HOW TO TAKE ORKAMBI®

People with CF pictured may 
or may not be taking ORKAMBI.

Take ORKAMBI every 12 hours as directed

Your healthcare provider will tell you exactly how much ORKAMBI you or your child should take and when it should be taken.

A fat-containing meal or snack helps the body better absorb the medicine. ORKAMBI should always be taken before or after a meal or snack that contains fat. Get recipes for fat-containing meals and snacks.

Avoid foods and drinks that contain grapefruit during your child's first week taking ORKAMBI because they can increase the amount of ORKAMBI in their body.

    How to Take ORKAMBI Oral Granules (Children Age 1 Through 5 Years)

    Select a button below for information and tips on your loved one ORKAMBI granules.

    WHAT TO TAKE WHEN

    GIVING YOUR
    LOVED ONE ORKAMBI

    HELPING YOUR LOVED
    ONE TAKE ORKAMBI

    What to take when

    Designed with a young child in mind

    ORKAMBI comes in single-use, oral granule packets for children age 1 through 5 years with cystic fibrosis (CF). The oral granules are similar in size to flour particles and should be mixed with 1 teaspoon (5 mL) of soft food or liquid.

    Dosage strengths for ORKAMBI oral granules

    ORKAMBI oral granules are prescribed based on weight and come in 3 different strengths.

    AGE WEIGHT-BASED DOSE RECOMMENDED DOSE
    1 through 2 years old
    		 15 lbs to less than 20 lbs (7 kg to less than 9 kg)
    lumacaftor 75 mg/ivacaftor 94 mg    		 1 packet every 12 hours
    (2 packets per day)
    20 lbs to less than 31 lbs (9 kg to less than 14 kg)
    lumacaftor 100 mg/ivacaftor 125 mg
    31 lbs or more (14 kg or more)
    lumacaftor 150 mg/ivacaftor 188 mg
    2 through 5 years old
    		 Less than 31 lbs (less than 14 kg)
    lumacaftor 100 mg/ivacaftor 125 mg
    31 lbs or more (14 kg or more)
    lumacaftor 150 mg/ivacaftor 188 mg
    AGE 1 THROUGH 2 YEARS OLD
    WEIGHT-BASED DOSE
    15 lbs to less than 20 lbs
    (7 kg to less than 9 kg)
    lumacaftor 75 mg/ivacaftor 94 mg
    20 lbs to less than 31 lbs
    (9 kg to less than 14 kg)
    lumacaftor 100 mg/ivacaftor 125 mg
    31 lbs or more (14 kg or more)
    lumacaftor 150 mg/ivacaftor 188 mg
    Recommended Dose
    1 packet every 12 hours
    (2 packets per day)

     

    AGE 2 THROUGH 5 YEARS OLD
    WEIGHT-BASED DOSE
    Less than 31 lbs (less than 14 kg) lumacaftor 100 mg/ivacaftor 125 mg
    31 lbs or more (14 kg or more) lumacaftor 150 mg/ivacaftor 188 mg
    Recommended Dose
    1 packet every 12 hours
    (2 packets per day)

    Your child should take ORKAMBI, plus all of your child's other CF therapies, exactly as your child's healthcare provider says they should be taken. 

    How ORKAMBI oral granules are packaged

    Every carton of ORKAMBI has 56 ORKAMBI oral granule packets, organized in 4 weekly wallets. Each wallet has 2 packets for each of the 7 days in a week.

