Longitudinal effects of ivacaftor and medicine possession ratio in people with the Gly551Asp mutation: a 5-year study

Ivacaftor gave acute benefits in cystic fibrosis with Gly551Asp mutation (n=35). FEV1 improved by 9.6% predicted by 6 months. At 5 yrs FEV1 returned to baseline; annual rate of FEV1 decline wasn’t altered (1.57% pre vs 1.82% post,p=0.74). Adherence was high but declined with time

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Thorax