CFTR and cystic fibrosis: COMP: comparator; SOC: standard of care; LUM: lumacaftor; IVA: ivacaftor; QALYs: quality-adjusted life-years; CFQ-R: Cystic Fibrosis Questionnaire-Revised; CF: cystic fibrosis; LE: average life expectancy; PEx: pulmonary exacerbations; muts: mutations; BL: baseline; 6MWT: six-minute walk test; FVC: forced vital capacity; FEV1: forced expiratory volume in one second; BMI: basal metabolic index; CFTR: cystic fibrosis transmembrane conductance regulator; HRQoL: health-related quality of life