Showing papers by "John M. Wilson published in 2017"

Apparent Improvement of Airway Injury after Gene-Potentiating Therapy in Cystic Fibrosis

Abstract: Patients with cystic fibrosis (CF) and suitable genotypes are now able to access gene-potentiating therapy that improves lung function, weight gain, and prolongs the time between pulmonary exacerbations. We report the case of a 74 year old woman who had CF with a R117H gating mutation. Lung function had declined to 38% predicted normal FEV1 and there was progressive bronchiectasis evident on CT scanning. After treatment with ivacaftor over 12 months, lung function has improved to FEV1 of 50% predicted normal. Repeat CF scanning showed marked improvement of bronchiectasis. This case report demonstrated that gene-potentiating therapy may be beneficial in CF patients with advancing age and that structural improvement may occur on treatment.