Cystic fibrosis pulmonary guidelines: Pulmonary complications: Hemoptysis and pneumothorax

Patrick A. Flume, Peter J. Mogayzel, Karen A. Robinson, Randall L. Rosenblatt, Lynne Quittell, Bruce C. Marshall, James Cunningham, Anne Downs, Jill Fleige, Chris Goss, Hector Gutierrez, Leslie Hazle, Robert Kuhn, Mary Lester, Kathryn Sabadosa, Robert L. Vender, Terry B. White, Donna Beth Willey-Courand, Melissa Chin, Carlos E. MillaJeff Wagener, Christopher M. Oermann, Thomas Ferkol, Wyn Hoover, Michelle S. Howenstine, Michael J. Rock, George Retsch-Bogart, Ronald Rubenstein, Michael Schechter, David M. Orenstein, John L. Colombo, Beryl Rosenstein, Henry L. Dorkin, James D. Acton, Susanna A. McColley, Felix Ratjen, Moira L. Aitken, Paul Mohabir, Jerry A. Nick, Marcia Katz, Daniel Rosenbluth, Randall K. Young, Aruna Sannuti, Guillermo A. DoPico, James R. Yankaskas, Douglas Holsclaw, Linda L. Wolfenden, Joseph M. Pilewski, Peter J. Murphy, Michael P. Boyle, Ahmet Uluer, Steven D. Strausbaugh, Patricia M. Joseph, Manu Jain, Elizabeth Tullis, Souheil Saddekni, Patricia Burrows, Mark Wholey, Charles Burke, Mark Sands, Sally Mitchell, R. Duane Davis, Thomas M. Egan, Charles B. Huddleston, Mike Mulligan, David McGiffin, Wickii Vigneswaran, Craig Lillehei

Research output: Contribution to journalReview article

130 Scopus citations

Abstract

Rationale: Cystic fibrosis (CF) is a recessive genetic disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance. As a result, individuals with the disease have difficulty clearing pathogens from the lung and experience chronic pulmonary infections and inflammation. There may be intermittent pulmonary exacerbations or acute worsening of infection and obstruction, which require more intensive therapies. Hemoptysis and pneumothorax are complications commonly reported in patients with cystic fibrosis. Objectives: This document presents the CF Foundation's Pulmonary Therapies Committee recommendations for the treatment of hemoptysis and pneumothorax. Methods: The committee recognized that insufficient data exist to develop evidence-based recommendations and so used the Delphi technique to formalize an expert panel's consensus process and develop explicit care recommendations. Measurements and Main Results: The expert panel completed the survey twice, allowing refinement of recommendations. Numeric responses to the questions were summarized and applied to a priori definitions to determine levels of consensus. Recommendations were then developed to practical treatment questions based upon the median scores and the degree of consensus. Conclusions: These recommendations for the management of the patient with CF with hemoptysis and pneumothorax are designed for general use in most individuals but should be adapted to meet specific needs as determined by the individuals, their families, and their health care providers. It ishopedthat the guidelines providedin this manuscript will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.

Original languageEnglish (US)
Pages (from-to)298-306
Number of pages9
JournalAmerican journal of respiratory and critical care medicine
Volume182
Issue number3
DOIs
StatePublished - Aug 1 2010

Keywords

  • Complications
  • Cystic fibrosis
  • Guidelines
  • Hemoptysis
  • Pneumothorax

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

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  • Cite this

    Flume, P. A., Mogayzel, P. J., Robinson, K. A., Rosenblatt, R. L., Quittell, L., Marshall, B. C., Cunningham, J., Downs, A., Fleige, J., Goss, C., Gutierrez, H., Hazle, L., Kuhn, R., Lester, M., Sabadosa, K., Vender, R. L., White, T. B., Willey-Courand, D. B., Chin, M., ... Lillehei, C. (2010). Cystic fibrosis pulmonary guidelines: Pulmonary complications: Hemoptysis and pneumothorax. American journal of respiratory and critical care medicine, 182(3), 298-306. https://doi.org/10.1164/rccm.201002-0157OC