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

Abstract

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. 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. 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. 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.