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Idiopathic Pulmonary Fibrosis: A Review of Disease, Pharmacological, and Nonpharmacological Strategies With a Focus on Symptoms, Function, and Health-Related Quality of Life.

Authors
  • Rozenberg, Dmitry1
  • Sitzer, Nicole2
  • Porter, Sandra3
  • Weiss, Andrea4
  • Colman, Rebecca5
  • Reid, W Darlene6
  • Shapera, Shane5
  • Fisher, Jolene2
  • Wentlandt, Kirsten7
  • 1 Division of Respirology, Department of Medicine, University Health Network, University of Toronto, Toronto, Ontario, Canada; Toronto Lung Transplant Program, University Health Network, Toronto, Ontario, Canada; Toronto General Hospital Research Institute, Toronto, Ontario, Canada. , (Canada)
  • 2 Division of Respirology, Department of Medicine, University Health Network, University of Toronto, Toronto, Ontario, Canada. , (Canada)
  • 3 Department of Pharmacy, University Health Network, Toronto, Ontario, Canada. , (Canada)
  • 4 Division of Palliative Care, Department of Supportive Care, University Health Network, Toronto, Ontario, Canada; Division of Palliative Care, Department of Family and Community Medicine, University of Toronto, Toronto, Ontario, Canada. , (Canada)
  • 5 Division of Respirology, Department of Medicine, University Health Network, University of Toronto, Toronto, Ontario, Canada; Toronto Lung Transplant Program, University Health Network, Toronto, Ontario, Canada. , (Canada)
  • 6 Interdepartmental Division of Critical Care, Department of Physical Therapy, University of Toronto, Toronto, Ontario, Canada; Interdepartmental Division of Critical Care Medicine, Toronto Rehabilitation Institute, University Health Network, University of Toronto, Toronto, Ontario, Canada. , (Canada)
  • 7 Division of Palliative Care, Department of Supportive Care, University Health Network, Toronto, Ontario, Canada; Division of Palliative Care, Department of Family and Community Medicine, University of Toronto, Toronto, Ontario, Canada. Electronic address: [email protected] , (Canada)
Type
Published Article
Journal
Journal of pain and symptom management
Publication Date
Jun 01, 2020
Volume
59
Issue
6
Pages
1362–1378
Identifiers
DOI: 10.1016/j.jpainsymman.2019.12.364
PMID: 31887400
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Despite several advances in treatment, idiopathic pulmonary fibrosis (IPF) remains a progressive, symptomatic, and terminal disease in patients not suitable for lung transplantation. With disease progression, IPF often leads to a constellation of symptoms, including dyspnea, cough, anxiety, and depression. Palliative care is appropriate to support these patients. However, traditional curriculum in palliative care has often focused on supporting patients with malignant disease, and clinicians are not universally trained to manage patients with progressive nonmalignant diseases such as IPF. Current antifibrotic therapies aim to slow disease progression but are not able to reduce symptoms or improve daily function and health-related quality of life (HRQL). Palliative care in this patient group requires an understanding of the clinical characteristics of IPF, comorbidities, common medications used, and nonpharmacological strategies that can be undertaken to improve daily function and HRQL. This review focuses on IPF management strategies and their effects on symptoms, exercise tolerance, HRQL, and survival. Pharmacological interactions and considerations related to commonly used palliative care medications are also reviewed. This review highlights the needs of patients with IPF and caregivers, psychosocial function, patient-reported assessment tools, and topics related to advance care planning. Copyright © 2019 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.

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