Ivacaftor

Oral Ivacaftor (VX-770) is an investigational drug that is designed to increase the time that activated cystic fibrosis transmembrane conductance regulator (CFTR) protein channels at the cell surface remain open.

  • It has been shown to decrease pulmonary exacerbations and other respiratory symptoms in cystic fibrosis patients

It is only to be used in cystic fibrosis patients with at least one copy of the G551D-CFTR mutation

  • It is hypothesized that modulating the CFTR protein may contribute to improved absorption of nutrients in patients with CF
  • This drug suggests that CFTR may be a valid therapeutic target for drug therapy in patients with cystic fibrosis

It has also been shown to increase weight gain in cystic fibrosis patients (which may prove beneficial as this patient population typically has difficulty gaining and maintaining weight)

Has been shown to slightly improve FEV1 in patients with CF

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