The Burden of Progressive Fibrosing Interstitial Lung Disease: A DELPHI Approach

Introduction: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour. Methods: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients' quality of life (QoL) and healthcare resource utilisation (HCRU). Annual costs were calculated for progressive and non-/slow-progressive fibrosing ILD for diagnosis, follow-up management, exacerbation management, and end-of-life care based on the survey data. Results: Physicians reported that progression in fibrosing ILD worsens QoL in both patients and their caregivers. Progression of fibrosing ILD was associated with a greater use of HCRU for follow-up visits and maintenance treatment compared with the non-/slow progression. The number of patients who suffered at least one acute exacerbation was reported to be more than three times higher in progressive fibrosing ILD patients than in patients with non-/slow-progressive fibrosing ILD. On average, annual estimated costs of progressive fibrosing ILD per patient were 1.8 times higher than those of the non-/slow-progressive form of the disease. Conclusions: Progression in fibrosing ILD causes a significant impact on QoL and HCRU and costs. These survey data underline the need for safe and effective therapies to slow the disease progression.</div

The Burden of Progressive Fibrosing Interstitial Lung Disease: A DELPHI Approach

Introduction: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour. Methods: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients’ quality of life (QoL) and healthcare resource utilisation (HCRU). Annual costs were calculated for progressive and non-/slow-progressive fibrosing ILD for diagnosis, follow-up management, exacerbation management, and end-of-life care based on the survey data. Results: Physicians reported that progression in fibrosing ILD worsens QoL in both patients and their caregivers. Progression of fibrosing ILD was associated with a greater use of HCRU for follow-up visits and maintenance treatment compared with the non-/slow progression. The number of patients who suffered at least one acute exacerbation was reported to be more than three times higher in progressive fibrosing ILD patients than in patients with non-/slow-progressive fibrosing ILD. On average, annual estimated costs of progressive fibrosing ILD per patient were 1.8 times higher than those of the non-/slow-progressive form of the disease.

Effectiveness of the Assessment of Burden of Chronic Obstructive Pulmonary Disease (ABC) tool: Study protocol of a cluster randomised trial in primary and secondary care

Abstract Background Chronic Obstructive Pulmonary Disease (COPD) is a growing worldwide problem that imposes a great burden on the daily life of patients. Since there is no cure, the goal of treating COPD is to maintain or improve quality of life. We have developed a new tool, the Assessment of Burden of COPD (ABC) tool, to assess and visualize the integrated health status of patients with COPD, and to provide patients and healthcare providers with a treatment algorithm. This tool may be used during consultations to monitor the burden of COPD and to adjust treatment if necessary. The aim of the current study is to analyse the effectiveness of the ABC tool compared with usual care on health related quality of life among COPD patients over a period of 18 months. Methods/Design A cluster randomised controlled trial will be conducted in COPD patients in both primary and secondary care throughout the Netherlands. An intervention group, receiving care based on the ABC tool, will be compared with a control group receiving usual care. The primary outcome will be the change in score on a disease-specific-quality-of-life questionnaire, the Saint George Respiratory Questionnaire. Secondary outcomes will be a different questionnaire (the COPD Assessment Test), lung function and number of exacerbations. During the 18 months follow-up, seven measurements will be conducted, including a baseline and final measurement. Patients will receive questionnaires to be completed at home. Additional data, such as number of exacerbations, will be recorded by the patients’ healthcare providers. A total of 360 patients will be recruited by 40 general practitioners and 20 pulmonologists. Additionally, a process evaluation will be performed among patients and healthcare providers. Discussion The new ABC tool complies with the 2014 Global Initiative for Chronic Obstructive Lung Disease guidelines, which describe the necessity to classify patients on both their airway obstruction and a comprehensive symptom assessment. It has been developed to classify patients, but also to provide visual insight into the burden of COPD and to provide treatment advice. Trial registration Netherlands Trial Register, NTR3788

Epidemiology of ILDs and their progressive-fibrosing behaviour in six European countries

The PERSEIDS study aimed to estimate incidence/prevalence of interstitial lung diseases (ILDs), fibrosing Interstitial lung diseases (F-ILDs), idiopathic pulmonary fibrosis (IPF), systemic sclerosis-associated ILD (SSc-ILD), other non-IPF F-ILDs and their progressive-fibrosing (PF) forms in six European countries, as current data are scarce.This retrospective, two-phase study used aggregate data (2014–2018). In Phase 1, incident/prevalent cases of ILDs above were identified from clinical databases through an algorithm based on codes/keywords, and incidence/prevalence was estimated. For non-IPF F–ILDs, the relative percentage of subtypes was also determined. In Phase 2, a subset of non-IPF F-ILD cases was manually reviewed to determine the percentage of PF behaviour and usual interstitial pneumonia-like (UIP-like) pattern. A weighted mean percentage of progression was calculated for each country and used to extrapolate incidence/prevalence of progressive-fibrosing ILDs (PF–ILDs).In 2018, incidence/105 person-years ranged between 9.4–83.6(ILDs), 7.7–76.2(F-ILDs), 0.4–10.3(IPF), 6.6–71.7(non-IPF F-ILDs) and 0.3–1.5(SSc-ILD); and prevalence/105 persons ranged between 33.6–247.4(ILDs), 26.7–236.8(F-ILDs), 2.8–31.0(IPF), 22.3–205.8(non-IPF F-ILDs) and 1.4–10.1(SSc-ILD). Among non-IPF F-ILDs, sarcoidosis was the most frequent subtype. PF behaviour and UIP-like pattern were present in a third of non-IPF F-ILD cases each and hypersensitivity pneumonitis showed the highest percentage of progressive behaviour. Incidence of PF-ILDs ranged between 2.1–14.5/105 person-years, and prevalence between 6.9–78.0/105 persons.To our knowledge, PERSEIDS is the first study assessing incidence, prevalence and rate of progression of ILDs across several European countries. Still below the threshold for orphan diseases, the estimates obtained were higher and more variable than reported in previous studies, but differences in study design/population must be considered.</p

