Advance care discussions with young people affected by life-limiting neuromuscular diseases: A systematic literature review and narrative synthesis

Abstract

End of life care policy in the UK advocates open discussions between health professionals and patients as the end of life approaches. Despite well documented understanding of the progression of life-limiting neuromuscular diseases, the majority of patients affected by such conditions die without a formal end of life plan in place. We performed a systematic review to investigate conversations regarding end of life care between healthcare professionals and younger adult patients with life-limiting neuromuscular diseases. The search strategy included terms that focused on death and dying along with other factors that could impact length of life. The review found a very limited body of literature regarding end of life care conversations between young people affected by neuromuscular diseases and health professionals. The views and preferences of patients themselves have not been investigated. There is a shared reluctance of patients, family carers and healthcare professionals to initiate end of life care discussions. There are many factors that need to be investigated further in order to develop a consensus that would allow healthcare professionals to engage patients in end of life care conversations allowing them to face the end of their lives with appropriate plans in place.

‘It’s a hard conversation to have’. Healthcare professionals’ views concerning advance care discussions with young people affected by life-limiting neuromuscular diseases: an interview study

“…Adults aged 20–45 with Duchenne muscular dystrophy report rarely being involved in end of life discussions,11 as found in our systematic review8: some adult patients have expressed reluctance to discuss such matters 12…”

“…Policy describes end of life care as applying to the last year of life, while acknowledging that it may cover periods from a few hours to many years 6. Within the specialist NMD literature, the palliative and end of life care needs of people with life-limiting NMDs are only briefly mentioned in condition-specific standards of care7; our recent systematic review found that the published literature concerning end-of-life care discussions for younger adult patients with NMDs is extremely limited 8…”

Health, wellbeing and lived experiences of adults with SMA: a scoping systematic review

“…There was no literature that described mental health care for adults with SMA or evaluated transition into the adult service. Palliative care services for adults with SMA have been described in two studies; however, limited evidence suggests a lack of co-ordination in the provision of such care [96,97]. Routine cardiac surveillance was not endorsed; two studies suggested that cardiac abnormalities were uncommon in type in SMA II and III patients [98,99].…”

Duchenne muscular dystrophy

“…Patients and carers can benefit from timely access to respite and palliative services and these options should be discussed early in the disease. Evidence shows a shared reluctance to discuss end-of-life care options among health professionals, young patients, and their carers 31…”