Kalydeco (ivacaftor) — CareFirst (Caremark)

Cystic fibrosis in patients age ≥ 1 month who have at least one CFTR gene mutation responsive to ivacaftor potentiation

Initial criteria

Genetic testing was conducted to detect a mutation in the CFTR gene
Member has one of the following CFTR gene mutations: A120T, A234D, A349V, A455E, A1067T, D110E, D110H, D192G, D579G, D924N, D1152H, D1270N, E56K, E193K, E822K, E831X, F311del, F311L, F508C, S1251N, F1052V, F1074L, G178E, G178R, G194R, G314E, G551D, G551S, G576A, G970D, G1069R, G1244E, G1249R, G1349D, H939R, H1375P, I148T, I175V, I807M, I1027T, I1139V, K1060T, L206W, L320V, L967S, L997F, L1480P, M152V, M952I, M952T, P67L, Q237E, Q237H, Q359R, Q1291R, R74W, R75Q, R117C, R117G, R117H, R117L, R117P, R170H, R347H, R347L, R352Q, R553Q, R668C, R792G, R933G, R1070Q, R1070W, R1162L, R1283M, S549N, S549R, S589N, S737F, S945L, S977F, S1159F, S1159P, S1251N, S1255P, T338I, T1053I, V232D, V562I, V754M, V1293G, W1282R, Y1014C, Y1032C, 711+3A→G, 2789+5G→A, 3272-26A→G, 3849+10kbC→T
Member is at least 1 month of age
Medication is prescribed by or in consultation with a pulmonologist
Kalydeco will not be used in combination with another CFTR modulator (e.g., Orkambi, Symdeko)

Member is experiencing benefit from therapy as evidenced by disease stability or disease improvement (e.g., improvement in FEV1 from baseline)

12 months