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

BACKGROUND: Motor Neurone Disease (MND) leads to muscle weakening, affecting movement, speech, and breathing. Home mechanical ventilation, particularly non-invasive ventilation (NIV), is used to alleviate symptoms and support breathing in people living with MND. While home mechanical ventilation can alleviate symptoms and improve survival, it does not slow the progression of MND. This study addresses gaps in understanding end-of-life decision-making in those dependent on home mechanical ventilation, considering the perspectives of patients, family members, and bereaved families. METHODS: A UK-wide qualitative study using flexible interviews to explore the experiences of people living with MND (n = 16), their family members (n = 10), and bereaved family members (n = 36) about the use of home mechanical ventilation at the end of life. RESULTS: Some participants expressed a reluctance to discuss end-of-life decisions, often framed as a desire to "live for the day" due to the considerable uncertainty faced by those with MND. Participants who avoided end-of-life discussions often engaged in 'selective decision-making' related to personal planning, involving practical and emotional preparations. Many faced challenges in hypothesising about future decisions given the unpredictability of the disease, opting to make 'timely decisions' as and when needed. For those who became dependent on ventilation and did not want to discuss end of life, decisions were often 'defaulted' to others, especially once capacity was lost. 'Proactive decisions', including advance care planning and withdrawal of treatment, were found to empower some patients, providing a sense of control over the timing of their death. A significant proportion lacked a clear understanding of the dying process and available options. CONCLUSIONS: The study highlights the complexity and evolution of decision-making, often influenced by the dynamic and uncertain nature of MND. The study emphasises the need for a nuanced understanding of decision-making in the context of MND.

Prolonged Mechanical Ventilation, Weaning, and the Role of Tracheostomy.

Depending on the definitional criteria used, approximately 5% to 10% of critical adults will require prolonged mechanical ventilation with longer-term outcomes that are worse than those ventilated for a shorter duration. Outcomes are affected by patient characteristics before critical illness and its severity but also by organizational characteristics and care models. Definitive trials of interventions to inform care activities, such as ventilator weaning, upper airway management, rehabilitation, and nutrition specific to the prolonged mechanical ventilation patient population, are lacking. A structured and individualized approach developed by the multiprofessional team in discussion with the patient and their family is warranted.

Development of respiratory care guidelines for Duchenne muscular dystrophy in the UK: key recommendations for clinical practice.

Significant inconsistencies in respiratory care provision for Duchenne muscular dystrophy (DMD) are reported across different specialist neuromuscular centres in the UK. The absence of robust clinical evidence and expert consensus is a barrier to the implementation of care recommendations in public healthcare systems as is the need to increase awareness of key aspects of care for those living with DMD. Here, we provide evidenced-based and/or consensus-based best practice for the respiratory care of children and adults living with DMD in the UK, both as part of routine care and in an emergency. METHODOLOGY: Initiated by an expert working group of UK-based respiratory physicians (including British Thoracic Society (BTS) representatives), neuromuscular clinicians, physiotherapist and patient representatives, draft guidelines were created based on published evidence, current practice and expert opinion. After wider consultation with UK respiratory teams and neuromuscular services, consensus was achieved on these best practice recommendations for respiratory care in DMD. RESULT: The resulting recommendations are presented in the form of a flow chart for assessment and monitoring, with additional guidance and a separate chart setting out key considerations for emergency management. The recommendations have been endorsed by the BTS. CONCLUSIONS: These guidelines provide practical, reasoned recommendations for all those managing day-to-day and acute respiratory care in children and adults with DMD. The hope is that this will support patients and healthcare professionals in accessing high standards of care across the UK.

International Survey of High-Flow Nasal Therapy Use for Respiratory Failure in Adult Patients.

(1) Background: High-flow nasal therapy (HFNT) has shown several benefits in addressing respiratory failure. However, the quality of evidence and the guidance for safe practice are lacking. This survey aimed to understand HFNT practice and the needs of the clinical community to support safe practice. (2) Method: A survey questionnaire was developed and distributed to relevant healthcare professionals through national networks in the UK, USA and Canada; responses were collected between October 2020 and April 2021. (3) Results: In the UK and Canada, HFNT was used in 95% of hospitals, with the highest use being in the emergency department. HNFT was widely used outside of a critical care setting. HFNT was mostly used to treat acute type 1 respiratory failure (98%), followed by acute type 2 respiratory failure and chronic respiratory failure. Guideline development was felt to be important (96%) and urgent (81%). Auditing of practice was lacking in 71% of hospitals. In the USA, HFNT was broadly similar to UK and Canadian practice. (4) Conclusions: The survey results reveal several key points: (a) HFNT is used in clinical conditions with limited evidence; (b) there is a lack of auditing; (c) it is used in wards that may not have the appropriate skill mix; and (d) there is a lack of guidance for HFNT use.

The variation of FiO2 with circuit type and peak inspiratory flow rate during non-invasive respiratory support using domiciliary ventilators and its significance during the COVID-19 pandemic.

