Impact of a program ensuring consistent response to acute drops in lung function in children with cystic fibrosis

Abstract

Background: Variation in CF pulmonary outcomes is multifactorial, but a significant component appears to be dependent upon differences in CF Center care. Previous investigations suggest that high performing CF centers are more consistent and proactive in the treatment of pulmonary exacerbations. We incorporated this approach into a program that could be bundled and shared with other CF Centers.

Methods: The reorganization of CF pulmonary care at the Children's Hospital of Richmond included the development of a pulmonary algorithm to define a standard response to changes in lung function and run charts to track process and outcome measures. We calculated the rolling average of the best percent predicted FEV1 (ppFEV1) over the previous 12 months as our primary outcome measure.

Results: The mean of the best ppFEV1 in the previous 12 months rose from 87% predicted (65% predicted for those 13-18 years, 97% predicted for those 6-13 years) in January 2013 to 98% predicted (95% predicted for those 13-18 years, 110% predicted for those 6-13 years) in January 2018. The ppFEV1 difference between children 6-13 years and adolescents 13-18 years dropped from 34 to 14 during that time.

Conclusions: Improvements in pulmonary outcomes can be accomplished rapidly using basic quality improvement principles, including interdisciplinary team goal setting, standardized and proactive approaches that ensure consistent recognition and treatment of pulmonary exacerbations, and the use of data to follow the effectiveness of the process. We believe that the steps involved would be easy for other CF Centers to adapt to their own settings.

Keywords: Algorithm; FEV1; Lung function; Pulmonary exacerbation; Quality improvement.