Ivacaftor, Oral

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KEY POINTS

• This medicine is taken by mouth to treat cystic fibrosis caused by a specific gene problem. Take it exactly as directed.
• Keep all appointments for tests to see how this medicine affects you.
• This medicine may cause unwanted side effects. Tell your healthcare provider if you have any side effects that are serious, continue, or get worse.
• Tell all healthcare providers who treat you about all the prescription medicines, nonprescription medicines, supplements, natural remedies, and vitamins that you take.

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What are other names for this medicine?

Type of medicine: cystic fibrosis treatment

Generic and brand names: ivacaftor, oral; Kalydeco

What is this medicine used for?

This medicine is taken by mouth to treat cystic fibrosis caused by a specific gene problem.

This medicine may be used to treat other conditions as determined by your healthcare provider.

What should my healthcare provider know before I take this medicine?

Before taking this medicine, tell your healthcare provider if you have ever had:

• An allergic reaction to any medicine
• Liver or kidney disease

Females of childbearing age: Tell your healthcare provider if you are pregnant or plan to become pregnant. Do not breast-feed while taking this medicine without your healthcare provider's approval.

How do I use it?

Check the label on the medicine for directions about your specific dose. Take this medicine exactly as your healthcare provider prescribes. Do not take more of it or for longer than prescribed. Taking too much increases the risk of serious side effects. Do not stop taking this medicine without your healthcare provider's approval.

Check with your healthcare provider before using this medicine in children under age 2.

Take this medicine with food that contains fat, such as eggs, butter, cheese pizza, or peanut butter. Do not take this medicine with grapefruit juice or Seville oranges. If you have the granules, shake the packet gently before you open it. Mix the contents with 1 teaspoon of cold soft food or liquid such as applesauce, pureed fruits or vegetables, yogurt, water, or milk. Take the mixture within 1 hour.

What if I miss a dose?

If you miss a dose and it is within 6 hours of when you usually take it, take your dose (with food that contains fat) as soon as possible. If you miss a dose and it is more than 6 hours after the time you usually take it, skip that dose and take the next dose when you usually take it. Do not take double doses. If you are not sure of what to do if you miss a dose, or if you miss more than one dose, contact your healthcare provider.

What if I overdose?

Symptoms of an acute overdose have not been reported.

What should I watch out for?

You will need to have blood tests regularly to see how this medicine affects you. Keep all appointments for these tests.

This medicine may increase the risk of cataracts. You should have regular eye exams while taking this medicine.

This medicine may make you dizzy. Do not drive or operate machinery unless you are fully alert.

If you need emergency care, surgery, lab tests, or dental work, tell the healthcare provider or dentist you are taking this medicine.

What are the possible side effects?

Along with its needed effects, your medicine may cause some unwanted side effects. Some side effects may be very serious. Some side effects may go away as your body adjusts to the medicine. Tell your healthcare provider if you have any side effects that continue or get worse.

Life-threatening (Report these to your healthcare provider right away. If you cannot reach your healthcare provider right away, get emergency medical care or call 911 for help.): Allergic reaction (hives; itching; rash; trouble breathing; tightness in your chest; swelling of your lips, tongue, and throat).

Serious (Report these to your healthcare provider right away.): Shortness of breath; severe diarrhea, nausea, or vomiting; yellowing of the skin or eyes; dark urine; unusual loss of appetite; light-colored bowel movements; chest pain.

Other: Rash, stomach pain, diarrhea, headache, dizziness, stuffy or runny nose, sore throat, joint or muscle pain, acne.

What products might interact with this medicine?

