Management of CF Lung Disease
Several clinical trials and epidemiologic studies have demonstrated that inflammation is a more important driver of declining lung function than is direct microbial damage.
A 4-year trial conducted on children showed that oral steroids (ie, prednisone) dosed on alternate days at 1 mg/kg was beneficial to pulmonary function, particularly in those patients colonized with Pseudomonas aeruginosa.
However, the benefit comes at significant expense in growth retardation and increased risks of glucose abnormalities, cataracts, and loss of bone density. At this time, the recommendation is that oral steroids not be used for long-term therapy.18,28
Therapy with inhaled steroids without significant systemic adverse events may be an option, but long-term data are needed before treatment for patients with CF can be recommended.18
Recent research into the use of marcrolides for the management of patients with CF has shown promise, although not for anti-infection because they are ineffective against strains of Pseudomonas aeruginosa. The macrolides show indirect anti-inflammatory effects.
In a large study treating patients with a three-times-per-week dose, there was clear evidence of a significant improvement in respiratory function.
There was a small increase in mild adverse events, mainly gastrointestinal, and there is concern regarding what resistance effect may be incurred in long-term therapy.28
High-dose ibuprofen slows the progression of lung disease in patients affected with mild CF, particularly children. Treating with ibuprofen requires monitoring of blood levels and for GI and renal adverse events.
Additionally, ibuprofen is not recommended for treatment of patients with moderate-to-severe lung disease because of the potential for increased hemoptysis.
18. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004 January;125(1 Suppl):1S-39S.
28. Smyth RL. Diagnosis and management of cystic fibrosis. Arch Dis Child Ed Pract 2005;90:1-6