Cystic fibrosis pulmonary guidelines: pulmonary complications: hemoptysis and pneumothorax.
BACKGROUND
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.


OBJECTIVE
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.


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 is hoped that the guidelines provided in this manuscript will facilitate the appropriate application of these treatments to improve and extend the lives of all individuals with cystic fibrosis.
Flume
Patrick A
PA
Department of Medicine, Medical University of South Carolina, Charleston, SC 29425, USA. flumepa@musc.edu
Mogayzel
Peter J
PJ
Robinson
Karen A
KA
Rosenblatt
Randall L
RL
Quittell
Lynne
L
Marshall
Bruce C
BC
D000280:Administration, Inhalation; D000401:Aircraft; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage; D001981:Bronchial Arteries; D001999:Bronchoscopy; D015505:Chest Tubes; D003550:Cystic Fibrosis / Q000150:complications* / Q000628:therapy; D003657:Decision Making; D003697:Delphi Technique; D004621:Embolization, Therapeutic; D006469:Hemoptysis / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D006760:Hospitalization; D006801:Humans; D008168:Lung / Q000601:surgery; D016040:Lung Transplantation; D018579:Patient Selection; D018700:Pleurodesis / Q000379:methods; D011030:Pneumothorax / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D011175:Positive-Pressure Respiration; D012462:Saline Solution, Hypertonic / Q000008:administration & dosage; D055502:Secondary Prevention; D012720:Severity of Illness Index; D013147:Spirometry; D014195:Travel; D014891:Weight Lifting
D000900:Anti-Bacterial Agents; D000894:Anti-Inflammatory Agents, Non-Steroidal; D012462:Saline Solution, Hypertonic