Kalydeco (ivacaftor tablets and oral granules – Vertex) — Cigna

Cystic Fibrosis

Initial criteria

Patient age ≥ 1 month; AND
Patient has at least ONE of the following cystic fibrosis transmembrane conductance regulator gene mutations that is considered to be a pathogenic or likely pathogenic variant: E56K, P67L, R74W, D110E, D110H, R117C, E193K, L206W, R347H, R352Q, A455E, D579G, S945L, S977F, F1052V, K1060T, A1067T, G1069R, R1070Q, R1070W, F1074L, D1152H, D1270N, G551D, G178R, S549N, S549R, G551S, G1244E, S1251N, S1255P, G1349D, 2789+5G—>A, 3272-26A—>G, 3849+10kbC—>T, 711+3A—>G, E831X, R117H, A120T, A234D, A349V, D192G, D924N, E882K, F311L, F311del, F508C, F508C;S1251N, G178E, G194R, G314E, G576A, G970D, G1249R, H939R, H1375P, I148T, I175V, I807M, I1027T, I1139V, L320V, L967S, L997F, L1480P, M152V, M9521, M952T, Q237E, Q237H, Q359R, Q1291R, R75Q, R117G, R117L, R117P, R170H, R347L, R553Q, R668C, R792G, R933G, R1162L, R1283M, S589N, S737F, S1159F, S1159P, T338I, T1053I, V232D, V562I, V754M, V1293G, W1282R, Y1014C, or Y1032C; AND
Patient meets at least ONE of the following: positive cystic fibrosis newborn screening test OR family history of cystic fibrosis OR clinical presentation consistent with signs and symptoms of cystic fibrosis (examples include meconium ileus, sino-pulmonary symptoms, bronchiectasis, sinusitis, failure to thrive, pancreatic insufficiency); AND
Patient has evidence of abnormal cystic fibrosis transmembrane conductance regulator function as demonstrated by at least ONE of the following: elevated sweat chloride test OR two cystic fibrosis-causing CFTR mutations OR abnormal nasal potential difference; AND
Medication is prescribed by or in consultation with a pulmonologist or a physician who specializes in the treatment of cystic fibrosis.

Approval duration

1 year