The relationship between pain and sleep in pediatric sickle cell disease.

BACKGROUND: Pain and sleep disturbances are prevalent complications experienced by pediatric patients with sickle cell disease (SCD). This study aims to identify associations between pain and sleep, and to characterize sleep chronotype and social jetlag in children and adolescent patients with SCD. METHODS: We performed a cross-sectional survey of 105 pediatric patients with SCD aged 8-17 years using PROMIS (Patient Reported Outcomes Measurement System) pain interference, sleep disturbance, and sleep-related impairment item banks. The µMCTQ (Ultra-short Munich Chronotype Questionnaire) assessed chronotype and social jetlag. Analyses were performed to assess associations between PROMIS measures, sleep patterns, and clinical variables. RESULTS: Female participants reported higher T-scores for sleep-related impairment than males (females: 56.7 ± 10 vs. males 50.2 ± 9.4, p = .0009). Patients with one or more emergency department (ED) visits for pain in the last 12 months reported greater sleep disturbance (55.0 ± 8.5 vs. 50.7 ± 10, p = .046) and sleep-related impairment (57.1 ± 9.3 vs. 52.1 ± 10.2, p = .03) than patients without any ED visits for pain in the last 12 months. Pain interference was significantly associated with both sleep disturbance (r = .49, p < .0001) and sleep-related impairment (r = .46, p < .0001). The average mid-sleep time was 4:14 ± 1:44 a.m. and the average social jetlag (hh:mm) was 2:32 ± 1:35. CONCLUSION: Our study demonstrates that pain interference is associated with both sleep disturbance and sleep-related impairment. PROMIS measures can identify patients that suffer from pain and sleep disturbances and highlights the need to conduct longitudinal prospective studies to define the directionality of pain and sleep in SCD.

The association between sleep disturbances and neurocognitive function in pediatric sickle cell disease.

BACKGROUND AND OBJECTIVE: Youth with sickle cell disease (SCD) without neurological complications continue to be at increased risk of neurocognitive difficulties. Nocturnal hypoxemia is associated with neurocognitive outcomes and has been identified as a chronic complication in youth with SCD. The objective of this study was to assess the relationship between sleep disturbances and neurocognitive functioning in youth with SCD, while taking into account demographic and socioeconomic factors. METHODS: Youth with SCD were identified through retrospective chart review who underwent a standardized polysomnography (PSG) and completed a neuropsychological testing battery to assess cognitive skills, including verbal comprehension, working memory, processing speed, and cognitive flexibility. Questionnaires were also collected to assess parent-reported concerns with their youth's executive and adaptive skills. RESULTS: Twenty-seven youth with SCD, ages 6-17, were identified who completed both a PSG and neuropsychological testing. Results demonstrated that verbal comprehension decreased by 2.37 standard points for every unit decrease in mean nocturnal oxygen saturation (SpO2) (p = 0.031). Working memory was also found to decrease by 1.46 standard points for each 1% increase in time spent under 90% oxygen saturation (pTST SpO2 < 90%) (p = 0.030). Sleep parameters did not significantly predict other cognitive scores or parent-reported executive or behavioral ratings. CONCLUSION: Our study found that sleep disturbance, mean nocturnal SpO2 and pTST SpO2 < 90%, significantly affected verbal comprehension and working memory performance, respectively. Overall, these findings have the potential to identify sleep needs in youth with SCD to promote sleep-targeted interventions as a modifiable factor to reduce neurocognitive deficits.

The Effects of Radiation and Sex Differences on Adaptive Functioning in Adult Survivors of Pediatric Posterior Fossa Brain Tumors.

OBJECTIVE: Radiation therapy (RT) improves rates of survival of patients with childhood brain tumors but increases deficits in cognition and independent living skills. Previous literature has studied difficulties in basic cognitive processes, but few explore impairment in higher-order skills such as adaptive functioning. Some studies identify females as at risk for cognitive deficits due to RT, but few investigate sex differences in adaptive functioning. It was hypothesized that females would exhibit poorer long-term independent living skills and core cognitive skills relative to males following RT. METHODS: Forty-five adult survivors of posterior fossa childhood brain tumors (24 females) completed the Wechsler Abbreviated Scale of Intelligence (WASI-II), Wechsler Memory Scale, Third Edition (WMS-III) Digit Span Forward (DSF) and Backward (DSB), and Oral Symbol Digit Modalities Test (OSDMT). Informants completed the Scales of Independent Behavior-Revised (SIB-R). RESULTS: DSF and OSDMT were positively correlated with all five SIB-R domains, full-scale IQ (FSIQ) was positively correlated with four SIB-R domains, and DSB was positively correlated with three SIB-R domains. There was an interaction between sex and RT for OSDMT and community living skills with trend level interactions for personal living skills and broad independent living skills, where females without RT had higher scores than females with RT. CONCLUSIONS: Female survivors were more affected by RT than males across the community living skills domain of adaptive functioning as well as processing speed. Processing speed deficits may have a cascading impact on daily living skills. Future studies should investigate how clinical and biological factors may contribute to personalized treatment plans between sexes. (JINS, 2019, 25, 729-739).