Kalydeco is used to treat cystic fibrosis in adults and children who are at least 4 months old and weigh at least 11 pounds (5 kilograms).

Kalydeco is for use only in patients with a specific gene mutation related to cystic fibrosis.

Before you take Kalydeco, you may need a medical test to make sure you have this gene mutation.

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

Before you take Kalydeco, tell your doctor if you have liver or kidney disease.

Kalydeco should be taken with a food that contains fat, such as butter, peanut butter, eggs, cheese pizza, or whole-milk dairy products such as whole milk, cheese, and yogurt. Follow your doctor's instructions very carefully.

Grapefruit, grapefruit juice, and Seville oranges may interact with Kalydeco and lead to unwanted effects. Discuss the use of grapefruit and orange products with your doctor.

To be sure Kalydeco is not causing harmful effects, your liver function will need to be checked with frequent blood tests. Visit your doctor regularly.

There are many other drugs that can interact with ivacaftor. Tell your doctor about all medications you use. This includes prescription, over-the-counter, vitamin, and herbal products. Do not start a new medication without telling your doctor. Keep a list of all your medicines and show it to any healthcare provider who treats you.

Kalydeco is usually taken every 12 hours. Follow all directions on your prescription label and read all medication guides or instruction sheets.

Take Kalydeco 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 Kalydeco 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 4 months to 5 years old should take oral granules. Children 6 years 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.

Usual Adult Dose for Cystic Fibrosis:

One 150 mg tablet taken orally every 12 hours with fat-containing food.

Usual Pediatric Dose for Cystic Fibrosis:

• Pediatric patients 6 years and older: One 150 mg tablet taken orally every 12 hours with fat-containing food.
• Pediatric patients 4 months to less than 6 years of age:
  • Weighing 5 kg to less than 7 kg: one 25 mg packet mixed with 1 teaspoon (5 mL) of soft food or liquid and administered orally every 12 hours with fat-containing food.
  • Weighing 7 kg to less than 14 kg: one 50 mg packet mixed with 1 teaspoon (5 mL) of soft food or liquid and administered orally every 12 hours with fat-containing food.
  • Weighing 14 kg or greater: one 75 mg packet mixed with 1 teaspoon (5 mL) of soft food or liquid and administered orally every 12 hours with fat-containing food.
• Pediatric patients less than 4 months of age: not recommended.

-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 4 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.

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

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

• rifabutin or rifampin;

• St. John's wort; or

• seizure medicine such as carbamazepine, phenobarbital, or phenytoin.

Tell your doctor if you have ever had:

• liver disease; or

• kidney disease.

It is not known whether this medicine 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.

Kalydeco should not be given to a child younger than 4 months old, or to a child with liver problems who is 4 months to less than 6 months old.

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.

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

Avoid driving or hazardous activity until you know how this medicine 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.

Get emergency medical help if you have signs of an allergic reaction to Kalydeco: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have:

• severe stomach pain;

• vision problems, eye pain;

• low blood sugar - headache, hunger, sweating, irritability, dizziness, nausea, fast heart rate, and feeling anxious or shaky; or

• liver problems - loss of appetite, stomach pain (upper right side), nausea, vomiting, dark urine, jaundice (yellowing of the skin or eyes).

Common Kalydeco side effects may include:

• dizziness;

• rash;

• headache;

• stomach pain, nausea, diarrhea; or

• cold symptoms such as stuffy nose, runny nose, sore throat.

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.

Tell your doctor about all your other medicines, especially:

• an antibiotic - ciprofloxacin, clarithromycin, erythromycin, telithromycin; or

• antifungal medicine - fluconazole, itraconazole, ketoconazole, posaconazole, voriconazole.

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