Tezacaftor and Ivacaftor

Class: Cystic Fibrosis Transmembrane Conductance Regulator Correctors
Chemical Name: 1-(2,2-difluoro-1,3-benzodioxol-5-yl)-N-[1-[(2R)-2,3-dihydroxypropyl]-6-fluoro-2-(1-hydroxy-2-methylpropan-2-yl)indol-5-yl]cyclopropane-1-carboxamide
Molecular Formula: C₂₆H₂₇F₃N₂O₆C₂₄H₂₈N₂O₃
CAS Number: 1152311-62-0
Brands: Symdeko

Medically reviewed by Drugs.com on Feb 8, 2022. Written by ASHP.

Introduction

Combination containing tezacaftor (cystic fibrosis transmembrane conductance regulator [CFTR] corrector) and ivacaftor (CFTR potentiator).

Uses for Tezacaftor and Ivacaftor

Cystic Fibrosis

Tezacaftor/ivacaftor combination therapy: Treatment of cystic fibrosis in patients homozygous for F508del mutation in the CFTR gene or having at least 1 mutation in the CFTR gene that is responsive to the combination drug regimen (i.e., E56K, P67L, R74W, D110E, D110H, R117C, E193K, L206W, R347H, R352Q, A455E, D579G, 711+3A→G, E831X, S945L, S977F, F1052V, K1060T, A1067T, R1070W, F1074L, D1152H, D1270N, 2789+5G→A, 3272-26A→G, 3849+10kbC→T).

Designated an orphan drug by FDA for this use.

Use FDA-approved cystic fibrosis mutation test to detect presence of CFTR mutations followed by verification with bidirectional sequencing when recommended by the mutation test instructions.

Tezacaftor and Ivacaftor Dosage and Administration

Administration

Oral Administration

Administer orally with fat-containing food (e.g., eggs, cheese, nuts, whole milk, meats, food prepared with butter or oils) to increase systemic absorption of the drug. (See Food under Pharmacokinetics.)

Dosage

Available as a kit containing 4 weekly blister cards of 7 tablets containing 100 mg of tezacaftor in fixed combination with 150 mg of ivacaftor copackaged with 7 tablets containing 150 mg of single-entity ivacaftor.

Pediatric Patients

Cystic Fibrosis

Oral

Children ≥12 years of age: Tezacaftor 100 mg/ivacaftor 150 mg once daily in the morning and single-entity ivacaftor 150 mg once daily in the evening (approximately 12 hours apart).

Dosage adjustment necessary when used concomitantly with moderate or potent inhibitors of CYP3A. (See Interactions.)

Adults

Cystic Fibrosis

Oral

Tezacaftor 100 mg/ivacaftor 150 mg once daily in the morning and single-entity ivacaftor 150 mg once daily in the evening (approximately 12 hours apart).

Dosage adjustment necessary when used concomitantly with moderate or potent inhibitors of CYP3A. (See Interactions.)

Special Populations

Hepatic Impairment

Mild hepatic impairment (Child-Pugh class A): Dosage adjustment not necessary.

Moderate hepatic impairment (Child-Pugh class B): Tezacaftor 100 mg/ivacaftor 150 mg once daily in the morning. Do not administer the evening dose of ivacaftor 150 mg in such patients.

Severe hepatic impairment (Child-Pugh class C): Use with caution and at a dosage of tezacaftor 100 mg/ivacaftor 150 mg once daily in the morning or less, after ...