Prospective Validation of Cough, Dyspnea, and Quality of Life Questionnaires in Patients With IPF

Study Overview
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Brief Summary
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The purpose of this study is to test cough, dyspnea (shortness of breath), and quality of life (QOL) questionnaires for their accuracy, sensitivity, and ability to reliably measure the severity of cough, breathlessness, and changes in cough and disease-related quality of life over time in Idiopathic Pulmonary Fibrosis (IPF) patients. These questionnaires have been used in other types of disease, but have not all been tested and validated in patients with cough due to IPF. Our hypothesis is that worsening of cough, dyspnea, and cough-related QOL questionnaire scores will correlate with physiologic markers of IPF severity and worsening of disease. Written, valid questionnaires measuring cough, dyspnea, and QOL are important to assess the benefit of investigational drugs under development to treat patients with IPF.

Detailed Description
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This study in patients with IPF will determine the validity, responsiveness, and reliability of two cough measures (the Leicester Cough Questionnaire (LCQ), as well as Visual Analogue Scales (VASs) for cough severity and distress; one dyspnea measure (the Baseline and Transition Dyspnea Index (BDI/TDI); and two health-related quality of life (HRQL) measures (the obstructive lung disease-specific Saint George's Respiratory Questionnaire (SGRQ) and the IPF-specific 'A Tool to Assess QOL in IPF' (ATAQ-IPF). Both the SGRQ and ATAQ include cough questions. Study participants will complete all questionnaires at baseline, 6, 12, and 18 months at the time of their usual clinic visits. Physiologic data will be collected at the same time of these visits including pulmonary function testing, exercise oxygen saturation, and changes in medications and health status. Changes in cough, dyspnea and QOL scores will be correlated with concurrent changes in physiologic markers of IPF severity. If a study participant has an acute worsening of their IPF, or undergoes lung transplantation, study questionnaires may be given at these additional timepoints when possible.

Official Title
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Prospective Validation of Cough, Dyspnea, and Quality of Life Questionnaires in Patients With Idiopathic Pulmonary Fibrosis (IPF)

Conditions
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Interstitial Lung Disease, Idiopathic Pulmonary Fibrosis

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Completion of informed consent. Adults over the age of 18. Diagnosis of IPF per ATS guidelines. Clinically stable at the time of enrollment defined as no antibiotics within the past month, with the exception of those patients currently listed for Lung Transplantation. No changes in immunosuppressive regimens (if applicable) over past month. Exclusion Criteria: Inability to understand or complete paper and pencil questionnaires. Patient not planning to return to Stanford for clinic visits.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| IPF-diagnosed patients<br>A group of up to 40 patients with a diagnosis of mild to severe IPF per American Thoracic Society (ATS) guidelines, either with no cough at baseline to severe cough at baseline, will be followed for at least a one-time assessment and every six months for up to 18 months to establish validity, responsiveness, and reliability of cough, dyspnea, and QOL instruments in patients with IPF. | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Correlation of LCQ scores with physiologic markers of IPF severity | Change scores for the LCQ cough-related quality of life instrument will be correlated with changes in pulmonary function tests | Baseline, 6, 12 and 18 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Interstitial Lung Disease (ILD), Idiopathic Pulmonary Fibrosis (IPF)