Holistic management of patients with progressive pulmonary fibrosis

Ana Oliveira; Gaia Fabbri; Thomas Gille; Elena Bargagli; Boris Duchemann; Rachel Evans; Hilary Pinnock; Anne E Holland; Elisabetta Renzoni; Magnus Ekström; Steve Jones; Marlies Wijsenbeek; Anh Tuan Dinh-Xuan; Guido Vagheggini

doi:10.1183/20734735.0101-2023

Holistic management of patients with progressive pulmonary fibrosis

Breathe (Sheff). 2023 Sep;19(3):230101. doi: 10.1183/20734735.0101-2023. Epub 2023 Sep 12.

Authors

Ana Oliveira^{1

2}, Gaia Fabbri³, Thomas Gille^{4

5}, Elena Bargagli³, Boris Duchemann^{4

6}, Rachel Evans⁷, Hilary Pinnock⁸, Anne E Holland⁹, Elisabetta Renzoni^{10

11}, Magnus Ekström¹², Steve Jones¹³, Marlies Wijsenbeek¹⁴, Anh Tuan Dinh-Xuan¹⁵, Guido Vagheggini^{16

17}

Affiliations

¹ Lab 3R Respiratory Research and Rehabilitation Laboratory, School of Health Sciences, University of Aveiro (ESSUA), IBMED Aveiro PT, Aveiro, Portugal.
² School of Rehabilitation Science, Faculty of Health Science, McMaster University, Hamilton, ON, Canada.
³ Respiratory Diseases and Lung Transplantation Unit, Department of Medical and Surgical Sciences and Neuro-Sciences, University of Siena, Siena, Italy.
⁴ Inserm U1272 "Hypoxia & the Lung", UFR SMBH Léonard de Vinci, Université Sorbonne Paris Nord, Bobigny, France.
⁵ Service Physiologie et Explorations Fonctionnelles, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Assistance Publique - Hôpitaux de Paris, Bobigny, France.
⁶ Service d'oncologie médicale et thoracique, Hôpitaux Universitaires de Paris Seine-Saint-Denis, Assistance Publique - Hôpitaux de Paris, Bobigny, France.
⁷ Department of Respiratory Sciences, University of Leicester, Glenfield Hospital, Leicester, UK.
⁸ Allergy and Respiratory Research Group, Usher Institute, The University of Edinburgh, Edinburgh, UK.
⁹ Central Clinical School, Monash University and Department of Physiotherapy, Alfred Health, Melbourne, Australia.
¹⁰ Interstitial Lung Disease Unit, Royal Brompton and Harefield Clinical Group, Guy's and St Thomas' NHS Foundation Trust, London, UK.
¹¹ Margaret Turner Warwick Centre for Fibrosing Lung Diseases, NHLI, Imperial College, London, UK.
¹² Lund University, Faculty of Medicine, Department of Clinical Sciences Lund, Respiratory Medicine, Allergology and Palliative Medicine, Lund, Sweden.
¹³ European Pulmonary Fibrosis Federation, Brussels, Belgium.
¹⁴ Centre for Expertise for Interstitial Lung Disease and Sarcoidosis, Department of Respiratory Medicine, Erasmus MC, University Medical Centre, Rotterdam, The Netherlands.
¹⁵ Service de Physiologie-Explorations Fonctionnelles, Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Université Paris Cité, Paris, France.
¹⁶ Department of Internal Medicine and Medical Specialties, Respiratory Failure Pathway, Azienda USL Toscana Nordovest, Pisa, Italy.
¹⁷ Fondazione Volterra Ricerche ONLUS, Volterra (PI), Italy.

Abstract

Progressive pulmonary fibrosis (PF) is a complex interstitial lung disease that impacts substantially on patients' daily lives, requiring personalised and integrated care. We summarise the main needs of patients with PF and their caregivers, and suggest a supportive care approach. Individualised care, education, emotional and psychological support, specialised treatments, and better access to information and resources are necessary. Management should start at diagnosis, be tailored to the patient's needs, and consider end-of-life care. Pharmacological and non-pharmacological interventions should be individualised, including oxygen therapy and pulmonary rehabilitation, with digital healthcare utilised as appropriate. Further research is needed to address technical issues related to oxygen delivery and digital healthcare.

Educational aims: To identify the main needs of patients with PF and their caregivers.To describe the components of a comprehensive approach to a supportive care programme for patients with PF.To identify further areas of research to address technical issues related to the management of patients with PF.

Publication types

Review