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Ivacaftor is gene‐specific oral therapy for patients with cystic fibrosis who have a cystic fibrosis transmembrane conductance regulator mutation,  G 551 D . To date, limited information is available about the benefit in patients with severe  CF  related lung disease, as such patients were excluded from the phase  III  trials. We report the early results on clinical outcomes, sweat electrolytes and  C ‐reactive protein in three adults with a  G 551 D  mutation and advanced lung disease. A mean increase of 6% in  FEV  1  was observed at 2 weeks and a mean reduction in sweat chloride of −48.9 mmol/L. While improvements in spirometry, weight gain and reduction in sweat electrolytes are similar with those reported in the phase III trials, a formal comparison was not performed.

Ivacaftor in severe cystic fibrosis lung disease and a G 551 D mutation