Impact of Spina Bifida on Sleep Quality: Current Insights.

Spina bifida (SB) is one of the most common birth defects in children. The care for patients with SB continues to evolve, and there has been notable improvement in survival outcomes, degree of disability and quality of life for these children. However, patients with SB continue to remain at higher risk for sleep-related breathing disorders (SRBD), unexplained sudden death, and potential alterations in their sleep chronotype. Previous studies report on abnormalities in the spinal cord, brainstem function, and dysfunction of upper airway maintenance as the likely mechanisms behind SRBD that is commonly seen in SB. Most studies looking at prevalence of SRBD in SB have been retrospective studies. A recent prospective study identified a prevalence as high as 42% when a polysomnography (PSG) was completed on all patients regardless of symptomatology. Treatment options vary depending on the type and severity of SRBD and can range widely. Despite advances in care for patients with SB and SRBD, a subset of these patients with myelomeningocele (MMC) continue to experience sudden unexplained death. Studies continue to evaluate ways to stratify which of these patients may be at higher risk of this devastating outcome. Given that SRBD is potentially treatable, early assessment and intervention could become an integral part of a multidisciplinary treatment strategy to optimize long-term medical and neurodevelopmental outcomes for this patient population. By understanding the impact that SB may have on a patient's sleep quality, their biological chronotype and their potential of developing SRBD, a provider may help to optimize the care a patient with SB receives from birth into adulthood.

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.

Acute chest syndrome in pediatric sickle cell disease: Associations with racial composition and neighborhood deprivation.

BACKGROUND: Acute chest syndrome (ACS) is the leading cause of death for children with sickle cell disease (SCD). Recurrent ACS has detrimental effects on pulmonary health and health care costs. Neighborhood characteristics affect the outcomes of many pediatric chronic diseases, but their role in SCD is not well studied. In this study, we investigated the effects of area-level socioeconomic deprivation and racial composition on the recurrence of ACS. STUDY DESIGN: We performed a retrospective cross-sectional analysis of clinical data from a large pediatric SCD center. Patients' residential addresses were geocoded and linked to a composite area deprivation index (ADI) and percent African American population at the level of Census block groups. The association of recurrent ACS with neighborhood characteristics was evaluated using logistic regression analysis. RESULTS: The sample included 709 children with SCD. Residence in a socioeconomically deprived neighborhood was associated with 27% less risk of recurrent ACS, and residence in a predominantly African American neighborhood was associated with 41% less risk of ACS recurrence. The racial composition explained the protective effect of living in a high-deprivation area after adjusting for sociodemographic and clinical covariates. Demographic and clinical factors associated with recurrent ACS included older age, male gender, asthma, hydroxyurea use, and chronic transfusion therapy. CONCLUSIONS: This is the first study to report a protective effect of residing in a predominantly African American community for ACS recurrence. Further prospective studies are needed to confirm the association and to understand the mechanisms of such relationship.

Nocturnal hypoxemia measured by polysomnogram is associated with acute chest syndrome in pediatric sickle cell disease.

STUDY OBJECTIVES: Nocturnal hypoxemia is associated with increased risk of sickle cell disease (SCD) complications. The association of nighttime hypoxemia and acute chest syndrome (ACS) in children with SCD has yet to be determined. METHODS: This is a retrospective study of children with SCD who underwent polysomnography at a SCD center. Univariate logistic regression was used to assess the association between nocturnal hypoxemia and ACS admissions. Multivariate logistic regression was performed to verify the effects of different clinical covariates on ACS. Secondary analysis comparing patients with one vs multiple ACS admissions was performed. RESULTS: One hundred ten individuals with SCD who completed their polysomnogram (mean age of 9.4 years) were identified. Fifty-nine (54%) had a history of at least one episode of ACS admission (mean age of 4.1 years), including 40 with multiple episodes. The percentage of total sleep time with O2 saturation < 90% was greater in the ACS group (P < .05). Similarly, mean nocturnal O2 saturation was lower in the ACS group (P < .0005). Mean nocturnal O2 saturation of < 97.3% and the percentage of total sleep time with O2 saturation < 90% higher than 2.7% were associated with ACS. There was no difference in nocturnal hypoxemia between patients with single and multiple ACS admissions. CONCLUSIONS: Nocturnal hypoxemia later in life is associated with previous ACS admissions in children with SCD. This can increase the yield of interpreting polysomnograms in this vulnerable population. Prospective studies are needed to determine the temporal relations of nocturnal hypoxemia and ACS, which may identify a modifiable risk for ACS.