Early Intervention in Pulmonary Exacerbation in Cystic Fibrosis

The study design is a single center, randomized non-blinded controlled clinical trial
comparing usual care to a program of early identification and treatment of pulmonary
exacerbation in adults with CF. Forty subjects will be enrolled a at the University of
Washington with 20 subjects randomized into the usual care arm, and 20 randomized into the
intervention arm.

Usual care is defined as the regular quarterly CF visits with acute visits triggered by
calls from the patient to the clinic. Neither home assessment of spirometry or standardized
home symptom assessment are a component of usual care. Treatment of a pulmonary exacerbation
will be based on a standard protocol employing a standard definition and treatment regimen.
This study will employ current standardized CF pulmonary exacerbation orders used at the
University of Washington. Usual care arm subjects will have 3 study visits at baseline,
midpoint, and final, over a period of 6 months.

The early intervention protocol will employ twice a week assessment of home spirometry and
CF respiratory symptoms; we will employ a home based monitoring system from Philips called
the Philips Remote Patient Monitoring system (TeleStation). The home monitoring system is
connected to the patient's phone line; this system is currently used in clinical practice in
the care of patients with diabetes and heart failure.

Subjects who do not have a home phone line will answer the CF respiratory questions via the
internet from their home computer.

The home spirometry will be done using a PiKo-6 hand held spirometer up to 84 spirometry
values. If the subject's spirometry falls by greater than 10% from baseline or the CF
respiratory symptoms change in 3 or more of the 8 respiratory symptoms captured in the
questionnaire, the subject will be called by the research staff and clinically evaluated by
the study PIs within three days. This evaluation will include a history and physical exam
along with repeat spirometry at the clinic. The intervention arm subjects will have 3 study
visits at baseline, midpoint, and final, over a period of 6 months. They may have additional
study visits depending on their respiratory symptoms or exacerbations during the 6 month
study period.

Inclusion Criteria:

- Male or female at least 18 years old

- Confirmed diagnosis of cystic fibrosis

- Chronically colonization wtih Pseudomonas defined by positive sputum or lower airway
cultures on 2 or more occasions in any 12 month period

- Ability to participate in an interview and administration of questionnaire lasting 60
minutes

- Clinically stable without IV antibiotic treatment for a pulmonary exacerbation in the
prior 2 weeks

- Ability to perform spirometry

- Have a home telephone line or home computer with internet connection

- Written informed consent

Exclusion Criteria:

- Colonization with burkholderia cepacia complex

- Confirmed diagnosis of allergic bronchopulmonary aspergillosis as defined by the CFF
guidance document

- Currently receiving antimicrobial therapy to treat non-tuberculous mycobacterium

- History of prior solid organ transplant

- Inability to speak and read the English language well enough to complete
questionnaire