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Elexacaftor, ivacaftor, and tezacaftor

Generic name: elexacaftor, ivacaftor, and tezacaftor (e LEX a KAF tor, EYE va KAF tor, and TEXZ a KAF for)
Brand name: Trikafta
Dosage forms: oral tablet (100 mg-75 mg-50 mg and ivacaftor 150 mg; 50 mg-37.5 mg-25 mg and ivacaftor 75 mg)
Drug class: CFTR combinations

Medically reviewed by Drugs.com on Jun 28, 2021. Written by Cerner Multum.

What is elexacaftor, ivacaftor, and tezacaftor (Trikafta)?

Trikafta is a combination medicine used to treat cystic fibrosis in adults and children at least 6 years old.

Trikafta is for use only in patients with a specific gene mutation related to cystic fibrosis. You may need a medical test to make sure you have this gene mutation.

Elexacaftor, ivacaftor, and tezacaftor may also be used for purposes not listed in this medication guide.

Warnings

Tell your doctor about all your other medicines. Some drugs should not be used with elexacaftor, ivacaftor, and tezacaftor.

Before taking this medicine

Some drugs should not be used with Trikafta. Your treatment plan may change if you also use:

Tell your doctor if you have ever had:

Tell your doctor if you are pregnant or breastfeeding.

How should I take Trikafta?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Use the medicine exactly as directed.

Trikafta is supplied as two separate daily tablets. The usual dose is 2 orange tablets in the morning and 1 blue tablet in the evening, 12 hours later. Follow your doctor's dosing instructions very carefully.

Swallow the tablet whole and do not crush, chew, or break it.

Doses are based on weight and may change if your child gains or loses weight.

Your dose may change if you also take certain other medications. Your doctor may tell you to take the orange tablets and the blue tablet on separate days. Do not change your dose or dosing schedule without your doctor's advice.

Always take elexacaftor, ivacaftor, and tezacaftor with food that contains fat, such as butter, peanut butter, eggs, nuts, meat, whole milk, cheese, or yogurt. Follow your doctor's dosing instructions very carefully.

You will need frequent blood tests to check your liver function.

A child using this medicine may need frequent eye exams to check for cataracts.

Store at room temperature away from moisture and heat. Keep each tablet in its daily blister strip until you are ready to take your dose.

What happens if I miss a dose?

Take the medicine as soon as you can, but do not take two doses at one time.

If you are more than 6 hours late in taking the orange tablets, take them as soon as you can but skip taking the blue tablet for that day.

If you are more than 6 hours late in taking the blue tablet, skip that missed dose and take the orange tablets at the regular time the next day.

Be sure to take a missed dose with a food that contains fat.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

What should I avoid while taking Trikafta?

Avoid driving or hazardous activity until you know how elexacaftor, ivacaftor, and tezacaftor will affect you. Your reactions could be impaired.

Grapefruit may interact with Trikafta and lead to unwanted side effects. Avoid the use of grapefruit products.

Trikafta side effects

Get emergency medical help if you have signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have:

  • vision changes; or

  • liver problems--loss of appetite, stomach pain (upper right side), dark urine, jaundice (yellowing of the skin or eyes).

Common side effects may include:

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Elexacaftor, ivacaftor, and tezacaftor dosing information

Usual Adult Dose for Cystic Fibrosis:

Morning dose: 2 (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) tablets orally each morning
Evening dose: 1 (ivacaftor 150 mg) tablet orally each evening

Comments:
-Doses should be taken approximately 12 hours apart; tablets should be taken with fat-containing food.
-If genotype is unknown, an FDA-cleared cystic fibrosis (CF) mutation test should be used to confirm the presence of at least one F508del mutation or a mutation that is responsive based on in vitro data.

Use: For the treatment of CF in patients who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene or a mutation in the CFTR gene that is responsive based on in vitro data.

Usual Pediatric Dose for Cystic Fibrosis:

12 years or older:
Morning dose: 2 (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) tablets orally each morning
Evening dose: 1 (ivacaftor 150 mg) tablet orally each evening

6 to 12 years:
Weight: Less than 30 kg:
-Morning dose: 2 (elexacaftor 50 mg/tezacaftor 25 mg/ivacaftor 37.5 mg) tablets orally each morning
-Evening dose: 1 (ivacaftor 75 mg) tablet orally each evening
Weight: 30 kg or more:
-Morning dose: 2 (elexacaftor 100 mg/tezacaftor 50 mg/ivacaftor 75 mg) tablets orally each morning
-Evening dose: 1 (ivacaftor 150 mg) tablet orally each evening

Comments:
-Doses should be taken approximately 12 hours apart; tablets should be taken with fat-containing food.
-If genotype is unknown, an FDA-cleared cystic fibrosis (CF) mutation test should be used to confirm the presence of at least one F508del mutation or a mutation that is responsive based on in vitro data.

Use: For the treatment of CF in patients aged 6 years or older who have at least one F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene or a mutation in the CFTR gene that is responsive based on in vitro data.

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What other drugs will affect Trikafta?

Sometimes it is not safe to use certain medications at the same time. Some drugs can affect your blood levels of other drugs you take, which may increase side effects or make the medications less effective.

Tell your doctor about all your other medicines, especially:

This list is not complete. Other drugs may affect Trikafta, including prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here.

Further information

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.