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

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.

Palliative care inpatients favour research participation irrespective of prognosis, performance or socioeconomic status: multicentre cohort study.

OBJECTIVES: Palliative care needs to embrace research to guide service development and effective symptom management. Healthcare professionals often feel research is too burdensome for patients who have poor performance status or are near the end of life. Many studies exclude these groups from participating.We aimed to identify whether specialist palliative care inpatients would wish to take part in research and whether preference varies according to study design, demographics, diagnosis, performance status and prognosis. METHODS: 100 inpatients in two National Health Service specialist palliative care units and one independent hospice in the Northeast of England completed a short questionnaire about preferences for involvement in research. RESULTS: 92% of participants stated they were interested in being involved in research. This was mostly unaffected by age, diagnosis, prognosis, performance and socioeconomic status. Three-quarters were within the last 3 months of life. Simple questions or interviews were the preferred methodology, whereas only half of patients would want further investigations or additional medications and fewer still wanted to participate in online activities, lifestyle change or group activities. CONCLUSIONS: Palliative care inpatients welcome the opportunity to be involved in research and should not be excluded on the grounds of advanced disease, poor prognosis and low performance status.

Palliative medicine trainees be should learn about frailty: meta-synthesis and Delphi study to establish curriculum content.

OBJECTIVES: Frailty is common and highly associated with morbidity and mortality, a fact that has been highlighted by COVID-19. Understanding how to provide palliative care for frail individuals is an international priority, despite receiving limited mention in Palliative Medicine curricula or examinations worldwide. This study aimed to synthesise evidence and establish expert consensus on what should be included in a Palliative-Medicine Specialist Training Curriculum for frailty. METHODS: Literature Meta-synthesis conducted by palliative medicine, frailty and education experts produced a draft curriculum with Bologna based Learning-Outcomes. A Delphi study asked experts to rate the importance of Learning-Outcomes for specialist-training completion and propose additional Learning-Outcomes. This process was repeated until 70% consensus was achieved for over 90% of Learning-Outcomes. Experts divided Learning-Outcomes into specific (for inclusion in a frailty subsection) or generic (applicable to other palliative conditions). The Delphi panel was Subject Matter Experts: Palliative-Medicine Consultants (n=14) and Trainees (n=10), representing hospital, community, hospice and care home services and including committee members of key national training organisations. A final reviewing panel of Geriatric Medicine Specialists including experts in research methodology, national training requirements and frailty were selected. RESULTS: The meta-synthesis produced 114 Learning-Outcomes. The Delphi Study and Review by Geriatric Medicine experts resulted in 46 essential and 33 desirable Learning-Outcomes. CONCLUSIONS: This frailty curriculum is applicable internationally and highlights the complex and unique palliative needs of frail patients. Future research is required to inform implementation, educational delivery and service provision.

Ventilator dependence complications in motor neuron disease.

Long-term dependence on non-invasive ventilation (NIV) without time for advance care planning can result in significant complications that may require innovative management strategies. We present the case of a man who was admitted with respiratory failure and required NIV. Despite effective treatment for community acquired pneumonia, attempts to wean NIV failed. While dependent on NIV, a diagnosis of motor neuron disease was made. Time without ventilation was not tolerated and consequently complications of: facial pressure ulceration, nasal septal prolapse, inspissated secretions and failure to feed occurred. This case illustrates the severity of complications that can result from NIV dependence; however, it also details how these can be effectively managed by the hospice multidisciplinary team, with support from experts both within and external to the hospice enabling the acquisition of appropriate skills and knowledge.