Lumacaftor and Ivacaftor

Lumacaftor and ivacaftor is used to treat certain types of cystic fibrosis (CF; an inherited condition that affects the lungs and other parts of the body). Lumacaftor is in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) correctors. Ivacaftor is in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) potentiators. Both of these medications work by improving the function of a protein in the body to decrease the build-up of thick mucus in the lungs and improve other symptoms of cystic fibrosis.

The combination of lumacaftor and ivacaftor comes as a tablet and as granules to take by mouth. Take with fatty foods twice a day, 12 hours apart. Take lumacaftor and ivacaftor at around the same times every day.

Take lumacaftor and 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 lumacaftor and ivacaftor granules, mix the entire packet of granules in 1 teaspoon (5 mL) of a soft food or liquid (cold or at room temperature) such as yogurt, applesauce, pudding, milk, or juice. Take the mixture within 1 hour of mixing the granules with food or a liquid.

Take lumacaftor and ivacaftor with fatty foods such as eggs, avocados, nuts, butter, peanut butter, cheese pizza, whole milk and other whole milk products such as cheese and full fat yogurt. Talk to your doctor about other fatty foods to eat with lumacaftor and ivacaftor.

Lumacaftor and ivacaftor controls cystic fibrosis but does not cure it. Continue to take lumacaftor and ivacaftor even if you feel well. Do not stop taking lumacaftor and ivacaftor without talking to your doctor.

Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

Before taking lumacaftor and ivacaftor,

• tell your doctor or pharmacist if you are allergic to this medication, any part of this medication, or any other medications, foods or substances. Tell your doctor or pharmacist about the allergy and what symptoms you had.
• tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take while taking lumacaftor and ivacaftor. Your doctor may need to change the doses of your medications or monitor you carefully for side effects.
• you should know that the following nonprescription or herbal products may interact with lumacaftor and ivacaftor tablets: lansoprazole, omeprazole, ranitidine, and St. John's Wort. Be sure to let your doctor and pharmacist know that you are taking these medications before you start taking lumacaftor and ivacaftor tablets. Do not start any of these medications while taking lumacaftor and ivacaftor without discussing with your healthcare provider.
• you should know that lumacaftor and ivacaftor may decrease the effectiveness of hormonal contraceptives (birth control pills, patches, rings, injections, implants, or intrauterine devices). Talk to your doctor about other methods of birth control that will work for you while you are taking lumacaftor and ivacaftor.
• tell your doctor if you have or have ever had breathing problems or conditions, an organ transplant, or liver or kidney disease.
• tell your doctor if you are pregnant, plan to become pregnant, or are breast-feeding. If you become pregnant while taking lumacaftor and ivacaftor, call your doctor.
• you should know that depression, anxiety, thinking about or committing self harm or suicide, and sleep difficulties have been reported with lumacaftor and ivacaftor. Before beginning lumacaftor and ivacaftor, tell your doctor if you have or have ever had depression, anxiety, thoughts of harming yourself or attempting suicide in the past, or sleep difficulties. During treatment, call your doctor right away if you start experiencing any of the following: changes in mood, mood swings, difficulties regulating emotion, trouble sleeping, anxious thoughts, or thoughts of harming yourself or others.

Do not eat grapefruit or drink grapefruit juice during your first week of treatment with lumacaftor and ivacaftor.

Unless your doctor tells you otherwise, continue your normal diet.

Take the missed dose as soon as you remember if it is within 6 hours of the missed dose. However, if it is more than 6 hours, skip the missed dose and continue your regular dosing schedule. Do not take a double dose to make up for a missed one. If you miss taking lumacaftor and ivacaftor for more than 7 days, call your doctor. Your doctor may need to change the dose of this medication of other medications you are taking.

Lumacaftor and ivacaftor may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

• diarrhea
• gas
• excessive tiredness
• irregular, missed, heavy or painful menstrual periods
• runny nose, sneezing, cough, sore throat, or flu-like symptoms
• headache

Some side effects can be serious. If you experience any of these symptoms, call your doctor immediately:

• pain in the upper right part of the stomach, dark urine, loss of appetite, nausea, vomiting, confusion, or yellowing of the skin or eyes
• rash; hives; itching; swelling of the face, eyes, lips, tongue, mouth, or throat; wheezing; difficulty breathing or swallowing; or lightheadedness or dizziness
• unusual headache, blurred vision, double vision, or vision loss
• chest tightness, trouble breathing, or shortness of breath

Lumacaftor and ivacaftor may cause cataracts (clouding of the lens of the eye that may cause vision problems) in children and teenagers. Children and teenagers taking lumacaftor and ivacaftor should see an eye doctor before and during their treatment. Talk to your child's doctor about the risks of giving lumacaftor and ivacaftor to your child.

Lumacaftor and ivacaftor may cause other side effects. Call your doctor if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (https://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

Keep this medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom).

Dispose of unneeded medications in a way so that pets, children, and other people cannot take them. Do not flush this medication down the toilet. Use a medicine take-back program. Talk to your pharmacist about take-back programs in your community. Visit the FDA's Safe Disposal of Medicines website https://goo.gl/c4Rm4p for more information.

Keep all medication out of sight and reach of children as many containers are not child-resistant. Always lock safety caps. Place the medication in a safe location – one that is up and away and out of their sight and reach. https://www.upandaway.org

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at https://www.poisonhelp.org/help. If the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services at 911.

Symptoms of overdose may include:

• headache
• rash

Keep all appointments with your doctor and the laboratory. Your doctor may order a lab test before you begin your treatment to see if this medication is right for you. Your doctor will also order certain lab tests before and during your treatment to check your body's response to lumacaftor and ivacaftor. Your blood pressure should be checked regularly while you are taking this medication.

Do not let anyone else take your medication. Ask your pharmacist any questions you have about refilling your prescription.

Keep a written list of all of the prescription and nonprescription (over-the-counter) medicines, vitamins, minerals, and dietary supplements you are taking. Bring this list with you each time you visit a doctor or if you are admitted to the hospital. You should carry the list with you in case of emergencies.

• Orkambi^®