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Ivacaftor and lumacaftor

Generic name: ivacaftor and lumacaftor [ EYE-va-KAF-tor-and-LOO-ma-KAF-tor ]
Brand name: Orkambi
Dosage forms: oral granule for reconstitution (125 mg-100 mg; 188 mg-150 mg; 94 mg-75 mg), oral tablet (125 mg-100 mg; 125 mg-200 mg)
Drug class: CFTR combinations

Medically reviewed by Drugs.com on Aug 22, 2023. Written by Cerner Multum.

What is ivacaftor and lumacaftor?

Ivacaftor and lumacaftor is a combination medicine used to treat cystic fibrosis in adults and children who are at least 1 year old.

Ivacaftor and lumacaftor tablet can be used in children aged 6 years and older. Ivacaftor and lumacaftor granules can be used in children at least 1 year old.

Your doctor will make sure you have the specific gene mutation related to cystic fibrosis to be treated with ivacaftor and lumacaftor.

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

Ivacaftor and lumacaftor 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.

Ivacaftor and lumacaftor may cause serious side effects. Call your doctor at once if you have:

Common side effects of ivacaftor and lumacaftor may include:

Some side effects are more common in children taking ivacaftor and lumacaftor such as increase in mucus, cough with mucus, headache, or stomach pain.

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.

Warnings

Use only as directed. Tell your doctor if you use other medicines or have other medical conditions or allergies.

Before taking this medicine

You should not be treated with ivacaftor and lumacaftor if you are allergic to it, or if you have or have ever had:

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

Ask a doctor if it is safe to breastfeed while using ivacaftor and lumacaftor.

Ivacaftor and lumacaftor can make hormonal birth control less effective, including birth control pills, injections, implants, skin patches, and vaginal rings. Use barrier birth control to prevent pregnancy: condom, diaphragm, cervical cap, or contraceptive sponge.

How should I take ivacaftor and lumacaftor?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.

Take this medicine with food that contains fat, such as eggs, milk products such as whole milk, cheese, yogurt, breast milk or infant formula. Follow your doctor's instructions carefully.

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

Mix the ivacaftor and lumacaftor granules with 1 teaspoon of soft food or liquid such as pureed fruits or vegetables, yogurt, applesauce, water, breast milk, infant formula, milk, or juice. The food or liquid should be at or below room temperature. Mix only 1 dose at a time and use within 1 hour after mixing. Feed the child a high-fat food after or before giving the granule mixture.

Take this medicine at evenly spaced intervals every 12 hours.

Doses are based on weight in children. Your child's dose may change if the child gains or loses weight.

You will need frequent blood tests to check your liver function, and your blood pressure may need to be checked often. You may also need other medical tests.

Your child may need frequent vision exams.

Store at room temperature away from moisture and heat. Keep the medicine in the original packaging.

Ivacaftor and lumacaftor dosing information

Usual Adult Dose for Cystic Fibrosis:

Lumacaftor 400 mg/Ivacaftor 250 mg orally every 12 hours with fat-containing food

Comments:
-A fat containing meal or snack should be consumed just before or just after dosing. Examples include eggs, avocados, nuts, butter, peanut butter, cheese pizza, or whole-milk dairy products.
-If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene; safety and efficacy in patients with cystic fibrosis other than homozygous F508del mutation has not been established.


Use: For the treatment of cystic fibrosis (CF) in patients who are homozygous for the F508del mutation in the CFTR gene.

Usual Pediatric Dose for Cystic Fibrosis:

Age: 2 through 5 years; weight less than 14 kg: Lumacaftor 100 mg/Ivacaftor 125 mg orally every 12 hours with fat-containing food
Age: 2 through 5 years: weight 14 kg or greater: Lumacaftor 150 mg/Ivacaftor 188 mg orally every 12 hours with fat-containing food

Age: 6 through 11 years: Lumacaftor 200 mg/Ivacaftor 250 mg orally every 12 hours with fat-containing food

Age: 12 years or older: Lumacaftor 400 mg/Ivacaftor 250 mg orally every 12 hours with fat-containing food

Comments:
-Pediatric patients 2 through 5 years of age should receive oral granules; pediatric patients over 6 years of age, should be dosed with tablets.
-A fat containing meal or snack should be consumed just before or just after dosing. Examples include eggs, avocados, nuts, butter, peanut butter, cheese pizza, or whole-milk dairy products.
-If the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene; safety and efficacy in patients with cystic fibrosis other than homozygous F508del mutation has not been established.

Use: For the treatment of cystic fibrosis (CF) in patients 2 years or older who are homozygous for the F508del mutation in the CFTR gene.

azithromycin, Zithromax, gentamicin, Creon, tobramycin

What happens if I miss a dose?

Take the medicine as soon as you can, with a fatty meal, but skip the missed dose if you are more than 6 hours late for the dose. Do not take two doses at one time.

Call your doctor for instructions if you stop taking ivacaftor and lumacaftor for more than 1 week.

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 and lumacaftor?

Grapefruit may interact with ivacaftor and lumacaftor and cause side effects. Avoid consuming grapefruit products during the first week of treatment.

What other drugs will affect ivacaftor and lumacaftor?

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

Tell your doctor about all your current medicines. Many drugs can affect ivacaftor and lumacaftor, especially:

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

Does ivacaftor/lumacaftor interact with my other drugs?

Enter other medications to view a detailed interaction report.

Popular FAQ

How do you take Orkambi?

Orkambi should be taken every 12 hours with fat-containing food such as eggs, avocados, nuts, butter, peanut butter, cheese, and whole-milk dairy products. Granules are available for younger children aged one through five years and these should be mixed with one teaspoon of soft food or liquid. Other therapies prescribed for CF - including bronchodilators, inhaled antibiotics, dornase alfa, and hypertonic saline - should continue to be used if you are prescribed Orkambi. Continue reading

More FAQ

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.