Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia

Abstract

Pain and acute chest syndrome (ACS) episodes are 2 of the most common causes of hospitalization in children with sickle cell anemia (SCA). However, very few potentially modifiable risk factors for either condition have been identified. In this prospective infant cohort study, we tested the hypothesis that asthma is associated with an increased incidence rate of pain and ACS episodes. An infant cohort was composed of 291 African American children with hemoglobin SS enrolled in the Cooperative Study for Sickle Cell Disease before age 6 months and followed beyond age 5 years. Asthma was defined by a physician diagnosis, an acute asthma event, or use of prescription asthma medications. The incidence rates of ACS and painful episodes were compared for children with and without asthma. A clinical diagnosis of asthma was made in 17% of the cohort. Asthma was associated with more frequent ACS episodes (0.39 vs 0.20 events per patient year, P < .001) and painful episodes (1.39 vs 0.47 events per patient year, P < .001). In conclusion, in children with SCA, asthma is associated with an increased incidence of sickle cell disease-related morbidity, including ACS and painful episodes. IntroductionPainful episodes and acute chest syndrome (ACS) are common complications of sickle cell anemia (SCA) and are the 2 leading causes of hospitalization among people with SCA. 1,2 ACS affects up to 50% of individuals with SCA and is the leading cause of premature death. [2][3][4] The pathogenesis of ACS is multifactorial and remains unclear. In a large prospective study, several etiologies were identified, but in approximately 50% of episodes, no cause was determined. 5 Known risk factors for the development of ACS include younger age, degree of anemia (higher steady-state hemoglobin level), lower hemoglobin F, and higher steady-state white blood cell count. 2,6 Asthma is a common chronic disease affecting approximately 15% to 20% of African American children. [7][8][9][10] Previous retrospective studies suggest a relationship between ACS and asthma among children with SCA. We previously reported a 4-fold higher risk of ACS among children with SCA and a prior doctor diagnosis of asthma within a cohort of children with SCA hospitalized for pain. 11 Knight-Madden et al 12 reported a 6-fold higher risk of recurrent ACS among children with SCA and a parental report of doctor-diagnosed asthma. Airway obstruction and airway lability have been previously described in patients with SCA, suggesting that asthma may contribute to the pulmonary complications of the disease. [13][14][15][16] These data suggest that in children with SCA, a diagnosis of asthma may be a risk factor for ACS. This study also seeks to determine whether asthma is also associated with painful episodes, a common complication in SCA that often precedes or occurs concurrently with ACS episodes.In a cohort of infants established and followed for 20 years by the Cooperative Study of Sickle Cell Disease (CSSCD), we tested the hypothesis that a concurrent diagnosis of asthma in p...

Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease

“…Readmission was less frequent in patients with many episodes of ACS and after transfusion. Asthma was associated with recurrent ACS in Jamaican children [18] and an increased rate of admission for ACS and painful crisis, but not prolonged hospitalization, in the infant cohort of the CSSCD [19]. However, most patients in our study who were treated with dexamethasone did not have a history of asthma or wheezing at the time of treatment, as recognized asthma exacerbations typically were treated with other corticosteroids (prednisone or prednisolone).…”

Original Research: Acute chest syndrome in sickle cell disease: Effect of genotype and asthma

“…Knight-Madden et al 4 reported that children with sickle cell disease were more than twice as likely to have asthma or bronchial hyperreactivity, and that asthma was more common in children who had suffered recurrent episodes of ACS. Boyd et al 5 reported from the Cooperative Study for Sickle Cell Disease that the frequency of ACS events was twice as high in patients with a diagnosis of asthma. Duckworth et al 6 reported a relationship between physician-diagnosed asthma and ACS.…”

“…[4][5][6][7][8] The presence of asthma in SCD has been associated with increased risk of developing ACS. In addition, the first ACS event tends to occur at a younger age when compared to patients who do not have asthma.…”

Assessment of Nutritional Status of Patients Suffering from Asthma

“…Recently Boyd et al [38] and Lewis et al [39] found lower haemoglobin and elevated white blood cells concentrations in asthmatics when compared with non-asthmatics. The disparity with regards to serum protein and serum calcium levels in blood among the two groups may be due to fewer intakes of calcium and protein rich food items in their diet.…”