Palliation, end of life care and ventilation withdrawal in neuromuscular disorders

Abstract

Background/objectives The role of palliative care in the support of patients with neuromuscular disorders (NMDs) is generally recognised in spite of the scarcity of condition-specific evidence in the literature. Methods We have focussed specifically on palliative and end-of-life care for patients whose neuromuscular disease has an impact on their respiratory function. Reviewing the literature, we have examined where existing palliative care knowledge can be applied to the specific challenges faced by patients with NMDs, identifying where lessons learnt during the management of one condition may need to be judiciously applied to others. Results We highlight lessons for clinical practice centring on six themes: management of complex symptoms; crisis support; relief of caregiver strain; coordination of care; advance care planning; and end of life care. Conclusions The principles of palliative care are well suited to addressing the complex needs of patients with NMDs and should be considered early in the course of illness rather than limited to care at the end of life. Embedding relationships with specialist palliative care services as part of the wider neuromuscular multidisciplinary team can facilitate staff education and ensure timely referral when more complex palliative care problems arise.

Long-term mechanical ventilation: State of the evidence and future directions

“…The series on long-term ventilation is comprised of three narrative reviews, on the topics of respiratory therapies for people with neuromuscular disease, specifically amyotrophic lateral sclerosis (ALS), 1 transitions in care for ventilator-assisted individuals, 2 and palliation and end of life care in neuromuscular disorders. 3 This series highlights current practices and optimal collaborative care plans for ventilator-assisted individuals, current evidence and avenues for research directions in the area of long-term ventilation.…”

“…Managing caregiver burden was also discussed in the context of palliative care, 3 and the increasing burden faced by caregivers when the health of their family member deteriorates. The importance of the healthcare team in supporting caregivers, including respite care, is emphasized as part of end of life care support.…”

“…Sales de Campos et al 1 describe how there has been a shift towards less reliance on pulmonary function testing during the pandemic, in the initiation and monitoring of NIV. Elverson et al 3 described how the pandemic highlighted inequities in access to palliative care, especially for individuals with neuromuscular disorders and malignancies. This may lead to improved access to end of life care in equity seeking groups.…”