Demographic characteristics, clinical and laboratory features, and the distribution of pathogenic variants in the CFTR gene in the Cypriot cystic fibrosis (CF) population demonstrate the utility of a national CF patient registry

Table 3 Eligibility for administration of CFTR modulators

CFTR modulator	Indications for use	Number (percentage, %) of currently eligible patients	Number (percentage, %) of futurely^a eligible patients
Elexacaftor/Tezacaftor/Ivacaftor	At least one p.Phe508del	23 (48.9%)	8 (17%)
Lumacaftor/Ivacaftor	Two p.Phe508del	9 (19.2%)	0 (0%)
Tezacaftor/Ivacaftor	Two p.Phe508del or at least one CFTR mutation responsive to therapy based on clinical and/or in vitro assay data	11 (23.4%)	5 (10.6%)
Ivacaftor	At least one CFTR mutation responsive to therapy based on clinical and/or in vitro assay data	7 (14.9%)	0 (0%)

^aEligible in the future when indications will include children less than 12 years of age

ISSN: 1750-1172