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Ivacaftor

Ivacaftor

Ivacaftor is a medication used to treat cystic fibrosis, a genetic disorder that affects the lungs and digestive system. It works by improving the function of the CFTR protein in patients with specific gene mutations associated with the disease. Ivacaftor helps enhance the movement of salt and water across cell membranes, leading to better lung function and overall health.

Indications

Ivacaftor is indicated for the treatment of cystic fibrosis in patients aged 4 months and older who have one of the specific mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.


Dosage

-Dosage varies depending on the age and weight of the patient. For adults and children aged 6 years and older, the standard dosage is 150 mg every 12 hours. For children under 6 years, dosages are weight-based and may range from 50 mg to 75 mg every 12 hours. -It should be taken with a fatty meal to improve absorption.


Contra-Indications

-Ivacaftor is contraindicated in patients with known hypersensitivity to the drug or its components. -It should not be used in patients with severe hepatic impairment.


Special Precautions

-Monitor liver function before starting ivacaftor and periodically thereafter. -Use caution when prescribing ivacaftor to patients with moderate hepatic impairment. -Adjustments in dosage may be required for patients taking certain medications that interact with ivacaftor.


Side Effects

-Common side effects include headache, upper respiratory tract infections, stomach pain, diarrhea, and rash. -Liver enzyme elevations and cataracts in children have been reported. -If side effects are severe or persistent, consult a healthcare provider.


Drug Interactions

-Ivacaftor may interact with strong CYP3A4 inhibitors or inducers, potentially requiring dose adjustments. -Avoid the concomitant use of grapefruit juice and Seville oranges as they may increase ivacaftor levels.


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