Cystic Fibrosis

Pryor JA. Physiotherapy for airway clearance in adults. Eur Respir J 1999;14:1418-24.  Somewhat cursory overview of common airway clearance techniques used in the setting of CF, neuromuscular disease, and other diseases associated with impaired secretion clearance. The author also touches on the paucity of evidence supporting the superiority of any one approach.  http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10624775

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;331:637-42. Large RCT found patients receiving a 24-week course of Pulmozyme had an improvement in FEV1 of 5% compared to placebo and decreased exacerbation rate (28 vs. 37% in placebo group). http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=7503821

Ramsey BW, Pepe MS, Quan JM, et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis. N Engl J Med 1999;340:23-9. Study found use of TOBI on alternating months improved lung function, decreased bacterial burden, and decreased the relative risk of hospitalization. The rate of acquired tobramycin resistance was about 7% over 24 weeks. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9878641

Saiman L, Marshall BC, Mayer-Hamblett N, et al. Azithromycin in patients with cystic fibrosis chronically infected with pseudomonas aeruginosa.  JAMA 2003;290:1749-56.  Large multicenter RCT of 6 months duration found chronic azithromycin resulted in a 4.4% improvement in FEV1% predicted compared to a 1.8% decline in placebo.  The azithromycin group had fewer exacerbations and gained more weight. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=14519709

Balfour-Lynn IM, Lees B, Hall P, et al. Multicenter randomized controlled trial of withdrawal of inhaled corticosteroids in cystic fibrosis.  Am J Respir Crit Care Med  2006; 173:1356-62. This study of 171 children and adults with mean baseline FEV1 of 76% predicted found no difference in to time to 1st exacerbation, or use of rescue bronchodilators and antibiotics over the first 6 months of withdrawing inhaled steroid.  These results suggest that many CF patients may be able to safely discontinue inhaled steroids. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16556691&query_hl=12&itool=pubmed_docsum

Aaron SD, Vandemheen KL, Ferris W, et al. Combination antibiotic susceptibility testing to treat exacerbations of cystic fibrosis associated with multiresistant bacteria: a randomised, double-blind, controlled clinical trial.  Lancet  2005; 366:463-71.  This study of 132 CF patients found selection of intravenous antibiotics based on multiple combination bactericidal susceptibility testing did not reduce the time to next exacerbation compared to antibiotic selection based on conventional culture and sensitivity tests and there was no difference in the rate of treatment failure.  http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16084254&query_hl=14&itool=pubmed_docsum

Elkins MR, Robinson M, Rose BR, et al.  A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis.  N Engl J Med  2006; 354:229-40. This study of 164 children and adults with CF found the combination of bronchodilator and 7% saline neb bid had only a modest impact on pulmonary function but reduced exacerbations (76% exacerbation free vs. 62% placebo, p = .03).  These results may not be applicable to patients on more aggressive baseline regimens than the study population. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=16421364&query_hl=17&itool=pubmed_docsum