Presently, effectiveness of treatments for CF lung disease is judged by improvement in lung
function (FEV1). However, in CF patients, FEV1 can range from severely decreased to normal,
and improvements may occur slowly. Thus, clinical trials require many patients over
prolonged periods to evaluate medications. As the pace of drug development accelerates, it
is no longer possible to test all of the promising candidate therapies using conventional
study designs. A sensitive technique for assessing lung inflammation has been developed
which uses the expression of genes located in circulating blood cells. Mononuclear cells
pass repeatedly through the blood vessels of the lung, and are exposed to many of the
inflammatory products that are present in the airways. Over the past 4 years the
investigators have identified a small group of candidate genes that are unregulated or
downregulated in response to antibiotic treatment. The investigators now propose to
prospectively test this method of quantifying lung inflammation in a large group of CF
patients undergoing treatment of pulmonary exacerbations. Blood will be sampled before and
after antibiotic treatment for a pulmonary exacerbation, and the relative change in gene
expression will be compared to improvement in FEV1 and other clinical responses, to
determine the utility of this method for use in studies. If successful, this technique could
allow for a rapid and noninvasive method to gauge immediate effects by new treatments, and
assist caregivers in determining optimal treatment strategies for the individual.
1. Documented diagnosis of CF.
2. Age 18 years old or greater.
3. Presentation at the start of treatment for a pulmonary exacerbation of CF.
4. Ability to perform reproducible Pulmonary Function Tests.
5. Willingness to comply with study procedure and willingness to provide written
1. Participation in an investigational drug study within one month of enrollment.
2. Presence of a condition or abnormality that, in the opinion of the Principal
Investigator (PI), would compromise the safety of the patient or the quality of the