Standards of care and educational gaps in adult cystic fibrosis units: an ERS survey

Abstract

Significant progress in the field of Cystic Fibrosis (CF) has substantially extended the life expectancy of patients with CF (pwCF). Consequently, the population of adult pwCF has outnumbered paediatric patients in most developed countries. Ageing is a new factor that can contribute to disease complexity and can require adaptation of CF Units. Therefore, the necessity for standardized, specialized, and multidisciplinary care is imperative. Concerns arise regarding the adequacy of current healthcare, therapeutic, and educational offerings.To address these concerns, a multinational survey was conducted to assess the current state of care in specialized multidisciplinary adult and paediatric CF units and identify areas for improvement. Responses were collected from 44 centres providing regular care to CF patients.The survey unveiled considerable disparities in the availability of critical resources, including diagnostic access, supplementary testing, treatment modalities, transplant and transition programs, and healthcare professionals’ training.This study underscores the urgent need to standardize care across these centres in order to minimize disparities in terms of available resources and training with a particular attention to adult pwCF that are becoming more numerous and showing different needs with ageing. The changing landscape of CF in adulthood will require a constant monitoring to ensure a proper adaptation of the current model of care.