What Patients With Idiopathic Pulmonary Fibrosis and Caregivers Want: Filling the Gaps With Patient Reported Outcomes and Experience Measures

Abstract Idiopathic pulmonary fibrosis is a progressive disease, with a high mortality within the first 3–5 years from diagnosis and a poor quality of life mainly because of the burden of symptoms, such as dyspnea and cough, occurring usually many months before the diagnosis. Although available antifibrotic therapies slow down disease progression, they have no impact on quality of life. Moreover, healthcare around idiopathic pulmonary fibrosis patients is very often "disease-centered", and relies on clinical surrogate outcomes that are poorly related to patients' quality of life and disease experience. Therefore, patients with idiopathic pulmonary fibrosis have several unmet needs in all domains of health that they wish to see recognized and addressed in the context of the treatment of their disease and its complications. In this review, we summarize the care pathway from the patients' perspective, identifying current gaps in care, education, support and communication between patients with idiopathic pulmonary fibrosis, their caregivers and care teams during the patient journey. The role of patients reported outcomes (PROs), PRO measures (PROMs) and patient reported experience measures (PREMs) in their care is discussed, as well as the need of disease specific PROs/PROMs/PREMs

Pulmonary Ossification Syndrome in a Patient with Chronic Thromboembolic Pulmonary Hypertension

First described more than 150 years ago, diffuse pulmonary ossification is a rare disease that can be idiopathic or secondary to an underlying chronic disorder. Commonly under-recognized and challenging to diagnose, clinical, radiographic and functional tests are often necessary. Given the increasing prevalence of chronic lung disease in the aging population, it is likely the pulmonary ossification will be encountered in clinics more often. This article describes the diagnosis of the condition in a 51-year-old man

Comparison of palliative care models in idiopathic pulmonary fibrosis

Introduction: Palliative care (PC) is recommended in idiopathic pulmonary fibrosis (IPF) patients but poorly implemented. Integration of PC into routine management by pulmonologists may improve overall and end-of-life (EOL) care, but the optimal model of PC delivery is unknown. Objective: To describe three PC care delivery models and their impact on EOL; the Multidisciplinary Collaborative ILD clinic, Edmonton, Canada (EC) and the Bristol ILD Service, UK (BC) that provide primary level PC; and the Queen’s University ILD Clinic, Kingston, Canada (QC), which refers IPF patients to a specialist PC Clinic using specific referral criteria. Methods: A multicenter retrospective observational study of IPF patients receiving care in the identified clinics (2012–2018) was designed. Demographics; PC delivery, including symptom management; advance care planning (ACP); and location of death data were examined. Results: 298 IPF patients were included (EC 95, BC 84, and QC 119). Median age was 71 years with 74% males. Overall, 63% (188) patients received PC. Primary PC approach in EC and BC led to more patients receiving PC (98% EC, 94% BC and 13% QC (p p p p p p p = 0.04; p = 0.01). EOL discussions occurred in 73% EC, 63% BC, and 4% QC decedents (p = 0.001). Fifty-nine% (57) died at home or hospice and 38% (36) in hospitals. Concordance rate between preferred and actual location of death was 58% in EC (0.29 (−0.02–0.51)) and 37% in BC models (−0.11 (−0.20–0.15)). Conclusions: Primary PC approach for IPF is feasible in ILD clinics with concurrent disease management and can improve access to symptom management, ACP, PC and EOL care. Reliance on PC specialist referral for PC initiation outside of the ILD clinic can result in delayed care

Patient-reported outcomes and patient-reported outcome measures in interstitial lung disease

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth.</p