Ivacaftor
.svg)
Drug Status
Government Approvals
US(FDA), UK(BNF)
WHO Essential Medicine
NO
Known Teratogen
NO
Pharmaceutical Class
None
Controlled Drug Substance
NO
Ivacaftor
Summary
Ivacaftor is used to treat cystic fibrosis in patients who have specific genetic mutations. It helps manage symptoms and improve quality of life but is not a cure.
Ivacaftor works by improving the function of a protein called CFTR. This helps reduce the buildup of thick mucus in the lungs, a common problem in cystic fibrosis.
Adults and children aged 6 years and older usually take 150 mg of Ivacaftor orally every 12 hours with food containing fat. For younger children, the dose is weight-based.
Common side effects of Ivacaftor include headache, upper respiratory tract infection, and abdominal pain. Serious adverse effects can include elevated liver enzymes and hypersensitivity reactions.
Ivacaftor can interact with certain medications and dietary supplements, so inform your doctor about everything you're taking. It's not recommended for people with severe liver impairment. Regular monitoring of liver enzymes is required.
Indications and Purpose
How does Ivacaftor work?
Ivacaftor is a CFTR potentiator that enhances the function of the CFTR protein, a chloride channel on cell surfaces. By increasing chloride transport, it helps reduce mucus build-up in the lungs and improves symptoms in cystic fibrosis patients with specific genetic mutations.
Is Ivacaftor effective?
Ivacaftor has been shown to improve lung function, reduce sweat chloride levels, and enhance quality of life in patients with cystic fibrosis who have specific genetic mutations. Clinical trials have demonstrated significant improvements in lung function and other health markers, supporting its effectiveness.
What is Ivacaftor?
Ivacaftor is used to treat cystic fibrosis in patients with specific genetic mutations. It works by improving the function of the CFTR protein, which helps reduce the build-up of thick mucus in the lungs and improves other symptoms. Ivacaftor is taken orally with fat-containing food to enhance absorption.
Directions for Use
For how long do I take Ivacaftor?
Ivacaftor is used as a long-term treatment for cystic fibrosis. It is important to continue taking it even if you feel well, as it controls the symptoms but does not cure the disease. Always follow your doctor's instructions regarding the duration of use.
How do I take Ivacaftor?
Ivacaftor should be taken with fat-containing food to improve absorption. Patients should avoid grapefruit and grapefruit juice, as they can interfere with the medication's effectiveness. Follow your doctor's instructions on dosage and timing.
How long does it take for Ivacaftor to start working?
Ivacaftor may start to improve symptoms within a few weeks of starting treatment, but the exact time can vary between individuals. Regular monitoring by a healthcare provider will help assess its effectiveness.
How should I store Ivacaftor?
Ivacaftor should be stored at room temperature, between 68°F to 77°F (20°C to 25°C). Keep it in its original container, tightly closed, and out of reach of children. Avoid storing it in the bathroom to prevent exposure to moisture.
What is the usual dose of Ivacaftor?
For adults and children aged 6 years and older, the usual dose of Ivacaftor is 150 mg taken orally every 12 hours with fat-containing food. For children aged 1 month to less than 6 years, the dose is weight-based and ranges from 5.8 mg to 75 mg every 12 hours, also taken with fat-containing food.
Warnings and Precautions
Can I take Ivacaftor with other prescription drugs?
Ivacaftor interacts with strong CYP3A inhibitors like ketoconazole, which can increase its levels in the body. It also interacts with CYP3A inducers like rifampin, which can decrease its effectiveness. Patients should avoid these medications or adjust their Ivacaftor dosage under medical supervision.
Can Ivacaftor be taken safely while breastfeeding?
There is no information on the presence of Ivacaftor in human milk or its effects on breastfed infants. Due to potential risks, breastfeeding mothers should consult their doctor to weigh the benefits and risks of using Ivacaftor while nursing.
Can Ivacaftor be taken safely while pregnant?
There is limited data on the use of Ivacaftor during pregnancy. Animal studies have not shown teratogenic effects, but the potential risk to humans is unknown. Pregnant women should only use Ivacaftor if the benefits outweigh the risks, and they should consult their doctor for personalized advice.
Is Ivacaftor safe for the elderly?
Ivacaftor has not been specifically studied in elderly patients, so there is limited information on its effects in this age group. However, as with any medication, elderly patients should use Ivacaftor with caution, and their treatment should be closely monitored by a healthcare professional.
Who should avoid taking Ivacaftor?
Important warnings for Ivacaftor include the risk of elevated liver enzymes, which requires regular monitoring. Patients should avoid grapefruit products and certain medications that interact with Ivacaftor. It is contraindicated in patients with severe liver impairment and should be used cautiously in those with moderate liver impairment.
