Knupp KG, Scarbro S, Wilkening G, Juarez-Colunga E, Kempe A, Dempsey A. Parental Perception of Comorbidities in Children With Dravet Syndrome. Pediatr Neurol. 2017 Nov;76:60-65.
We hypothesized that children with Dravet syndrome may have additional common features beyond seizures and cognitive impairment. To address this gap in knowledge, we conducted a survey of caregivers of children with Dravet syndrome to identify and quantify their perception of associated symptoms in this population.
An electronic survey was developed in REDcap (Research Electronic Data Capture) and sent via e-mail to the participants on the Dravet Syndrome Foundation e-mail list. Questions focused on eating, sleep, behavior, and other symptoms that might be related to Dravet syndrome. The questions were assessed using a four-point Likert scale (e.g., strongly agree to strongly disagree). Results were later dichotomized for analysis. Logistic regression was used to calculate odds ratios of various demographic factors potentially associated with symptoms. Multivariable models were constructed using backward elimination to assess the relationship among a variety of symptoms.
There were 202 respondents, 96% were parents of a child with Dravet syndrome (the remainder were grandparents or guardians); 90.5% were female. The median age of the affected child was eight years (interquartile range five to 14), 50% were male, and 90.5% were reported to have a known SCN1A mutation. At least one symptom associated with appetite was reported in 99% of respondents, 82% reported a disturbance of sleep, one third reported autonomic symptoms, and 75% reported problems with gait. Inattention and perseveration were reported more commonly than other behavioral disturbances.
Caregivers have the perception of many symptoms in children with Dravet syndrome in addition to those that have been previously reported, including appetite, sleep, gait, and behavior. Many of these can significantly affect quality of life for both the child and the caregiver.