Introduction: Motor neurone disease (MND) is characterized by rapidly progressive motor neurone degeneration which leads to muscle wasting. Mortality and morbidity are due to respiratory muscle failure which may be offset by ventilation. The aim of this observational study was to quantify the number and characteristics of patients living with MND choosing tracheostomy ventilation (TV) in the UK. Methods: Long-term ventilation services in the UK were invited to undertake a retrospective 5-year audit of MND patients under their care between April 2013 and March 2018 who had TV. Patient characteristics, the time spent on ventilation, hospital length of stay, discharge destination, and survival data were collected. Results: Sixty-eight MND patients were initiated on TV over the 5-year period. Eighty-one percent of patients received TV in an emergency setting with more than a third of these undiagnosed at presentation. Patients choosing elective TV were more likely to be male (85%) have a bulbar presentation (54%) and an increased survival of 10 months over the observation period. The mean length of hospital stay post TV was 136 days. Two-thirds of patients were discharged to their own home. Conclusion: Very few patients living with MND in the UK are currently receiving TV. In those who choose TV, there may be a survival advantage to planning an elective procedure. Despite the long inpatient stay and high care costs involved a majority of patients survived and were discharged to their own home.

Tracheostomy ventilation (TV) can increase survival time for people living with motor neurone disease (MND); however, the use of TV varies between countries. Concerns regarding anticipated quality of life (QoL) are among the reasons given by healthcare professionals for not recommending this intervention, yet little is known about QoL in this context. This scoping review was conducted to examine the evidence on QoL for those with MND who use TV and family members involved in their care. Using the methodological guidance of the Joanna Briggs Institute, 23 papers were identified for inclusion, and findings were inductively analysed to identify key themes. We found that people living with MND tend to rate QoL post TV more positively than anticipated by healthcare professionals or family members. QoL was found to be related to positive relationships and activities the person could maintain. Feeling able to make a choice and an adequate level of financial resources were also important factors. Family members tended to experience lower QoL, associated with the uncertainty surrounding an emergency procedure and the complexity of subsequently required care. More evidence on QoL from the perspectives of people with MND who use TV is needed to support decision making and inform guidance.

Objectives: This qualitative study aimed to increase understanding of how healthcare practitioners (HCPs) perceive quality of life for people with ALS who use tracheostomy ventilation (TV) and the extent to which such views inform discussions regarding future treatment and care. Methods: A thematic analysis was applied to data derived from online semi-structured interviews with a professionally diverse group of 24 HCPs with experience of supporting people with ALS to use TV. Results: Four main themes relating to TV use emerged: i) Positive benefits; ii) Risks and challenges; iii) Factors influencing HCP perspectives; iv) Concepts informing HCP discussions. HCPs acknowledged that TV has the potential to extend life but were concerned with prolonging a serious decline in physical and cognitive functioning. HCPs tried to identify the 'tipping point' between quantity and quality of life for the individual and their family, taking into account the likelihood of a higher burden of care. HCPs drew on prior experience to assess anticipated quality of life, yet most HCPs in the UK have limited experience of TV for this group. HCPs also drew on principles of person-centered care, patient autonomy and value for money to guide their approach to discussing TV. Conclusions: HCP experience of positive outcomes of TV can foster a more proactive approach to initiating conversations about its potential use. Sharing best practice and improving guidance for HCPs may support early and on-going future care planning and enable people with ALS to make choices which are informed and in their best interests.

To examine the experiences of family members caring for people with motor neurone disease (MND) who use tracheostomy ventilation. Drawing on a constructivist interpretivist approach, qualitative interviews with family members from England, Scotland, and Northern Ireland were conducted. Data were thematically analysed to interpret meaning and identify key themes. Sixteen family members took part. Four themes are presented: (1) Decision-making and implementation: The decision about undergoing tracheostomy was driven by the person with MND. Tracheostomy ventilation was often initiated in an emergency, leaving families unprepared and distressed. (2) Impact on quality of life for family members: Responsibilities intensified once tracheostomy ventilation was in place, leading to a gradual erosion of personal autonomy for family caregivers. (3) Redefining family place and space: The continuous presence of paid carers and the medicalisation of the home impacted family dynamics. (4) Support for family members: Family members took on many roles with little support yet found meaning in the extended life of the person with MND. Enhanced psychological, social, and practical support is urgently needed for families caring for someone with tracheostomy ventilation for MND. Greater awareness of its long-term impact, realistic home assessments, and structured support networks are essential.

Introduction: The use of non-invasive ventilation (NIV) in patients with Motor Neurone Disease (MND) is recommended by the National Institute for Clinical Excellence (NICE). Some UK centres also offer tracheostomy ventilation (TV). Previous studies estimate 1% of MND patients having TV in the UK. Aim: To evaluate the use of TV in patients with MND in the UK. Method: UK ventilation centres were approached to undertake a retrospective audit of the use of TV in MND between 1st April 2013 and 31st March 2018. Data were obtained by retrospective case-note review. Results: 69 patients had been set up on TV for MND, 81% (n=56) were commenced as a result of an emergency admission due to respiratory failure. 36% (n=20) were not previously diagnosed with MND. Not all patients were managed with TV into the long term, a wean was attempted in 54% (n=37) of emergencies; 8 were then managed with NIV until death; mean survival post extubation in this group was 28.8 months. 13 patients received elective tracheostomy. Elective tracheostomy placement was more likely to occur due to bulbar dysfunction or upper airway difficulties compared to those having emergency procedures. These patients were younger (53.8v61.3 years) with a male predominance (85%v15%). They had shorter mean length of stay (40v136 days) and a tendency to increased survival (34v24 months). Conclusion: TV in the UK to manage ventilatory failure in patients living with MND is limited, with less than 1% of all such patients receiving it. Elective tracheostomy for TV is incredibly rare with those choosing to opt for this procedure having a tendency towards better outcomes with a shorter length of hospital stay and increased survival. This may represent a self-selecting group.

Introduction: Some UK centres offer tracheostomy ventilation (TV) to patients with MND. There is no UK data on survival in these patients. Aim: There have been no formal studies to evaluate survival in patients with MND in the UK who have TV. It is hypothesised that in patients with MND, TV will lead to improved survival as these patients usually die from respiratory compromise. Method: UK ventilation centres were approached to retrospectively review MND patients who had started TV between 1st April 2013 and 31st March 2018. Results: 69 MND patients commenced TV in 5 years. 81% (n=56) was commenced following emergency admission due to respiratory failure. 8 patients were weaned and managed with NIV until death, mean survival in those weaned was 28.8 months. As of 31st March 2018, 25 patients had died, 12 were described as sudden. 1 year survival was 76% for all patients who received TV. For those who had non-invasive ventilation (NIV) and then TV, 95% were alive 1 year after initiation of NIV. 13 patients received elective tracheostomy, these had a tendency to improved survival compared to those receiving emergency tracheostomy (mean 34v24 months). Conclusion: Use of TV in MND in the UK is associated with an excellent 1 year survival; elective patients demonstrate improved survival over those admitted acutely. In addition, weaning from TV onto NIV is possible and is associated with comparable 1 year survival to those who continue to have TV.