SYMDEKO (tezacaftor-ivacaftor)

SYMDEKO (tezacaftor-ivacaftor)

SELF ADMINISTRATION - ORAL

Indication for Prior Authorization:

SYMDEKO® is a combination of tezacaftor and ivacaftor, indicated for the treatment of cystic fibrosis (CF) in patients age 6 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.

Coverage Criteria:

For the treatment of CF:

• Patient is 6 years of age or older; AND
• Prescribed by or in consultation with a pulmonologist or a physician who specializes in the treatment of CF; AND
• Symdeko will not be used in combination with another agent containing tezacaftor or ivacaftor (e.g. Orkambi®, Kalydeco®, Trikafta®); AND
• The patient meets ONE of the following conditions:
  • The patient has at least one of the “CFTR Gene Mutations that Produce CFTR Protein and are Responsive to SYMDEKO.” (See SYMDEKO® package labeling for list of acceptable gene mutations); OR
  • The patient has 2 copies of the F508del mutation

Dosing:

Pediatric patients age 6 to less than 12 years weighing less than 30 kg:

• one tablet (containing tezacaftor 50 mg/ivacaftor 75 mg) in the morning and one tablet (containing ivacaftor 75 mg) in the evening, approximately 12 hours apart. SYMDEKO® should be taken with fat-containing food.

Adults and pediatric patients age 12 years and older or pediatric patients age 6 to less than 12 years weighing 30 kg or more:

• one tablet (containing tezacaftor 100 mg/ivacaftor 150 mg) in the morning and one tablet (containing ivacaftor 150 mg) in the evening, approximately 12 hours apart. SYMDEKO® should be taken with fat-containing food.

Coverage Duration:

1 year.

Authorization is Not Covered for the Following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics Committee.

Additional Information:

• 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.

Review History:

• 12/30/2020 – Annual review; format updated; age limit changed to match FDA approval.
• 06/21/2019 – Approved by FDA for patients 6yrs and older.
• 04/23/2019 – Annual review.
• 02/12/2018 – Initial FDA approve.

References:

• Mogayzel Jr PJ, Naureckas ET, Robinson KA, Mueller G, Hadjiliadis D, Hoag JB, Lubsch L, Hazle L, Sabadosa K, Marshall B, Pulmonary Clinical Practice Guidelines Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. American journal of respiratory and critical care medicine. 2013 Apr 1;187(7):680-9.
• Ren CL, Morgan RL, Oermann C, Resnick HE, Brady C, Campbell A, DeNagel R, Guill M, Hoag J, Lipton A, Newton T. Cystic fibrosis foundation pulmonary guidelines. Use of cystic fibrosis transmembrane conductance regulator modulator therapy in patients with cystic fibrosis. Annals of the American Thoracic Society. 2018 Mar;15(3):271-80.
• SYMDEKO [package insert]. Boston, MA: Vertex Pharmaceuticals Inc.; December 2020.V