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Optimising treatment of CF pulmonary exacerbation: a tough nut to crack
  1. Sonya L Heltshe1,2,
  2. Christopher H Goss1,2,3
  1. 1Department of Pediatrics, University of Washington School of Medicine, Seattle, Washington, USA
  2. 2Cystic Fibrosis Foundation Therapeutics Development Network Coordinating Center, Seattle Children's Research Institute, Seattle, Washington, USA
  3. 3Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington School of Medicine Seattle, Seattle, Washington, USA
  1. Correspondence to Dr Christopher H Goss, Professor of Medicine and Pediatrics, University of Washington Medical Center, Campus Box 356522, 1959 N.E. Pacific, Seattle, WA 98195, USA; goss{at}

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Pulmonary exacerbations (PExs) are a common occurrence in cystic fibrosis (CF) and their impact real, detrimentally affecting quality of life,1 ,2 morbidity3 ,4 and mortality.5 Treatment often includes a prolonged course of intravenous aminoglycosides, beta-lactams and increased airway clearance but there is neither consensus nor standard protocol for therapeutic options in the setting of CF PEx. The United States CF Foundation conducted a systematic review and published guidelines6 ,7 for the treatment of a CF PEx; the data presented in the systematic review highlighted the paucity of evidence that guides current clinical management. Recent advances in CF chronic therapies such as inhaled antibiotics, cystic fibrosis transmembrane conductance regulator (CFTR) modulators, mucolytics and macrolides have proven to reduce the occurrence of PEx or delay the time to next exacerbation; however, very limited research8 has occurred in the area of acute management of PEx, including the development of novel therapeutics. This is due, in part, to the challenges in conducting such studies. Randomised, controlled trials of acute PEx management are mired by challenges, including variability in treatment location (home vs inpatient vs both), duration and selection of antibiotics and use of mucolytics or corticosteroids—many of which are driven by differences in physician goals or patient preferences. Conducting a trial in an environment with this constellation of variable treatment factors can be a daunting task, but without such trials, we can never improve the care of acute PEx for our patients with CF.

Dentice et al9 report findings of a randomised, blinded, placebo controlled trial of hypertonic saline (HS) administered during a hospital admission for PEx in CF adults. HS is an osmotic agent …

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  • Contributors Drafting the manuscript for important intellectual content: all authors.

  • Funding CHG receives funding from the Cystic Fibrosis Foundation, the NIH (R01HL103965, R01HL113382, R01AI101307, U M1HL119073, P30DK089507) and the FDA (R01FD003704). SLH receives funding from the Cystic Fibrosis Foundation and the NIH (P30DK089507, R01DK095738, UM1HL119073, R01DK095869).

  • Competing interests None declared.

  • Provenance and peer review Commissioned; externally peer reviewed.

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