WHAT'S KNOWN ON THIS SUBJECT: Obstructive sleep apnea syndrome (OSAS) prevalence in children with sickle cell anemia is not well described. Although these children often experience nocturnal oxygen desaturation, it is unclear whether they are more likely to have OSAS.WHAT THIS STUDY ADDS: Children with sickle cell anemia have a high prevalence of OSAS with typical symptoms, beyond just nocturnal oxyhemoglobin desaturation. This study supports the need for increased efforts to screen for, diagnose, and treat OSAS in this vulnerable population. abstract OBJECTIVE: To ascertain the prevalence of and risk factors for obstructive sleep apnea syndrome (OSAS) in children with sickle cell anemia (SCA). METHODS:Cross-sectional baseline data were analyzed from the Sleep and Asthma Cohort Study, a multicenter prospective study designed to evaluate the contribution of sleep and breathing abnormalities to SCArelated morbidity in children ages 4 to 18 years, unselected for OSAS symptoms or asthma. Multivariable logistic regression assessed the relationships between OSAS status on the basis of overnight inlaboratory polysomnography and putative risk factors obtained from questionnaires and direct measurements. RESULTS:Participants included 243 children with a median age of 10 years; 50% were boys, 99% were of African heritage, and 95% were homozygous for b S hemoglobin. OSAS, defined by obstructive apnea hypopnea indices, was present in 100 (41%) or 25 (10%) children at cutpoints of $1 or $5, respectively. In univariate analyses, OSAS was associated with higher levels of habitual snoring, lower waking pulse oxygen saturation (SpO 2 ), reduced lung function, less caretaker education, and non-preterm birth. Lower sleep-related SpO 2 metrics were also associated with higher obstructive apnea hypopnea indices. In multivariable analyses, habitual snoring and lower waking SpO 2 remained risk factors for OSAS in children with SCA. CONCLUSIONS:The prevalence of OSAS in children with SCA is higher than in the general pediatric population. Habitual snoring and lower waking SpO 2 values, data easily obtained in routine care, were the strongest OSAS risk factors. Because OSAS is a treatable condition with adverse health outcomes, greater efforts are needed to screen, diagnose, and treat OSAS in this high-risk, vulnerable population.

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Objective To identify factors associated with asthma associated with increased sickle cell anemia (SCA). Study design Children with SCA (n=187; mean age 9.6 years, 48% male) were classified as having “asthma” based on parent report of doctor diagnosis plus prescription of asthma medication (n=53) or “no asthma” based on the absence of these features (n=134). Pain and acute chest syndrome (ACS) events were collected prospectively. Results Multiple variable logistic regression model identified three factors associated with asthma: parent with asthma (p=0.006), wheezing causing shortness of breath (p=0.001), and wheezing after exercise (p < 0.001). When two or more features were present, model sensitivity was 100%. When none of the features were present, model sensitivity was 0%. When only one feature was present, model sensitivity was also 0% and presence of 2 or more positive allergy skin tests, airway obstruction on spirometry, and bronchodilator responsiveness did not improve clinical utility. ACS incident rates were significantly higher in individuals with asthma than those without asthma (IRR 2.21, CI 1.31-3.76); pain rates were not (IRR 1.28, CI 0.78-2.10). Conclusions For children with SCA, having a parent with asthma and specific wheezing symptoms are the best features to distinguish those with and without parent report of a doctor diagnosis of asthma and identify those at higher risk for ACS events. The value of treatment for asthma in prevention of SCA morbidity needs to be studied.