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