Perspectives on Palliative Care Among Duchenne Muscular Dystrophy Patients and Their Families in Singapore

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, crosssectional 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. Mostpatients 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. Key words: Advance care planning, Palliative support services, Quality of life.

Role of pharmacists in the context of rare diseases: a scoping review protocol

“…Regardless of important innovations in diagnostic and therapeutic areas, many rare health conditions still lack specific therapies, which increases the need for preventive measures and multidisciplinary follow-ups. While practice standards for interprofessional care have been published for some rare conditions, patients can receive care from providers who have little knowledge or experience with the disease 23–26. In this sense, improving therapeutic management in services, including that of available pharmacological treatments, is a fundamental demand that has the potential to reduce costs and provide a better quality of life for those with rare health conditions.…”

Palliative care in Duchenne muscular dystrophy: Goals of care discussions and beyond

“…Palliative care engagement is recommended throughout the lifespan of these individuals 1 ; however, practices surrounding these topics and palliative care team involvement in DMD is highly variable across centers. The few published studies consistently showed underutilization of palliative care services in DMD 3‐8 . The reasons for this are multilayered, and may include limited resources, time constraints, lack of awareness among providers or families, and lack of willingness of patients and their families to engage with these services.…”

“…Traditional quantitative approaches, such as randomized controlled trials or observational studies, may not be feasible when gathering individual viewpoints on care goals and advance directives in this population, due to the sensitive nature of the topics and complexities surrounding data generation. The small number of currently available studies that focused on palliative care services in the DMD population used such qualitative research methodologies primarily by interviewing patients and their families/caregivers 3‐8 …”

“…The few published studies consistently showed underutilization of palliative care services in DMD. [3][4][5][6][7][8] The reasons for this are multilayered, and may include limited resources, time constraints, lack of awareness among providers or families, and lack of willingness of patients and their families to engage with these services. Even when palliative care services are available, our understanding of the unique needs and perceptions of goals of care is limited for those with DMD and their caregivers.…”

“…The small number of currently available studies that focused on palliative care services in the DMD population used such qualitative research methodologies primarily by interviewing patients and their families/caregivers. [3][4][5][6][7][8] Grossoehme et al 9 interviewed 13 individuals with DMD, aged 11 to 33 years, at different disease stages together with 17 caregivers. Their findings revealed that the adolescents and young adults with DMD were unlikely to have explicit goals of care discussions with their providers and generally deferred such evaluation because of delays in diagnoses and a gradual disease progression.…”