was for Derek’s case to be presented at the multidisciplinary Epilepsy Surgery Conference, held bimonthly. This conference includes the epileptologists, neurosurgeons, neuroradiologists and neuropsychologists.
As part of his pre-surgical work-up, Derek had several multi-modal imaging studies. These included high-resolution and functional MRI studies, a CT scan and PET imaging, which were integrated to provide optimal information for locating the source of Derek’s seizures.
Once the conference team agreed that Derek was a good surgical candidate, he advanced to pre-operative visits with the surgical and anesthesia teams.
However, in order for Derek to be able to successfully undergo removal of the dysfunctioning region of his brain generating the seizures, the area had to be carefully located with surgical precision. This can only be done by directly implanting electrodes into the brain.
Christian Kaufman, MD, a pediatric neurosurgeon who specializes in epilepsy surgery, used a new, state-of-the-art surgical navigation and positioning system known as the ROSA® Brain, to accurately pinpoint the source of seizure activity without having to perform a large, open surgery. Children’s Mercy is one of only 15 pediatric hospitals in the nation currently using ROSA.
“ROSA allows us to collect information from the brain in a minimally disruptive way,” Dr. Kaufman said. “Instead of removing a large portion of the patient’s skull, now we can get that same information through very small incisions that are not disruptive or cosmetically disfiguring.”
The ROSA robot acts like a GPS system, guiding the surgeon in the placement of multiple EEG electrodes via small burr holes in the patient’s skull. These electrodes monitor seizure activity, identifying the location of the seizures in a less invasive way. ROSA also increases the number of patients who may be candidates for this procedure, versus an open craniotomy.
In Derek’s case, the ROSA-implanted electrodes identified more than 10 seizures during his stay. “We only witnessed a couple of the seizures,” Sarah said. “We were worried the team wouldn’t have enough information to move forward with the resection.”
But Lalit Bansal, MD, pediatric neurologist, explained that Derek was having frequent seizure activity that Mike and Sarah couldn’t see.
“That’s when I realized the seizures we could see were just the tip of the iceberg,” Mike said. “Derek’s brain was experiencing constant abnormal brain activity.”
A few days later during the same hospitalization, and guided by the data gathered from the ROSA implanted electrodes, Dr. Kaufman removed the small section of Derek’s brain responsible for most of the seizure activity. After a total of eight days, Derek was released from Children’s Mercy.
“That wasn’t a moment too soon for Derek,” Sarah said. “He was ready to go home.”
Life after surgery
It’s now been more than six months since Derek’s resection surgery, and six months since his last seizure!
“We are hopeful that we can potentially cure Derek’s seizure activity,” Dr. Kaufman said. “At the very least, we hope he will see a significant improvement in his quality of life.”
“Our ultimate goal is for Derek to be seizure-free,” Sarah said. “But even if that isn’t possible, it would be wonderful if he could have fewer seizures and take fewer medications.”
Functionally, Derek’s parents say he’s doing better than ever, and so is their family!
“We’ve noticed that Derek is much quicker to respond to questions, and his teachers say he expresses himself much more in class,” Sarah said.
“I’ve noticed his motor skills have improved too,” Mike added. “We can play catch now.”
Jason, Mike and Sarah also are more relaxed and worry less about Derek when he spends the night at a friend’s or visits his grandparents.
“As a mom, you always worry, but it’s not as bad as it used to be,” Sarah said. “Before surgery, we wouldn’t leave Derek home alone. Now, if we need to run to the store or be away for a couple of hours, we are more comfortable doing that. It’s so nice to be off that roller coaster.”
As Derek prepares to enter the 8th grade, he’s enjoying his new-found independence, participating in scouting and camping, and looking forward to the possibility of driving one day.
“A year ago the team’s neuropsychologist, told us that driving was not an option for Derek,” Mike said. “But he’s come a long way in the past year. Now it may be.”
And though it was tough to see Derek go through brain surgery, Sarah and Mike say it wasn’t a hard decision to make.
“Knowing the possible outcome and the improvement in Derek’s quality of life after surgery made it all worthwhile,” Mike said.
“We had great confidence in the Children’s Mercy Epilepsy team,” Sarah added, “and we’ve had an amazing experience. Everyone, from Erin D. Fecske, CPNP, Derek’s advanced practice provider, to Dr. Kaufman and the neurosurgery team, to Child Life, to Social Work, to the EEG tech, they were all easy to work with. They answered every question and put us at ease.
“And it was wonderful to be able to have Derek’s surgery so close to home,” Sarah added. “We had the opportunity to go to the Mayo Clinic, but being an hour from Topeka during an eight-day hospital stay was convenient.
“Mike and I took turns staying with Derek, and Jason was able to visit his brother frequently. That wouldn’t have been possible if we had gone to Minnesota. We’re just thankful Children’s Mercy is so close to us.”
Pediatric epilepsy: Gary's story
Christian Kaufman, MD, Children’s Mercy pediatric neurosurgeon, performs an intricate surgery to remove abnormal brain tissue, which should help stop severe epileptic seizures for Gary. Months later, Gary is seizure-free, and is slowly being taken off his anti-seizure medications.
Pediatric Neurosurgeon; Assistant Professor of Surgery, University of Missouri-Kansas City School of Medicine; Clinical Assistant Professor of Neurosurgery, University of Kansas School of Medicine