Cystic fibrosis: pulmonary and multidisciplinary management — branching viva

Opening

Examiner: A 9-year-old with cystic fibrosis attends routine clinic. She is at her personal best FEV1 and growing well, but her cough swab has grown Pseudomonas for the first time. How do you approach her overall pulmonary care and this new result? [11]

Candidate: I would frame her care around four pillars delivered daily: airway clearance physiotherapy, mucoactive nebulisers such as dornase alfa and hypertonic saline, an anti-infective strategy, and CFTR modulator therapy, all coordinated by the multidisciplinary team with nutrition. The new result is a first Pseudomonas isolate, and my priority is immediate eradication rather than observation, because preventing chronic Pseudomonas infection protects her long-term lung function. [11] [7]

Branch 1 — the first Pseudomonas isolate

Examiner: Why not simply repeat the culture and watch, since she is well? [11]

Candidate: Because a first Pseudomonas isolate is a window of opportunity. Early eradication with a regimen such as inhaled tobramycin, with or without oral ciprofloxacin, clears the organism in most children and delays or prevents chronic infection, which is one of the strongest predictors of accelerated lung decline. Watching risks letting a treatable early infection become an established biofilm that we can only suppress, not clear. [11] [7]

Examiner (probe): How do the mucoactive nebulisers help, and how do they differ? [4]

Candidate: Dornase alfa is recombinant human DNase that cleaves the extracellular DNA released by neutrophils in cystic fibrosis sputum, thinning the mucus and improving lung function and exacerbation rates. Hypertonic saline works by a different mechanism, osmotically drawing water back onto the dehydrated airway surface to improve clearance. They are complementary and both given regularly, not only when unwell. [4] [7]

Branch 2 — the exacerbation and its emergencies

Examiner: A year later she returns with increased cough, purulent sputum, weight loss and a fall in FEV1 from best. What is happening and what do you do? [7]

Candidate: This is a pulmonary exacerbation, defined by the change from her personal best. I would intensify airway clearance, send sputum cultures, and start antibiotics — oral for a mild episode, but for this more significant fall I would admit for two intravenous anti-pseudomonal agents for about 14 days, guided by her prior isolates, and optimise her nutrition throughout. Chronic azithromycin also has an anti-inflammatory role in children with chronic Pseudomonas. [7] [6]

Examiner (probe): During admission she suddenly coughs up a large volume of fresh blood. How do you manage this? [7]

Candidate: Massive haemoptysis is an emergency, usually from a hypertrophied bronchial artery. I would protect the airway, give oxygen, position her with the bleeding side down if known, resuscitate the circulation and correct any coagulopathy, withhold physiotherapy and non-essential nebulisers, and arrange urgent bronchial artery embolisation, which is the definitive treatment for significant or recurrent bleeding. I would involve respiratory and interventional radiology teams early. [7] [11]

Branch 3 — disease-modifying therapy and the team

Examiner: She is a Phe508del homozygote. What disease-modifying option would you discuss with the family? [1]

Candidate: She is eligible for the triple CFTR modulator elexacaftor-tezacaftor-ivacaftor, which corrects and potentiates the defective CFTR protein and produces large, sustained improvements in lung function, fewer exacerbations and better weight and quality of life. I would explain that it treats the underlying defect but is added to, not a substitute for, her airway clearance, infection management and nutrition, and that the team will monitor her response and side effects. [1] [7]

Examiner (probe): Who else in the team is essential to her care, and why? [1]

Candidate: The dietitian is central, because nutrition and lung function track together, managing pancreatic enzyme replacement, a high-calorie diet and fat-soluble vitamins. The physiotherapist teaches and supervises airway clearance, the CF nurse coordinates care and adherence, the pharmacist manages the complex regimen, and the social worker and psychologist support the family with a heavy treatment burden. Infection segregation between patients is also a shared team responsibility. [1] [6]

Close

Examiner: Summarise your approach to cystic fibrosis pulmonary care in one line. [1]

Candidate: Deliver the four pillars every day — airway clearance, mucoactive nebulisers, anti-infective therapy and CFTR modulators — through a multidisciplinary team that protects nutrition, eradicate the first Pseudomonas isolate, treat exacerbations early, and stay alert for the haemoptysis, pneumothorax and allergic bronchopulmonary aspergillosis that turn a chronic illness into an emergency. [1] [7]