Table 1.

Study Objectives

Primary Objective	To determine the efficacy of home lung function testing and symptom diary use for early intervention in the treatment of adolescent and adult CF acute pulmonary exacerbations. The primary outcome is lung function as measured with forced expiratory volume in the first second (FEV1). We hypothesize that if pulmonary exacerbations are identified and treated earlier, the progression of lung disease will be slowed, indicated by a slower annual decline in FEV1.
Secondary Objectives	1. Assess the efficacy of the multi-faceted intervention compared to usual care using a standardized treatment on health related quality of life as measured by the CFQ-R.
	2. Assess the efficacy of the multi-faceted intervention compared to usual care using a standardized treatment on respiratory symptoms as measured by the CFRSD.
	3. Assess whether the intervention will be associated with improved intermediate outcome measures (earlier identification of acute pulmonary exacerbation, change in lung function and respiratory symptoms from onset of acute pulmonary exacerbation to the 14 day follow-up visit, number of hospitalization days for acute pulmonary exacerbation, time from the onset of acute pulmonary exacerbation to onset of subsequent pulmonary exacerbation).
	4. Assess the safety of the multi-faceted intervention compared to usual care.
	5. Assess the tolerability as measured by treatment burden of the multi-faceted intervention compared to usual care.
	6. Assess the adherence to the multi-faceted intervention.