Tezacaftor-ivacaftor use in routine care of adults with cystic fibrosis: a medicine use evaluation.

European Journal of Hospital Pharmacy. Science and Practice
Iona PatersonGordon MacGregor

Abstract

Cystic fibrosis is a devastating life-limiting genetic condition characterised by a progressive decline in lung function, respiratory infections and premature death. Tezacaftor-ivacaftor is a combined cystic fibrosis transmembrane conductance regulator (CFTR) modulator that targets the underlying cause of the disease. This study aimed to assess the impact of tezacaftor-ivacaftor use in routine clinical practice for adults with cystic fibrosis. A retrospective observational longitudinal cohort study design was applied to examine the clinical effect of tezacaftor-ivacaftor in routine practice in the West of Scotland Adult Cystic Fibrosis Unit. Adults receiving tezacaftor-ivacaftor for at least 4 weeks were included in this medicine use evaluation.A standardised data form was used to collect patient-level data: demographics, genotype, complications of cystic fibrosis, medicine access process. Fifty-two weeks pre and post tezacaftor-ivacaftor initiation data: lung function, body mass index (BMI), days spent in hospital, days receiving antibiotic treatment for respiratory exacerbations. Anonymised data were collated and analysed using SPSS V.26. Of 121 potential patients, 45 received treatment with tezacaftor-ivacaftor; median age 3...Continue Reading

References

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Nov 2, 2019·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Damian G Downey, Jennifer Taylor-Cousar

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