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.