Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues

Burgel, Pierre-Régis; Southern, Kevin W; Addy, Charlotte; Battezzati, Alberto; Berry, Claire; Bouchara, Jean-Philippe; Brokaar, Edwin; Brown, Whitney; Azevedo, Pilar; Durieu, Isabelle; Ekkelenkamp, Miquel; Finlayson, Felicity; Forton, Julian; Gardecki, Johanna; Hodkova, Pavla; Hong, Gina; Lowdon, Jacqueline; Madge, Su; Martin, Clémence; McKone, Edward; Munck, Anne; Ooi, Chee Y; Perrem, Lucy; Piper, Amanda; Prayle, Andrew; Ratjen, Felix; Rosenfeld, Margaret; Sanders, Don B; Schwarz, Carsten; Taccetti, Giovanni; Wainwright, Claire; West, Natalie E; Wilschanski, Michael; Bevan, Amanda; Castellani, Carlo; Drevinek, Pavel; Gartner, Silvia; Gramegna, Andrea; Lammertyn, Elise; Landau, Eddie (Edwina) C; Plant, Barry J; Smyth, Alan R; van Koningsbruggen-Rietschel, Silke; Middleton, Peter G.

doi:10.1016/j.jcf.2024.01.005

Journal of Cystic Fibrosis

2024

DOI: 10.1016/j.jcf.2024.01.005

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Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues

Pierre-Régis Burgel,

Kevin W Southern,

Charlotte Addy

et al.

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2024

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Cited by 3 publications

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Coping with a chronic disease in childhood and adolescence using the example of cystic fibrosis

Gardecki

2024

Monatsschr Kinderheilkd

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Coping with a chronic disease in childhood and adolescence using the example of cystic fibrosis

Gardecki

2024

Monatsschr Kinderheilkd

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Standards for the care of people with cystic fibrosis (CF); Planning for a longer life

Gramegna,

Addy,

Allen

et al. 2024

Journal of Cystic Fibrosis

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Considerations for the use of inhaled antibiotics forPseudomonas aeruginosain people with cystic fibrosis receiving CFTR modulator therapy

Burgel,

Ballmann,

Drevinek

et al. 2024

BMJ Open Resp Res

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The major cause of mortality in people with cystic fibrosis (pwCF) is progressive lung disease characterised by acute and chronic infections, the accumulation of mucus, airway inflammation, structural damage and pulmonary exacerbations. The prevalence ofPseudomonas aeruginosarises rapidly in the teenage years, and this organism is the most common cause of chronic lung infection in adults with cystic fibrosis (CF). It is associated with an accelerated decline in lung function and premature death. NewP. aeruginosainfections are treated with antibiotics to eradicate the organism, while chronic infections require long-term inhaled antibiotic therapy. The prevalence ofP. aeruginosainfections has decreased in CF registries since the introduction of CF transmembrane conductance regulator modulators (CFTRm), but clinical observations suggest that chronicP. aeruginosainfections usually persist in patients receiving CFTRm. This indicates that pwCF may still need inhaled antibiotics in the CFTRm era to maintain long-term control ofP. aeruginosainfections. Here, we provide an overview of the changing perceptions ofP. aeruginosainfection management, including considerations on detection and treatment, the therapy burden associated with inhaled antibiotics and the potential effects of CFTRm on the lung microbiome. We conclude that updated guidance is required on the diagnosis and management ofP. aeruginosainfection. In particular, we highlight a need for prospective studies to evaluate the consequences of stopping inhaled antibiotic therapy in pwCF who have chronicP. aeruginosainfection and are receiving CFTRm. This will help inform new guidelines on the use of antibiotics alongside CFTRm.

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Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues

Cited by 3 publications

References 173 publications

Coping with a chronic disease in childhood and adolescence using the example of cystic fibrosis

Coping with a chronic disease in childhood and adolescence using the example of cystic fibrosis

Standards for the care of people with cystic fibrosis (CF); Planning for a longer life

Considerations for the use of inhaled antibiotics forPseudomonas aeruginosain people with cystic fibrosis receiving CFTR modulator therapy

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