Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients’ perspectives of the disease and treatment

Russell et al. BMC Pulmonary Medicine (2016) 16:10 DOI 10.1186/s12890-016-0171-y RESEARCH ARTICLE Open Access Qualitative European survey of patients with idiopathic pulmonary fibrosis: patients’ perspectives of the disease and treatment Anne-Marie Russell1*, Elena Ripamonti2 and Carlo Vancheri3 Abstract Background: ‘Living with IPF and an exploration of Esbriet® – a new treatment’ was an exploratory, qualitative, real-world survey of European patients with idiopathic pulmonary fibrosis (IPF) who were receiving treatment with pirfenidone prior to its commercial availability. The aim of the survey was to probe the impact of IPF on patients’ quality of life; the role of healthcare professionals and caregivers; the information needs of both patients and their caregivers; and patients’ perceptions of pirfenidone as a new treatment option for IPF. Methods: Patients from the UK, Germany and Italy, with a diagnosis of IPF (duration >3 months), who were being treated with pirfenidone, were recruited from patient support groups, specialist centres and advocacy groups. Semi-structured, qualitative, in-depth patient interviews of 1-h duration were conducted by an independent researcher. Patients were initially asked about their experiences of living with IPF and then prompted to describe their experiences of taking pirfenidone. Techniques utilised included: the bubble-speech technique; the icon cards projective exercise; and the free association exercise. All interviews were transcribed and analysed by an independent researcher. Results: Forty-five patients (71 % male) were interviewed (mean age 68.5 years; mean time since diagnosis 3.5 years); 87 % of patients reported that diagnosis took >1 year. Patients reported that IPF had a significant physical and emotional impact on their quality of life. The beneficial role played by caregivers and interstitial lung disease specialist nurses (where available) was specifically highlighted. Although most patients were keen for information on IPF, this was often of poor quality, out of date, or in English only. Patients’ perceptions of pirfenidone were largely positive and associated with ‘hope’ but were also influenced by the level of side effects experienced. Conclusions: This survey highlights the impact of IPF on patients’ lives, and the need to adequately support both patients and their caregivers. These findings demonstrate the value of seeking patients’ perspectives of a chronic disease such as IPF and how this information can be used to guide improvements in care, to best support the needs of patients with this devastating condition. Keywords: Caregiver, Idiopathic pulmonary fibrosis, Impact, Information, Interview, Needs, Patients, Perspectives, Pirfenidone, Survey * Correspondence: 1 National Heart & Lung Institute, Imperial College & Royal Brompton Hospital, Respiratory Epidemiology, Occupational Medicine and Public Health, 1b Manresa Road, London SW3 6LR, UK Full list of author information is available at the end of the article © 2016 Russell et al. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Russell et al. BMC Pulmonary Medicine (2016) 16:10 Background Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and irreversible interstitial lung disease (ILD) with a poor prognosis (2–5 years) [1–5]. IPF is predominant in men aged >50 years and, although idiopathic, risk factors may include gastroesophageal reflux, smoking and environmental or occupational exposures [1, 3]. IPF imposes limitations on many daily activities, necessitating a change in lifestyle. In addition to impacts on physical and social function, patients may also experience increased anxiety, depression and a reduced quality of life [6–8]. Increased sleep disruption and a poorer quality of sleep contributes to fatigue, further impacting the emotional and physical well-being of patients [9–11]. The impact of IPF affects not only patients but also caregivers, family and other members of patients’ support networks, all of whom have a role to play in helping to manage the burden of the disease [12, 13]. Until recently, the standard of care for patients with IPF was limited to oxygen therapy, pulmonary rehabilitation, palliative care and, in a small minority of patients, lung transplantation [1, 14]. Pirfenidone (Esbriet®), an antifibrotic drug with anti-inflammatory properties, was approved by the European Medicines Agency (EMA) in 2011 for the treatment of adult patients with IPF based on the favourable benefit-risk profile observed in Phase III clinical trials [15, 16]. The US Food and Drug Administration simultaneously approved pirfenidone and a tyrosine kinase inhibitor, nintedanib, for the treatment of patients with IPF in the USA, in October 2014 [17–20]; the EMA subsequently approved nintedanib in January 2015 [21]. Here, we report findings from ‘Living with IPF and an exploration of Esbriet® – a new treatment’, an exploratory, qualitative, real-world survey of European patients with IPF who were receiving treatment with pirfenidone prior to its commercial availability. The aims of this survey were to: explore patients’ experiences of living with IPF and how this impacted on their quality of life; determine the availability and impact of support systems, specifically that of healthcare professionals (HCPs) and caregivers; establish the information needs of both patients with IPF and their caregivers; and explore patients’ perceptions of pirfenidone as a new treatment option. Methods Design and patients Patients were invited to enrol through patient support groups (UK), specialist centres (Italy) or an advocacy group (Germany). Eligible patients had a multidisciplinary team-confirmed diagnosis of IPF with disease duration >3 months. All patients were being treated with pirfenidone as part of a Named Patient Program (NPP) under the supervision of an ILD physician. Page 2 of 7 Patients completed a screening questionnaire to verify their IPF diagnosis and that they were receiving treatment with pirfenidone. Forced vital capacity was not recorded as part of the questionnaire and, while the NPP was in principle limited to patients with mild-tomoderate disease, the decision to ultimately treat each patient was the responsibility of each participating physician, and, therefore, the inclusion of patients with more advanced disease cannot be excluded. All patients provided consent for their participation in the survey (...truncated)