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Kalydeco (Granules)

Generic name: Ivacaftor Granules (eye va KAF tor)
Brand name: Kalydeco
Drug class: CFTR potentiators

Medically reviewed by Last updated on Nov 5, 2020.

Uses of Kalydeco:

  • It is used to treat cystic fibrosis.

What do I need to tell my doctor BEFORE I take Kalydeco?

For all patients taking Kalydeco (ivacaftor granules):

  • If you are allergic to Kalydeco (ivacaftor granules); any part of Kalydeco (ivacaftor granules); or any other drugs, foods, or substances. Tell your doctor about the allergy and what signs you had.
  • If you are taking any of these drugs: Carbamazepine, phenobarbital, phenytoin, rifabutin, rifampin, or St. John's wort.


  • If your child is younger than 6 months of age. Do not give Kalydeco (ivacaftor granules) to an infant younger than 6 months of age.

This is not a list of all drugs or health problems that interact with Kalydeco (ivacaftor granules).

Tell your doctor and pharmacist about all of your drugs (prescription or OTC, natural products, vitamins) and health problems. You must check to make sure that it is safe for you to take Kalydeco (ivacaftor granules) with all of your drugs and health problems. Do not start, stop, or change the dose of any drug without checking with your doctor.

What are some things I need to know or do while I take Kalydeco?

  • Tell all of your health care providers that you take Kalydeco (ivacaftor granules). This includes your doctors, nurses, pharmacists, and dentists.
  • Avoid driving and doing other tasks or actions that call for you to be alert until you see how Kalydeco (ivacaftor granules) affects you.
  • Have blood work checked as you have been told by the doctor. Talk with the doctor.
  • Have your lung function checked as you have been told by your doctor. Talk with your doctor.
  • Avoid grapefruit and grapefruit juice.
  • Avoid Seville oranges.
  • Cataracts have happened in children taking Kalydeco (ivacaftor granules). Have an eye exam before starting Kalydeco (ivacaftor granules) and while taking it. Talk with your doctor
  • Tell your doctor if you are pregnant, plan on getting pregnant, or are breast-feeding. You will need to talk about the benefits and risks to you and the baby.

How is this medicine (Kalydeco) best taken?

Use Kalydeco (ivacaftor granules) as ordered by your doctor. Read all information given to you. Follow all instructions closely.

  • Mix the contents of the packet with 1 teaspoon (5 mL) of soft food or liquid. This includes pureed fruits or vegetables, yogurt, applesauce, water, breast milk, baby formula, milk, or juice. Food or liquid needs to be at or below room temperature.
  • Take Kalydeco (ivacaftor granules) within 1 hour of mixing.
  • Take Kalydeco (ivacaftor granules) just before or just after fatty foods. This includes eggs; butter; peanut butter; cheese pizza; or whole-milk dairy products like whole-milk, cheese, or yogurt.
  • Keep taking Kalydeco (ivacaftor granules) as you have been told by your doctor or other health care provider, even if you feel well.

What do I do if I miss a dose?

  • Take a missed dose as soon as you think about it.
  • If it has been 6 hours or more since the missed dose, skip the missed dose and go back to your normal time.
  • Do not take 2 doses at the same time or extra doses.
  • If you are not sure what to do if you miss a dose, call your doctor.

What are some side effects that I need to call my doctor about right away?

WARNING/CAUTION: Even though it may be rare, some people may have very bad and sometimes deadly side effects when taking a drug. Tell your doctor or get medical help right away if you have any of the following signs or symptoms that may be related to a very bad side effect:

  • Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.
  • Signs of liver problems like dark urine, feeling tired, not hungry, upset stomach or stomach pain, light-colored stools, throwing up, or yellow skin or eyes.
  • Signs of high blood sugar like confusion, feeling sleepy, more thirst, more hungry, passing urine more often, flushing, fast breathing, or breath that smells like fruit.
  • Change in eyesight.
  • Chest pain.

What are some other side effects of Kalydeco?

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away:

These are not all of the side effects that may occur. If you have questions about side effects, call your doctor. Call your doctor for medical advice about side effects.

You may report side effects to the FDA at 1-800-332-1088. You may also report side effects at

If OVERDOSE is suspected:

If you think there has been an overdose, call your poison control center or get medical care right away. Be ready to tell or show what was taken, how much, and when it happened.

How do I store and/or throw out Kalydeco?

  • Store at room temperature.
  • Store in a dry place. Do not store in a bathroom.
  • Keep all drugs in a safe place. Keep all drugs out of the reach of children and pets.
  • Throw away unused or expired drugs. Do not flush down a toilet or pour down a drain unless you are told to do so. Check with your pharmacist if you have questions about the best way to throw out drugs. There may be drug take-back programs in your area.

Consumer information use

  • If your symptoms or health problems do not get better or if they become worse, call your doctor.
  • Do not share your drugs with others and do not take anyone else's drugs.
  • Some drugs may have another patient information leaflet. Check with your pharmacist. If you have any questions about Kalydeco (ivacaftor granules), please talk with your doctor, nurse, pharmacist, or other health care provider.
  • If you think there has been an overdose, call your poison control center or get medical care right away. Be ready to tell or show what was taken, how much, and when it happened.

Frequently asked questions

Further information

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