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Important Information for Your Healthcare Provider

You should talk with your healthcare provider prior to taking this medication if you have:
  • Liver disease, such as hepatitis, cirrhosis, or liver failure
  • Kidney disease, such as kidney failure (renal failure)
  • Any allergies, including to foods, dyes, or preservatives.
Also, let your healthcare provider know if you are:
Make sure to tell your healthcare provider about all other medications you are taking, including prescription and nonprescription medicines, vitamins, and herbal supplements.
(Click Precautions and Warnings With Ivacaftor to learn more, including information on who should not take the drug.)

How Does It Work?

In people with certain mutations of the CFTR (cystic fibrosis transmembrane conductance regulator) protein, the CFTR ion channels have a much lower likelihood of opening, compared to normal CFTR channels.
Ivacaftor is a CFTR protein potentiator. This means that it helps to increase the probability that the CFTR channel opens to allow flow of chloride ions across cell membranes. This helps to correct the main problem, rather than just addressing symptoms, in people with this type of cystic fibrosis.

Clinical Effects of Ivacaftor

In studies, people who took ivacaftor had improved lung function, improved cystic fibrosis symptoms, and more weight gain, compared to people taking a placebo (a "sugar pill" that does not contain any active ingredients). Ivacaftor also decreased the chance of pulmonary exacerbations.

Ivacaftor Drug Information

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