Kalydeco

Ivacaftor is usually taken every 12 hours. Follow all directions on your prescription label. Do not take this medicine in larger or smaller amounts or for longer than recommended.

Read all patient information, medication guides, and instruction sheets provided to you. Ask your doctor or pharmacist if you have any questions.

Take ivacaftor with a food that contains fat, such as butter, peanut butter, eggs, cheese pizza. Follow your doctor's instructions very carefully.

For children 2 to 6 years old who take ivacaftor oral granules: Mix the granules with 1 teaspoon of liquid or soft food such as pudding, yogurt, applesauce, water, fruit juice, or milk. Mix only 1 dose at a time, and use the mixture within 1 hour after mixing. Then feed the child a high-fat food such as eggs, peanut butter, cheese, yogurt, or whole milk.

You will need frequent blood tests to check your liver function during treatment.

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.

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

Take the missed dose as soon as you remember. If you are more than 6 hours late, skip the missed dose. Do not take extra medicine to make up the missed dose.

Oral Tablets

Take with fat-containing food (eg, eggs, butter, peanut butter, cheese pizza)

Oral Granules

The entire contents of each packet of oral granules should be mixed with 1 teaspoon (5 mL) of age-appropriate soft food or liquid and completely consumed

Food or liquid should be at or below room temperature

Once mixed, the product has been shown to be stable for 1 hour, and therefore should be consumed during this period

Some examples of soft foods or liquids may include puréed fruits or vegetables, yogurt, applesauce, water, milk, or juice

Each dose should be administered just before or just after eating fat-containing food (eg, eggs, butter, peanut butter, cheese pizza)

Included as part of the PRECAUTIONS section.

KALYDECO
(kuh-LYE-deh-koh)
(ivacaftor) Film-Coated Tablets and Oral Granules

Read this Patient Information before you start taking KALYDECO and each time you get a refill. There may be new information. This information does not take the place of talking to your doctor about your medical condition or your treatment.

What is KALYDECO?

KALYDECO is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients age 2 years and older who have at least one mutation in their CF gene that is responsive to KALYDECO.

Talk to your doctor to learn if you have an indicated CF gene mutation.

It is not known if KALYDECO is safe and effective in children under 2 years of age.

Who should not take KALYDECO?

Do not take KALYDECO if you take certain medicines or herbal supplements such as:

• the antibiotics rifampin (Rifamate®, Rifater®) or rifabutin (Mycobutin®)
• seizure medications such as phenobarbital, carbamazepine (Tegretol®, Carbatrol®, Equetro®) or phenytoin (Dilantin®, Phenytek®)
• St. John's wort

Talk to your doctor before taking KALYDECO if you take any of the medicines or supplements listed above.

What should I tell my doctor before taking KALYDECO?

Before you take KALYDECO, tell your doctor if you:

• have liver or kidney problems
• drink grapefruit juice, or eat grapefruit or Seville oranges
• are pregnant or plan to become pregnant. It is not known if KALYDECO will harm your unborn baby. You and your doctor should decide if you will take KALYDECO while you are pregnant.
• are breastfeeding or planning to breastfeed. It is not known if KALYDECO passes into your breast milk. You and your doctor should decide if you will take KALYDECO while you are breastfeeding.

KALYDECO may affect the way other medicines work, and other medicines may affect how KALYDECO works.

Tell your doctor about all the medicines you take, including prescription and non-prescription medicines, vitamins, and herbal supplements, as the dose of KALYDECO may need to be adjusted when taken with certain medications.

Ask your doctor or pharmacist for a list of these medicines if you are not sure.

Especially tell your doctor if you take:

• antifungal medications such as ketoconazole (e.g., Nizoral®), itraconazole (e.g., Sporanox®), posaconazole (e.g., Noxafil®), voriconazole (e.g., Vfend®), or fluconazole (e.g., Diflucan®)
• antibiotics such as telithromycin (e.g., Ketek®), clarithromycin (e.g., Biaxin®), or erythromycin (e.g., Ery-Tab®)

Know the medicines you take. Keep a list of them to show your doctor and pharmacist when you get a new medicine.

