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Orkambi

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

What is Orkambi?

Orkambi contains a combination of ivacaftor and lumacaftor. Orkambi is used to treat cystic fibrosis in adults and children who are at least 6 years old.

Orkambi is for use only in patients with a specific gene mutation related to cystic fibrosis.

Before you take Orkambi, you may need a medical test to make sure you have this gene mutation.

Important information

Orkambi can cause serious side effects. High liver enzymes in the blood, which can be a sign of liver injury, have been reported in patients receiving this medicice. Your doctor will do blood tests to check your liver.

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

Before taking this medicine

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

Some medicines can interact with Orkambi 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.

Orkambi 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.

Orkambi is not approved for use by anyone younger than 6 years old.

How should I take Orkambi?

Orkambi 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 Orkambi 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 Orkambi may need frequent eye exams.

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

Orkambi dosing information

Usual Adult Dose for Cystic Fibrosis:

Two tablets (ivacaftor 125 mg-lumacaftor 200 mg) orally every 12 hours with fat-containing food

Comments:
-If a dose is missed within 6 hours of the usual scheduled time, take the dose immediately with a fat-containing meal; if more than 6 hours have elapsed, skip that dose and resume the normal schedule for the following dose.

Use: For the treatment of cystic fibrosis (CF) in patients who are homozygous for the F508del mutation in the CFTR gene; if the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene

Usual Pediatric Dose for Cystic Fibrosis:

6 to 11 years: Two tablets (ivacaftor 125 mg-lumacaftor 100 mg) orally every 12 hours with fat-containing food

12 years or older: Two tablets (ivacaftor 125 mg-lumacaftor 200 mg) orally every 12 hours with fat-containing food

Comments:
-If a dose is missed within 6 hours of the usual scheduled time, take the dose immediately with a fat-containing meal; if more than 6 hours have elapsed, skip that dose and resume the normal schedule for the following dose.

Use: For the treatment of CF in patients age 6 years and older who are homozygous for the F508del mutation in the CFTR gene; if the patient's genotype is unknown, an FDA-cleared CF mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene.

See also: Dosage Information (in more detail)

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 Orkambi 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 Orkambi?

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

Orkambi side effects

Get emergency medical help if you have signs of an allergic reaction to Orkambi: 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 Orkambi 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)

What other drugs will affect Orkambi?

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 Orkambi.
  • Remember, keep this and all other medicines out of the reach of children, never share your medicines with others, and use Orkambi only for the indication prescribed.
  • Disclaimer: Every effort has been made to ensure that the information provided is accurate, up-to-date, and complete, but no guarantee is made to that effect. 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 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 1996-2016 Cerner Multum, Inc. Version: 1.03. Revision Date: 2016-11-10, 1:19:21 PM.

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