THE EFFECT OF MODULATORS ON LUNG FUNCTION FOLLOWING INPATIENT TREATMENT
FOR CF EXACERBATIONS
Abstract
Background: Modulator therapy restores CFTR function and has
led to health benefits for persons with CF (PwCF) including lower rates
of pulmonary exacerbations. It is unknown if modulators affect lung
function trajectories after inpatient treatment of pulmonary
exacerbations (PEx). Methods: We conducted a retrospective
review of hospital encounters for PEx for subjects 6-25 years old
admitted to a large tertiary care center from 2014-2021 in order to
capture hospitalizations of individual PwCF before and after starting
modulators. Descriptive analyses were used to characterize the
population and lung function findings. Logistic regression analyses were
conducted to assess the association between modulators and FEV1pp
outcomes. Results: The study sample included 575 encounters
representing 149 unique PwCF. Hospital encounters of PwCF taking
modulator were associated with higher mean FEV1pp at baseline, midway,
discharge, and follow-up assessments. Mean FEV1pp increased during
inpatient treatment of APE with loss of lung function at follow-up
regardless of modulator use. At follow-up, hospitalizations of PwCF
taking modulators were associated with significantly higher probability
of sustained improvement in FEV1pp from discharge (average treatment
effect (ATE) 0.118, p<0.05). Conclusions:
Hospitalizations for PwCF taking modulators were associated with higher
lung function at all assessments. Inpatient treatment for PEx was
associated with lung function recovery at discharge followed by loss of
function at follow-up that was partially ameliorated by taking
modulators.