Clinical Health Updates

Guideline for Medications in Cystic Fibrosis

Clinical Question:
What medications are effective in managing patients with cystic fibrosis?

Bottom Line:
In this evidence-based guideline, patients with cystic fibrosis should be treated with inhaled tobramycin, hypertonic saline, and dornase alfa. Patients with an FEV1 of more than 60% of predicted may benefit from ibuprofen, and those with persistent Pseudomonas aeruginosa may benefit from azithromycin. The guideline identified many areas in which the existing research is not helpful.

Reference:
Flume PA, O’Sullivan BP, Robinson KA, et al, and the Cystic Fibrosis Foundation, Pulmonary Therapies Committee. Cystic fibrosis pulmonary guidelines: chronic medications for maintenance of lung health. Am J Respir Crit Care Med 2007;176(10):957-969.

Study Design:
Practice guideline

Synopsis:
The Cystic Fibrosis Foundation commissioned a committee to develop evidence-based guidelines for drug therapy in managing patients with cystic fibrosis. Only 4 of the 15 members had potential conflicts of interest. The committee developed a set of questions that were addressed in 1 of 3 ways: a commissioned systematic review, a modified systematic review, or a summary of existing Cochrane reviews. From these questions, the committee made a set of recommendations using the US Preventive Service Task Force rating scheme to grade them. Here is a summary of their main recommendations, all applied to patients aged 6 years or older.

A recommendations
For patients with moderate to severe disease:
Chronic use of inhaled tobramycin reduces exacerbations and improves lung function
Chronic use of dornase alfa reduces exacerbations and improves lung function

B recommendations
For patients with mild disease or who are asymptomatic:
Chronic use of inhaled tobramycin reduces exacerbations and improves lung function
Chronic use of dornase alfa reduces exacerbations and improves lung function
Chronic use of inhaled hypertonic saline reduces exacerbations and improves lung function
Chronic oral ibuprofen slows the loss of lung function in patients whose FEV1 is more than 60% predicted
Chronic azithromycin reduces exacerbations and improves lung function in patients with persistent Pseudomonas aeruginosa

D recommendations
Inhaled corticosteroids provide no benefit in patients with cystic fibrosis and should not be used
Prophylactic oral antistaphylococcal antibiotics should not be used