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Review
. 2017 Mar;11(3):157-169.
doi: 10.1177/1753465816686743. Epub 2017 Jan 1.

Optimizing quality of life in patients with idiopathic pulmonary fibrosis

Mirjam J G van Manen  1 , J J Miranda Geelhoed  1 , Nelleke C Tak  1 , Marlies S Wijsenbeek  2
Affiliations
Review

Optimizing quality of life in patients with idiopathic pulmonary fibrosis

Mirjam J G van Manen et al. Ther Adv Respir Dis. 2017 Mar.

Erratum in

  • Corrigendum for 686743.
    [No authors listed] [No authors listed] Ther Adv Respir Dis. 2017 Jun;11(6):245. doi: 10.1177/1753465817707441. Epub 2017 May 1. Ther Adv Respir Dis. 2017. PMID: 28459174 Free PMC article. No abstract available.

Abstract

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.

Keywords: health-related quality of life; idiopathic pulmonary fibrosis; pulmonary fibrosis care.

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Conflict of interest statement

Conflict of interest statement: MM, JG, NT declare that they have no conflicts of interest. MW has received speaker fees, advisory board fees, and unrestricted research grants from Intermune, Hoffman la Roche, and Boehringer Ingelheim outside the submitted work. All fees were paid to her institution.

Figures

Figure 1.
Figure 1.
Balance wheel of symptoms, perception and reaction in patients with idiopathic pulmonary fibrosis.
Figure 2.
Figure 2.
ABCDE of IPF care. GERD, gastro-esophageal reflux disease; OSA, obstructive sleep apnoea.
See this image and copyright information in PMC

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