Elexacaftor, Tezacaftor, And Ivacaftor

Class: Cystic Fibrosis Transmembrane Conductance Regulator Correctors
Chemical Name: N-(1,3-Dimethyl-1H-pyrazole-4-sulfonyl)-6-[3-(3,3,3-trifluoro-2,2-dimethylpropoxy)-1H-pyrazol-1-yl]-2-[(4S)-2,2,4-trimethylpyrrolidin-1-yl]pyridine-3-carboxamide
Molecular Formula: C₂₆H₃₄F₃N₇O₄SC₂₆H₂₇F₃N₂O₆C₂₄H₂₈N₂O₃
CAS Number: 2216712-66-0
Brands: Trikafta

Medically reviewed by Drugs.com on Nov 1, 2021. Written by ASHP.

Introduction

The combination of elexacaftor, tezacaftor, and ivacaftor increases the quantity and function of the cystic fibrosis transmembrane conductance regulator (CFTR) protein at the cell surface, resulting in increased CFTR activity as measured by CFTR mediated chloride transport.

Uses for Elexacaftor, Tezacaftor, And Ivacaftor

Elexacaftor, tezacaftor, and ivacaftor has the following uses:

The combination of elexacaftor, tezacaftor, and ivacaftor is indicated for the treatment of cystic fibrosis (CF) in patients aged 12 years and older who have at least one F508del mutation in the CFTR gene.

If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to confirm the presence of at least one F508del mutation.

Elexacaftor, Tezacaftor, And Ivacaftor Dosage and Administration

General

The combination of elexacaftor, tezacaftor, and ivacaftor is available in the following dosage form(s) and strength(s):

• Co-packaged as elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg fixed-dose combination tablets and ivacaftor 150 mg tablets.

Dosage

It isessentialthat the manufacturer's labeling be consulted for more detailed information on dosage and administration of this drug. Dosage summary:

• The combination of elexacaftor, tezacaftor, and ivacaftor is administered orally.

• Instruct patients to swallow the tablets whole.

• Elexacaftor, tezacaftor, and ivacaftor should be taken with fat-containing food. Examples of meals or snacks that contain fat are those prepared with butter or oils or those containing eggs, cheeses, nuts, whole milk, or meats.

Pediatric Patients

Dosage in Pediatric Patients 12 Years of Age and Older

• The recommended starting dosage is 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, approximately 12 hours apart.

• Should not be used in patients with severe hepatic impairment. Use not recommended in patients with moderate hepatic impairment unless the benefit exceeds the risk. Reduce dose if used in patients with moderate hepatic impairment. Liver function tests should be closely monitored.

• Reduce dose when co-administered with drugs that are moderate or strong CYP3A inhibitors.

Adults

Dosage in Adults

• The recommended starting dosage is two ...