Ivacaftor (eye-va-KAF-tor), Lumacaftor (loo-ma-KAF-tor)

Treats cystic fibrosis.

Brand Name(s):

Orkambi

When This Medicine Should Not Be Used:

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, including avocados, butter, cheese pizza, eggs, nuts, peanut butter, breast milk, baby formula, or whole-milk dairy products (including whole milk, cheese, yogurt). This helps your body absorb the medicine better.
• Granules: Mix the entire content of each packet with 1 teaspoon (5 milliliters [mL]) of cool to room temperature soft food (including puréed fruits, flavored yogurt, pudding, applesauce) or liquid (including water, milk, breast milk, baby formula, juice). Take the mixture within 1 hour after mixing.
• 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 lessfrom 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 medicines can affect how lumacaftor/ivacaftor works. Tell your doctor if you are using any of the following:
  • Carbamazepine, clarithromycin, cyclosporine, digoxin, erythromycin, everolimus, ibuprofen, itraconazole, ketoconazole, midazolam, montelukast, phenobarbital, phenytoin, posaconazole, rifabutin, rifampin, sirolimus, St John's wort, tacrolimus, telithromycin, triazolam, voriconazole
  • Birth control pills or other hormone-based contraceptives (including implants, injections, patches, rings)
  • Blood thinner (including warfarin)
  • Medicine to treat depression (including citalopram, escitalopram, sertraline)
  • Oral diabetes medicine (including glimepiride, glipizide, glyburide, repaglinide)
  • Steroid medicine (including methylprednisolone, prednisone)
  • Stomach medicine (including esomeprazole, lansoprazole, omeprazole, ranitidine)

Warnings While Using This Medicine:

• Tell your doctor if you are pregnant or breastfeeding, or if you have kidney disease, liver disease, lung problems, or high blood pressure. Tell your doctor if you have had an organ transplant.
• This medicine may cause the following problems:
  • Liver problems
  • Lung or breathing problems
  • High blood pressure
  • Eye or vision problems
• 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
• Blurred vision, changes in vision
• Dark urine or pale stools, nausea, vomiting, loss of appetite, stomach pain, yellow skin or eyes
• Fast, slow, or pounding heartbeat
• Trouble breathing, chest tightness or discomfort

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

• Cough, stuffy or runny nose, sore throat
• Irregular, missed, or abnormal menstrual periods
• Mild nausea, diarrhea, gas

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