Phase-3 studies have investigated the safety and clinical efficacy of LUM/IVA therapy in both adults and children (Wainwright et al., 2015; Konstan et al., 2017; Ratjen et al., 2017; McNamara et al., 2019) and in a previous prospective multicentre study (Graeber et al., 2018), our group was able to evaluate the effect of LUM/IVA on CFTR function in different organ systems in patients with CF who are F/F over 12 years of age using the CFTR biomarkers SCC, nPD and ICM. This evidence concerns the gene CFTR and cystic fibrosis.