    For children age 1 through 2 years
    weighing 15 lbs to less than 20 lbs
     (7 kg to less than 9 kg)

    ORKAMBI oral granules for children age 1 through 2 years weighing 15 lbs to less than 20 lbs (7 kg to less than 9 kg)

    For children age 1 through 2 years weighing
    20 lbs to less than 31 lbs (9 kg to less than 14 kg)

    and children age 2 through 5 years
    weighing less than 31 lbs (less than 14 kg)

    ORKAMBI oral granules for children age 1 through 2 years weighing 20 lbs to less than 31 lbs (9 kg to less than 14 kg) and children age 2 through 5 years weighing less than 31 lbs (less than 14 kg)

    For children age 1 through 2 years
    weighing 31 lbs or more (14 kg or more)

    and children age 2 through 5 years
    weighing 31 lbs or more (14 kg or more)

    ORKAMBI oral granules for children age 1 through 2 years weighing 31 lbs or more (14 kg or more) and children age 2 through 5 years weighing 31 lbs or more (14 kg or more)

    What if your child misses a dose of ORKAMBI?

    • If your child misses a dose of ORKAMBI and it is within 6 hours of when they usually take it, have your child take the dose of ORKAMBI as prescribed with fat-containing food as soon as possible
    • If your child misses a dose and it is more than 6 hours after the time they usually take it, have your child skip that dose only and take the next dose with fat-containing food when they usually take it. Do not take 2 doses at the same time to make up for a missed dose
    • Tell your child’s healthcare provider if your child stops taking ORKAMBI for more than 1 week. The healthcare provider may need to change your child’s ORKAMBI dose or other medicines your child takes

    How to give your child ORKAMBI oral granules

    STEP 1: Preparation

    STEP 1: Preparation

    • Hold the packet with the cut line on top
    • Shake the packet gently to settle the ORKAMBI granules
    • Tear or cut packet open along cut line
    • Carefully pour all of the ORKAMBI granules in the packet into 1 teaspoon (5 mL) of soft food such as flavored pudding or yogurt, or liquid such as juice, in a small container. Food or liquid should be at or below room temperature
    • Here are examples of foods and liquids you can mix ORKAMBI granules into. Remember, every child is different, so be sure to talk to your healthcare provider about what the best options are for your child
      • Breast milk
      • Applesauce
      • Infant formula
      • Water
      • Puréed fruit or vegetables
      • Milk
      • Yogurt or pudding (flavored if desired)
      • Juice
      • Breast milk
      • Infant formula
      • Puréed fruit or vegetables
      • Yogurt or pudding (flavored if desired)
      • Applesauce
      • Water
      • Milk
      • Juice
    • Then, mix the granules with the 1 teaspoon of food or liquid

    STEP 2: Administration

    STEP 2: Administration

    • Within 1 hour of mixing, give ORKAMBI to your child
    • Make sure the entire medicine mixture is taken

    STEP 3: Fat-containing food before or after dose

    STEP 3: Fat-containing food before or after dose

    Always take ORKAMBI with fat-containing food

    • Make sure to always give your child food that contains fat just before or after the dose of ORKAMBI oral granules. This helps the body absorb ORKAMBI better

    Here are suggestions for fat-containing foods to give your child:

    • Breast milk
    • Eggs
    • Cheese pizza
    • Infant formula
    • Whole-milk cheese
    • Nuts
    • Whole milk
    • Butter
    • Avocados
    • Whole-milk yogurt
    • Peanut butter
    • Breast milk
    • Infant formula
    • Whole milk
    • Whole-milk yogurt
    • Eggs
    • Whole-milk cheese
    • Butter
    • Peanut butter
    • Cheese pizza
    • Avocados
    • Nuts
       
    • Talk to your healthcare provider about fat-containing foods appropriate for your child when taking ORKAMBI granules

    HELPING YOUR CHILD TAKE ORKAMBI

    Taking ORKAMBI oral granules with Cammy and Sam

    Watch this educational video with your child as you learn how ORKAMBI oral granules should be prepared and taken.

    Tips for helping your child take ORKAMBI oral granules

    Your child may find that ORKAMBI oral granules have a bitter taste. Every dose matters, so it's important to make sure your child completely finishes the ORKAMBI mixture at each dose. Here are a few tips that may help.

    Chocolate pudding

    Mix the granules with 1 teaspoon (5 mL) of soft food or liquid that is sweet or rich, like pudding, chocolate sauce, caramel sauce, or melted ice cream.