Illness perceptions and coping determine quality of life in COPD patients

Jitske Tiemensma,1 Erin Gaab,2 Maarten Voorhaar,3,4 Guus Asijee,3,5 Adrian A Kaptein6 1Psychological Sciences, 2Health Sciences Research Institute, University of California, Merced, CA, USA; 3Department of Family Medicine, Maastricht University, Care and Public Health Research Institute School for Public Health and Primary Care, Maastricht, the Netherlands; 4Boehringer Ingelheim, Alkmaar, the Netherlands; 5Boehringer Ingelheim, Amsterdam, the Netherlands; 6Department of Medical Psychology, Leiden University Medical Centre, Leiden, the Netherlands Background: A key goal of chronic obstructive pulmonary disease (COPD) care is to improve patients&rsquo; quality of life (QoL). For outcomes such as QoL, illness perceptions and coping are important determinants.Aim: The primary aim was to assess the associations between illness perceptions, coping and QoL in COPD patients. A secondary aim was to compare illness perceptions and coping of patients with reference values derived from the literature.Patients and methods: A total of 100 patients were included in the study. Patients were asked to complete the Brief Illness Perception Questionnaire (B-IPQ), the Utrecht Proactive Coping Competence scale (UPCC), and a QoL item. Correlations and linear regression models were used to analyze the data. Student&rsquo;s t-tests were used to compare patients with COPD with reference values derived from the literature.Results: Patients with better understanding of COPD utilized more proactive coping strategies (P=0.04). A more intense emotional response to COPD was related to less proactive coping (P=0.02). Patients who reported using more proactive coping techniques also reported to have a better QoL (P&lt;0.01). Illness perceptions were also related to QoL: more positive illness perceptions were related to a better QoL (all P&lt;0.05). Patients with COPD reported more negative illness perceptions than people with a common cold or patients with asthma (all P&lt;0.01), but reported similar perceptions compared with patients with diabetes.Conclusion: Patients with COPD reported a moderate QoL, but appeared to be proficient in proactive coping. Illness perceptions, coping, and QoL were all associated with each other. Patients reported more strongly affected illness perceptions compared to people with a cold and patients with asthma. We postulate that a self-management intervention targeting patients&rsquo; illness perceptions leads to improved QoL. Keywords: COPD, illness perceptions, coping, QoL, common sense mode

COPD depicted - patients drawing their lungs

Ad A Kaptein,1 Jitske Tiemensma,2 Elizabeth Broadbent,3 Guus M Asijee,4,5 Maarten Voorhaar4,5 1Medical Psychology, Leiden University Medical Centre (LUMC), Leiden, the Netherlands; 2Psychological Sciences, University of California, Merced, CA, USA; 3Psychology Department, Auckland University Medical School, Auckland, New Zealand; 4CAPHRI School for Public Health and Primary Care, Maastricht University, Maastricht, the Netherlands; 5Boehringer Ingelheim Pharma GmbH &amp; Co. KG, Ingelheim am Rhein, Germany Background: Given the increasing importance of patient-reported outcomes (PRO) in quality medical care, we examined the value and feasibility of an innovative method for assessing patients&rsquo; illness perceptions, represented in drawings made by patients with COPD of their lungs. Aim: The aim of our study was: to study patients&rsquo; representation of COPD as reflected in their drawings of their lungs; and to examine scores on a validated measure that assesses illness perceptions (ie, Brief Illness Perception Questionnaire [B-IPQ]). Patients and methods: One hundred outpatients with COPD, mean age 70 years, selected from a pharmacy database, participated and 98 filled out the B-IPQ. Eighty-seven patients completed the drawing task. Results: The illness perceptions as reflected in the responses to the B-IPQ scales represented a quite optimistic view of COPD and its consequences. The drawings of the lungs reflected a considerable discordance between patients&rsquo; representations and medically accepted representations of lungs of a person with COPD. Conclusion: Assessing illness perceptions in clinical care and research about COPD offers opportunities to identify goals for patient education and self-management. Inviting patients to draw their illness is an innovative and promising approach to assessing PRO. Keywords: COPD, drawings, illness perceptions, PRO, quality of life, self-managemen

A TASMAN Expedition: Development of a Questionnaire to Assess Specific Self-Management Abilities

Introduction: Self-management (SM) is a core component of well-being and perceived health for patients with chronic obstructive pulmonary disease (COPD). Most theories on SM share that self-efficacy, illness-perception and coping are determinants of SM behavior. Optimal support to improve SM should be tailored to the individual patient’s level of these determinants as SM abilities vary between patients. To tailor SM support, it is therefore necessary to assess the scores on these determinants. Unfortunately, no such instrument exists for clinical use. Therefore, the first goal of this study was to verify presumed correlations between SM and the determinants thereof. The second goal was to develop an instrument to assess the SM abilities. Methods: In this cross-sectional, observational study, COPD patients completed the General Self-Efficacy Scale (GSES), Brief Illness Perception Questionnaire (B-IPQ) and the Utrech