Optimizing quality of life in patients with idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive and ultimately fatal lung disease. The combination of poor prognosis, uncertainty of disease course and severe symptom burden heavily impacts patients’ and their families’ quality of life. Though new antifibrotic drugs have been shown...

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Bibliographic Details
Published in:Therapeutic Advances in Respiratory Disease 2017-03, Vol.11 (3), p.157-169
Main Authors: van Manen, Mirjam J. G., Geelhoed, J. J. Miranda, Tak, Nelleke C., Wijsenbeek, Marlies S.
Format: Article
Language:English
Subjects:
Delivery of Health Care - methods
Disease Progression
Family - psychology
Humans
Idiopathic Pulmonary Fibrosis - physiopathology
Idiopathic Pulmonary Fibrosis - therapy
Patient Education as Topic - methods
Prognosis
Pulmonary fibrosis
Quality of Life
Reviews
Terminal Care - methods
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Summary:Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive and ultimately fatal lung disease. The combination of poor prognosis, uncertainty of disease course and severe symptom burden heavily impacts patients’ and their families’ quality of life. Though new antifibrotic drugs have been shown to decrease disease progression, the effect on health-related quality of life (HRQOL) has not been convincingly demonstrated. In a relentless disease such as IPF, striving to optimize HRQOL should complement the endeavour to prolong life. Unfortunately, there is a paucity of interventions improving symptoms and functionality for patients with IPF, and research focusing on symptom improvement, and assessing and optimizing HRQOL, is limited. This review summarizes the most recent insights into measuring and improving quality of life for patients with IPF, and discusses challenges in the management of this devastating disease. Moreover, we postulate a new model for continuous care in IPF – ‘the ABCDE of IPF care’: Assessing patients’ needs; Backing patients by giving information and support; delivering Comfort care by focusing on treating symptoms and taking into account Comorbidities; striving to prolong life by Disease modification; helping and preparing patients and their caregivers for the eventual End-of-life events that are likely to occur.
ISSN:1753-4666
1753-4658
1753-4666
DOI:10.1177/1753465816686743