Tezacaftor/Ivacaftor & Ivacaftor (Symdeko®)

SELF ADMINISTRATION

Indications for Prior Authorization:

  • Treatment of patients with cystic fibrosis (CF) aged 12 years and older who are homozygous for the F508del mutation or who have at least one mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to Tezacaftor/Ivacaftor based on in vitro data and/or clinical evidence
  • If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test instructions for use

Patients must meet the following criteria for the indication(s) above:

  • Patient is 12 years of age or older, AND
  • Prescribed by or in consultation with a pulmonologist or a physician who specializes in the treatment of cystic fibrosis (CF), AND
  • The patient meets ONE of the following conditions:
    • The patient has at least one of the following mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene: E56K, P67L, R74W, D110E, D110H, R117C, E193K, L206W, R347H, R352Q, A455E, D579G, 711+3A → G, S945L, S977F, F1052V, E831X, K1060T, A1067T, R1070W, F1074L, D1152H, D1270N, 2789+5G → A, 3272-26A → D, or 3849 + 10kbC → T, OR
    • The patient has 2 copies of the F508del mutation, AND
  • Symdeko will not be used in combination with Orkambi or Kalydeco

Dosing:

  • One tablet (Tezacaftor 100mg/Ivacaftor 150mg) in the morning and one tablet (Ivacaftor 150mg) in the evening, 12 hours apart, with fat-containing food)
  • The concomitant use with CYP3A inducers is not recommended
  • Reduce dose in patients with moderate and severe hepatic impairment
  • Reduce dose when co-administered with moderate or strong CYP3A inhibitors

Approval:

  • 1 year

Last review date: April 23, 2019