Synopsis
Coordinate specialist multisystem care through transition and adulthood, recognise pulmonary and gastrointestinal emergencies, individualise airway and nutritional treatment, and use CFTR modulators safely by genotype.
- Cystic fibrosis is an autosomal-recessive CFTR disorder affecting airway mucus, pancreas, gut, liver, sinuses, sweat and reproduction; modern modulator therapy changes but does not erase multisystem surveillance.
- Adolescents should move through a planned, developmentally appropriate transition to a specialist adult CF centre, with increasing autonomy, medicines competence and attention to education, work and mental health.
- A late diagnosis remains possible in adults with bronchiectasis, recurrent pancreatitis, malabsorption, male infertility or characteristic organisms; use sweat chloride and CFTR testing through a specialist pathway.
Key red flags
A large fresh bleed, sudden unilateral pleuritic pain, marked desaturation or acute respiratory distress requires immediate imaging, airway assessment and CF-respiratory escalation; suspend forceful clearance until reviewed.
Investigation priorities
Assess CFTR dysfunction in a person with a compatible phenotype or newborn-screen follow-up.
Management branches
An adolescent is developing the capacity to manage treatment and prepare for adult services.
- Begin planning over several years, assess understanding and practical skills, include private consultation time, and agree how parents or carers remain supportive without displacing the young person's voice.
- Transfer a complete summary of genotype, best lung function, microbiology, allergies, venous access, complications, modulator history, fertility discussion, mental health and emergency plans to the adult centre.
Key medicines
Elexacaftor–tezacaftor–ivacaftor plus ivacaftor for eligible people aged at least 12 yearsThe current Kaftrio tablet SmPC uses two 75 mg/50 mg/100 mg combination tablets in the morning and one ivacaftor 150 mg tablet in the evening, about 12 hours apart with fat-containing food.
Pancreatic enzyme replacement therapyDose lipase units individually with every fat-containing meal, snack and supplement using the CF dietitian's plan; adjust to weight, stool and dietary response and remain within the current product and CF-centre safety ceiling.