The benefits of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) for people with cystic fibrosis (pwCF) with at least 1 F508del variant are well documented.1 ELX and TEZ increase the presence of corrected CFTR at the cell surface, whereas IVA improves chloride ion transportation.1 Although most pwCF may be eligible for CFTR modulator treatment based on United States Food and Drug Administration (FDA) criteria, which currently include those with at least 1 F508del allele or another CFTR mutation proven to be responsive through in vitro evidence, the in vivo effects of these treatments in rare genotypes are still being studied.