New clinical standards on the treatment of patients with cystic fibrosis (CF) have been released following an exhaustive review of all available literature.
The guidelines, a result of a two-year review of original research and systematic review literature from 1983 to 2006, were published in the second issue for November of the American Thoracic Society's American Journal of Respiratory and Critical Care Medicine.
CF is a complex genetic disease affecting the lungs and other organs, and is characterized by an unusually thick, sticky mucus that renders CF victims virtually incapable of fending off infections and pathogens. CF is always fatal, but thanks to advances in treatment in the last 60 years, life expectancy has increased from just a few years to 36 years. Lung disease accounts for 85 percent of the mortality among CF patients.
"Physicians treating patients with CF are faced with a growing number of treatment options" wrote lead author of the guidelines, Patrick A. Flume, M.D. "We are hopeful that clinicians will find these recommendations to be useful in their care of patients with CF."
The pulmonary guidelines committee assessed the clinical efficacy and safety of the use of aerosolized antibiotics, recombinant human DNase (dornase alfa), hypertonic saline inhalation, anti-inflammatory agents, macrolide antibiotics, the use of bronchodilators and N-acetylcysteine. They analyzed the clinical results with regard to the number of studies, strength of the study designs and the quality and consistency of results to make their final recommendations. Because of the general dearth of studies on treatments in children under the age of six, the committee restricted their recommendations to CF patients six years of age and older.
The treatments with the strongest, most consistent results yielding an "A"-grade positive recommendation were:
"B" grade recommendations by the committee are for:
The guidelines recommend against:
All other treatments lacked sufficient evidence to recommend for or against their use.
The article includes recommendations for physicians to help determine which therapies to use, and the order in which they should be introduced. "There is a challenge for the clinician to decide what therapy to start first, or when to add another therapy," explained Dr. Flume. "We are putting together an educational program to assist the community in implementation of the guidelines and hope to launch a web-based program in January."
Still, much work remains to be done. "We were able to support our recommendations based on the quality of the evidence," said Dr. Flume. "But also we could see clearly where we were lacking data. This will allow us to lay the foundation for how those studies should be performed."
The committee plans to update the guidelines every five years.
American Journal of Respiratory and Critical Care Medicine