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ivacaftor and lumacaftor

Generic Name: ivacaftor and lumacaftor (EYE va KAF tor and LOO ma KAF tor)
Brand Name: Orkambi

What is ivacaftor and lumacaftor?

Ivacaftor and lumacaftor is used to treat cystic fibrosis in adults and children who are at least 12 years old.

Ivacaftor and lumacaftor is for use only in patients with a specific gene mutation related to cystic fibrosis. Before you take ivacaftor, 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 is the most important information I should know about ivacaftor and lumacaftor?

Serious drug interactions can occur when certain medicines are used together with ivacaftor and lumacaftor. Tell each of your healthcare providers about all medicines you use now, and any medicine you start or stop using.

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 interact with ivacaftor and lumacaftor and should not be used at the same time. Your doctor may need to change your treatment plan if you use any of the following drugs:

  • rifabutin or rifampin;

  • St. John's wort;

  • medicine to prevent organ transplant rejection--everolimus (Zortress), sirolimus, tacrolimus;

  • a sedative--triazolam or oral midazolam; or

  • seizure medicine--carbamazepine, phenobarbital, phenytoin.

To make sure ivacaftor and lumacaftor is safe for you, tell your doctor if you have:

  • liver disease;

  • kidney disease;

  • high blood pressure;

  • asthma or other lung disease; or

  • if you have had an organ transplant.

This medicine is not expected to harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

Use a barrier form of birth control (condom or diaphragm with spermicide). Hormonal contraception (birth control pills, injections, implants, and skin patches, and vaginal rings) may not be effective enough to prevent pregnancy during your treatment.

It is not known whether ivacaftor and lumacaftor passes into breast milk or if it could harm a nursing baby. Tell your doctor if you are breast-feeding a baby.

Ivacaftor and lumacaftor is not approved for use by anyone younger than 12 years old.

How should I take ivacaftor and lumacaftor?

Ivacaftor and lumacaftor is usually taken every 12 hours. Follow all directions on your prescription label. Do not take this medicine in larger or smaller amounts or for longer than recommended.

Read all patient information, medication guides, and instruction sheets provided to you. Ask your doctor or pharmacist if you have any questions.

Take this medicine with a 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.

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

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 missed dose as soon as you remember. If you are more than 6 hours late, skip the missed dose. Do not take extra medicine to make up the missed dose.

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?

This medicine may impair your thinking or reactions. Be careful if you drive or do anything that requires you to be alert.

Ivacaftor and lumacaftor side effects

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.

See also: Side effects (in more detail)

Ivacaftor and lumacaftor dosing information

Usual Adult Dose for Cystic Fibrosis:

12 years of age and older: 2 tablets (ivacaftor 125 mg/ lumacaftor 200 mg) orally every 12 hours with fat containing food

Comments:
-This combination drug should be taken with fat containing food
-If a patient misses a dose and remembers the missed dose within 6 hours, the patient should take the dose with fat containing food. If more than 6 hours elapsed after the usual dosing time, the patient should skip that dose and resume the normal schedule for the following dose. A double dose should not be taken to make up for the forgotten dose.

Use: For the treatment of cystic fibrosis (CF) in patients age 12 years and older who are homozygous for the F508del mutation in the CFTR gene

Usual Pediatric Dose for Cystic Fibrosis:

12 years of age and greater: 2 tablets (ivacaftor 125 mg/ lumacaftor 200 mg) orally every 12 hours with fat containing food

Comments:
-This combination drug should be taken with fat containing food
-If a patient misses a dose and remembers the missed dose within 6 hours, the patient should take the dose with fat containing food. If more than 6 hours elapsed after the usual dosing time, the patient should skip that dose and resume the normal schedule for the following dose. A double dose should not be taken to make up for the forgotten dose.

Use: For the treatment of cystic fibrosis (CF) in patients age 12 years and older who are homozygous for the F508del mutation in the CFTR gene

What other drugs will affect ivacaftor and lumacaftor?

Tell your doctor about all your current medicines and any you start or stop using, especially:

  • an antibiotic--clarithromycin, erythromycin, telithromycin; or

  • antifungal medicine--itraconazole, ketoconazole, posaconazole, voriconazole.

Other drugs may interact with ivacaftor and lumacaftor, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell each of your health care providers about all medicines you use now and any medicine you start or stop using.

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.
  • Disclaimer: 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 1996-2012 Cerner Multum, Inc. Version: 1.02. Revision Date: 2016-07-05, 11:51:15 AM.

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