Patient discussions

Will vary dramatically according to the patient's age and clinical presentation. However, there are commonalities that can be highlighted.

Patients and their families should always be encouraged to take prescribed therapies, particularly in times of good health, because maintaining good health is crucial to long-term survival. Patients should be instructed on the proper administration of therapies, particularly supplemental pancreatic enzymes and chest physiotherapy. Instruction should begin within the first two visits of diagnosis and should be reinforced as often as necessary.

Patients and their families should be instructed to maintain good communication with both a primary care provider and their CF care team.

Transition from paediatric to adult care

Medical advancements mean that individuals with CF are now living into adulthood. There is a need for a safe and planned transition to bridge from paediatric to adult care. The concept of transition should be introduced early so that the patient and the family can plan and prepare for this process. Transition should be individualised, gradually giving patients more responsibility from young adulthood (e.g., self-care, disease knowledge, and decision-making). A designated adult CF centre should be identified before transfer to the adult health system.

Successful transition requires effective communication and partnership between paediatric and adult care teams. Establishing transition programmes can facilitate this process.[181]

Exercise

Exercise improves aerobic capacity, lung function, and health‐related quality of life, while reducing disease severity and improving both prognosis and survival.[182][183][184][185]

An expert consensus statement concluded that physical activity and exercise present minimal risk if the patient receives appropriate guidance about hydration, nutrition, exercise intensity, and duration.[186]​ Although exercise with huff or cough may improve mucus clearance, this should not replace other airway clearance techniques.[184]​[186]​​

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