During the seizures – which would strike at any hour throughout the day and night – Ruth would spend hours simply holding Rachael and comforting her. Over time, Rachael had tried 14 different medications to reduce her daily seizures. While the medications brought her seizure count down from about 50 per day to 10 to 15 per day, more needed to be done. The medication was working, but not well enough to give Rachael the quality of life she deserved.
It got to the point where Rachael visited Children’s Hospitals and Clinics of Minnesota at least once a month due to long or very frequent seizures. She also had a high risk of pneumonia – very common in children with epilepsy.
One in 26 people will develop epilepsy in their lifetime, making it more common than most people realize. Of that population, one in three have difficult-to-control seizures that don’t always respond to medication – patients like Rachael. Rachael suffers from a rare neurological disorder called Aicardi Syndrome, characterized by seizures and a partial or complete absence of the corpus callosum, the structure that connects the left part of the brain to the right part of the brain. Aicardi Syndrome affects about 4,000 people worldwide, most of them newborn girls who first show signs of the condition through “infantile spasms.” Today, Rachael has significant developmental delays, including the inability to speak.
Nevertheless, Steve and Ruth have a special way of communicating with their daughter. She smiles at her parents responsively, makes eye contact and enjoys activities children her age enjoy, such as attending festivals, football games or community celebrations. But medication often made Rachael drowsy and limited her interactions with others, including her parents. She needed therapy that could better treat her seizures and give her a better chance to engage with life.
Looking beyond medication
That’s why Steve and Ruth were willing to try a device called the AspireSR – a non-medicinal approach they viewed as a last resort for reducing Rachael’s seizures.
Implanted in the chest, the device detects oncoming seizures by sensing a change in heart rate. When the device detects a rapid rise in heart rate (oncoming seizure), it then stimulates the vagus nerve (one of two extremely long cranial nerves that extend from the brain to the abdomen) and provides information to the brain in time to interrupt or significantly shorten the seizure. The device also comes with a magnet that caregivers can swipe over the device to stop a seizure without medication. For patients like Rachael who don’t always respond to medication, devices such as this have major implications for an improved quality of life. In clinical studies, seizures were resolved in patients 60 percent of the time. In addition, 50 percent to 60 percent of patients using the device see seizures cut in half or more in the first year of treatment, and may see additional improvement over time.
On July 30, 2015, Rachael underwent surgery at Children’s Minnesota to have the device implanted in her chest, making her one of the first pediatric patients in the country and the first patient in Minnesota to receive the device.
How the smallest improvement can be the biggest achievement
When Rachael first began experiencing seizures at six months, her parents’ lives changed drastically. Their days were suddenly filled with hospitalizations, doctor visits and injecting Rachael with adrenocorticotropic hormone (ACTH) to control her infantile spasms. As Rachael grew older her infantile spasms turned into seizures, and the Malezes continued to seek the best treatment possible. Since receiving the device, Rachael is more alert, interactive, and visits the hospital less frequently. Knowing the device responds in realtime to seizures, Steve and Ruth feel more confident not being in the same room with Rachael, such as when she’s riding the school bus or sleeping at night. These small improvements have made a huge impact on the Malez’s day-to-day life and Steve and Ruth look forward to watching Rachael experience more of the joys of being a kid.
It’s only the beginning
For a child, the only thing worse than being in the hospital is returning; the goal for Rachael is that she can stay home with her parents and out of the hospital. Children’s Minnesota continues to seek new neurological diagnostic services and treatment, like AspireSR, for patients with epilepsy, and is engaged in research aimed at delivering new products and treatments in this field. Kids just want to be kids, and as doctors in this field, we have a responsibility and drive to seek effective treatments for these children – the most amazing people on earth.
Dr. Meysam A. Kebriaei is a pediatric neurosurgeon at Children’s Hospitals and Clinics of Minnesota. He received his bachelor of science degree from University of Minnesota Twin Cities. Dr. Kebriaei attended medical school at the University of Nebraska Medical Center and completed his neurosurgery residency at the University of Nebraska Medical Center. He completed his fellowship in pediatric neurosurgery at Emory University. Dr. Kebriaei was inducted into the prestigious Alpha Omega Alpha medical honor society while a chief resident at the University of Nebraska. He has published several peer reviewed articles and is involved in ongoing research projects.
Dr. Kebriaei’s interests include brain and spinal cord tumors; vascular malformations of the brain and spine; cerebral vascular surgery; minimally invasive spine and pediatric spine surgery with and without instrumentation; congenital anomalies of brain and spine; hydrocephalus, congenital and post hemorrhagic; endoscopic surgery; Chiari malformation; craniosynostosis; epilepsy surgery; perinatal consultation; traumatic brain and spine surgery; and spinal dysraphism.
Dr. Dimitrios Arkilo is a pediatric neurologist at Minnesota Epilepsy Group, P.A. He is board certified in pediatrics and neurology with special qualifications in child neurology and epilepsy. He attended Semmelweis University of Medicine in Budapest, Hungary and completed his child neurology residency at Tufts University-Floating Hospital for Children in Boston. Dr. Arkilo received several awards for excellence in teaching and research from Tufts University Department of Neurology while he was a chief resident at Floating Hospital for Children. He completed his fellowship in neurophysiology at the University of Minnesota-Twin Cities. Dr. Arkilo has published many peer reviewed articles and is involved in ongoing research projects.
Dr. Arkilo’s interests include pharmacogenomics and genetics in epilepsy and the treatment of rare epilepsies. He is the director of the chromosome 15 dup clinic at Minnesota Epilepsy Group, P.A. and serves on the genomics committee at Children’s Hospitals and Clinics of Minnesota.
The views, opinions and positions expressed within these guest posts are those of the author alone and do not represent those of Becker’s Hospital Review/Becker’s Healthcare. The accuracy, completeness and validity of any statements made within this article are not guaranteed. We accept no liability for any errors, omissions or representations. The copyright of this content belongs to the author and any liability with regards to infringement of intellectual property rights remains with them.