    Honey

    Give your child something sweet, like maple syrup or honey, after they completely finish the dose. It may help with the aftertaste.

    Ice pop

    Numb the taste buds before taking ORKAMBI by having your child eat an ice pop or another cold treat before each dose.

    Put some control in your child’s hands by letting them choose a favorite fat-containing food to eat before or after taking ORKAMBI.

    Does your child have a favorite doll or toy? Holding a special toy while taking ORKAMBI may make your child feel more comfortable.

    Try creating a rewards system for finishing ORKAMBI, such as collecting stickers. You know best how to keep your child motivated!

    How to Take ORKAMBI Tablets (People Age 6 Years and Older)

    Dosage strengths for ORKAMBI tablets

    AGEEACH TABLETEACH DOSETOTAL DAILY DOSE
    Children age 6 
    through 11 years    		Lumacaftor 100 mg/
    ivacaftor 125 mg     		2 tablets
    every 12 hours    		4 tablets
    per day
    People age 12 years 
    and older    		Lumacaftor 200 mg/
    ivacaftor 125 mg    		2 tablets
    every 12 hours    		4 tablets
    per day
    AGE
    Children age 6 through 11 years
    EACH TABLET
    Lumacaftor 100 mg/
    ivacaftor 125 mg
    EACH DOSE
    2 tablets
    every 12 hours
    TOTAL DAILY DOSE
    4 tablets
    per day

     

    AGE
    People age 12 years
    and older
    EACH TABLET
    Lumacaftor 200 mg/
    ivacaftor 125 mg
    EACH DOSE
    2 tablets
    every 12 hours
    TOTAL DAILY DOSE
    4 tablets
    per day

    Make sure to take ORKAMBI before or after a meal or snack that contains fat.

    Find new and delicious fat-containing food ideas.

    How ORKAMBI tablets are packaged

    Each carton of ORKAMBI has 4 weekly wallets. Inside each weekly wallet are 7 daily blister strips. Every blister strip contains 4 tablets: 2 for the morning, and 12 hours later, 2 for the evening. When taking ORKAMBI, remove 2 tablets from the blister strip (do not push tablet through backing).

    You can cut along the blister strip’s dotted line to separate your or your child’s morning and evening doses, which contain the same amount of medicine.

    ORKAMBI tablet packaging

    What if you or your child misses a dose of ORKAMBI?

    • If you or your child misses a dose of ORKAMBI and it is within 6 hours of when it's usually taken, you or your child should take the dose as prescribed with fat-containing food as soon as possible
    • If you or your child misses a dose and it is more than 6 hours after the time it's usually taken, you or your child should skip that dose only and take the next dose with fat-containing food at the usual time. Do not take 2 doses at the same time to make up for a missed dose
    • Tell your healthcare provider if you or your child stops taking ORKAMBI for more than 1 week. Your healthcare provider may need to change the ORKAMBI dose or other medicines
    FAQs

    If I mix the ORKAMBI oral granules with a fat-containing food, do I still need to give my child fat-containing food afterwards?

    Yes, your child should still eat fat-containing food just before or just after taking the entire mixture.

    Does the temperature of the food that I mix with ORKAMBI oral granules matter? Can the granules be mixed in foods that are hot or cold?

    The granules should be mixed with soft food or liquid at room temperature or below. The granules should not be mixed in items that are frozen or hot.

    Can my child swallow the granules without mixing them in soft food or liquid?

    The entire contents of each packet should be mixed with 1 teaspoon (5 mL) of age-appropriate soft food or liquid. The mixture should be taken within 1 hour of being mixed. Make sure all medicine is taken.

    My child vomited after taking the dose of ORKAMBI oral granules. What should I do?

    You should contact your child’s healthcare provider to discuss this. Your pharmacist may also be a good resource.

    If my child is napping when it is time for ORKAMBI oral granules, should I wait until they’re awake to administer them?

