2026-04-17T15:43:44Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/115352025-10-24T10:22:59Zcom_2072_378070com_2072_378040col_2072_378092

Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues Southern, Kevin BATTEZZATI, ALBERTO Berry, Claire Bouchara, Jean-Philippe Gartner Tizzano, Silvia Burgel, Pierre Regis Addy, Charlotte Pacients - Satisfacció Fibrosi quística - Tractament Atenció primària DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis Other subheadings::Other subheadings::/therapy HEALTH CARE::Environment and Public Health::Public Health::Epidemiologic Measurements::Demography::Health Status::Quality of Life HEALTH CARE::Health Services Administration::Quality of Health Care::Quality Indicators, Health Care::Standard of Care ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística Otros calificadores::Otros calificadores::/terapia ATENCIÓN DE SALUD::ambiente y salud pública::salud pública::medidas epidemiológicas::demografía::estado de salud::calidad de vida ATENCIÓN DE SALUD::administración de los servicios de salud::gestión de la atención al paciente::atención integral de salud::atención primaria de la salud Complications; Cystic fibrosis; Lung infection Complicacions; Fibrosi quística; Infecció pulmonar Complicaciones; Fibrosis quística; Infección pulmonar This is the third in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation. Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family. CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey. 2025-10-24T10:22:59Z 2025-10-24T10:22:59Z 2025-10-24T10:22:59Z 2024-05-31T11:24:07Z 2024-05-31T11:24:07Z 2024-03 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion http://hdl.handle.net/11351/11535 Journal of Cystic Fibrosis;23(2) https://doi.org/10.1016/j.jcf.2024.01.005 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess Elsevier Scientia