Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.
2 min synopsisUK scopeSources checked 27 Aug 2026Clinical review pending
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Respiratory failure or major haemoptysis
Severe hypoxaemia, exhaustion, sepsis, pneumothorax or haemoptysis compromising airway or circulation is an emergency in a child with CF or bronchiectasis.
Action: Call senior paediatric respiratory and intensive-care help, give oxygen, support ventilation and obtain intravenous access and cultures without delaying treatment. Position significant unilateral bleeding with the suspected bleeding lung down when known, avoid routine airway-clearance manoeuvres during active major haemoptysis and involve interventional radiology or surgery. Treat sepsis and pneumothorax through specialist pathways.
Synopsis
Recognise cystic fibrosis and non-CF bronchiectasis from chronic wet cough, growth and infection patterns, confirm CF with specialist sweat and genetic testing and bronchiectasis with thin-section CT, investigate treatable causes, and coordinate airway clearance, culture-directed antibiotics, nutrition and CFTR-modulator care.
Cystic fibrosis is an autosomal-recessive CFTR disorder causing dehydrated secretions in lung, pancreas, gut, biliary and reproductive tracts. Bronchiectasis is irreversible bronchial dilatation from repeated infection and inflammation and has many causes.
Think CF with chronic wet cough, recurrent infection, poor weight gain, steatorrhoea, meconium ileus, rectal prolapse, salt-loss episodes, nasal polyps or affected sibling.
Newborn screening uses immunoreactive trypsin with mutation testing, but it is screening rather than diagnosis. Symptomatic children still require a specialist CF pathway.
Key red flags
Chronic daily wet cough is pathological; clubbing, crackles, poor growth or recurrent pneumonia requires respiratory investigation.
Investigation priorities
01
Reference CF test: sweat chlorideFirst step
Demonstrate CFTR dysfunction after positive screening or compatible symptoms.
Management branches
Suspect CFRefer despite screening history
Multisystem CF features or an affected sibling is present.
Refer to a specialist CF centre and obtain accredited sweat testing.
Use CFTR genetics and functional interpretation when sweat is intermediate or discordant.
National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 27 Aug 2026; clinical approval remains outstanding.