Orkambi (as a combination product containing Lumacaftor and Ivacaftor)

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.

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); diazepam (Diastat, Valium); 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 breastfeeding. 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.

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

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.

• Kalydeco^®
• Orkambi^® (as a combination product containing Lumacaftor and Ivacaftor)