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ivacaftor and lumacaftor

Pronunciation: EYE va KAF tor and LOO ma KAF tor

Brand: Orkambi

What is the most important information I should know about ivacaftor and lumacaftor?

Tell your doctor about all your current medicines and any you start or stop using. Many drugs can interact, and some drugs should not be used together.

What is ivacaftor and lumacaftor?

Ivacaftor and lumacaftor is used to treat cystic fibrosis in adults and children who are at least 2 years old.

Ivacaftor and lumacaftor is for use only in patients with a specific gene mutation related to cystic fibrosis. Before you take this medicine, you may need a medical test to make sure you have this gene mutation.

Ivacaftor and lumacaftor may also be used for purposes not listed in this medication guide.

What should I discuss with my healthcare provider before taking ivacaftor and lumacaftor?

You should not use this medicine if you are allergic to ivacaftor or lumacaftor.

Some medicines can cause unwanted or dangerous effects when used with ivacaftor and lumacaftor. Your doctor may change your treatment plan if you also use:

  • rifabutin or rifampin;
  • St. John's wort;
  • medicine to prevent organ transplant rejection --cyclosporine, everolimus (Zortress), sirolimus, tacrolimus;
  • a sedative --triazolam or midazolam; or
  • seizure medicine --carbamazepine, phenobarbital, phenytoin.

Tell your doctor if you have ever had:

  • liver disease;
  • kidney disease;
  • high blood pressure;
  • asthma or other lung disease; or
  • an organ transplant.

It is not known whether this medicine will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

Ivacaftor and lumacaftor can make hormonal birth control less effective (birth control pills, injections, implants, skin patches, vaginal rings). Use a condom or diaphragm with spermicide to prevent pregnancy.

It may not be safe to breast-feed while using this medicine. Ask your doctor about any risk.

Ivacaftor and lumacaftor is not approved for use by anyone younger than 2 years old.

How should I take ivacaftor and lumacaftor?

Follow all directions on your prescription label and read all medication guides or instruction sheets. Use the medicine exactly as directed.

Take this medicine with food that contains fat, such as butter, peanut butter, eggs, avocados, nuts, whole milk, cheese, yogurt, or cheese pizza. Follow your doctor's dosing instructions very carefully.

To use the oral granules: Mix the granules with 1 teaspoon of milk, juice, or soft food such as applesauce, yogurt, or pudding. Mix only 1 dose at a time, and use the mixture within 1 hour after mixing. Feed the child a high-fat food just before or just after giving the granule mixture.

Take your doses at regular intervals, 12 hours apart. This will keep a steady amount of the drug in your body at all times.

Ivacaftor and lumacaftor doses are based on age and weight in children. Children from 2 to 5 years old should take oral granules. Children 6 and older should take tablets.

You will need frequent blood tests to check your liver function. Your blood pressure may also need to be checked often.

A child using this medicine may need frequent eye exams.

Store at room temperature away from moisture and heat. Keep each tablet in its daily blister strip until you are ready to take your dose.

What happens if I miss a dose?

Take the medicine as soon as you can, but skip the missed dose if you are more than 6 hours late for the dose. Do not take two doses at one time.

Be sure to take a missed dose with a food that contains fat.

If you miss doses or stop taking ivacaftor and lumacaftor for more than 1 week, call your doctor before you start taking the medicine again. You may need a different dose.

What happens if I overdose?

Seek emergency medical attention or call the Poison Help line at 1-800-222-1222.

What should I avoid while taking ivacaftor and lumacaftor?

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

What are the possible side effects of ivacaftor and lumacaftor?

Get emergency medical help if you have signs of an allergic reaction: hives; difficult breathing; swelling of your face, lips, tongue, or throat.

Call your doctor at once if you have:

  • shortness of breath, tightness in your chest; or
  • liver problems --nausea, upper stomach pain, itching, tired feeling, loss of appetite, dark urine, clay-colored stools, jaundice (yellowing of the skin or eyes).

Common side effects may include:

  • breathing problems;
  • feeling tired;
  • nausea, gas, diarrhea;
  • rash;
  • flu symptoms;
  • changes in your menstrual periods; or
  • cold symptoms such as runny or stuffy nose, sinus pain, sore throat.

This is not a complete list of side effects and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

What other drugs will affect ivacaftor and lumacaftor?

Tell your doctor about all your other medicines, especially:

  • an antibiotic --clarithromycin, erythromycin, telithromycin; or
  • antifungal medicine --itraconazole, ketoconazole, posaconazole, voriconazole.

This list is not complete. Other drugs may affect ivacaftor and lumacaftor, including prescription and over-the-counter medicines, vitamins, and herbal products. Not all possible drug interactions are listed here.

Where can I get more information?

Your pharmacist can provide more information about ivacaftor and lumacaftor.

Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use this medication only for the indication prescribed.

Every effort has been made to ensure that the information provided by Cerner Multum, Inc. ('Multum') is accurate, up-to-date, and complete, but no guarantee is made to that effect. Drug information contained herein may be time sensitive. Multum information has been compiled for use by healthcare practitioners and consumers in the United States and therefore Multum does not warrant that uses outside of the United States are appropriate, unless specifically indicated otherwise. Multum's drug information does not endorse drugs, diagnose patients or recommend therapy. Multum's drug information is an informational resource designed to assist licensed healthcare practitioners in caring for their patients and/or to serve consumers viewing this service as a supplement to, and not a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners. The absence of a warning for a given drug or drug combination in no way should be construed to indicate that the drug or drug combination is safe, effective or appropriate for any given patient. Multum does not assume any responsibility for any aspect of healthcare administered with the aid of information Multum provides. The information contained herein is not intended to cover all possible uses, directions, precautions, warnings, drug interactions, allergic reactions, or adverse effects. If you have questions about the drugs you are taking, check with your doctor, nurse or pharmacist.

Copyright 1996-2019 Cerner Multum, Inc. Version: 2.01. Revision date: 9/20/2018.

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