Pulmonary Disorders II

Continuation of patient’s current chronic airway clearance therapy

It is recommended to continue nebulized airway clearance therapies during an exacerbation (Table

2); there is limited data to describe the impact of this practice.

The only airway clearance therapy that has been studied specifically during pulmonary

exacerbations is hypertonic saline. This study of 132 patients, randomized to either 7% hypertonic

saline or control, failed to detect a difference in the primary outcome of hospital length of stay,

but did find patients in the treatment group were more likely to return to baseline FEV1 and have

significantly improved symptoms at discharge (Thorax 2016;71:141-7).

Data remains unclear on the role of continuing patient’s chronic antimicrobial regimens during an

exacerbation and practice will vary.

The typical order of inhaled medication delivery is a bronchodilator followed by hypertonic saline,

followed by chest physiotherapy, dornase alfa, and then inhaled antibiotics (Chest. 2019;155(1):202-214).

Corticosteroids

Med 2009;180:802-8; J Cyst Fibros. 2020;19(3):344-354).

However, current practice appears at odds with the 2009 guidelines. Though data from several small

studies have been mixed, the STOP trial observed 21% of patients were treated with corticosteroids

during a pulmonary exacerbation (J Cyst Fibros 2017;16:600-6).

The Prednisone in Cystic Fibrosis Pulmonary Exacerbations (PIPE) trial compared 7 days of

oral prednisone 1 mg/kg twice daily (maximum 60 mg per day) with placebo in patients with

pulmonary exacerbation receiving intravenous antibiotics and who had not recovered to greater

than 90% of their baseline FEV1 % predicted after 7 days of antibiotics. The primary outcome

was the difference in the proportion of subjects who recovered more than 90% baseline FEV1 %

predicted at day 14 of intravenous antibiotic therapy. There was no difference in FEV1 % predicted

recovery between the prednisone and placebo groups in those not responding at day 7 of intravenous

antibiotic therapy. No significant differences in adverse effects were observed between groups (Eur

Respir J. 2024;63(6):2302278).

Therapy

Mechanism

Clinical benefit(s)

Nebulized dornase alfaa

Nebulized hypertonic salinea

Osmotically active expectorant

Nebulized mannitolb

Osmotically active expectorant

2013;187:680-9.

bFlume PA, Amelina E, Daines CL, et al. Efficacy and safety of inhaled dry-powder mannitol in adults with cystic fibrosis: an international, randomized controlled study.

J Cyst Fibros 2021;20:1003-9.