The Living with Pulmonary Fibrosis questionnaire in progressive fibrosing interstitial lung disease

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.

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

“…Limited data reported the humanistic burden of PF-ILD. Those studies identified showed that non-IPF PF-ILD negatively impacts both patients' and unpaid carers' quality of life (QoL), affecting their mental and physical health (9,11,19,31,32). These results are summarized below.…”

“…In the Living with Pulmonary Fibrosis (L-PF) study (n = 20), common symptoms reported by patients included dyspnea (95.0%), cough (95.0%), and fatigue (90.0%) (9). Similarly, patients in the King's Brief Interstitial Lung Disease (K-BILD) study (n = 20), commonly reported dyspnea (100.0%), chest tightness (85.0%), and chest wheezing/whistling (80.0%) (32).…”

“…Dyspnea, cough and fatigue are strong drivers of HRQoL impairment in PF-ILD (9,19). In IPF and other PF-ILDs, cough can affect sleep and willingness to participate in social activities, dyspnea can limit patients' ability to carry out physical activities, and fatigue can lead to decreased social participation, physical deconditioning, low mood and isolation (19).…”

“…Clinical events such as acute exacerbations (AEs) and respiratory hospitalizations indicate disease worsening (1,7). Common and burdensome symptoms of PF-ILD include dyspnea, cough and fatigue (9). AEs of PF-ILD are clinically-meaningful events characterized by rapid respiratory deterioration (10), associated with a poor prognosis and increased mortality (11).…”

Health Related Quality of Life in Interstitial Lung Disease: Can We Use the Same Concepts Around the World?

“…The Living with IPF questionnaire (L-IPF) (107), developed in the English language, has published initial validation data in a cohort of patients with IPF and has recently expanded applicability as the Living with Pulmonary Fibrosis questionnaire (L-PF) (108). The Patient Experiences and Satisfaction with Medications questionnaire (PESaM) is a unique instrument evaluating patients' expectations, experiences, and satisfaction with disease-modifying drugs (109,110).…”