CFTR Modulators and Substance Use
With significant improvements in lung and overall health with highly
effective modulator therapy, we investigated the prevalence of substance
use in pwCF on CFTR modulators. We found that e-cigarette and marijuana
use were more than two times higher in those on CFTR modulators than
those not on CFTR modulators. Although these associations did not reach
statistical significance after adjusting for other factors, these
associations are likely clinically meaningful as there was a strongly
positive trend of the odds ratio, and essentially rule out that pwCF on
CFTR modulators have lower substance use. There were no significant
differences in CBD and cigarette use between those on and not on CFTR
modulators. The increased trend in e-cigarette and marijuana use among
those on CFTR modulators poses significant concerns as there is ample
data on lung injuries associated with e-cigarettes14.
Tobacco smoke exposure can cause impairment in CFTR functional
expression, which may mitigate the benefit of CFTR
modulators14. The impact of marijuana smoke exposure
on CFTR expression has not been studied. With social influence and
increased access to these agents, it is critical that drug use
assessment and education be implemented in routine CF care, especially
as pwCF live longer in the era of effective modulator therapy to prevent
adverse outcomes from substance use.