The Use of Mechanical Ventilation Support at the End of Life in Motor Neurone Disease/Amyotrophic Lateral Sclerosis: A Scoping Review

Abstract

There has been an increase in the use of mechanical ventilation (MV) in motor neurone disease (MND) to alleviate symptoms related to hypoventilation. Little is known about its use at the end of life, and the withdrawal of MV is a challenging topic. A scoping review was conducted to map the existing evidence of ventilation use at the end of life in MND. The Joanna Briggs Institute methodological framework was used, and a total of 31 documents were included in the data extraction. Boarder themes around place, planning, cause and comments on death were identified. Our findings show that the focus of the literature has been on the process of the withdrawal of ventilation, creating the subthemes of timing and reason for withdrawal, along with ethical, emotional, and practical issues, medications used and the time until death. There is a foundation of evidence to guide the process and discuss the ethical and emotional issues of withdrawing ventilation. However, there remains limited evidence from patient and family member perspectives. Importantly, there is almost no evidence exploring dying with ventilation in place, the active or passive decisions around this process, how the interface might impact the dying process, or what families think about this.

End of life decision making when home mechanical ventilation is used to sustain breathing in Motor Neurone Disease: patient and family perspectives

“…Little is known about the extent or nature of end-of-life discussions between patients, families, and healthcare professionals, what informs decisions, or how patients and families are supported through the experience (17). While some patients who are dependent on HMV wish to remain so until their death, a proportion wish to stop their HMV sooner (18-21).…”

“…Patients ultimately make decisions about commencing HMV but these are rarely made in isolation or without consideration for their families (9,10). However, little is known about decision-making regarding HMV use at the end-of-life, once a plwMND is dependent on HMV, or 'fully ventilated' (7,17). This study explored this end-of-life decisionmaking from the perspectives of plwMND, family members, and bereaved family members.…”

End of life decision making when home mechanical ventilation is used to sustain breathing in Motor Neurone Disease: patient and family perspectives

“…Little is known about the extent or nature of end-of-life discussions between patients, families, and healthcare professionals, what informs decisions, or how patients and families are supported through the experience [ 18 ]. While some patients who are dependent on HMV wish to remain so until their death, a proportion wish to stop their HMV sooner [ 19 – 22 ].…”

“…Patients ultimately make decisions about commencing HMV but these are rarely made in isolation or without consideration for their families [ 10 , 11 ]. However, little is known about decision-making regarding HMV use at the end-of-life, once a plwMND is dependent on HMV, or ‘fully ventilated’ [ 8 , 18 ]. This study explored this end-of-life decision-making about HMV use from the perspectives of plwMND, family members, and bereaved family members.…”