Benefit of Pulmonary Subspecialty Care for Children with Sickle Cell Disease and Asthma

Abstract

Objective: Asthma is a recognized comorbidity in children with sickle cell disease (SCD). It increases risk of acute chest syndrome (ACS), vaso-occlusive episodes, and early mortality. We aim to determine whether evaluation and management of children with sickle cell disease (SCD) and asthma by a pulmonologist reduces rate of asthma exacerbation and ACS. Methods: The study included 192 patients with SCD (0-21 years) followed at Children’s Hospital of Philadelphia Hematology between 01/01/2015 and 12/31/2018 with a diagnosis of asthma, wheeze, or cough. Patients were placed in two groups: those evaluated by a pulmonologist (SCD-A-P) and those not (SCD-A). Rates of emergency department (ED) visits and hospitalizations for asthma exacerbation and ACS were compared between groups and before/after initial pulmonology visit. Results: SCD-A-P (n=70) had lower baseline pulmonary function, hemoglobin, and hematocrit compared to SCD-A (n=122). SCD-A-P had a higher average rate of hospital visits for asthma exacerbation and ACS per year compared to SCD-A: 0.84 [0.56-1.12] versus 0.31 [0.18-0.43], (p<0.001). For SCD-A-P patients with at least one hospital visit (n=48), the average rate decreased from 3.93 [1.57-6.29] before initial pulmonary visit to 0.85 [0.48-1.23] after the visit (p=0.014). Conclusion: SCD-A-P had more severe baseline disease and higher rates of ED visits and hospitalizations for asthma exacerbation and ACS compared to SCD-A, but the rates significantly decreased following pulmonology consultation. These findings support the pulmonologist’s role in the multidisciplinary care of SCD patients. Further studies are needed to help establish evidence-based asthma guidelines for children with SCD.