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Ivacaftor

Generic Name: ivacaftor (EYE va KAF tor)
Brand Name: Kalydeco

Medically reviewed by Drugs.com on Sep 24, 2018 – Written by Cerner Multum

What is ivacaftor?

Ivacaftor is used to treat cystic fibrosis in adults and children who are at least 12 months old and weigh at least 15 pounds (7 kilograms).

Ivacaftor is for use only in patients with a specific gene mutation related to cystic fibrosis. Before you take ivacaftor, you may need a medical test to make sure you have this gene mutation.

Ivacaftor may also be used for purposes not listed in this medication guide.

Important Information

Tell your doctor about all your current medicines and any you start or stop using. Many drugs can interact, and some drugs should not be used together.

Before taking this medicine

You should not use ivacaftor if you are allergic to it.

Some medicines can cause unwanted or dangerous effects when used with ivacaftor. Your doctor may change your treatment plan if you also use:

Tell your doctor if you have ever had:

It is not known whether ivacaftor will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

It may not be safe to breast-feed while using this medicine. Ask your doctor about any risk.

Ivacaftor is not approved for use by anyone younger than 12 months old.

How should I take ivacaftor?

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

Take ivacaftor with food that contains fat, such as butter, peanut butter, eggs, cheese, whole milk, yogurt, or cheese pizza. Follow your doctor's instructions very carefully.

To use ivacaftor oral granules: Mix the granules with 1 teaspoon of liquid or soft food such as water, juice, milk, pudding, yogurt, or applesauce. Mix only 1 dose at a time, and use the mixture within 1 hour after mixing. Feed the child a high-fat food just before or just after giving the granule mixture.

Take your doses at regular intervals, 12 hours apart.

Ivacaftor doses are based on age and weight in children. Children from 12 months to 5 years old should take oral granules. Children 6 and older should take tablets.

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

A child using this medicine may need frequent eye exams.

Store at room temperature away from moisture and heat. Keep each tablet in the foil blister pack until you are ready to take it.

What happens if I miss a dose?

Use the medicine as soon as you can, but skip the missed dose if you are more than 6 hours late for the dose. Do not use two doses at one time.

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 ivacaftor?

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

Grapefruit and Seville oranges may interact with ivacaftor and lead to unwanted side effects. Avoid the use of grapefruit products and orange marmalades.

Ivacaftor 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:

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.

Ivacaftor dosing information

Usual Adult Dose for Cystic Fibrosis:

150 mg orally every 12 hours

Comments:
-This drug should be taken with fat-containing food, examples include: eggs, butter, peanut butter, cheese pizza, and whole milk dairy products.
-If the patient's genotype is unknown, an approved CF mutation test should be used to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test instructions for use.

Uses:
-For the treatment of cystic fibrosis in patients having one mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.

Usual Pediatric Dose for Cystic Fibrosis:

Age: 12 months to less than 6 years:
Weight: 7 to less than 14 kg: 50 mg orally every 12 hours
Weight: 14 kg or greater: 75 mg orally every 12 hours

Age: 6 years and older:
150 mg orally every 12 hours

Comments:
-This drug should be taken with fat-containing food, examples include: eggs, butter, peanut butter, cheese pizza, and whole milk dairy products.
-The oral granule packets are recommended for patients less than 6 years old and the tablet formulation is recommended for patients 6 years and older.
-If the patient's genotype is unknown, an approved CF mutation test should be used to detect the presence of a CFTR mutation followed by verification with bi-directional sequencing when recommended by the mutation test instructions for use.

Uses:
-For the treatment of cystic fibrosis in patients 12 months or older having one mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.

What other drugs will affect ivacaftor?

Tell your doctor about all your other medicines, especially:

This list is not complete. Other drugs may affect ivacaftor, 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.

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