Desmoid fibromatosis through the patients' eyes: time to change the focus and organisation of care?
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Date
2019-03-01Author
Husson, O
Younger, E
Dunlop, A
Dean, L
Strauss, DC
Benson, C
Hayes, AJ
Miah, A
van Houdt, W
Zaidi, S
Smith, M
Williams, J
Jones, RL
van der Graaf, WTA
Type
Journal Article
Metadata
Show full item recordAbstract
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.
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Subject
Humans
Fibromatosis, Aggressive
Pain
Fatigue
Focus Groups
Depression
Empathy
Decision Making
Quality of Life
Social Support
Adult
Aged
Middle Aged
Delivery of Health Care
Female
Male
Young Adult
Delayed Diagnosis
Research team
Clinical and Translational Sarcoma
Sarcoma Clinical Trials (R Jones)
Sarcoma and Melanoma Surgery
Targeted Therapy
Language
eng
Date accepted
2018-07-26
License start date
2019-03
Citation
Supportive care in cancer : official journal of the Multinational Association of Supportive Care in Cancer, 2019, 27 (3), pp. 965 - 980
Publisher
SPRINGER