Cystic fibrosis (CF) is traditionally associated with considerable and progressive multisystem pathology, onerous treatment burden, complex psychosocial challenges, and reduced life-expectancy [1-9].This decade has seen transformative change in management for many, but not all, people with CF. The most notable change comes from Cystic Fibrosis Transmembrane Receptor (CFTR) modulators, which bring significant benefits for people who are eligible for, and able to access, them [10]. However alongside, or perhaps because of, this exciting progress, the past few years have also brought important novel challenges to the psychosocial wellbeing of people with CF. This article, written as a collaboration between CF psychologists, social workers, physicians and nurses aims to provide an accessible overview of the novel psychosocial challenges now faced by children, their families, and adults with CF, and to invite consideration of their changing psychosocial requirements to inform future holistic care. Themes include geopolitical stressors such as the pandemic and its wake, a growing divide between those able or unable to access CFTR modulators, potential rapid changes in life expectancy secondary to these drugs and the inevitable associated challenges this brings; evolving body image, mental health side effects of CFTR modulators, the challenges of adherence in apparently well children and young adults, as well as the diagnostic conundrum and associated anxiety of the cystic fibrosis screen positive inconclusive diagnosis (CFSPID) label. It also highlights some unmet research and service delivery needs in the area.
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http://dx.doi.org/10.1016/j.prrv.2024.08.001 | DOI Listing |
BMJ Sex Reprod Health
December 2024
Department of Obstetrics and Gynecology, Oregon Health & Science University, Portland, Oregon, USA.
J Med Genet
December 2024
Molecular Genetics, Virtus Diagnostics, Revesby, New South Wales, Australia.
Background: Targeted cystic fibrosis (CF) carrier screening panels may lack sensitivity in non-European ancestry groups. This study aims to evaluate the sensitivity of various panels in Australian CF carriers identified through sequencing.
Methods: The following panels were evaluated in 869 CF carriers: Asuragen, Elucigene, Devyser, American College of Medical Genetics and Genomics and Victorian Clinical Genetics Services.
BMJ Support Palliat Care
December 2024
Section of Palliative Medicine, Department of Oncology, Rigshospitalet, Kobenhavn, Denmark
Background: Cystic fibrosis (CF) is an incurable, progressive disease that affects multiple organs, causing burdensome symptoms. This study aimed to explore the palliative care needs in patients with CF, focusing on health-related quality of life (HRQOL), fatigue, anxiety and depression.
Methods: From October 2019 to March 2020, a cross-sectional questionnaire survey was conducted with outpatients with CF at the Infectious Medicine Clinic in a Danish University Hospital.
Pediatr Pulmonol
December 2024
Department of Pediatrics and Professor of Biomedical Engineering, Virginia Commonwealth University, Richmond, Virginia, USA.
Pediatr Pulmonol
December 2024
Department of Pediatrics, Virginia Commonwealth University, Richmond, Virginia, USA.
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