Abbott, Janice ORCID: 0000-0001-9851-1236 and Bilton, Diana
(2015)
Adherence to Ivacaftor is suboptimal.
Journal of Cystic Fibrosis, 14
(5).
pp. 547-548.
ISSN 1569-1993
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Official URL: http://dx.doi.org/10.1016/j.jcf.2015.08.001
Abstract
Ivacaftor, the first cystic fibrosis (CF) transmembrane conductance regulator (CFTR) potentiator provides a remarkable example of personalised medicine that has the capacity to transform lives and patient care. For those with a CFTR-G551D mutation, two phase three trials (adult and paediatric age 6+ years) and a follow-up open label trial have demonstrated remarkable and sustained improvements in CFTR function, FEV1 (approximately 10% points) and a reduction in pulmonary exacerbations with Ivacaftor 150 mg every 12 h [1–3].
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