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Factors predicting future ACS episodes in children with sickle cell anemia
Michael R DeBaun1, Mark Rodeghier, Robyn Cohen
1Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, Tennessee.
Insights
Early acute chest syndrome (ACS) in children with sickle cell anemia (SCA), wheezing, and multiple allergies predict future ACS events. Identifying these risk factors aids in managing SCA complications.
Area of Science:
- Pediatrics
- Hematology
- Pulmonology
Background:
- Asthma diagnosis increases pain and acute chest syndrome (ACS) risk in children with sickle cell anemia (SCA).
- Limited understanding of specific asthma characteristics influencing future morbidity in pediatric SCA patients.
Purpose of the Study:
- To evaluate the association between asthma risk factors and clinical factors with prospective pain and ACS episodes in children with SCA.
- To identify predictors of future ACS events in a pediatric SCA cohort.
Main Methods:
- Prospective cohort study of 159 children with SCA from birth to a median of 14.7 years.
- Assessed asthma risk factors: respiratory symptoms, maternal history, allergy skin tests, spirometry.
- Analyzed early-life ACS, gender, wheezing, spirometry, and skin test results as predictors.
Main Results:
- Early ACS (before age 4), female gender, and wheezing with shortness of breath predicted future ACS rates.
- ≥ 2 positive allergy skin tests were significant predictors of future ACS events.
- Spirometry results (obstruction, bronchodilator response) did not significantly predict future ACS.
Conclusions:
- Early ACS, wheezing-induced shortness of breath, and multiple positive allergy skin tests are key predictors of future ACS in children with SCA.
- These findings can inform targeted interventions for managing ACS risk in pediatric SCA.
- Further research into the interplay of asthma and SCA pathophysiology is warranted.
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 (i) asthma risk factors at the time of a clinical visit (respiratory symptoms, maternal history of asthma, allergy skin tests, spirometry results) and (ii) 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.
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