TRIKAFTA (elexacaftor, tezacaftor, and ivacaftor)

TRIKAFTA (elexacaftor, tezacaftor, and ivacaftor)

SELF ADMINISTRATION - ORAL

Indications for Prior Authorization:

Cystic fibrosis (CF) - treatment of CF in patients aged 2 years and older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene or a mutation in the CFTR gene that is responsive based on in vitro data.

Coverage criteria:

For diagnosis of cystic fibrosis (CF):

• Dose does not exceed the FDA labeled maximum (see below):
  • Age 2 to less than 6 years (less than 14 kg): one packet (containing elexacaftor 80 mg, tezacaftor 40 mg, and ivacaftor 60 mg) taken in the morning and one ivacaftor packet (containing ivacaftor 59.5 mg) taken in the evening;
  • Age 2 to less than 6 years (14 kg or more): one packet (containing elexacaftor 100 mg, tezacaftor 50 mg, and ivacaftor 75 mg) taken in the morning and one ivacaftor packet (containing ivacaftor 75 mg) taken in the evening;
  • Age 6 to less than 12 years (less than 30 kg): two tablets (each containing elexacaftor 50 mg, tezacaftor 25 mg, and ivacaftor 37.5 mg) taken in the morning and one ivacaftor tablet (containing ivacaftor 75 mg) taken in the evening;
  • Age 6 to less than 12 years (30 kg or more): two tablets (each containing elexacaftor 100 mg, tezacaftor 50 mg, and ivacaftor 75 mg) taken in the morning and one ivacaftor tablet (containing ivacaftor 150 mg) taken in the evening;
  • 12 years of age and older: two tablets (each containing elexacaftor 100 mg, tezacaftor 50 mg, and ivacaftor 75 mg) taken in the morning and one ivacaftor tablet (containing ivacaftor 150 mg) taken in the evening; AND
• Patient is 2 years of age or older; AND
• Prescribed by or in consultation with a pulmonologist or physician who specializes in the treatment of CF; AND
• Patient has at least one of the following mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene as detected by a FDA-cleared cystic fibrosis mutation test or a test performed at a Clinical Laboratory Improvement Amendments (CLIA)-approved facility:*
  • F508del mutation
  • A mutation in the CFTR gene that is responsive based on in vitro data 
• Trikafta will not be used in combination with Orkambi (lumacaftor/ivacaftor), Kalydeco (ivacaftor), or Symdeko (tezacaftor/ivacaftor).

Reauthorization Criteria:

For diagnosis of cystic fibrosis (CF):

• Documentation of a positive clinical response to therapy (e.g., improvement in lung function [percent predicted forced expiratory volume in one second {PPFEV1}] or decreased number of pulmonary exacerbations)

Coverage Duration:

• Initial: 1 year
• Reauthorization: 1 year

Authorization is not covered for the following:

Indications that are not listed in this policy or not approved by the Food and Drug Administration (FDA) do not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics Committee.

Additional Information:

• Reduce dose when co-administered with drugs that are moderate or strong CYP3A inhibitors.
• Should not be used in patients with severe hepatic impairment. Reduce the daily dose if benefit exceeds the risk in patients with moderate hepatic impairment. Consult the FDA approved drug labeling for specific dosing recommendations.

Policy Updates:

• 09/21/2020 - annual review; updated formatting
• 12/08/2021 - updated minimum age from 12 to 6 years to reflect FDA-approved labeling; updated dose recommendations based on FDA approved labeling; updated formatting.
• 11/14/2023 - updated minimum age from 6 to 2 years to reflect FDA-approved labeling; added reauthorization criteria; added max dosing requirement for oral granules formulation to reflect FDA-approved labeling.

References:

1. Trikafta Prescribing Information. Vertex Pharmaceuticals Inc. Boston, MA. October 2021.