Feasibility of Home-based Exercise Program for Adults With Cystic Fibrosis

The main objective of this clinical pilot study is to evaluate the feasibility and impact of a home-based exercise program on clinical and patient-centered outcomes in adult with cystic fibrosis (CF) and inform the design of a larger clinical trial.

This pilot and feasibility study is a randomized controlled trial comparing exercise intervention with a usual care group. Participants will be randomized based on a randomization table developed by a biostatistician who is not involved in the study. The exercise intervention group will be delivered with a telehealth platform using internet (Zoom HIPAA-Compliant video), an app, phone, and email/text support. Participants will be randomly allocated to either an exercise coaching program or a usual care arm. The exercise intervention arm will follow guidelines established by the American Association of Cardiovascular and Pulmonary Rehabilitation (AACVPR) and complement best clinical practices established by the American Thoracic Society/European Respiratory Society (ATS/ERS). The usual care arm will be provided a handout on resources for engaging in physical activity.

Aim 1. Examine the feasibility of a home-based Tele-rehab intervention. Indicators of feasibility will include recruitment and adherence. Hypothesis 1: Based on previous trials, the investigators predict that \>50% of eligible participants will consent to the study. Hypothesis 2: Again, based on previous trials, \>70% of participants in the intervention arm will adhere to \>70% of their weekly prescribed exercise. Also, the investigators will assess reasons for; 1) eligible patients who don't enroll, 2) participants' non-adherence, and 3) non-completers.

Aim 2. Evaluate the impact of a home-based exercise program in adults with CF for improving clinical and patient-centered outcomes. Hypothesis 1: There will be a greater improvement in exercise capacity (measured by a modified shuttle test), a novel measure of lung function 129Xenon MRI (129Xe MRI), forced expiratory volume in 1 second (FEV1), and quality of life with participants in the intervention arm when compared to usual care. Hypothesis 2: Changes in exercise capacity and 129Xe MRI will be associated with exercise adherence, as noted in Aim 1.

Eligibility Criteria:
Inclusion Criteria:

* cystic fibrosis patients 18 years of age and older, confirmed diagnosis of CF (two CF mutations or sweat chloride \> 60 mmol/L)
* stable while either on/off Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapy and no plan to start/discontinue CFTR modulator therapy
* clearance from their CF physician to participate in exercise
* have access to the internet
* not involved in an exercise intervention in the previous 6 months, and not performing structured exercise \> 150 minutes per week.

Exclusion Criteria:

* pregnancy
* history of solid organ transplant
* active treatment for mycobacterial infections
* significant untreated hypoxemia, oxygen dependent at rest or with exercise
* FEV1 \< 40% of predicted or clinical evidence of cor pulmonale
* untreated arterial hypertension (resting systolic blood pressure \>140 mm Hg, diastolic blood pressure \> 90 mmHg)
* systolic blood pressure less than 90 mm Hg while standing
* congestive heart failure
* active treatment for Allergic Bronchopulmonary Aspergillosis (ABPA)
* acute upper or lower respiratory infection or pulmonary exacerbation within 4 weeks prior to Day 1
* changes in therapy (including antibiotics) for pulmonary disease within 4 weeks prior to Day 1
* significant hemoptysis within 4 weeks prior to Day 1 (≥ 5 mL of blood in one coughing episode or \> 30 mL of blood in a 24 hour period
* ongoing participation in an investigational drug study within 60 days prior to Day 1