Sleep Health in Children and Adolescent with Sickle Cell Disease
Authors: Rani Seema , Chidekel Aaron , Strang Abigail , Miller Robin E , DiVirgilio Nicole , Lewis Amanda M. , Hildenbrand Aimee K. , Canter Kimberly , Patterson Freda 
Published: December 2024

Background: Youth with sickle cell disease (SCD) are at increased risk for sleep-related breathing disorders, sleep disturbances, and other sleep impairments including reduced sleep duration, increased nocturnal awakenings, and excessive daytime sleepiness. Poor sleep is associated with increased risk for Vaso-occlusive crisis (VOC) and poor health-related quality of life.
Aim: We aimed to describe multiple aspects of sleep health in youth with SCD compared to healthy controls.
We also sought to examine the relationship between indicators of sleep health and healthcare utilization (HCU) for VOC among youth with SCD.
Methods: Cross-sectional study was conducted between November 2022 and June 2024. Youth between the ages of 8-17 years with a diagnosis of SCD (any genotype) in their baseline state of health were eligible to participate. Age-, race-, and sex-matched children without chronic illness and in their baseline state of health without conditions causing chronic pain or inflammation were enrolled as controls. During an in-person or remote study visit, participants completed assessments including the Patient-Reported Outcomes Measurement Information System (PROMIS) Sleep Disturbance (SD) Short Form 8A, PROMIS Sleep-Related Impairment (SRI) Short Form 8A, Pediatric Sleep Questionnaire (PSQ), Epworth Sleepiness Scale in Children and Adolescents (ESS-CHAD), Child Sleep Hygiene Scale (CSHS) and Adolescent Sleep Hygiene Scale (ASHS), and Pediatric Quality of Life Inventory (PedsQL). Electronic health records were reviewed to obtain data on emergency department, day medicine, and SCD clinic visits as well as hospital admissions for VOC.
Results: Analytic sample included 50 youth with SCD (60% female, Mean age=12.7 years old, 66% HbSS) and 25 youth without chronic illness (60% female, Mean age=13.3 years old). Groups did not differ in age, race, sex, height, weight, or BMI.
PROMIS SD scores did not differ between youth with SCD (M=55.9, SD 9.2) and controls (M=52.1, SD 7.1, p=.07).
However, children with SCD reported higher sleep-related impairment (PROMIS SRI M=54.9, SD 8.4) than controls (M=50.3; SD 8.5, p=.03). Children with SCD reported worse sleep hygiene (CSHS M=19.27) than control children (M=21.35; p=.05); there were no significant differences between adolescents with SCD and control adolescents in sleep hygiene (ASHS; p=0.9). While there were no differences between groups in daytime sleepiness (ESS-CHAD, p=0.1), 38% of youth with SCD had scores indicating increased daytime sleepiness (i.e., &gt;10) compared to 28% of youth in the control group. There was also a trend towards higher risk for sleep-disordered breathing (PSQ) among youth with SCD (p=.06). Youth with SCD reported worse overall health-related quality of life (PedsQL M=48.3) than controls (M=55.7, p=.02). About 51% of youth with SCD had HCU for VOC in the preceding year, which ranged from 0 to 6 visits (median= 1). There was no significant association between HCU for VOC and sleep health indices among youth with SCD.
Conclusions: Youth with SCD in their baseline state of health reported overall similar sleep quality and hygiene when compared to healthy youth without chronic illness. However, we found increased impairment related to sleep and worse health-related quality of life in youth with SCD compared to controls. Indices of sleep health were not associated with HCU for VOC among youth with SCD. Larger studies using longitudinal designs and multiple methods of sleep assessment (e.g., patient-reported questionnaires, polysomnogram or device estimates of sleep) are needed to evaluate relationships between sleep and health outcomes in youth with SCD,
Acknowledgements:
Research reported in this publication was supported by the National Institute of General Medical Sciences of the National Institutes of Health under Award Number P20GM109021
Research reported in this publication is supported by the National Institute of General Medical Sciences of the NIH under grant number P20GM144270. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health