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Lumacaftor and Ivacaftor

pronounced as (loo'' ma kaf' tor) and (eye'' va kaf' tor)

Why is this medication prescribed?

Lumacaftor 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 1 year of age and older. Lumacaftor is 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. Both of 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 improving other symptoms of cystic fibrosis.

How should this medicine be used?

The combination of lumacaftor and ivacaftor comes as a tablet and as granules to take by mouth. It is usually taken with fatty foods twice a day, 12 hours apart. Take lumacaftor and ivacaftor at around 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 lumacaftor and ivacaftor exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.

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

Take lumacaftor and ivacaftor with fatty foods such as eggs, avocados, nuts, butter, peanut butter, cheese pizza, whole milk and other whole milk products such as cheese and full fat yogurt. Talk to your doctor about other fatty foods to eat with lumacaftor and ivacaftor.

Lumacaftor and ivacaftor controls cystic fibrosis but does not cure it. Continue to take lumacaftor and ivacaftor even if you feel well. Do not stop taking lumacaftor and ivacaftor without talking to your doctor.

If you do not take lumacaftor and ivacaftor for 7 days or longer, do not start taking it again without talking to your doctor. Your doctor may need to change your dose of this medication or other medications you are taking.

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

Other uses for this medicine

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

What special precautions should I follow?

Before taking lumacaftor and ivacaftor,

  • tell your doctor and pharmacist if you are allergic to lumacaftor and ivacaftor, any other medications, or any of the ingredients in lumacaftor and ivacaftor tablets or granules. Ask your pharmacist for a list of the ingredients.
  • tell your doctor and pharmacist what medications you are taking or plan to take while taking lumacaftor and ivacaftor. Your doctor may need to change the doses of your medications or monitor you carefully for side effects.
  • the following nonprescription or herbal products may interact with lumacaftor and ivacaftor tablets: lansoprazole, (Prevacid), omeprazole (Prilosec), St. John's Wort, and ranitidine (Zantac). Be sure to let your doctor and pharmacist know that you are taking these medications before you start taking lumacaftor and ivacaftor tablets. Do not start any of these medications while taking lumacaftor and ivacaftor without discussing with your healthcare provider.
  • tell your doctor if you have or have ever had breathing problems or conditions, an organ transplant, or liver or kidney disease.
  • you should know that lumacaftor and ivacaftor may decrease the effectiveness of hormonal contraceptives (birth control pills, patches, rings, injections, implants, or intrauterine devices). Talk to your doctor about other methods of birth control that will work for you while you are taking lumacaftor and ivacaftor.
  • tell your doctor if you are pregnant, plan to become pregnant, or are breast-feeding. If you become pregnant while taking lumacaftor and ivacaftor, call your doctor.

What special dietary instructions should I follow?

Do not eat grapefruit or drink grapefruit juice during your first week of treatment with lumacaftor and ivacaftor.

Unless your doctor tells you otherwise, continue your normal diet.

What should I do if I forget a dose?

If you remember the missed dose within 6 hours of the time you were scheduled to take it, take the missed dose right away. However, if more than 6 hours have passed since the time you usually take lumacaftor and ivacaftor, skip the missed dose and continue your regular dosing schedule. Do not take a double dose to make up for a missed one.

What side effects can this medication cause?

Lumacaftor and ivacaftor may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

  • shortness of breath
  • chest tightness or pain
  • breathing problems
  • diarrhea
  • gas
  • excessive tiredness
  • rash
  • irregular, missed, heavy or painful menstrual periods, especially in women taking hormonal contraceptives
  • runny nose, sneezing, cough, sore throat, or flu-like symptoms
  • headache

Some side effects can be serious. If you experience any of these symptoms, call your doctor immediately:

  • nausea
  • vomiting
  • loss of appetite
  • pain in the upper right part of the stomach
  • dark urine
  • confusion
  • yellowing of the skin or eyes
  • rash; hives; itching; swelling of the face, eyes, lips, tongue, mouth, or throat; wheezing; difficulty breathing or swallowing; or lightheadedness

Lumacaftor and ivacaftor may cause cataracts (clouding of the lens of the eye that may cause vision problems) in children and teenagers. Children and teenagers taking lumacaftor and ivacaftor should see an eye doctor before and during their treatment. Talk to your child's doctor about the risks of giving lumacaftor and ivacaftor to your child.

Lumacaftor and ivacaftor may cause other side effects. Call your doctor if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online ( or by phone (1-800-332-1088).

What should I know about storage and disposal of this medication?

Keep this medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom).

Unneeded medications should be disposed of in special ways to ensure that pets, children, and other people cannot consume them. However, you should not flush this medication down the toilet. Instead, the best way to dispose of your medication is through a medicine take-back program. Talk to your pharmacist or contact your local garbage/recycling department to learn about take-back programs in your community. See the FDA's Safe Disposal of Medicines website ( for more information if you do not have access to a take-back program.

It is important to keep all medication out of sight and reach of children as many containers (such as weekly pill minders and those for eye drops, creams, patches, and inhalers) are not child-resistant and young children can open them easily. To protect young children from poisoning, always lock safety caps and immediately place the medication in a safe location – one that is up and away and out of their sight and reach.

In case of emergency/overdose

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at If the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services at 911.

Symptoms of overdose may include:

  • headache
  • rash

What other information should I know?

Keep all appointments with your doctor and the laboratory. Your doctor will order a lab test before you begin your treatment to see whether your condition can be treated with lumacaftor and ivacaftor. Your doctor will also order certain lab tests before and during your treatment to check your body's response to lumacaftor and ivacaftor. Your blood pressure should be checked regularly while you are taking this medication.

Do not let anyone else take your medication. Ask your pharmacist any questions you have about refilling your prescription.

It is important for you to keep a written list of all of the prescription and nonprescription (over-the-counter) medicines you are taking, as well as any products such as vitamins, minerals, or other dietary supplements. You should bring this list with you each time you visit a doctor or if you are admitted to a hospital. It is also important information to carry with you in case of emergencies.

Brand names

  • Orkambi®
Last Revised - 10/15/2023