Pulmonary exacerbations in CF patients with early lung disease.

Author(s): Anstead M(1), Saiman L(2), Mayer-Hamblett N(3), Lands LC(4), Kloster M(3), Goss CH(5), Rose L(6), Burns JL(7), Marshall B(8), Ratjen F(9).

Affiliation(s): Author information: (1)Kentucky Children's Hospital, University of Kentucky, Lexington, KY United States. Electronic address: mianst0@uky.edu. (2)Department of Pediatrics, Columbia University, New York, NY, United States. (3)University of Washington, Seattle, WA, United States. (4)Department of Pediatrics, McGill University, Montreal, Canada. (5)Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Washington, Seattle, WA, United States. (6)Cystic Fibrosis Therapeutics Network Coordinating Center, Seattle Children's Research Institute, Seattle, WA, United States. (7)Division of Infectious Diseases, Seattle Children's Hospital, Seattle, WA, United States. (8)Cystic Fibrosis Foundation, Bethesda, DC, United States. (9)Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Canada; Program in Physiology and Experimental Medicine, SickKids Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, Canada.

Publication date & source: 2014, J Cyst Fibros. , 13(1):74-9

BACKGROUND: Current definitions of pulmonary exacerbation (PE) in cystic fibrosis are based on studies in participants with significant lung disease and may not reflect the spectrum of findings observed in younger patients with early lung disease. METHODS: We used data from a recent trial assessing the efficacy of azithromycin in children to study signs and symptoms associated with PEs and related changes in lung function and weight. RESULTS: While increased cough was present in all PEs, acute weight loss and reduction in oxygen saturation were not observed. Changes in lung function did not differ between subjects who did experience a PE and those who were exacerbation-free. CONCLUSIONS: Cough was the predominant symptom in CF patients with early lung disease experiencing a PE. There was no significant difference in mean 6-month change in lung function or weight among subjects with one or more exacerbations and those without an exacerbation.