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.