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Supportive Care in Cancer
Published in: Supportive Care in Cancer 3/2019

Open Access 01-03-2019 | Original Article

Desmoid fibromatosis through the patients’ eyes: time to change the focus and organisation of care?

Authors: Olga Husson, Eugenie Younger, Alison Dunlop, Lucy Dean, Dirk C. Strauss, Charlotte Benson, Andy J. Hayes, Aisha Miah, Winan van Houdt, Shane Zaidi, Myles Smith, John Williams, Robin L. Jones, Winette T. A. van der Graaf

Published in: Supportive Care in Cancer | Issue 3/2019

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Abstract

Purpose

Desmoid fibromatosis (DF) is a rare, unpredictable disease with no established, evidence-based treatments. Individual management is based on consensus algorithms. This study aimed to examine the specific health-related quality of life challenges faced by DF patients, current experiences and expectations of care.

Methods

Twenty-seven DF patients were purposively sampled from The Royal Marsden Hospital. Two focus groups and 13 interviews (males 12, females 15; mean age at study 39.5 years) explored health-related quality of life issues and experiences of healthcare. Thematic content was analysed.

Results

Discussions revealed four key themes (diagnostic pathway; treatment pathway; living with DF; supportive care). Diagnostic delay resulted from lack of recognition by patients and healthcare professionals. Some patients received an initial diagnosis of cancer, causing significant distress. Treatment decisions were challenging, and patients experienced uncertainty among clinicians about optimal therapies. Side-effects of treatment were severe, including fatigue, nausea, anorexia, low libido and depression. Pain was the most debilitating symptom and dependency on painkillers was a significant concern. Functional limitation and restricted mobility frequently affected daily activities. Patients experienced difficulty accomplishing their role in society; relationship problems, caring for children, employment and financial difficulties. Social isolation and lack of understanding were common. The psychological impact of this “life-changing and life-long” condition was profound. All patients requested knowledgeable healthcare professionals, more information, continuity of care and peer support.

Conclusions

DF patients face complex physical, psychological and practical challenges. Comprehensive care services are needed. Increasing awareness may help to improve diagnostic pathways and overall patient experience.
Appendix
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Metadata
Title
Desmoid fibromatosis through the patients’ eyes: time to change the focus and organisation of care?
Authors
Olga Husson
Eugenie Younger
Alison Dunlop
Lucy Dean
Dirk C. Strauss
Charlotte Benson
Andy J. Hayes
Aisha Miah
Winan van Houdt
Shane Zaidi
Myles Smith
John Williams
Robin L. Jones
Winette T. A. van der Graaf
Publication date
01-03-2019
Publisher
Springer Berlin Heidelberg
Published in
Supportive Care in Cancer / Issue 3/2019
Print ISSN: 0941-4355
Electronic ISSN: 1433-7339
DOI
https://doi.org/10.1007/s00520-018-4386-8

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