How should I take KALYDECO?

• Take KALYDECO exactly as your doctor tells you to take it.
• Take your doses of KALYDECO 12 hours apart.
• If you miss a dose of KALYDECO and it is within 6 hours of when you usually take it, take your dose of KALYDECO as prescribed with fat-containing food as soon as possible.
• If you miss a dose of KALYDECO and it is more than 6 hours after the time you usually take it, skip that dose only and take the next dose when you usually take it. Do not take 2 doses at the same time to make up for your missed dose.

KALYDECO Tablets (ages 6 years and older):

• Always take KALYDECO tablets with food that contains fat. Examples of fat-containing foods include eggs, butter, peanut butter, cheese pizza, and whole-milk dairy products such as whole milk, cheese, and yogurt.
• Each KALYDECO box contains 4 individual blister cards.
• Each blister card contains 14 pills—7 morning doses and 7 evening doses.
• In the morning, unpeel the paper backing from a blister card to remove 1 KALYDECO tablet and take it with food that contains fat.
• In the evening, 12 hours later, open another blister card to remove 1 KALYDECO tablet and take it with food that contains fat.
• You may cut along the dotted line to separate your doses from the blister card.

KALYDECO Oral Granules (ages 2 to under 6 years old):

• Hold the packet with cut line on top.
• Shake the packet gently to settle the KALYDECO granules.
• Tear or cut packet open along cut line.
• Carefully pour all of the KALYDECO granules in the packet into 1 teaspoon of soft food or liquid. Food or liquid should be at or below room temperature. Some examples of soft foods or liquids include puréed fruits or vegetables, yogurt, applesauce, water, milk, or juice.
• Mix the KALYDECO granules with food or liquid.
• After mixing, give KALYDECO within 1 hour. Make sure all medicine is taken.
• Give a child fat-containing food just before or just after the KALYDECO granules dose. Examples of fat-containing foods include eggs, butter, peanut butter, cheese pizza, and whole-milk dairy products such as whole milk, cheese, and yogurt.

What should I avoid while taking KALYDECO?

• KALYDECO can cause dizziness in some people who take it. Do not drive a car, use machinery or do anything that needs you to be alert until you know how KALYDECO affects you.
• You should avoid food containing grapefruit or Seville oranges while you are taking KALYDECO.

What are the possible side effects of KALYDECO?

KALYDECO can cause serious side effects.

High liver enzymes in the blood have been reported in patients receiving KALYDECO. Your doctor will do blood tests to check your liver:

• before you start KALYDECO
• every 3 months during your first year of taking KALYDECO
• every year while you are taking KALYDECO

For patients who have had high liver enzymes in the past, the doctor may do blood tests to check the liver more often.

Call your doctor right away if you have any of the following symptoms of liver problems:

• pain or discomfort in the upper right stomach (abdominal) area
• yellowing of your skin or the white part of your eyes
• loss of appetite
• nausea or vomiting
• dark, amber-colored urine

Abnormality of the eye lens (cataract) has been noted in some children and adolescents receiving KALYDECO.

Your doctor should perform eye examinations prior to and during treatment with KALYDECO to look for cataracts.

The most common side effects of KALYDECO include:

• headache
• upper respiratory tract infection (common cold), including:
  • sore throat
  • nasal or sinus congestion
  • runny nose
• stomach (abdominal) pain
• diarrhea
• rash
• nausea
• dizziness

Tell your doctor if you have any side effect that bothers you or that does not go away.

These are not all the possible side effects of KALYDECO. For more information, ask your doctor or pharmacist.

Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

How should I store KALYDECO?

• Store KALYDECO at room temperature between 68°F to 77°F (20°C to 25°C).
• Do not use KALYDECO after the expiration date on the package.

Keep KALYDECO and all medicines out of the reach of children.

General information about the safe and effective use of KALYDECO

Medicines are sometimes prescribed for purposes other than those listed in a Patient Information leaflet. Do not use KALYDECO for a condition for which it was not prescribed. Do not give KALYDECO to other people, even if they have the same symptoms you have. It may harm them.

