Targeted Intervention for Patient Centered Outcome in Patients With Idiopathic Pulmonary Fibrosis

Targeted Intervention for Patient Centered Outcome in Patients With Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF), a chronic fibrotic lung disease of unknown cause, is characterized by relentless progression, with a three-year mortality of up to 50%. IPF has high morbidity, with 90% of patients reporting dyspnea at the time of diagnosis and this is strongly correlated with quality of life and mortality. As IPF progress, breathlessness worsens, physical functional capacity declines, and health-related quality of life deteriorates. Pulmonary rehabilitation (PR) can improve well-being in patients with other chronic lung disease, but little is known regarding PR in IPF.

The hypothesis is that the provision of a comprehensive pulmonary rehabilitation program to patients with IPF will significantly improve participant health related quality of life. The objective is to develop a comprehensive, multi-disciplinary 12-week PR program with disease specific educational components for IPF patients. The specific aims of the proposal is to evaluate the effect of the comprehensive PR program on 1) improvements in quality of life using the Medical Outcomes Study 36-Item Short Form 36 (SF-36), a quality of life instrument and 2) improvements in patient self-reported dyspnea scores using the chronic respiratory questionnaire. The investigator intends to evaluate sustained benefits of the PR intervention, changes in depression, anxiety, stress and functional physical capacity.

Inclusion Criteria:

• Diagnosis of IPF confirmed by lung biopsy or by characteristic pattern on high resolution computed tomogram (HRCT)
• No identifiable cause of lung fibrosis
• PR not received in the past year
• Ability to walk

Exclusion Criteria:

• History of unstable angina
• Deterioration cardiac or neurological disease
• Pregnancy or lactation
• Degenerative arthritis or other limitation to mobility
• PR in the past 12 months