When you take this medicine with other medicines, it can change the way this or any of the other medicines work. Nonprescription medicines, vitamins, natural remedies, and certain foods may also interact. Using these products together might cause harmful side effects. Talk to your healthcare provider if you are taking:

• Antianxiety medicines such as alprazolam (Xanax), clorazepate (Gen-Xene, Tranxene), diazepam (Valium), lorazepam (Ativan), midazolam (Versed), and oxazepam
• Antibiotics such as clarithromycin (Biaxin), rifampin (Rifadin), and telithromycin (Ketek)
• Antifungal medicines such as clotrimazole, fluconazole (Diflucan), itraconazole (Sporanox), ketoconazole (Nizoral), luliconazole (Luzu), miconazole (Monistat, Oravig), posaconazole (Noxafil), and voriconazole (Vfend)
• Antipsychotic medicines such as aripiprazole (Abilify), haloperidol (Haldol), paliperidone (Invega), and pimozide (Orap)
• Antiseizure medicines such as carbamazepine (Carbatrol, Epitol, Equetro, Tegretol), perampanel (Fycompa), phenobarbital, phenytoin (Dilantin, Phenytek), and primidone (Mysoline)
• Antiviral medicines such as ledipasvir/sofosbuvir (Harvoni) and simeprevir (Olysio)
• Aprepitant (Emend)
• Bosentan (Tracleer)
• Cancer medicines such as abiraterone (Zytiga), afatinib (Gilotrif), bicalutamide (Casodex), bosutinib (Bosulif), crizotinib (Xalkori), dabrafenib (Tafinlar), dasatinib (Sprycel), doxorubicin (Doxil), enzalutamide (Xtandi), everolimus (Afinitor, Zortress), idelalisib (Zydelig), imatinib (Gleevec), osimertinib (Tagrisso), palbociclib (Ibrance), pazopanib (Votrient), siltuximab (Sylvant), sunitinib (Sutent), topotecan (Hycamtin), vemurafenib (Zelboraf), and vincristine (Marqibo)
• Cimetidine (Tagamet)
• Colchicine (Colcrys)
• Conivaptan (Vaprisol)
• Dexamethasone
• Flibanserin (Addyi)
• Heart medicines such as amiodarone (Cordarone, Pacerone), digoxin (Lanoxin), diltiazem (Cardizem, Cartia, Tiazac), disopyramide (Norpace), dronedarone (Multaq), nicardipine (Cardene), quinidine, and verapamil (Calan, Covera, Verelan)
• HIV medicines such as atazanavir (Reyataz), delavirdine (Rescriptor), efavirenz (Sustiva), indinavir (Crixivan), lopinavir/ritonavir (Kaletra), nelfinavir (Viracept), nevirapine (Viramune), ritonavir (Norvir), and saquinavir (Invirase)
• Immunosuppressants such as cyclosporine (Gengraf, Neoral, Sandimmune) and tacrolimus (Astagraf, Prograf, Protopic)
• Lomitapide (Juxtapid)
• Medicines to treat or prevent blood clots such as dabigatran (Pradaxa), rivaroxaban (Xarelto), and warfarin (Coumadin)
• Naloxegol (Movantik)
• Silodosin (Rapaflo)
• St. John's wort

Ask your healthcare provider or pharmacist if you need to avoid products that contain grapefruit, Seville oranges, and tangelos while you are taking this medicine. These fruits and juices can affect the way this medicine works and may increase your risk of serious side effects.

If you are not sure if your medicines might interact, ask your pharmacist or healthcare provider. Keep a list of all your medicines with you. List all the prescription medicines, nonprescription medicines, supplements, natural remedies, and vitamins that you take. Be sure that you tell all healthcare providers who treat you about all the products you are taking.

How should I store this medicine?

Store this medicine at room temperature. Keep the container tightly closed. Protect it from heat, high humidity, and bright light.

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This advisory includes selected information only and may not include all side effects of this medicine or interactions with other medicines. Ask your healthcare provider or pharmacist for more information or if you have any questions.

Ask your pharmacist for the best way to dispose of outdated medicine or medicine you have not used. Do not throw medicine in the trash.

Keep all medicines out of the reach of children.

Do not share medicines with other people.