What are Ivacaftor and Lumacaftor?
Ivacaftor is a combination medication used to treat cystic fibrosis in adults and children older than 1 year. Children aged 6 and over can use Ivacaftor or Lumacaftor tablets. Ivacaftor or Lumacaftor can be given to children who are at least one year old.
You will be tested by your doctor to determine if you have a specific gene mutation that is related to cystic fibrosis. If so, you can receive ivacaftor or lumacaftor. This medication guide does not list all possible uses for lumacaftor or ivacaftor.
Side effects of Ivacaftor and Lumacaftor
If you experience any of the following symptoms of an allergic reaction, seek immediate medical attention: difficulty breathing, swelling in your face, lips, or throat, or hives.
Ivacaftor or lumacaftor can cause serious side effects. If you experience:
- Severe headache; blurred vision; or a pounding sensation in the neck or ears
- Eye pain or redness; seeing halos around lights
- Chest tightness and difficulty breathing
- Liver problems: loss of appetite; nausea; vomiting; confusion; upper right-side stomach pain; fatigue; itching. Dark urine and clay-coloured stools. Jaundice.
Some of the common side effects associated with ivacaftor or lumacaftor include:
- Breathing problems
- Feeling tired
- Nausea, gas, diarrhea;
- Rash;
- Heavy menstrual bleeding.
- Flu or symptoms of a cold, such as sneezing or a stuffy nose; a sore neck or
- Abnormal blood tests
Children taking ivacaftor or lumacaftor are more likely to experience side effects such as an increase in mucus (mucusy cough), headaches, and stomach pain.
There may be other side effects. For medical advice on side effects, call your doctor. The FDA can be contacted at 1-800-FDA-1088 to report side effects.
Similar/related drugs
Azthromycin, Zithromax, Gentamicin, Creon, and Tobramycin
Warnings
Do not use it other than as directed. Inform your doctor of any other medications you are taking or if you have allergies or other medical conditions.
Before you take this drug
If you have an allergy to ivacaftor or lumacaftor, or if:
- High blood pressure
- Lung disease;
- An organ transplant;
- Liver or kidney disease
The effects of ivacaftor or lumacaftor on an unborn child are not yet known. Inform your doctor immediately if you are pregnant or planning on becoming pregnant. Ask your doctor if you can safely breastfeed while taking ivacaftor or lumacaftor.
Ivacaftor or lumacaftor may reduce the effectiveness of hormonal contraception, such as birth controls, injections, and skin patches. Barrier birth control can be used to prevent pregnancy.
How to take Ivacaftor or lumacaftor?
Read all the instructions or guides that come with your medication and follow the directions. Sometimes, your doctor will change the dose. Your medication must be taken exactly as prescribed. You can take this medication with foods that contain fats, like eggs and dairy products such as milk, whole milk, yogurt, or breast milk. Please follow your doctor's advice. Do not chew, crush, or break the tablet.
Mix the ivacaftor or lumacaftor with 1 teaspoon of liquid or soft food such as yogurt, applesauce, or pureed vegetables. Food or liquids should be below or at room temperature. Use within an hour of mixing only one dose. After or before administering the mixture, give the child high-fat foods.
This medicine should be taken every 12 hours at regular intervals. Weight is used to determine the dose for children. If your child loses or gains weight, the dose may change.
Your blood pressure and liver function may be tested frequently. Other medical tests may be required. You may need to have your child's vision checked frequently. Store away from heat and moisture at room temperature. Keep the medicine in its original packaging.
Details on dosage
Adult dose for cystic fibrosis:
Lumacaftor/Ivacaftor, 400 mg/250 mg, orally every 12 hours with fat-containing foods
Comments:
A meal or snack containing fat should be eaten just before or after the dose. Eggs, avocados, and nuts are all examples. Butter, peanut butter, pizza with cheese, or dairy products made from whole milk can also be used.
If a patient's genotype has not yet been determined, an FDA-cleared CF mutation test should be used to detect f508del on both alleles in the CFTR gene. The safety and efficacy of this test for patients with cystic fibrosis who do not have homozygous f508del mutants haven't been proven.
Use: To treat cystic fibrosis in patients with homozygous mutations in the CFTR gene (f508del).
The usual pediatric dose for cystic fibrosis:
Weight less than 14 kg in children aged 2 to 5 years: Lumacaftor/Ivacaftor, 100 mg orally every twelve hours with fat-containing foods
Weight: 14 kg or more Age 2 to 5 years, take Lumacaftor or Ivacaftor every 12 hours orally with fat-containing foods.
Age: 6–11 years Lumacaftor/Ivacaftor: 200 mg/250 mg orally, every 12 hours.
Age: 12 or older Lumacaftor/Ivacaftor: 400 mg/250 mg orally, every 12 hours.
Comments:
Children aged 2 to 5 years should receive tablets.
A meal or snack containing fat should be eaten just before or after the dose. Eggs, avocados, and nuts are all examples. Butter, peanut butter, pizza with cheese, or dairy products made from whole milk can also be used.
If the genotype of the patient is unknown, a CF mutation test cleared by the FDA should be used to detect f508del on both alleles in the CFTR gene. The safety and efficacy of this drug for patients with cystic fibrosis who do not have homozygous f508del have yet to be established.
Use: To treat cystic fibrosis in patients aged 1 year and older with homozygous mutations in the CFTR gene.
What happens if I miss the dose?
If you miss the dose by more than six hours, skip it. Never take two doses in one go.
If you have been taking ivacaftor or lumacaftor for longer than a week, call your doctor to get instructions.
What happens if I overdose?
Call 1-800-222-1222 for poison help or seek immediate medical attention.
What should be avoided?
Grapefruit can cause side effects when combined with ivacaftor or lumacaftor. Avoid grapefruit products for the first week after treatment.
Interaction with other drug
It is sometimes not safe to take certain medications at the same time. Some drugs may affect the blood levels of drugs that you are taking, increasing side effects or making them less effective.
Tell your doctor all the medicines you are taking. Many drugs may affect ivacaftor or lumacaftor.
- John's Wort;
- Seizure medicines such as carbamazepine, phenobarbital, and phenytoin
- Medicine to prevent organ transplant rejection: cyclosporine, everolimus (Zortress), sirolimus, and tacrolimus;
- A sedative—triazolam or midazolam;
- An antibiotic—clarithromycin, erythromycin, telithromycin, rifabutin, rifampin;
- Antifungal medicine: itraconazole, ketoconazole, posaconazole, and voriconazole.
This list is incomplete. Other drugs, such as vitamins and herbs, may also affect ivacaftor or lumacaftor. This list does not include all possible drug interactions.
