Corresponding Author
Richard De Vuyst
Oklahoma Children’s Hospital, Pulmonology and Cystic Fibrosis Clinic
1200 Children’s Ave #9A, Oklahoma City, OK 73104
Richard-Devuyst@ouhsc.edu
To the editor,
Over the last seven decades, the life expectancy of people with cystic fibrosis (PwCF) has increased from less than two years to greater than 50 years1. A significant contributor to this improvement has been the development of inhaled therapies such as dornase alfa and hypertonic saline. Adherence to treatment regimens including these therapies is associated with improved outcomes2, but challenges inherent in maintaining complex treatment regimens including inhaled therapies has led to poor adherence overall.
The introduction of elexacaftor/ivacaftor/tezacaftor (ETI) has profoundly changed clinical outcomes in treatment eligible PwCF. This has generated considerable interest in exploring possible reductions in treatment burden such as the recent SIMPLFY study3. While a prior small study suggested that PwCF were choosing to reduce their use of chronic therapies4, the extent to which this occurs and clinical consequences within the CF population are unknown. To provide insight, we examined medication utilization among a large cohort of PwCF on ETI therapy within our adult and pediatric CF Clinical Centers.