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

Abstract

ObjectiveThe 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.Patients and methodsFace-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.ResultsSimilarities 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).ConclusionThe 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.

The importance of social zeitgeber in paediatric type 1 narcolepsy: What we can learn from the COVID‐19 restrictions adopted in Italy?

“…Eighteen NT1 children and adolescents (11 males, mean age: 14.44 ± 2.01 years, range 10–17 years) accepted to participate and to wear an actigraph continuously for 14 days. Actigraphs (Micro Motionlogger Watch, Ambulatory Monitoring) have been sent to participants’ homes, along with the Epworth Sleepiness Scale for children and adolescents (ESS‐CHAD) and the Italian version of the consensus sleep diary, which participants have to fill in daily (Carney et al, 2012 ; Wang, Benmedjahed, & Lambert, 2017 ). Moreover, participants were asked to report their height and weight upon receiving the device.…”

Evaluation and Treatment of Children and Adolescents With Excessive Daytime Sleepiness

“…40 Instruments used specifically to screen for EDS in the pediatric population include the Pediatric Daytime Sleepiness Scale 41 and Modified Epworth Sleepiness Scale for Children and Adolescents. 42,43 Another screening tool that is useful in identifying potential causes of EDS is a 24-hour, 2-week, parent-recorded (or self-recorded for adolescents) sleep diary. 13 Sleep diaries allow detailed documentation of variations in sleep patterns and sleep-wake cycles in real time compared with descriptive histories recalled in the clinic.…”

In-field assessment of sodium oxybate effect in pediatric type 1 narcolepsy: an actigraphic study

“…At baseline and at 1 year follow-up evaluations, children underwent a semistructured interview and completed the Epworth Sleepiness scale for children and adolescents (ESS-CHAD) [28], and the Italian version of the reduced Morningness-Eveningness questionnaire for children and adolescents (rMEQ-CA) [29].…”