Study of Ibuprofen to Preserve Lung Function in Patients With Cystic Fibrosis

PROTOCOL OUTLINE:

This is an open label study. Patients are randomized into 5 arms, each consisting of 10 healthy volunteers and 5 patients with cystic fibrosis, based on the amount of ibuprofen received during the treatment period.

The study period lasts for at least 15 days and consists of 3 periods: baseline (days 1-3), treatment (days 3-12), and recovery (days 13-15 or longer). During the treatment period patients receive ibuprofen orally every 12 hours (except for a control arm that receives no ibuprofen).

PROTOCOL ENTRY CRITERIA:

--Disease Characteristics--

Confirmed diagnosis of cystic fibrosis (CF) based on: Sweat chloride greater than 60 mEq/L AND Typical pulmonary and/or gastrointestinal manifestation of CF OR Healthy volunteers

--Prior/Concurrent Therapy--

At least 30 days since medications with anti-neutrophil or anti-inflammatory effect (e.g., aspirin, nonsteroidal anti-inflammatory drugs [NSAIDs], corticosteroids, macrolide antibiotics)

--Patient Characteristics--

Age: Patients with cystic fibrosis (CF): 5 and over; Healthy volunteers: 18 and over

Hematopoietic: No significant history of hematologic disease

Hepatic: No significant history of hepatic disease

Renal: No significant history of renal disease

Cardiovascular: No significant history of cardiovascular disease

Pulmonary: See Disease Characteristics

Neurologic: No significant history of neurologic disease

Other: Not pregnant; No significant history of peptic ulcer disease; Patients with CF free of any acute illness within 14 days; No prior hypersensitivity to any NSAID