Paediatric palliative care in advanced cystic fibrosis: from prognosis-based to needs-based care across the disease trajectory

Advances in cystic fibrosis care, particularly highly effective cystic fibrosis transmembrane conductance regulator modulator therapies, have transformed disease trajectories for many children and adolescents, improving health and survival. However, advanced disease, symptom and treatment burden, psychosocial distress, and uncertainty persist, requiring continued attention to palliative care needs. This narrative review examines palliative care in children and adolescents with advanced cystic fibrosis, focusing on multidimensional symptom burden, communication, shared decision-making, advance care planning, lung transplantation, and integration within multidisciplinary care. Building on the established longitudinal, needs-based framework for palliative care in cystic fibrosis, we examine its application specifically to paediatric care in the era of highly effective modulator therapies. Palliative care can complement disease-directed therapies throughout the disease trajectory rather than being reserved for end-of-life care. In children and adolescents, needs are shaped not only by disease severity but also by developmental stage, evolving autonomy, family circumstances, education and social participation, transplantation, and transition to adulthood. Multidisciplinary cystic fibrosis teams can provide primary palliative care within their expertise, with specialist paediatric palliative care added when needs become complex or refractory. In the era of highly effective modulator therapies, the challenge is therefore to operationalise longitudinal, needs-based palliative care for children and families across increasingly heterogeneous disease trajectories. Future research should define approaches to needs assessment, escalation of palliative support, specialist referral, and implementation within multidisciplinary cystic fibrosis services, with the aim of improving quality of life alongside longer survival.