Assessing narcolepsy with cataplexy in children and adolescents: development of a cataplexy diary and the ESS-CHAD

• Published in: Nature and science of sleep 2017-01, Vol.9, p.201-211
• Main Authors: Wang, Y Grace, Benmedjahed, Khadra, Lambert, Jérémy, Evans, Christopher J, Hwang, Steve, Black, Jed, Johns, Murray W
• Format: Article
• Language: English
• Subjects: Adolescent psychology; adolescents; Adults; assessment; Care and treatment; cataplexy; Child development; Child psychology; children; Diagnosis; Narcolepsy; Original Research; Patients; Pediatrics; Product development; Psychological research; Self-rated health; Sleep disorders; sleepiness; Sodium; Teenagers; Terminology
• ISSN: 1179-1608; 1179-1608
• DOI: 10.2147/NSS.S140143

The aim of this study was to qualitatively evaluate concepts for incorporation into a daily diary to capture cataplexy frequency and to assess the content validity of the Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD) in pediatric patients with narcolepsy. Face-to-face concept elicitation and cognitive interviews were conducted with children (7-9 years; n=13) and adolescents (10-17 years; n=16) who have narcolepsy with cataplexy, and their parents/caregivers. Similarities and differences were noted between narcolepsy concepts described by children and their parents/caregivers, suggesting some different but complementary perspectives; parents may not recognize cataplexy symptoms/triggers as well as children, but parents have greater recognition of the circumstances of falling asleep. Cataplexy diary modifications included changes in definitions and examples of cataplexy, using child-friendly terminology, adding a quantitative question to determine daily frequency, and standardizing the questionnaire for evening administration with self-completion by the child. Modifications were made to ESS-CHAD for child-friendly wording and to ensure that items reflect activities (eating, watching TV/video) and environments (school, bus/car transport) in which children are likely to participate. Two ESS-CHAD versions were proposed: one with a 1-month recall period, for general use, and the other with a recall period of "since your last study visit," for research, which could be shorter or longer than 1 month (as short as 1 week). The cataplexy diary and ESS-CHAD were modified for the assessment of children and adolescents. Further psychometric validation is recommended. These measures are being used in a Phase III, placebo-controlled clinical trial of sodium oxybate in children and adolescents with narcolepsy.