Outcomes before and after providing interdisciplinary hematology and pulmonary care for children with sickle cell disease

Abstract

People with sickle cell disease (pwSCD) are at risk of developing lung conditions that complicate their sickle cell disease (SCD) but often face healthcare access barriers. An interdisciplinary SCD-pulmonary clinic was created in 2014 at Nationwide Children's Hospital (NCH) to address access barriers that may prevent optimized treatment. We hypothesize that pwSCD and pulmonary disease would have fewer hospitalizations for acute chest syndrome (ACS), asthma, and vaso-occlusive episodes (VOEs) in the two years after their initial SCD-pulmonary clinic visit compared to the two years prior. From 2014-2020,119 pwSCD were evaluated in the SCD-pulmonary clinic and followed at NCH for at least two years before and after this visit. Acute care outcomes, pulmonary function, polysomnography, echocardiogram, laboratory, and medication prescribing data were collected and analyzed using the Wilcoxon signed ranked and McNemar's tests. The median number of acute care visits for ACS (p<0.001) and asthma (p=0.006) were significantly lower during the two years after pwSCD's initial SCD-pulmonary clinic evaluation compared to the two years prior. Asthma and allergic rhinitis were more frequently diagnosed and prescriptions for hydroxyurea (p=0.005) and inhaled corticosteroids (p=0.005) were more common in the post SCD-pulmonary clinic period. The median number of prescribed systemic corticosteroids was lower in the two years following SCD-pulmonary clinic evaluation (p<0.0001). Lactate dehydrogenase and white blood cell counts also significantly decreased. Implementing a multidisciplinary SCD-pulmonary clinic is feasible and may allow improved management of pulmonary problems and lead to improvements in health and acute care utilization.