Lumacaftor; Ivacaftor Tablets

What is this medication?

LUMACAFTOR; IVACAFTOR (LOO ma KAF tor; EYE va KAF tor) treats cystic fibrosis (CF). It works by improving the function of a protein in your body, which helps balance the level of salt and water in the lungs. This prevents mucus from building up in your lungs, which may improve symptoms of CF. It is not a cure for CF.

This medicine may be used for other purposes; ask your health care provider or pharmacist if you have questions.

COMMON BRAND NAME(S): ORKAMBI

What should I tell my care team before I take this medication?

They need to know if you have any of these conditions:

• High blood pressure
• Kidney disease
• Liver disease
• An unusual reaction to lumacaftor, ivacaftor, other medications, foods, dyes, or preservatives
• Pregnant or trying to get pregnant
• Breastfeeding

How should I use this medication?

Take this medication by mouth with fat-containing foods such as eggs, avocados, nuts, butter, peanut butter, or cheese pizza. Breast milk, infant formula, whole-milk dairy products (such as whole milk, cheese, and yogurt) may also be used. Take it as directed on the prescription label. Keep taking it unless your care team tells you to stop.

Do not take this medication with grapefruit juice.

Talk to your care team about the use of this medication in children. While it may be prescribed for children as young as 6 years of age for selected conditions, precautions do apply.

Overdosage: If you think you have taken too much of this medicine contact a poison control center or emergency room at once.

NOTE: This medicine is only for you. Do not share this medicine with others.

What if I miss a dose?

If you miss a dose, take it as soon as you can. If your next dose is to be taken in less than 6 hours, then do not take the missed dose. Take the next dose at your regular time. Do not take double or extra doses.

What may interact with this medication?

Do not take this medication with any of the following:

• Artemether, lumefantrine
• Boceprevir
• Cobicistat, elvitegravir, emtricitabine, tenofovir
• Cobicistat, elvitegravir, emtricitabine, tenofovir alafenamide
• Dasabuvir, ombitasvir, paritaprevir, ritonavir
• Emtricitabine, rilpivirine, tenofovir
• Lurasidone
• Nifedipine
• Ombitasvir, paritaprevir, ritonavir
• Praziquantel
• Ranolazine
• Rilpivirine

This medication may also interact with the following:

• Certain antibiotics, such as clarithromycin, erythromycin, telithromycin
• Certain medications for depression, anxiety, or sleep
• Certain medications for diabetes, such as glipizide or repaglinide
• Certain medications for fungal infections, such as fluconazole, itraconazole, ketoconazole, posaconazole, voriconazole
• Certain medications for immunosuppression, such as cyclosporine, everolimus, sirolimus, or tacrolimus
• Certain medications for seizures, such as carbamazepine, phenobarbital, phenytoin
• Certain medications for stomach problems, such as cimetidine, famotidine, omeprazole, lansoprazole
• Digoxin
• Estrogen or progestin hormones
• Grapefruit juice
• Ibuprofen
• Montelukast
• Rifabutin
• Rifampin
• St. John's Wort
• Steroid medications, such as prednisone or methylprednisolone
• Warfarin

This list may not describe all possible interactions. Give your health care provider a list of all the medicines, herbs, non-prescription drugs, or dietary supplements you use. Also tell them if you smoke, drink alcohol, or use illegal drugs. Some items may interact with your medicine.

What should I watch for while using this medication?

Visit your care team for regular checks on your progress. Tell your care team if your symptoms do not start to get better or if they get worse.

This medication may cause cataracts, especially in children. You should have regular eye exams while taking this medication. Tell your care team if you have changes in your eyesight.

Estrogen and/or progestin hormones may not work as well while you are taking this medication. If you are using these hormones for contraception, talk to your care team about using a second type of contraception. A barrier contraceptive, such as a condom or diaphragm, is recommended.

What side effects may I notice from receiving this medication?

Side effects that you should report to your care team as soon as possible:

• Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat
• Change in vision
• Increase in blood pressure
• Liver injury—right upper belly pain, loss of appetite, nausea, light-colored stool, dark yellow or brown urine, yellowing skin or eyes, unusual weakness or fatigue
• Shortness of breath or chest pain

Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):

• Diarrhea
• Gas
• Fatigue
• Headache
• Irregular menstrual cycles or spotting
• Nausea
• Stomach pain

This list may not describe all possible side effects. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Where should I keep my medication?

Keep out of the reach of children and pets.

Store at room temperature between 20 and 25 degrees C (68 and 77 degrees F). Get rid of any unused medication after the expiration date.

To get rid of medications that are no longer needed or have expired:

• Take the medication to a medication take-back program. Check with your pharmacy or law enforcement to find a location.
• If you cannot return the medication, check the label or package insert to see if the medication should be thrown out in the garbage or flushed down the toilet. If you are not sure, ask your care team. If it is safe to put it in the trash, empty the medication out of the container. Mix the medication with cat litter, dirt, coffee grounds, or other unwanted substance. Seal the mixture in a bag or container. Put it in the trash.

NOTE: This sheet is a summary. It may not cover all possible information. If you have questions about this medicine, talk to your doctor, pharmacist, or health care provider.