The psychological implications and health risks of cystic fibrosis pre- and post- CFTR modulator therapy

Abstract

Objectives: Cystic Fibrosis (CF) care is entering a period of personalised medicine with the emergence of CF transmembrane conductance regulator (CFTR) modulator therapies. Anecdotally individuals are reporting life-changing effects of modulator therapies, proposing an important area of study. Methods: Twenty adult participants (males: 8, age range: 22–51 years, average FEV1: 53.45%) were recruited via social media to participate in a semi-structured interview; 17 participants were currently taking Elexacaftor/Tezacaftor/Ivacaftor (Kaftrio). Results: An appreciation of a “normal life” post-modulator therapy is paramount, with improvements in symptoms and quality-of-life bringing a more urgent imperative for the provision of effective support to encourage positive health and lifestyle choices. Discussion: In this new era of CF care, there remains many challenges present for the CF community, with participants suggesting that proactive psychological support is required along with proactive awareness regarding health risk behaviours for the current and future CF generations.