Asthma morbidity and treatment in children with sickle cell disease.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, Washington University School of Medicine, Saint Louis, MO, USA.
Expert Review of Respiratory Medicine 10/2011; 5(5):635-45. DOI: 10.1586/ers.11.64
Source: PubMed

ABSTRACT Children with sickle cell disease (SCD) and a comorbid condition of asthma have increased numbers of vaso-occlusive pain and acute chest syndrome episodes, and all-cause mortality. When assessed systematically, asthma prevalence is probably similar among children with SCD when compared with the general African-American population. With increasing recognition of the importance of asthma in the management of SCD, hematologists must become familiar with asthma and develop a multidisciplinary approach, including early recognition, appropriate management and referral to asthma specialists.

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    ABSTRACT: While a doctor-diagnosis of asthma is associated with an increased risk of pain and acute chest syndrome (ACS) in children with sickle cell anemia (SCA), little is known about the relationship between specific asthma characteristics and clinical factors and future morbidity in children with SCA. We evaluated the relationship between 1) asthma risk factors at the time of a clinical visit (respiratory symptoms, maternal history of asthma, allergy skin tests, spirometry results) and 2) the known risk factor of ACS early in life, on prospective pain and ACS episodes in a cohort of 159 children with SCA followed from birth to a median of 14.7 years. An ACS episode prior to 4 years of age, (incidence rate ratio (IRR) =2.84; p<0.001), female gender (IRR=1.80; p=0.009), and wheezing causing shortness of breath (IRR=1.68; p=0.042) were associated with future ACS rates. We subsequently added spirometry results (obstruction defined as FEV1 /FVC less than the lower limits of normal; and bronchodilator response, FEV1 ≥12%) and prick skin test responses to the model. Only ≥2 positive skin tests had a significant effect (IRR 1.87; p = 0.01). Thus, early in life ACS events, wheezing causing shortness of breath, and ≥2 positive skin tests predict future ACS events.
    American Journal of Hematology 11/2014; 89(11). DOI:10.1002/ajh.23819 · 3.48 Impact Factor
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    ABSTRACT: 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 physician 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 3 factors associated with asthma: parent with asthma (P = .006), wheezing causing shortness of breath (P = .001), and wheezing after exercise (P < .001). When ≥2 features were present, model sensitivity was 100%. When none of the features were present, model sensitivity was 0%. When only 1 feature was present, model sensitivity was also 0%, and presence of ≥2 of 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 in those without asthma (incident rate ratio 2.21, CI 1.31-3.76), but pain rates were not (incident rate ratio 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 physician diagnosis of asthma and to identify those at higher risk for ACS events. The value of treatment for asthma in the prevention of SCA morbidity needs to be studied.
    The Journal of pediatrics 04/2014; 164(4):821–826.e1. DOI:10.1016/j.jpeds.2013.11.034 · 4.02 Impact Factor
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    ABSTRACT: The purpose of this article is to provide a comprehensive review of wheezing in sickle cell disease (SCD), including epidemiology, pathophysiology, associations between wheezing and SCD morbidity and finally the clinical approach to evaluation and management of individuals with SCD who wheeze. Wheezing is common in SCD and in some individuals represents an intrinsic component of SCD-related lung disease rather than asthma. Emerging data suggest that, regardless of the cause, individuals with SCD and with recurrent wheezing are at increased risk for subsequent morbidity and premature mortality. We believe individuals who acutely wheeze and have respiratory symptoms should be managed with a beta agonist and short-term treatment of oral steroids, typically less than 3 days to attenuate rebound vaso-occlusive disease. For those who wheeze and have a history or examination associated with atopy, we consider asthma treatment and monitoring per National Heart, Lung and Blood Institute asthma guidelines. Wheezing in SCD should be treated aggressively both in the acute setting and with controller medications. Prospective SCD-specific clinical trials will be necessary to address whether anti-inflammatory asthma therapies (leukotriene antagonists, inhaled corticosteroids) can safely mitigate the sequelae of wheezing in SCD.
    Current opinion in pediatrics 12/2013; 26(1). DOI:10.1097/MOP.0000000000000045 · 2.74 Impact Factor

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