Cystic Fibrosis (CF)

Drugs/Brands/Classes

Lumacaftor

Class: CFTR Corrector

Tezaxaftor

Class: CFTR Corrector

Elexacaftor

Class: CFTR Corrector

Vanzacaftor

Class: CFTR Corrector

Ivacaftor

Brand: Kalydeco

Class: CFTR Potentiator

>1 month

Deutivacaftor

Class: CFTR Potentiator

Lumacaftor + Ivacaftor

Brand: Orkambi

Class: CFTR Modulator + Potentiator

>1 years

Monitor liver toxicity

Tezacaftor + Ivacaftor

Brand: Symdeko

Class: CFTR Modulator + Potentiator

>6 years

Monitor liver toxicity

Elexacaftor + Tezacaftor + Ivacaftor

Brand: Trikafta

Class: CFTR Modulator + Potentiator

>2 years

Vanzacaftor + Tezacaftor + Deutivacaftor

Brand: Alyftrek

Class: CFTR Modulator + Potentiator

>6 years

Dosed 1 time daily

Ursodiol

Increases bile flow and may protect liver cells from cytotoxic salts and bile ducts

Azithromycin

Anti-inflammatory

Albuterol

Class: Bronchodilator

Dilates the lungs

Hypertonic Saline

Brand: HyperSal

Hydrates the lungs

Pulmozyme

Brand: Dornase alfa

Thins mucus

Do not mix with other nebulized medications

Tobramycin

Class: Antibiotic

Use: P. aeruginosa

>6 years

28 days, then 28 days off (cycled)

Aztreonam

Brand: Cayston

Class: Antibiotic '

Use: P. aeruginosa

Colistin

Class: Antibiotic

Use: P. aeruginosa (off-label)

Amikacin

Brand: Arikayce

Class: Antibiotic

Use: M. avium complex

Pancreatic Enzyme Replacement Therapy (PERT)

Brand: Creon, Pancreaze, Viokase, etc.

Administer before meals, snacks, and fat-soluble vitamins

Vitamins A, D, E, K

Class: fat-soluble vitamins

N-Acetylcysteine

Brand: Mucosol

Class: mucolytic