Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive and ultimately fatal lung disease. The combination of poor prognosis, uncertainty of disease course and severe symptom burden heavily impacts patients' and their families' quality of life. Though new antifibrotic drugs have been shown to decrease disease progression, the effect on health-related quality of life (HRQOL) has not been convincingly demonstrated. In a relentless disease such as IPF, striving to optimize HRQOL should complement the endeavour to prolong life. Unfortunately, there is a paucity of interventions improving symptoms and functionality for patients with IPF, and research focusing on symptom improvement, and assessing and optimizing HRQOL, is limited. This review summarizes the most recent insights into measuring and improving quality of life for patients with IPF, and discusses challenges in the management of this devastating disease. Moreover, we postulate a new model for continuous care in IPF - 'the ABCDE of IPF care': Assessing patients' needs; Backing patients by giving information and support; delivering Comfort care by focusing on treating symptoms and taking into account Comorbidities; striving to prolong life by Disease modification; helping and preparing patients and their caregivers for the eventual End-of-life events that are likely to occur.

Pulmonary fibrosis greatly impacts patients and their partners. Unmet needs of patients are increasingly acknowledged; the needs of partners often remain unnoticed. Little is known about the best way to educate patients and partners. We investigated pulmonary fibrosis patients' and partners' perspectives and preferences in care, and the differences in these between the Netherlands and Germany. Additionally, we evaluated whether interactive interviewing could be a novel education method in this population.Patients and partners were interviewed during pulmonary fibrosis patient information meetings. In the Netherlands, voting boxes were used and results were projected directly. In Germany, questionnaires were used.In the Netherlands, 278 patients and partners participated; in Germany, 51. Many participants experienced anxiety. Almost all experienced misunderstanding, because people do not know what pulmonary fibrosis is. All expressed a need for information, psychological support and care for partners. Use of the interactive voting system was found to be pleasant (70%) and informative (94%).This study improves the knowledge of care needs of patients with pulmonary fibrosis and their partners. There were no major differences between the Netherlands and Germany. Interactive interviewing could be an attractive method to acquire insights into the needs and preferences of patients and partners, while providing them with information at the same time.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease with a poor prognosis. The quality of life of patients is often impaired [1, 2]. In other chronic lung diseases, the use of eHealth to improve clinical outcomes has been increasingly investigated [3-5]. eHealth is defined as "the use of information and communication technologies for health" [6]. Use of eHealth may improve understanding of disease, promote self-management and facilitate longitudinal data collection for both care and research [3, 7]. Experience with eHealth tools in IPF is scarce and people are often hesitant to start online initiatives in this mostly elderly population. However, collecting data at home and facilitating consultations at a distance could hold great benefits for these patients, as they often struggle to visit hospital because of reduced mobility, dyspnoea and extra oxygen needs. Together with patients we developed IPF-online, an eHealth tool for patients with IPF, and evaluated the feasibility and user satisfaction of this tool.

BackgroundThe aim of this study was to develop, together with the Lung Foundation Netherlands and Dutch Kidney Patients Association, patients and clinicians, a measure to evaluate patient experiences with the orphan drugs pirfenidone (for idiopathic pulmonary fibrosis [IPF]) and eculizumab (for atypical haemolytic uraemic syndrome [aHUS]), as well as a generic measure of patient experiences and satisfaction with medications.MethodsDevelopment of the Patient Experiences and Satisfaction with Medications (PESaM) questionnaire consisted of four phases: literature review (phase I); focus groups and individual patient interviews (phase II); item generation (phase III); and face and content validity testing (phase IV). Literature review aimed to identify existing disease-specific and generic patient experience measures to provide guidance on the domains of medication use relevant to patients, the number of items and type of response categories, and to generate an initial pool of items. Subsequent focus groups and patient interviews were conducted to gain insight into the perceived effectiveness of the therapies, the burden of side effects, and how the medication impacted on a patient’s daily life. Focus groups and interviews were recorded and transcribed verbatim. Coding was carried out by highlighting passages in the text and assigning each passage a code representing the following predefined categories: (1) perceived effectiveness; (2) side effects; (3) ease of use; and (4) impact of medication. Using data from phase I and II, a panel of experts selected items relevant for inclusion in the questionnaire. Individual patient interviews with IPF and aHUS patients (n = 18), using a retrospective verbal probing technique, were conducted to assess face validity, time needed to fill out the questionnaire, and content validity.ResultsThe PESaM questionnaire that was developed consisted of two disease-specific modules that assessed patient experiences with pirfenidone for the treatment of IPF, and eculizumab for the treatment of aHUS, a generic module, applicable to any medication, and a module to assess patient expectations. Review of the literature identified multiple disease- or medication-specific questionnaires and two generic patient satisfaction questionnaires. Common domains across most questionnaires were effectiveness, side effects, ease of use and overall satisfaction. Patient interviews revealed the social impact (e.g. unable to go outside) of side effects such as photosensitivity associated with pirfenidone and the risk of infection associated with eculizumab. Each PESaM module focuses on patients’ perceived effectiveness of the medication, side effects, and ease of use, and the impact these aspects have on physical and emotional health and daily life. The generic module additionally includes items related to satisfaction with the medication. Individual interviews with patients in phase IV confirmed, in general, that questions and response options of the modules were clear and content validity was good. The mean ...

Idiopathic pulmonary fibrosis (IPF) is a progressive, deadly disease with a devastating impact on patients' and their partners' quality of life (QoL) [1]. Many studies have shown the need for support groups, better information resources and disease education in IPF [2][3][4][5][6]. Although these needs have been identified, few studies currently exist concerning interventions that can fulfil them and possibly improve QoL for patients and their partners [7]. In our study, we determined the effect of a short multidisciplinary empowerment programme on the QoL for patients with IPF and their partners.In 2014 and 2015, consecutive IPF outpatients and their partners at the Erasmus MC, in Rotterdam, were asked to participate in a Patient and Partner Empowerment Programme for IPF, called PPEPP. "Partner" was broadly defined as spouse, partner, family member or close friend. PPEPP consisted of three afternoon meetings, divided over three consecutive weeks and focuses on coping with IPF. A psychologist who is experienced in group therapy leads PPEPP. A pulmonologist, specialised interstitial lung disease nurse, oxygen supplier, social worker and physiotherapists also contribute to the sessions. The protocol and content were designed by the participating disciplines. Moreover, two patients with IPF, a former physiotherapist and a vicar, gave their input on the protocol and content of the programme. A comprehensive description of the programme and the contribution of each discipline can be found in the supplementary material.