Modification of a Parent-report Sleep Scale for Individuals with CDKL5 Deficiency Disorder: A Psychometric Study
Abstract number :
3.105
Submission category :
11. Behavior/Neuropsychology/Language / 11B. Pediatrics
Year :
2024
Submission ID :
471
Source :
www.aesnet.org
Presentation date :
12/9/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Jacinta Saldaris, PhD – Telethon Kids Institute
Scott Demarest, MD – Children's Hospital Colorado
Peter Jacoby, MSc – Telethon Kids Institute
Heather Olson, MD, MS – Boston Children's Hospital, Harvard Medical School
Kiran Maski, MD – Boston Children’s Hospital
Elia Pestana Knight, MD – Cleveland Clinic Epilepsy Center
Dana Price, MD – NYU Langone Health
Rajsekar Rajaraman, MD, MS – UCLA Mattel Children's Hospital
Bernhard Suter, MD – Baylor College of Medicine
Judith Weisenberg, MD – Washington University of St. Louis
Helen Leonard, MBChB – Telethon Kids Institute
Eric Marsh, MD PhD – Children's Hospital of Philadelphia
Tim Benke, MD – University of Colorado School of Medicine
Jenny Downs, PhD – Telethon Kids Institute
Rationale:
Sleep difficulties are common in CDKL5 deficiency disorder (CDD; 87%), a developmental and epileptic encephalopathy (DEE), and are rated as a high priority by parents. The Sleep Disturbance Scale for Children (SDSC) was developed and validated for children in the general population but has been used in populations with cerebral palsy, autism and other intellectual disabilities. The SDSC has 26 items that load to six domains that describe “Disorders of Maintaining Sleep” (DIMS), “Sleep Breathing Disorders” (SBD), “Disorders of Arousal and Nightmares” (DAN) “Sleep Wake Transition Disorders” (SWTD), “Disorders of Excessive Somnolence” (DOES), and “Sleep Hyperhidrosis” (SHY). Because of the potential clinical relevance, this study evaluated the factor structure of the DIMS, DOES and SBD domains of the SDSC for CDD.
Methods:
A cross-sectional psychometric study design was used. Data were collected for 125 individuals aged 3 years or older who attended a US Center of Excellence clinic for CDD or were registered with the International CDKL5 Disorder Database. Psychometric analysis included confirmatory factor analysis (CFA), and evaluation of item factor loadings, internal consistency, Average Variance Extracted (AVE), discriminant validity and goodness of fit.
Results:
The median age was 10.3 years (range 3.2 – 40.7 years) and 105 (84%) were female. Two of the three SBD items were not observed by most respondents, so CFA was restricted to the DIMS and DOES domains. Using all DIMS and DOES items in the initial CFA, two items in the DIMS domain and one item in the DOES domain loaded poorly (lower confidence interval < 0.4). After deleting these items and repeating the analysis, item loading (0.524-0.814) and internal consistency (DIMS: 0.78, DOES: 0.76) statistics were good. The square of the inter-domain correlation coefficient was 0.17, less than AVE values for both domains, indicating good discriminant validity of the model domains. The Tucker-Lewis and Comparative Fit indices were slightly lower than the threshold of >0.9 for establishing goodness of fit.
Conclusions:
Our study suggests that modification of the existing DIMS and DOES domains of the SDSC yielded acceptable psychometric characteristics. Whilst SBD may be important for some children with CDD, there was very little variation in responses (majority scoring “never”) suggesting that it is a poor measure of this concept for this population. The DIMS and DOES domains could be suitable clinical outcome assessments of insomnia and daytime sleepiness in CDD and potentially other DEE conditions. However, further analyses with a larger and diverse sample, potentially with other DEEs, would be required.
Funding: This research was supported by funding from the International Foundation for CDKL5 Research and the NIH/NINDS (U01NS114312).
Behavior