Elexacaftor, Tezacaftor, and Ivacaftor

The combination of elexacaftor, tezacaftor, and ivacaftor is used to treat certain types of cystic fibrosis (an inborn disease that causes problems with breathing, digestion, and reproduction) in adults and children 2 years of age and older. It should be used only in people with a certain genetic make-up. Your doctor may order a blood test to help decide if this medication is right for you. Elexacaftor and tezacaftor are in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) correctors. Ivacaftor is in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) potentiators. These medications work by improving the function of a protein in the body to decrease the build-up of thick mucus in the lungs and improve other cystic fibrosis symptoms.

🔔 How should this medicine be used?

Elexacaftor, tezacaftor, and ivacaftor come as tablets and as granules to take by mouth. It is usually taken with fatty foods twice a day, 12 hours apart. Take elexacaftor, tezacaftor, and ivacaftor at the same times every day. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take these medications exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.

If you are taking the tablets, each daily dose is usually given as 2 different tablets that have different ingredients. One tablet (combination of elexacaftor, tezacaftor, and ivacaftor) is orange and the other tablet (ivacaftor) is blue. Your doctor will probably tell you to take elexacaftor, tezacaftor, and ivacaftor (2 orange tablets) every morning and ivacaftor (1 blue tablet) every evening.

Swallow the tablets whole; do not split, chew, or crush them.

If you are taking the granules, each daily dose is usually given as 2 different packets of granules: one packet contains granules of elexacaftor, tezacaftor, and ivacaftor and the other packet contains granules of ivacaftor. Your doctor will probably tell you to take 1 packet of elexacaftor, tezacaftor, and ivacaftor granules every morning and 1 packet of ivacaftor granules every evening.

To prepare a dose of elexacaftor, tezacaftor, and ivacaftor granules, mix the entire packet of granules in 1 teaspoon (5 mL) of cold or room temperature soft food or liquid, such as pureed fruits or vegetables, yogurt, applesauce, water, milk, or juice. Take the mixture within 1 hour of mixing the granules with food or a liquid.

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Take elexacaftor, tezacaftor, and ivacaftor tablets and granules with fatty foods such as eggs, butter, nuts, peanut butter, cheese pizza, and whole-milk dairy products (such as whole milk, cheese, and yogurt). Talk to your doctor about other fatty foods to eat with these medications.

The combination of elexacaftor, tezacaftor, and ivacaftor works to control cystic fibrosis, but does not cure it. Continue to take these medications even if you feel well. Do not stop taking these medications without talking to your doctor.

Ask your pharmacist or doctor for a copy of the manufacturer’s information for the patient.