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Copyright © 2020 Annals, Academy of Medicine, Singapore
Perspectives on Palliative Care Among Duchenne Muscular Dystrophy Patients
and Their Families in Singapore
Sarah Jane Corpuz Tapawan,
1
MD, MRCPCH (UK), Furene SJ Wang,
2
MBBS, MMed (Paeds), MRCPCH (UK), Ming Wei Lee,
1
MBBS, MMed (Paeds), MRCPCH (UK),
Aaron QH Chua,
1
MBBS, MMed (Int Med), MRCP (UK), Jeremy BY Lin,
2
MBBS, MMed (Paeds), MRCPCH (UK), Velda Han,
2
MBBS, MMed (Paeds), MRCPCH (UK),
Michael TC Lim,
2
MBBS, FRCPCH (UK), FCCP (USA), Hian Tat Ong,
2
MBBS, MMed (Paeds), Stacey KH Tay,
1,2
MBBS, MMed (Paeds), FRCPCH (UK)
1
Department of Paediatrics, Yong Loo Lin School of Medicine, National University of Singapore, Singapore
2
Khoo Teck Puat-National University Children’s Medical Institute, National University Health System, Singapore
Address for Correspondence: Dr Sarah Jane Corpuz Tapawan, Unit 9 45-49 Harbourne Road, Kingsford, NSW 2032, Australia.
Email: janesa0301@gmail.com
Introduction
Duchenne muscular dystrophy (DMD) is an X-linked,
progressive neuromuscular disorder that causes loss of
mobility and comorbidities including cardiomyopathy,
contractures, respiratory insufficiency, scoliosis and
eventual loss of ability to care for oneself.
1,2
Despite
advances in medicine, DMD remains incurable.
3
Without non-invasive ventilation (NIV), mean age of
death in DMD patients in the past was 14.4 years (95%
confidence interval, 11.9–16.82 years).
4
However,
nocturnal ventilation has improved the likelihood of
survival of up to 25 years in DMD patients.
4
In the late stage of the disease, DMD patients are
typically wheelchair-bound, on NIV support and
dependent on others for their care. Their nutritional
status is also greatly compromised from progressive
swallowing dysfunction and they experience pain
from scoliosis and contractures with poor sleep.
5–7
Beyond the direct costs incurred by DMD patients for
medical intervention, the impact of the disease on their
caregivers and families can be huge since their extensive
health needs can cause much emotional, financial and
psychological stress on their resources and coping
abilities.
8,9
Increasingly, as DMD patients enjoy longer
life expectancy, it means that the duration of nutritional,
physical and social issues that significantly impact their
quality of life has been extended.
Consequently, efforts to destigmatise and effect a
change in the perceptions of the general population of
palliative care as “giving up hope” still remains very
much a work in progress. Poor awareness of “palliative
care” in DMD patients and even health professionals
Palliative Care in DMD Patients—Sarah Jane Corpuz Tapawan et al
Original Article
Abstract
Introduction: With better medical care, patients with Duchenne muscular dystrophy
(DMD) now live longer but face more complex medical and social needs. This study
described the perceptions of DMD patients and their families of disease-specific
palliative care services in Singapore. Materials and Methods: A multicentre, cross-
sectional study involving DMD patients and their families was carried out. Structured
questionnaires were administered to them to collect data on their understanding
of palliative care, health services accessed and desired by them and quality of life.
Results: A total of 30 pairs of DMD patients and their caregivers responded. Most
patients were >13 years old (70%) and non-ambulant (86%). Most of them and
their families (70%) were also not aware of palliative care and support services
that were available to them in Singapore. Additionally, they perceived greater
financial assistance and better transport services as resources that could better
meet their care needs. The presence of scoliosis and need for ventilatory support
were associated with lower quality of life in patients. Conclusion: There is a need
to improve awareness and provision of palliative care services for DMD patients in
Singapore where discussion of end-of-life care is often considered taboo. Prevention
and correction of scoliosis and provision of appropriate ventilatory support may
improve quality of life in DMD patients.
Ann Acad Med Singapore 2020;49:72–7
Key words: Advance care planning, Palliative support services, Quality of life
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