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Ivacaftor

(eye'' va kaf' tor)

Brand Name(s): Kalydeco®

WHY is this medicine prescribed?

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 6 months of age and older. Ivacaftor 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. Ivacaftor is in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) potentiators. It works 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.

Are there OTHER USES for this medicine?

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

HOW should this medicine be used?

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

Take each dose of ivacaftor with a fatty food such as eggs, butter, 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 ivacaftor.

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

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

What SPECIAL PRECAUTIONS should I follow?

Before taking ivacaftor,

  • tell your doctor and pharmacist if you are allergic to ivacaftor, any other medications, or any of the ingredients in ivacaftor tablets. Ask your pharmacist for a list of the ingredients.
  • tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, you are taking or plan to take. Be sure to mention any of the following: certain antibiotics such as clarithromycin (Biaxin, in PrevPac), erythromycin (E.E.S., E-Mycin, Ery-Tab, Erythrocin), and telithromycin (Ketek); certain antifungals such as fluconazole (Diflucan), itraconazole (Onmel, Sporanox), ketoconazole (Nizoral), posaconazole (Noxafil), and voriconazole (Vfend); cyclosporine (Gengraf, Neoral, Sandimmune); digoxin (Lanoxin); certain medications for seizures such as carbamazepine (Carbatrol, Epitol, Tegretol, others), phenobarbital, and phenytoin (Dilantin, Phenytek); midazolam; rifabutin (Mycobutin); rifampin (Rifadin, in Rifamate, in Rifater, Rimactane); or tacrolimus (Astagraf, Prograf). Your doctor may need to change the doses of your medications or monitor you carefully for side effects. Many other medications may also interact with ivacaftor, so be sure to tell your doctor about all the medications you are taking, even those that do not appear on this list.
  • tell your doctor what herbal products you are taking, especially St. John's wort.
  • tell your doctor if you have or have ever had liver or kidney disease.
  • tell your doctor if you are pregnant, plan to become pregnant, or are breast-feeding. If you become pregnant while taking ivacaftor, call your doctor.
  • you should know that ivacaftor may make you dizzy. Do not drive a car or operate machinery until you know how this medication affects you.

What SPECIAL DIETARY instructions should I follow?

Do not eat grapefruit or Seville oranges or drink grapefruit juice while taking this medication.

What should I do IF I FORGET to take 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 with a fat-containing food. However, if more than 6 hours have passed since the scheduled time, 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 medicine cause?

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

  • headache
  • rash
  • joint pain
  • mouth and throat pain
  • fever, sore throat, runny nose, or other signs of infection
  • diarrhea
  • dizziness

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
  • extreme tiredness
  • unusual bleeding or bruising
  • yellowing of the skin or eyes
  • vision changes

Ivacaftor may increase the risk that you or your child may develop cataracts. Talk to your doctor or your child's doctor about the risks of taking this medication.

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 (http://www.fda.gov/Safety/MedWatch) 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 (http://goo.gl/c4Rm4p) 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. http://www.upandaway.org

What should I do in case of OVERDOSE?

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at https://www.poisonhelp.org/help. 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 the following:

  • dizziness
  • diarrhea

What OTHER INFORMATION should I know?

Keep all appointments with your doctor and the laboratory. Your doctor will order an eye exam and certain lab tests before and during your treatment to check your body's response to ivacaftor.

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.


This report on medications is for your information only, and is not considered individual patient advice. Because of the changing nature of drug information, please consult your physician or pharmacist about specific clinical use.

The American Society of Health-System Pharmacists® represents that the information provided hereunder was formulated with a reasonable standard of care, and in conformity with professional standards in the field. The American Society of Health-System Pharmacists® makes no representations or warranties, express or implied, including, but not limited to, any implied warranty of merchantability and/or fitness for a particular purpose, with respect to such information and specifically disclaims all such warranties. Users are advised that decisions regarding drug therapy are complex medical decisions requiring the independent, informed decision of an appropriate health care professional, and the information is provided for informational purposes only. The entire monograph for a drug should be reviewed for a thorough understanding of the drug's actions, uses and side effects. The American Society of Health-System Pharmacists® does not endorse or recommend the use of any drug. The information is not a substitute for medical care.

Pronunciation Guide for Drug Names is used with permission. © 2009. The United States Pharmacopeial Convention. All Rights Reserved.

AHFS® Patient Medication Information™. © Copyright, 2020. The American Society of Health-System Pharmacists®, 4500 East-West Highway, Suite 900, Bethesda, Maryland. All Rights Reserved. Duplication for commercial use must be authorized by ASHP.

Selected Revisions: October 15, 2019.