Paeds Cases · respiratory-sleep-and-airway
Explain daily cystic fibrosis treatment and a new CFTR modulator to a parent — OSCE
OSCE communication and shared-planning station: explaining to a parent why daily airway clearance, nebulisers, enzymes and antibiotics all matter in cystic fibrosis, what a new CFTR modulator does and does not replace, why the first Pseudomonas is treated at once, and when to seek help urgently.
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You are the paediatric registrar in the cystic fibrosis clinic. A 7-year-old boy, homozygous Phe508del, has grown Pseudomonas for the first time and is about to start the CFTR modulator elexacaftor-tezacaftor-ivacaftor. His mother has ten minutes with you. She wants to understand, in plain language: (1) why he still needs all his daily physiotherapy, nebulisers and enzymes; (2) what the new modulator tablet does and whether it replaces everything else; (3) why the new chest bug needs treating now when he seems well; and (4) when she should seek help urgently. Explain with empathy, check understanding, and agree a plan. [8]
References4ShowHide
- [1]Middleton PG, Mall MA, Dřevínek P, et al. Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele. N Engl J Med, 2019.PMID 31697873
- [4]Fuchs HJ, Borowitz DS, Christiansen DH, et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med, 1994.PMID 7503821
- [8]Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med, 2013.PMID 23540878
- [11]Treggiari MM, Retsch-Bogart G, Mayer-Hamblett N, et al. Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis. Arch Pediatr Adolesc Med, 2011.PMID 21893650