Gaps in care of patients living with pulmonary fibrosis: a joint patient and expert statement on the results of a Europe-wide survey

Abstract

IntroductionPulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey.MethodsTwo online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries.ResultsThe surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptom-centred management, adequate information, trial information and increasing awareness of PF/IPF is required.ConclusionsThe surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience.

Scutellarin ameliorates pulmonary fibrosis through inhibiting NF-κB/NLRP3-mediated epithelial–mesenchymal transition and inflammation

“…Pulmonary fibrosis is a chronic, progressive lung disease, featured with rapid and lethal respiratory failure and high mortality 31 . IPF, the most common form of pulmonary fibrosis, affects 8-14% of patients each year [32][33][34] .…”

Educational aspects of rare and orphan lung diseases

“…Participants expressed the need for printed or online educational materials, and support staff for educating patients [ 34 ]. In a European-wide survey about gaps in care for pulmonary fibrosis, distributed through the ERN-LUNG network, less than half (39%) of participating healthcare professionals responded that they received education on how to communicate information on diagnosis and treatment plan with their patients [ 35 ]. One of the challenges for physicians managing rare diseases is that patients or family members can sometimes become experts on their own disease, knowing more about it than professionals [ 36 ].…”

“…Moreover, educated patients are more independent and empowered for dealing with a chronic debilitating disease, such as rare lung disease. In fact, studies on the supportive care needs of caregivers and patients with pulmonary fibrosis show that the most frequently reported needs are information and education on disease progression and disease management [ 35 , 40 ]. Nevertheless, many patients feel that they receive incomplete information, both before and after diagnosis [ 41 ].…”

“…Almost all respondents thought that patients should be involved in the education of professionals [ 32 ]. In a survey among healthcare professionals caring for patients with pulmonary fibrosis, 65% answered that their hospital offers educational activities, such as face-to-face patient information meetings, support groups, eHealth programmes, and education sessions led by specialist nurses; 39% of surveyed patients was involved in any of these activities [ 35 ].…”

The world is not enough – the value of increasing registry data in idiopathic pulmonary fibrosis

“…Larger datasets may especially be of added value to enhance our knowledge and understanding of rare events, such as acute exacerbations [29,30]. Finally, a Europe-wide, or even better, global registry could also facilitate analysis of geographical variations in care and outcomes, and differences between healthcare systems, which will hopefully improve equal access to care for patients in the near future [31]. It should however be noted that registries are currently not available in all countries; thus, registry data might still not be completely generalizable.…”