This Patient Information summarizes the most important information about KALYDECO. If you would like more information, talk with your doctor. You can ask your pharmacist or doctor for information about KALYDECO that is written for health professionals.

For more information, go to www.kalydeco.com or call 1-877-752-5933.

What are the ingredients in KALYDECO?

Active ingredient: ivacaftor

Inactive ingredients:

KALYDECO Tablets are light blue, film-coated, capsule-shaped tablets for oral administration and contain the following inactive ingredients: colloidal silicon dioxide, croscarmellose sodium, hypromellose acetate succinate, lactose monohydrate, magnesium stearate, microcrystalline cellulose, and sodium lauryl sulfate.

The tablet film coat contains: carnauba wax, FD&C Blue #2, PEG 3350, polyvinyl alcohol, talc, and titanium dioxide.

The printing ink contains: ammonium hydroxide, iron oxide black, propylene glycol, and shellac.

KALYDECO Oral Granules are white to off-white granules for oral administration (sweetened but unflavored) and contain the following inactive ingredients: colloidal silicon dioxide, croscarmellose sodium, hypromellose acetate succinate, lactose monohydrate, magnesium stearate, mannitol, sucralose, and sodium lauryl sulfate.

This Patient Information has been approved by the U.S. Food and Drug Administration.

Kalydeco is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients age 2 years and older who have at least one mutation in their CF gene that is responsive to this medication.  

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

• Take Kalydeco exactly as your doctor tells you to take it.
• Kalydeco comes in tablet form and granules to be taken by mouth. 
• Your doses of Kalydeco should be taken 12 hours apart.
• If you miss a dose of Kalydeco and it is within 6 hours of when you usually take it, take your dose of Kalydeco as prescribed with fat-containing food as soon as possible.
• If you miss a dose of Kalydeco and it is more than 6 hours after the time you usually take it, skip that dose only and take the next dose when you usually take it. Do not take 2 doses at the same time to make up for your missed dose. 

Kalydeco Tablets (ages 6 years and older):

• Always take Kalydeco Tablets with food that contains fat. Examples of fat-containing foods include eggs, butter, peanut butter, cheese pizza, and whole-milk dairy products such as whole milk, cheese, and yogurt.
• Each Kalydeco box contains 4 individual blister cards.
• Each blister card contains 14 pills—7 morning doses and 7 evening doses.
• In the morning, unpeel the paper backing from a blister card to remove 1 Kalydeco tablet and take it with food that contains fat. 
• In the evening, 12 hours later, open another blister card to remove 1 Kalydeco tablet and take it with food that contains fat.
• You may cut along the dotted line to separate your doses from the blister card.

Kalydeco Oral Granules (ages 2 to under 6 years old):

• Hold the packet with cut line on top.
• Shake the packet gently to settle the Kalydeco granules. 
• Tear or cut packet open along cut line.
• Carefully pour all of the Kalydeco granules in the packet into 1 teaspoon of soft food or liquid. Food or liquid should be at or below room temperature. Some examples of soft foods or liquids include puréed fruits or vegetables, yogurt, applesauce, water, milk, or juice.
• Mix the Kalydeco granules with food or liquid.
• After mixing, give Kalydeco within 1 hour. Make sure all medicine is taken.
• Give a child fat-containing food just before or just after the Kalydeco granules dose. Examples of fat containing foods include eggs, butter, peanut butter, cheese pizza, and whole-milk dairy products such as whole milk, cheese, and yogurt.

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

Some medicines can interact with ivacaftor and should not be used at the same time. Your doctor may need to change your treatment plan if you use any of the following drugs:

• rifabutin or rifampin;

• St. John's wort; or

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

To make sure ivacaftor is safe for you, tell your doctor if you have:

• 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.

Ivacaftor may pass into breast milk and could harm a nursing baby. Tell your doctor if you are breast-feeding a baby.

Ivacaftor is not approved for use by anyone younger than 2 years old.

