Ivacaftor and lumacaftor

Ivacaftor and lumacaftor is a combination medicine used to treat cystic fibrosis in adults and children who are at least 1 year old.

Ivacaftor and lumacaftor tablet can be used in children aged 6 years and older. Ivacaftor and lumacaftor granules can be used in children at least 1 year old.

Your doctor will confirm that you have the specific gene mutation related to cystic fibrosis to be treated with ivacaftor and lumacaftor.

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

Follow all directions on your prescription label and read all medication guides or instruction sheets. Your doctor may occasionally change your dose. Use the medicine exactly as directed.

Take this medicine with food that contains fat, such as eggs, whole milk, cheese, yogurt, breast milk, or infant formula. Follow your doctor's instructions carefully.

Swallow the tablet whole and do not crush, chew, or break it.

Mix the ivacaftor and lumacaftor granules with 1 teaspoon of soft food or liquid such as pureed fruits or vegetables, yogurt, applesauce, water, breast milk, infant formula, milk, or juice. The food or liquid should be at or below room temperature. Mix only 1 dose at a time and use it within 1 hour after mixing. Feed your child a high-fat food after or before giving the granule mixture.

Take this medicine at evenly spaced intervals every 12 hours.

Doses are based on weight in children. Your child's dose may change if the child gains or loses weight.

You will need frequent blood tests to check your liver function, and your blood pressure may need to be checked often. You may also need other medical tests.

Your child may need frequent vision exams.

Store at room temperature away from moisture and heat. Keep the medicine in the original packaging.

Use only as directed. Inform your doctor if you take other medicines, have other medical conditions, or have allergies.

You should not be treated with ivacaftor and lumacaftor if you are allergic to it, or if you have or have ever had:

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

It is not known if ivacaftor and lumacaftor will harm an unborn baby. Tell your doctor if you are pregnant or plan to become pregnant.

Ask a doctor if it is safe to breastfeed while using ivacaftor and lumacaftor.

Ivacaftor and lumacaftor can make hormonal birth control less effective, including birth control pills, injections, implants, skin patches, and vaginal rings. Use barrier birth control to prevent pregnancy: condom, diaphragm, cervical cap, or contraceptive sponge.

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

Ivacaftor and lumacaftor may cause serious side effects. Call your doctor right away if you have:

• severe headache, blurred vision, pounding in your neck or ears;
• eye pain or redness, seeing halos around lights;
• chest tightness, trouble breathing; or
• liver problems—loss of appetite, nausea, vomiting, confusion, stomach pain (upper right side), tiredness, itching, dark urine, clay-colored stools, jaundice (yellowing of the skin or eyes).

Common side effects of ivacaftor and lumacaftor may include:

• breathing problems;
• feeling tired;
• nausea, gas, diarrhea;
• rash;
• changes in your menstrual periods, heavy menstrual bleeding;
• flu or cold symptoms such as stuffy or runny nose, sneezing, sore throat; or
• abnormal blood tests.

Some side effects are more common in children taking ivacaftor and lumacaftor, such as increase in mucus, cough with mucus, headache, or stomach pain.

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.

Usual Adult Dose for Cystic Fibrosis:

Lumacaftor 400 mg-ivacaftor 250 mg orally every 12 hours with fat-containing food Comments:-Administer each dose with a fat-containing meal or snack consumed just before or after dosing. -If the patient's genotype is unknown, an FDA-cleared cystic fibrosis (CF) mutation test should be used to detect the presence of the F508del mutation on both alleles of the CFTR gene. -The safety and efficacy of this drug in patients with cystic fibrosis other than homozygous F508del mutation has not been established. Use: For the treatment of CF in patients who are homozygous for the F508del mutation in the CFTR gene

Usual Pediatric Dose for Cystic Fibrosis:

AGE 1 TO 2 YEARS:-Weight 7 kg to less than 9 kg: Lumacaftor 75 mg-ivacaftor 94 mg orally every 12 hours-Weight 9 kg to less than 14 kg: Lumacaftor 100 mg-ivacaftor 125 mg orally every 12 hours-Weight 14 kg or greater: Lumacaftor 150 mg-ivacaftor 188 mg orally every 12 hoursAGE 2 THROUGH 5 YEARS:-Weight less than 14 kg: Lumacaftor 100 mg-ivacaftor 125 mg orally every 12 hours -Weight 14 kg or greater: Lumacaftor 150 mg-ivacaftor 188 mg orally every 12 hours AGE 6 THROUGH 11 YEARS: Lumacaftor 200 mg-ivacaftor 250 mg orally every 12 hours AGE 12 YEARS OR OLDER: Lumacaftor 400 mg-ivacaftor 250 mg orally every 12 hours Comments:-Administer each dose with a fat-containing meal or snack consumed just before or after dosing. -Pediatric patients 1 through 5 years of age should receive oral granules. -Pediatric patients over 6 years of age should be dosed with oral tablets. -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. -The safety and efficacy of this drug in patients with cystic fibrosis other than homozygous F508del mutation has not been established. Use: For the treatment of CF in patients 1 year or older who are homozygous for the F508del mutation in the CFTR gene

Take the medicine as soon as you can, with a fatty meal. If you are more than 6 hours late for the dose, skip the missed dose. Do not take two doses at one time.

Call your doctor for instructions if you stop taking ivacaftor and lumacaftor for more than 1 week.

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

• Grapefruit may interact with ivacaftor and lumacaftor and cause side effects.
• Avoid consuming grapefruit products during the first week of treatment.

Sometimes it is not safe to use certain medicines together. Some drugs can change the levels of other drugs in your blood, which may increase side effects or make the medicines less effective.

Tell your doctor about all the medicines you are currently taking. Many drugs can affect ivacaftor and lumacaftor, especially:

• St. John's wort;
• seizure medicines such as carbamazepine, phenobarbital, phenytoin;
• medicines to prevent organ transplant rejection--cyclosporine, everolimus (Zortress), sirolimus, tacrolimus;
• sedatives--triazolam or midazolam;
• antibiotics--clarithromycin, erythromycin, telithromycin, rifabutin, rifampin; or
• antifungal medicines--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.

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