The Impact of Sleep on Neurologic and Neurocognitive Complications in Children With Sickle Cell Disease: A Scoping
Rachel R Rice1, Ambika G Chidambaram2, Kiran Nandalike2
1Department of Pediatrics, Pediatric Residency Program, University of California, Davis, California, USA.
Insights
Sleep-disordered breathing in children with sickle cell disease worsens neurologic and cognitive issues. Early screening and treatment of sleep disorders are vital for better outcomes in these children.
Area of Science:
- Pediatric Neurology
- Sleep Medicine
- Hematology
Background:
- Sleep-disordered breathing (SDB) is common in children with sickle cell disease (SCD).
- SDB involves sleep fragmentation and hypoxia, impacting neurologic health.
- The link between SDB and neurologic/neurocognitive outcomes in pediatric SCD is a growing concern.
Purpose of the Study:
- To review and synthesize data on the impact of sleep disruption and SDB on neurologic and neurocognitive complications in children with SCD.
- To enhance understanding of the relationship between SDB and adverse outcomes in pediatric SCD.
- To inform clinical practice regarding sleep disorders in children with SCD.
Main Methods:
- Comprehensive literature search of Medline, PubMed, and Cochrane databases (inception to March 2023).
- Inclusion of studies assessing neurologic outcomes or cognitive function in pediatric SCD patients with SDB.
- Screening of 542 studies, with 21 studies meeting inclusion criteria.
Main Results:
- SDB is linked to increased stroke risk, higher transcranial Doppler velocities, and cerebral vasculopathy in children with SCD.
- Nocturnal hypoxemia and fragmented sleep correlate with neurocognitive deficits (reduced IQ, verbal comprehension, executive function).
- Interventions like adenotonsillectomy and CPAP improved sleep and mitigated neurocognitive deficits.
Conclusions:
- SDB exacerbates the neurologic burden in pediatric SCD via hypoxia, oxidative stress, and inflammation.
- Screening for sleep disorders in children with SCD is crucial.
- Timely intervention for SDB can improve neurologic and cognitive outcomes in this population.
Abstract:
Sleep-disordered breathing (SDB), characterized by sleep fragmentation and hypoxia, is a prevalent yet underappreciated complication in children with sickle cell disease (SCD). The interplay between SDB and neurologic outcomes in SCD, including stroke and cognitive impairment, is an area of emerging clinical concern. The aim of this review is to provide an overview and synthesis of available data to improve understanding of the impact sleep disruption and SDB have on neurologic and neurocognitive complications in children with SCD. We conducted a comprehensive literature search across databases including Medline, PubMed, and Cochrane from inception to March 2023. Studies assessing neurologic outcomes (e.g., stroke, silent cerebral infarcts) or cognitive function in children with SCD in relation to SDB were included. A total of 542 studies were screened, with 21 meeting inclusion criteria. SDB was associated with adverse neurologic outcomes, including increased stroke risk, higher transcranial Doppler velocities, and cerebral vasculopathy. Neurocognitive deficits, including reduced IQ, verbal comprehension, and executive function, were linked to nocturnal hypoxemia and fragmented sleep. Treatment interventions such as adenotonsillectomy and positive airway pressure improved sleep quality and mitigated neurocognitive deficits. SDB exacerbates the neurologic burden in children with SCD through pathways involving hypoxia, oxidative stress, and inflammation. Screening and timely intervention for sleep disorders in this population are crucial to improving neurologic and cognitive outcomes.
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