Cystic Fibrosis

Treatment of cystic fibrosis in patients with G551D mutation in the CFTR gene (designated an orphan drug by FDA for this use).1 2 3 4 12

Use an FDA-approved cystic fibrosis mutation test to detect presence of G551D mutation if genotype of patient unknown.1

Not effective in patients with cystic fibrosis homozygous for F508del mutation in the CFTR gene.1 5 F508del mutation in the CFTR gene is most common mutation causing cystic fibrosis; patients homozygous for this mutation account for about 50% of disease population.5

Not studied in other patient populations with cystic fibrosis.1

In the U.S.

• Kalydeco

Available Dosage Forms:

• Granule
• Tablet

Therapeutic Class: Respiratory Agent

Potential for other drugs to affect ivacaftor

Inhibitors of CYP3A

Ivacaftor is a sensitive CYP3A substrate. Co-administration with ketoconazole, a strong CYP3A inhibitor, significantly increased ivacaftor exposure [measured as area under the curve (AUC)] by 8.5-fold. Based on simulations of these results, a reduction of the Kalydeco dose is recommended when co-administered with strong CYP3A inhibitors, such as ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, and clarithromycin, as follows: in patients 6 years and older reduce dose to one 150 mg tablet twice a week; in patients 2 to less than 6 years with body weight less than 14 kg, reduce dose to one 50 mg packet of granules twice a week; and in patients 2 to less than 6 years with body weight 14 kg or greater, reduce dose to one 75 mg packet of granules twice a week.

Co-administration with fluconazole, a moderate inhibitor of CYP3A, increased ivacaftor exposure by 3-fold. Therefore, a reduction of the Kalydeco dose is recommended for patients taking concomitant moderate CYP3A inhibitors, such as fluconazole and erythromycin, as follows: in patients 6 years and older reduce dose to one 150 mg tablet once daily; in patients 2 to less than 6 years with body weight less than 14 kg, reduce dose to one 50 mg packet of granules once daily; and in patients 2 to less than 6 years with body weight 14 kg or greater, reduce dose to one 75 mg packet of granules once daily.

Co-administration of Kalydeco with grapefruit juice, which contains one or more components that moderately inhibit CYP3A, may increase exposure of ivacaftor. Therefore, food containing grapefruit or Seville oranges should be avoided during treatment with Kalydeco [see Clinical Pharmacology (12.3)].

Inducers of CYP3A

Co-administration with rifampin, a strong CYP3A inducer, significantly decreased ivacaftor exposure (AUC) by approximately 9-fold. Therefore, co-administration with strong CYP3A inducers, such as rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, and St. John's wort is not recommended [see Warnings and Precautions (5.2) and Clinical Pharmacology (12.3)].

Ciprofloxacin

Co-administration of Kalydeco with ciprofloxacin had no effect on the exposure of ivacaftor. Therefore, no dose adjustment is necessary during concomitant administration of Kalydeco with ciprofloxacin [see Clinical Pharmacology (12.3)].

Potential for ivacaftor to affect other drugs

CYP3A and/or P-gp Substrates

Ivacaftor and its M1 metabolite have the potential to inhibit CYP3A and P-gp. Co-administration with oral midazolam, a sensitive CYP3A substrate, increased midazolam exposure 1.5-fold, consistent with weak inhibition of CYP3A by ivacaftor. Co-administration with digoxin, a sensitive P-gp substrate, increased digoxin exposure by 1.3-fold, consistent with weak inhibition of P-gp by ivacaftor. Administration of Kalydeco may increase systemic exposure of drugs that are substrates of CYP3A and/or P-gp, which may increase or prolong their therapeutic effect and adverse events. Therefore, caution and appropriate monitoring are recommended when co-administering Kalydeco with sensitive CYP3A and/or P-gp substrates, such as digoxin, cyclosporine, and tacrolimus [see Clinical Pharmacology (12.3)].

NDC 51167-200-01
Rx only

Kalydeco®
(ivacaftor) tablets

150 mg per tablet

Carton contains
4 individual blister cards
of 14 tablets per card.

56 tablets