Abstract
The Living with Idiopathic Pulmonary Fibrosis (L-IPF) questionnaire was developed with substantial input from patients with IPF to assess symptoms and health-related quality of life (HRQoL). Because IPF is the prototypical chronic fibrosing interstitial lung disease (ILD) with a progressive phenotype, we expanded applicability of the L-IPF by deleting the word “idiopathic”, creating the L-PF (Living with Pulmonary Fibrosis) questionnaire, and then assessed its relevance among patients with progressive fibrosing ILDs in one-to-one interviews.
Patients in the USA and Germany with any progressive fibrosing ILD other than IPF were asked about their disease and symptoms, completed the 44-item L-PF questionnaire (comprising two modules that assess symptoms and impacts of disease) and then answered a series of debriefing questions. Interviews were recorded, transcribed and coded for qualitative content analysis.
Twenty patients were interviewed, but time constraints meant not all were asked about all items. The most frequent diagnoses were rheumatoid arthritis-associated ILD (25%) and mixed connective tissue disease-associated ILD (20%). Almost all patients endorsed the symptoms assessed by the L-PF: shortness of breath (19/20), cough (19/20 patients) and fatigue (18/20). Most patients endorsed impacts of progressive fibrosing ILD on activities of daily living, physical well-being, sleep, emotional well-being, and social aspects of their lives. Most patients had an overall positive impression of the Symptoms module and understood items as intended. All seven patients asked understood the items of the Impacts module.
The L-PF contains concepts relevant and important to patients with progressive fibrosing ILD, and items are understood as intended.
Footnotes
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Conflict of interest: Dr. Swigris is a paid consultant for Lung Therapeutics, an unpaid consultant for Boehringer Ingelheim, and he receives grant support from Genentech and Boehringer Ingelheim. He is on the advisory board of Live Fully, Inc. which has a patent pending.
Conflict of interest: Ms. Cutts reports grants from Boehringer Ingelheim, during the conduct of the study.
Conflict of interest: N. Male is an employee of Boehringer Ingelheim.
Conflict of interest: M. Baldwin is an employee of Boehringer Ingelheim.
Conflict of interest: K.B. Rohr is an employee of Boehringer Ingelheim.
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- Received March 25, 2020.
- Accepted February 24, 2021.
- Copyright ©The authors 2021
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