Navigating Change – UK specialised adult cystic fibrosis service delivery in the highly effective modulator therapy era

Background Cystic Fibrosis (CF) is a life limiting inherited disease that primarily effects the respiratory and gastrointestinal systems. Patients with CF have complex healthcare needs requiring multidisciplinary specialist centre care, as this is associated with improved health outcomes. Recent introduction of highly effective modulator therapies has significantly improved the health and life expectancy of this population. This study aimed to explore CF healthcare professionals experience of the impact of highly effective modulator therapies on specialist services. Methods Qualitative semi-structured online focus groups (n = 5) and individual (n = 8) interviews were conducted with 10 dietitians, 6 physiotherapists,4 clinical nurse specialists and 3 doctors working in adult CF centres. Interview data were analysed using the framework method. Themes and subthemes were mapped to Bronfenbrenner’s socio-ecological model to characterise the individual and interpersonal (micro), organisational (meso) and policy levels (macro) and chrono (time) influence of highly effective modulator therapies on the CF healthcare service model. Results Five main themes were identified; existing challenges (timeline -chrono), generalist vs specialist (individual - micro), relationships and roles (interpersonal - micro ) , model of care ( organisational – meso), normalising amid uncertainty (CF culture and society - macro). Conclusions Since the introduction of highly effective modulator therapies, specialised CF multidisciplinary teams have experienced a change in the healthcare needs and life expectancy of their patients due to fewer respiratory exacerbations, improved lung function, and weight gain. Services are now transitioning from acute care to the management of a chronic condition. As people with CF live longer and experience co-morbidities increasingly similar to the general population, services will need to incorporate approaches that prevent and manage co-morbidities associated with overweight, obesity and ageing. Targeted training, role development, and a change to infrastructure will be required to ensure CF services remain responsive to the evolving health needs of their patients. Partnership with primary care and clearer referral pathways to other specialist services will be essential to delivering effective care in the most appropriate settings. Research to develop effective behaviour change interventions and optimal nutritional targets for patients prescribed highly effective modulator therapies is required to direct clinical practice.