Jan 14, 2022
stubborn seizures lead to rare condition
Emalyne had her first seizure when she was only 3 months old. New parents, Michele and Jeffery Wolfe, didn't understand what was happening to their baby or why one side of her face was twitching. Just as they secured Emalyne in her car seat to go to the emergency department, her arm and leg on the opposite side of her body began twitching as well. By the time they reached Dayton Children's Hospital, Emalyne had been seizing for an hour.
Her seizure stopped once she settled into her emergency department room and providers quickly went to work running a variety of tests. All test results came back inconclusive.
The following month, Emalyne had another seizure, leading her family to the emergency department at Dayton Children's once again. This time, the providers in the emergency department referred Emalyne to be seen by a neurologist.
Gogi Kumar, MD, division chief of neurology at Dayton Children's Hospital saw Emalyne shortly after her second trip to the emergency department. Dr. Kumar started Emalyne on seizure medication in hopes of reducing the frequency and severity of her seizures. Unfortunately, Emalyne's body didn't respond to the medication. More medication was introduced, and different seizure types started to present. These symptoms led Dr. Kumar to order more testing for Emalyne because this was not your typical epilepsy.
Eight months and countless tests after her first seizure, Emalyne was diagnosed with Dravet syndrome. Dravet syndrome is a lifelong, rare, drug-resistant epilepsy that begins in the first year of life in an otherwise healthy infant. Seizures in Dravet syndrome are difficult to control. As seizures increase, the pace of acquiring skills slows and children start to lag in development. Children with Dravet syndrome may also experience changes in eating, appetite, balance, and a crouched gait (walking).
"Everyone's experience is different with Dravet syndrome," said Michele. "But the most important thing to me is my child's quality of life, and Dr. Kumar understands this. She continues to look for the best ways to manage Emalyne's medication and condition."
When Emalyne was 7 years old, she received her first vagus nerve stimulator (VNS) treatment. VNS is used when surgery is not possible or does not work. A small electrical generator is implanted under the skin over the chest and is placed on a set schedule to stimulate the vagus nerve. Over time this helps to reduce the number or severity of seizures a child has.
As of today, Emalyne has undergone three surgeries for vagus nerve stimulator implantation and only has about one seizure per month, and her recovery time only takes five to ten minutes. Prior to her VNS implantations, Emalyne was having several seizures each month. And with each of those seizures she would need a day to sleep and recover.
Emalyne is now a sophomore in high school. She attends school with her seizure alert dog Sif and has a nurse who takes care of her and knows how to react if a seizure occurs. She continues to see Dr. Kumar every two to three months to manage her medication and make sure she has the best quality of life. Emalyne also sees Dr. Pfeifer in endocrinology for her thyroid and Dr. Mowery in PM&R for her crouched gait.
Although the management of Emalyne’s seizures is under control, Michele is still hopeful that a new medication will become available to give Emalyne an even better quality of life.