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:
- Inhaled tobramycin, an antibiotic, to suppress chronic Pseudomonas aeruginosa infections in CF patients with moderate to severe disease to improve lung function and reduce exacerbations; and
- Dornase alfa, which degrades the free DNA that accumulates in CF mucus, thereby loosening the mucus, promoting airway clearance, improving lung function and reducing exacerbations.
"B" grade recommendations by the committee are for:
- Inhaled tobramycin, to suppress chronic Pseudomonas aeruginosa infections in CF patients with mild disease or who are asymptomatic, to reduce exacerbations;
- Dornase alfa, for CF patients with mild disease or who are asymptomatic, to improve lung function and reduce exacerbations;
- Hypertonic saline, which hydrates surface liquid in patients with CF, thereby improving lung function and decreasing exacerbations; and
- £]2-adrenergic receptor agonists, which relax smooth muscles and dilate bronchial passages, which improve lung-function in CF patients, and were well-tolerated.
The guidelines recommend against: