Dyspnea in Patients With Pulmonary Fibrosis
Dyspnea in Patients With Pulmonary Fibrosis
This study has two aims:

 1. To determine the relationship of shortness of breath (dyspnea) to other conditions
 present in patients with pulmonary fibrosis.

 2. To define the relationship between shortness of breath and rate of functional decline in
 patients with pulmonary fibrosis.
Idiopathic Pulmonary Fibrosis is the most common form of chronic fibrosing lung disease seen
 by pulmonologists, with an estimated 128,000 cases in the United States alone. It is almost
 surely a disorder related to aging, with a median age at the time of diagnosis of
 approximately 65 years; IPF is almost unheard of under the age of 50. Dyspnea is common in
 patients with IPF, and is often the primary symptom of the disease. It is tightly linked to
 quality of life in IPF, suggesting that the experience of dyspnea has wide-ranging and
 clinically-significant consequences. Despite its importance, surprisingly little is known
 about the etiology or functional impact of dyspnea in this disease.

 This research proposal focuses on defining the relationship of dyspnea to comorbidity and the
 rate of functional decline in patients with IPF

 Aim 1: To determine the relationship of dyspnea to other conditions present in patients with
 IPF.

 Dyspnea is a complex symptom, related to both mechanical and cognitive factors. The
 mechanisms of dyspnea in IPF remain unknown, but there are several likely contributors that
 are both IPF and non-IPF related. Although IPF is a chronic disease of the elderly, no one
 has investigated the relationship between common geriatric conditions and dyspnea. It is well
 established that the perception of dyspnea depends equally on factors that influence the
 intensity of the experience of breathlessness (such as thoracic restriction and weakness) and
 the distress which that intensity produces. Gender, ethnicity, anxiety, pain, and depression
 all may contribute to the distress caused by dyspnea (and therefore its intensity) in IPF.

 Aim 2: To define the relationship between dyspnea and rate of functional decline in IPF.

 Numerous studies have looked at predictors of survival in patients with IPF, and both
 baseline and change in dyspnea over time have been shown to be significantly associated. The
 prediction of future morbidity, however, is largely unstudied. Established markers of
 functional decline in geriatric patients include weight loss, decline in walking speed over 4
 meters, and the onset of disability as defined by the ability to perform activities of daily
 living (ADLs) and instrumental activities of daily living (IADLs). Dyspnea likely impacts
 patients' activity levels and/or motivation to stay active, and may contribute to worsening
 functional decline.
Inclusion Criteria:

 -  18 years of age and older with pulmonary fibrosis, and able to travel to University of
 California San Francisco for study visits

 Exclusion Criteria:

 -  None