Experts Release New Cystic Fibrosis Guidelines

Includes updated info on which drugs work, which don't

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THURSDAY, Nov. 15 (HealthDay News) -- Experts on Thursday issued new clinical standards for the treatment of cystic fibrosis (CF).

The guidelines were based on a two-year review of published research on patients aged six and older.

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"Physicians treating patients with CF are faced with a growing number of treatment options. We are hopeful that clinicians will find these recommendations to be useful in their care of patients with CF," the guidelines' lead author, Dr. Patrick A. Flume, said in a prepared statement.

CF is a genetic disease that affects the lungs and other organs. It's characterized by thick, sticky mucus that makes it almost impossible for CF patients to fight off germs and infections. The disease is always fatal, and lung disease accounts for 85 percent of deaths among CF patients. However, advances in treatment in the last 60 years have increased life expectancy from just a few years to about 36 years.

Flume and his colleagues looked at a number of treatments and rated their effectiveness. Those with the strongest, most consistent results were given an "A" grade recommendation. They are:

• 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.
• 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 were given for:

• Inhaled tobramycin, to suppress Pseudomonas aeruginosa infections in CF patients with mild disease or who are asymptomatic, to reduce exacerbations.
• Dornase alfa, for CF patients with mild disease 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.
• Beta 2-adrenergic receptor agonists, which relax smooth muscles and dilate bronchial passages, which improve lung function in CF patients, and are well-tolerated.

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-- Robert Preidt