    You should give your child ORKAMBI oral granules every 12 hours or as prescribed by your child’s healthcare provider.

    What can I do if my child doesn't like the taste of ORKAMBI granules?

    It's important to make sure your child completely finishes the ORKAMBI mixture at each dose. If they don’t like the taste, there are a few tips that may help.

    Start by mixing the granules into a food that is sweet or rich, like pudding, chocolate sauce, caramel sauce, or melted ice cream. You can also try giving your child something sweet, like maple syrup or honey, after the dose. Giving your child an ice pop or cold treat before they take ORKAMBI may help by numbing their taste buds.

    For a different approach, put some control in your child’s hands by letting them choose a favorite fat-containing food to eat before or after taking ORKAMBI. Letting your child hold a special toy while taking ORKAMBI may make them feel more comfortable. You could also try creating a rewards system for finishing ORKAMBI, such as collecting stickers or something else that motivates your child.

    How are ORKAMBI oral granules packaged?

    Every carton has 56 ORKAMBI oral granule packets, organized in 4 weekly wallets. Each wallet has 2 packets for each of the 7 days in a week.

    Are the oral granules packets child proof?

    The packets are child-resistant.

    How should ORKAMBI oral granules and tablets be stored?

    ORKAMBI should be stored at 68°F to 77°F (20°C to 25°C). Do not use ORKAMBI after the expiration date on the package. Keep ORKAMBI and all medicines out of the reach of children.

    How are ORKAMBI tablets packaged?

    Each carton of ORKAMBI has 4 weekly wallets. Inside each weekly wallet are 7 daily blister strips. Every blister strip contains 4 tablets: 2 for the morning, and 12 hours later, 2 for the evening.

    Can I separate the doses on the blister cards?

    You may cut along the dotted line to separate your doses from the blister card.

    My child is over 6 years old and has trouble swallowing pills. Can my child take oral granules?

    ORKAMBI oral granules are for patients age 2 through 5 years old. ORKAMBI tablets are for people age 6 years and older.

    What should my child avoid while taking ORKAMBI?

    Avoid foods and drinks that contain grapefruit during your child's first week taking ORKAMBI because they can increase the amount of ORKAMBI in their body.

    IMPORTANT SAFETY INFORMATION

    Before taking ORKAMBI, tell your doctor about all of your medical conditions, including if you:

    • have or have had liver problems
    • are allergic to ORKAMBI or any ingredients in ORKAMBI. See the Patient Information for a list of ingredients
    • have kidney problems
    • have lung problems
    • have had an organ transplant
    • are using birth control (hormonal contraceptives, including oral, injectable, transdermal, or implantable forms). Hormonal contraceptives should not be used as a method of birth control when taking ORKAMBI. Talk to your doctor about the best birth control method you should use while taking ORKAMBI
    • are pregnant or plan to become pregnant. It is not known if ORKAMBI will harm your unborn baby. You and your doctor should decide if you will take ORKAMBI while you are pregnant
    • are breastfeeding or planning to breastfeed. It is not known if ORKAMBI passes into your breast milk. You and your doctor should decide if you will take ORKAMBI while you are breastfeeding

    What is ORKAMBI® (lumacaftor/ivacaftor)?

    What is ORKAMBI®
    (lumacaftor/ivacaftor)?

    ORKAMBI is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients aged 1 year and older who have two copies of the F508del mutation (F508del/F508del) in their CFTR gene.

    ORKAMBI should not be used in patients other than those who have two copies of the F508del mutation in their CFTR gene.

    It is not known if ORKAMBI is safe and effective in children under 1 year of age.

    Tell your doctor about all the medicines you take, including prescription and over-the-counter medicines, vitamins, and herbal supplements.

    ORKAMBI may affect the way other medicines work, and other medicines may affect how ORKAMBI works. The dose of ORKAMBI may need to be adjusted when taken with certain medicines. Ask your doctor or pharmacist for a list of these medicines if you are not sure.

