When This Medicine Should Not Be Used:
This medicine is not right for everyone. Do not use it if you had an allergic reaction to ivacaftor.

How to Use This Medicine:
Granule, Tablet

• Your doctor will tell you how much medicine to use. Do not use more than directed.
• Take this medicine with food that contains fat, such as cheese pizza, eggs, peanut butter, or whole-milk dairy products. This helps your body absorb the medicine.
• Oral granules: Shake the packet gently before you open it. Pour the contents into 1 teaspoon of cold soft food or liquid (such as applesauce, pureed fruits or vegetables, yogurt, water, milk, or juice) and mix together. Give this mixture within 1 hour and make sure all medicine is taken. Give food that contains fat right before or right after you give the mixture.
• Read and follow the patient instructions that come with this medicine. Talk to your doctor or pharmacist if you have any questions.
• Missed dose: If it is 6 hours or less from the time you normally take your medicine, take a dose as soon as you can. If it is more than 6 hours, skip the missed dose and take your next dose at the regular time. Do not take extra medicine to make up for a missed dose.
• Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light.

Drugs and Foods to Avoid:
Ask your doctor or pharmacist before using any other medicine, including over-the-counter medicines, vitamins, and herbal products.

• Some foods and medicines can affect how ivacaftor works. Tell your doctor if you are using any of the following:
  
  • Carbamazepine, clarithromycin, cyclosporine, digoxin, erythromycin, fluconazole, itraconazole, ketoconazole, midazolam, phenobarbital, phenytoin, posaconazole, rifabutin, rifampin, St John's wort, tacrolimus, telithromycin, or voriconazole
• Do not eat or drink anything that contains grapefruit or Seville oranges.

Warnings While Using This Medicine:

• Tell your doctor if you are pregnant or breastfeeding, or if you have kidney disease, liver disease, or cataracts.
• This medicine may make you dizzy. Do not drive or do anything that could be dangerous until you know how this medicine affects you.
• Your doctor will do lab tests at regular visits to check on the effects of this medicine. Keep all appointments.
• Keep all medicine out of the reach of children. Never share your medicine with anyone.

Possible Side Effects While Using This Medicine:
Call your doctor right away if you notice any of these side effects:

• Allergic reaction: Itching or hives, swelling in your face or hands, swelling or tingling in your mouth or throat, chest tightness, trouble breathing
• Changes in vision
• Dark urine or pale stools, nausea, vomiting, loss of appetite, stomach pain, yellow skin or eyes

If you notice these less serious side effects, talk with your doctor:

• Cough, stuffy or runny nose, sore throat
• Headache
• Mild rash

If you notice other side effects that you think are caused by this medicine, tell your doctor.
Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088