What is Ivacaftor?
Ivacaftor can be used to treat cystic fibrosis among adults and children aged at least one month and weighing at least six pounds (3 kilograms). Ivacaftor tablets can be given to children 6 years old and older. Ivacaftor Granules are suitable for children from 1 month up to 6 years of age.
You will be tested by your doctor to determine if you have the gene mutation that is related to cystic fibrosis. Ivacaftor can be used in other ways not mentioned in this guide.
Side effects of Ivacaftor
If you experience any of the following symptoms of an allergic reaction, seek immediate medical attention: difficulty breathing, hives, swelling in your lips, face, throat, or tongue.
Ivacaftor can cause serious side effects. If you experience:
- Blurred vision, redness or pain in the eyes, and seeing halos around lights are all symptoms of blurred vision.
- Low sugar—headache; sweating; confusion, irritability, or dizziness. Rapid heart rate.
- Liver problems: loss of appetite; nausea; vomiting; stomach pain (upper-right side); tiredness. Itching. Dark urine and clay-coloured stools. Jaundice.
Some of the common side effects associated with ivacaftor include:
- Joint or muscle pain
- Dizziness;
- Rash, acne;
- Headache;
- Mouth pain
- Diarrhea, stomach pain, or nausea?
- Cold symptoms, including stuffy noses, sneezing, and runny nasal passages.
There may be other side effects. For medical advice regarding side effects, always seek medical advice from your physician first. You can contact the FDA at 1-800-FDA-1088 if any serious adverse events have been experienced and reported by calling 1-800FDA-1088.
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 are allergic or if
- Liver or kidney disease
The effects of ivacaftor on an unborn child are not yet known. Inform your physician immediately if you are pregnant or planning on becoming pregnant. Ask your doctor if you can safely breastfeed if you are using ivacaftor.
How to take Ivacaftor?
Read all the instructions or guides that come with your medication and follow the directions. Sometimes, your doctor will change the dose. Your medications must be taken exactly as prescribed.
If you are taking ivacaftor along with a food product that contains fat (e.g., eggs, dairy products like whole milk, yogurt, cheese, or breast milk), make sure to avoid eating foods high in fat. Please follow your doctor's directions. Do not chew or crush the tablet.
Mix the ivacaftor granules in 1 teaspoon of a soft food, such as pureed fruit or vegetables, yogurt, or applesauce. You can also mix it with water, breastmilk, infant formula, or milk. Mix 1 dose only at a given time. Use within an hour of mixing. 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 liver will require frequent blood tests. 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:
Take 150 mg every 12 hours along with a fat-containing meal.
Comments:
Examples of foods that contain fat include butter, peanut butter, cheese pizza, and dairy products made from whole milk.
If the genotype of the patient is unknown, a CFTR-mutation test approved by the FDA should be used. This test will detect if there is a CFTR mutation. The test results can then be verified with bi-directional sequences based on the instructions for the use of the test.
Uses:
for the treatment of cystic fibrosis when a patient has a mutation in the CFTR gene, which is responsive to ivacaftor based on data from clinical or in vitro tests.
The usual pediatric dose for cystic fibrosis:
Oral Granules
Age: less than six months (4 months)
Weight: 5 kg and greater: 25 mg every 12 hours
Use is not recommended if there is a hepatic impairment or if you are taking moderate or strong CYP450-3A inhibitors.
Age: 6 months and older
Weight: 5 kg to less: 25 mg every 12 hours
Weight: 7–14 kg: 50 mg every 12 hours
Weight: 14 kg and greater: 75 mg every 12 hours
Oral Tablet
Age: 6 years old and older: 150 mg orally every 12 hours
Comments:
Doses should be taken before or just after consuming a fat-containing meal. Examples of foods that contain fat include eggs and butter.
Dose adjustments may be recommended for patients aged 6 months and older who have hepatic dysfunction or are taking moderate or strong CYP450 3A inhibitors concomitantly.
If the genotype of the patient is unknown, a CFTR-mutation test cleared by the FDA should be used. This test will detect a CFTR mutation, and then bi-directional sequences can be performed if recommended in the instructions for using the test.
Uses:
for treatment of cystic fibrosis in patients aged 4 months and older with a mutation in the CFTR gene. This mutation is responsive to ivacaftor based on data from clinical or in vitro tests.
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.
What happens if I overdose?
Call 1-800-222-1222 for poison help or seek immediate medical attention.
What should be avoided?
You should avoid driving and other hazardous activities until you understand how Ivacaftor affects you. Dizziness, or drowsiness, can lead to accidents or serious injuries.
Grapefruit can interact with ivacaftor, causing side effects. Avoid consuming grapefruit products.
Interaction with other drug
It is sometimes not safe to take certain medications at the same time. Using certain drugs together can cause side effects and/or reduce the effectiveness of other medications.
Tell your doctor what other medications you are taking, including:
- John's Wort;
- Seizure medication such as carbamazepine or phenobarbital
- An antibiotic—clarithromycin, erythromycin, telithromycin, rifabutin, rifampin; or
- Antifungal medicine: fluconazole, itraconazole, ketoconazole, posaconazole, and voriconazole.
This list is incomplete. Ivacaftor may be affected by other drugs, such as vitamins and herbs. This list does not include all possible drug interactions.