    Especially tell your doctor if you take:

    • antibiotics: rifampin (RIFAMATE®, RIFATER®) or rifabutin (MYCOBUTIN®)
    • seizure medicines: phenobarbital, carbamazepine (TEGRETOL®, CARBATROL®, and EQUETRO®), or phenytoin (DILANTIN®, PHENYTEK®)
    • sedatives and anti-anxiety medicines: triazolam (HALCION®) or midazolam (DORMICUM®, HYPNOVEL®, and VERSED®)
    • immunosuppressant medicines: cyclosporine, everolimus (ZORTRESS®), sirolimus (RAPAMUNE®), or tacrolimus (ASTAGRAF XL®, ENVARSUS XR®, PROGRAF®, and PROTOPIC®)
    • St. John’s wort
    • antifungal medicines including ketoconazole, itraconazole (such as SPORANOX®), posaconazole (such as NOXAFIL®), or voriconazole (such as VFEND®)
    • antibiotics including telithromycin, clarithromycin (such as BIAXIN®), or erythromycin (such as ERY-TAB®)

    What should I avoid while taking ORKAMBI?

    • Do not eat or drink grapefruit products during your first week of treatment with ORKAMBI. Eating or drinking grapefruit products can increase the amount of ORKAMBI in your blood

    What are the possible side effects of ORKAMBI?

    ORKAMBI can cause serious side effects, including:

    • Worsening of liver function in people with severe liver disease. The worsening of liver function can be serious or cause death. Talk to your doctor if you have been told you have liver disease as your doctor may need to adjust the dose of ORKAMBI
    • High liver enzymes in the blood, which can be a sign of liver injury in people receiving ORKAMBI. Your doctor will do blood tests to check your liver:
    • before you start ORKAMBI
    • every 3 months during your first year of taking ORKAMBI
    • every year while you are taking ORKAMBI

    Call your doctor right away if you have any of the following symptoms of liver problems:

    • pain or discomfort in the upper right stomach (abdominal) area
    • yellowing of your skin or the white part of your eyes
    • loss of appetite
    • nausea or vomiting
    • dark, amber-colored urine
    • confusion
    • Serious allergic reactions have happened to people who are treated with ORKAMBI. Call your doctor or go to the emergency room right away if you have any symptoms of an allergic reaction. Symptoms of an allergic reaction may include:
    • rash or hives
    • tightness of the chest or throat or difficulty breathing
    • swelling of the face, lips, and/or tongue, or difficulty swallowing
    • light-headedness or dizziness
    • Breathing problems such as trouble breathing, shortness of breath or chest tightness in patients when starting ORKAMBI, especially in patients who have poor lung function. Call your doctor right away if you experience these symptoms
    • An increase in blood pressure in some people receiving ORKAMBI. Call your doctor right away if you have an increase in blood pressure
    • Abnormality of the eye lens (cataract) in some children and adolescents receiving ORKAMBI. If you are a child or adolescent, your doctor should perform eye examinations before and during treatment with ORKAMBI to look for cataracts

    The most common side effects of ORKAMBI include:

    • breathing problems such as shortness of breath and chest tightness
    • nausea
    • diarrhea
    • fatigue
    • increase in a certain blood enzyme called creatine phosphokinase
    • rash
    • gas
    • common cold, including sore throat, stuffy or runny nose
    • flu or flu-like symptoms
    • irregular, missed, or abnormal periods (menses) and increase in the amount of menstrual bleeding

    Side effects seen in children are similar to those seen in adults and adolescents. Additional common side effects seen in children include:

    • cough with sputum
    • stuffy nose
    • headache
    • stomach pain
    • increase in sputum

    Tell your doctor if you have any side effect that bothers you or that does not go away.

    These are not all the possible side effects of ORKAMBI. Call your doctor for medical advice about side effects.

    You may report side effects to FDA at 1-800-FDA-1088.

    For further information, please see full Prescribing Information, including Patient Information.