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A Review of Sleep Concerns in Pediatric Sickle Cell Disease

Oncology & Hematology Review, 2012;8(1):83–7 DOI:


Children with sickle cell disease (SCD) are at an increased risk of sleep disorders as compared with healthy children, possibly because of disease processes, pain, ethnic minority status, and living in an urban environment. Adequate sleep is an essential component of typical child development, mood-and-affect regulation, and health maintenance, but more research is needed to understand the contribution of sleep to health outcomes in children with SCD. SCD is a chronic disease that can be influenced by environmental, health, and behavioral factors. Understanding the impact of the disease on sleep is important to maximise the quality of life in these children. If SCD causes poor sleep quality, then children may be at risk of a host of developmental and psychosocial problems beyond those caused by the disease. Also, poor sleep may affect the disease course, thus exacerbating symptoms. In this article, prevalent sleep disorders in this population are reviewed, including sleep-disordered breathing, periodic limb movement disorder, restless legs syndrome, and nocturnal enuresis. Also, the contribution of the disease symptoms—such as pain, hypoxemia, and daytime tiredness and fatigue—to disrupted sleep are examined. Finally, the effects of sociodemographic factors, such as poverty and ethnic minority status, are described, as these contextual factors significantly impact sleep across several chronic conditions in pediatric patients. Frequent monitoring for sleep disruptions can be essential to improving health outcomes and quality of life in children with SCD.
Keywords: Sickle cell disease, pediatrics, sleep, sleep-disordered breathing, enuresis, pain, socioeconomic status
Disclosure: The authors have no conflicts of interest to declare.
Received: December 05, 2011 Accepted: January 23, 2012
Correspondence: Lauren C Daniel, PhD, The Children’s Hospital of Philadelphia, 34th Street and Civic Center Blvd, CHOP North 1482, Philadelphia, PA 19104. E:

The symptoms of sickle cell disease (SCD) as well as underlying disease processes affect sleep across development, which has important implications for academic and behavioural functioning and overall health outcomes in children and adolescents with SCD. High frequencies of arousals and fragmented sleep related to sleep disorders that are more prevalent in SCD coupled with sleep-interfering disease symptoms (i.e. pain, enuresis, hypoxaemia) result in chronic sleep disruption that can affect development and health outcomes. Rates of sleep-disordered breathing conditions, including obstructive sleep apnoea (OSA),1–3 periodic limb movements (PLMS),4 restless legs syndrome4 and nocturnal enuresis5,6 have been documented in children and adolescents with SCD compared to healthy peers. In children without chronic illness, sleep problems have been linked to behaviour problems,7 poor academic outcomes,8 depression8 and reduced cognitive performance.9 A greater understanding of the interplay between SCD, health outcomes and sociodemographic factors, which are known to affect sleep,10 is necessary to target and improve sleep in children with SCD. This review describes highly prevalent sleep disorders in SCD, disease factors associated with disrupted sleep and the sociodemographic contributions to sleep patterns in children and adolescents with SCD.
Sleep Disorders Prevalent in Paediatric Sickle Cell Disease
Sleep-disordered Breathing
Sleep-disordered breathing is one of the most frequently observed sleep disorders in children with SCD and can significantly impact health and behavioural outcomes in child development. Sleep-disordered breathing refers to the category of disorders of abnormal respiration during the night, either based on ventilation patterns or quantity of ventilation throughout the night.11 OSA is the most common of these disorders, caused by a collapse of the upper airway that restricts airflow and causes hypoxia, disruptions in sleep, hypercapnia (increased carbon dioxide) and reduced neurocognitive performance.11 Sleep-disordered breathing contributes to behavioural problems and learning difficulties (for a review see Owens12), pulmonary hypertension, arterial hypertension, nocturnal enuresis and reductions in growth.13
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Keywords: Sickle cell disease, pediatrics, sleep, sleep-disordered breathing, enuresis, pain, socioeconomic status