Background: The COVID-19 pandemic has resulted in increased admissions with respiratory failure and there have been reports of oxygen failure and shortages of machines to deliver ventilation and Continuous Positive Airway Pressure (CPAP). Domiciliary ventilators which entrain room air have been widely used during the pandemic. Poor outcomes reported with non-invasive respiratory support using ventilators which lack an oxygen blender could be related to an unreliable Fraction of inspired O2 (FiO2). Additionally, with concerns about oxygen failure, the variety of ventilator circuits used as well as differing peak inspiratory flow rates (PIFR) could impact on the FiO2 delivered during therapy with domiciliary ventilators. Methods: In a series of bench tests, we tested the effect of choice of circuit and different PIFR on the FiO2 achieved during simulation of ventilation and CPAP therapy using domiciliary ventilators. Results: FiO2 was highly dependent upon the type of circuit used with circuits with an active exhalation valve achieving similar FiO2 at lower oxygen flow rates than circuits using an exhalation port. During CPAP therapy, high PIFR resulted in significantly lower FiO2 than low PIFR. Conclusions: This study has implications for oxygen usage as well as delivery of non-invasive respiratory support during therapy with domiciliary ventilators when these are used during the second wave of COVID-19.

An observational study of Pseudomonas aeruginosa in adult long-term ventilation.

Introduction: Pseudomonas aeruginosa increases morbidity and mortality in respiratory disease. To date the long-term ventilation population does not have clear guidelines regarding its management. Method: We undertook a retrospective observational study in a regional long-term ventilation population (837 patients). We defined the primary outcome as P. aeruginosa isolation. In addition positive cultures for copathogens (Serratia, Proteus species, Stenotrophomonas, Burkholderia cepacia complex and nontuberculous mycobacteria) were recorded. Logistic regression and odds ratios were calculated. Results: 17.6% of the cohort isolated P. aeruginosa, and this pathogen was cultured more frequently in patients with a tracheostomy (logistic regression coefficient 2.90, p≤0.0001) and cystic fibrosis/bronchiectasis (logistic regression coefficient 2.48, p≤0.0001). 6.3% of patients were ventilated via tracheostomy. In the P. aeruginosa positive cohort 46.9% of patients were treated with a long-term macrolide, 36.7% received a nebulised antibiotic and 21.1% received both. Tracheostomised P. aeruginosa positive patients received a nebulised antibiotic more frequently (OR 2.63, 95% CI 1.23-5.64, p=0.013). Copathogens were isolated in 33.3% of the P. aeruginosa cohort. In this cohort patients with a tracheostomy grew a copathogen more frequently than those without (OR 2.75, 95% CI 1.28-5.90). Conclusions: P. aeruginosa isolation is common within the adult long-term ventilation population and is significantly associated with tracheostomy, cystic fibrosis and bronchiectasis. Further research and international guidelines are needed to establish the prognostic impact of P. aeruginosa and to guide on antimicrobial management. The increased risk of P. aeruginosa should be considered when contemplating long-term ventilation via tracheostomy.

Developing an intrasalivary gland botox service for patients receiving long-term non-invasive ventilation at home: a single-centre experience.

INTRODUCTION: Sialorrhoea is a debilitating symptom in neurological disease and there is a growing literature for the use of intrasalivary gland Botulinum Toxin (botox) injections in its management. However, provision of intrasalivary gland botox remains inconsistent and sialorrhoea is often poorly controlled in motor neuron disease (MND).Sialorrhoea in association with bulbar dysfunction can cause intolerance of non-invasive ventilation (NIV) and respiratory infection, so its treatment is critical within a home ventilation service (HVS).This treatment can also be used to enable tracheostomy cuff deflation to facilitate weaning from ventilation. We report on the outcomes of intrasalivary gland botox in our HVS. METHODS: In 2015, we set up an intrasalivary gland botox service for patients under our HVS. Under ultrasound guidance, we injected submandibular gland(SMG), parotid gland (PG) or both. RESULTS: 109 intrasalivary gland botox procedures were performed in 72 patients. Diagnostic groups included MND 32Cerebral Palsy 8 and Weaning 14. Glands injected were, SMG (6%), PG (47%) and both (47%). The majority (84%) received the Dysport preparation with mean dose 273 units. 94% were ultrasound guided. 81% of injections resulted in a positive treatment effect, with 47% patients requesting repeat injections. Complications were angioedema (0.9%) and worsening dysphagia (3.7% following SMG injection). Mean survival following treatment was 40 months with 53% patients still alive. CONCLUSIONS: Intrasalivary gland botox appears effective across a range of neurological conditions requiring long-term NIV with few complications. Dysphagia may be an important complication of SMG injection. A randomised controlled trial may help establish the evidence base.

Tracheostomy ventilation in motor neurone disease: a snapshot of UK practice.

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.

A retrospective cohort study of idiopathic diaphragmatic palsy: a diagnostic triad, natural history and prognosis.

BACKGROUND: Isolated diaphragmatic palsy in the absence of progressive neuromuscular disease is uncommon. It poses diagnostic challenges and limited data are available regarding prognosis. We present retrospective cohort data from two large teaching hospitals in the United Kingdom. METHOD: 60 patients who were assessed either as inpatients or outpatients were included in this study. Patients with progressive neuromuscular disease were excluded. Clinical presentation, tests of respiratory muscle function (sitting/supine vital capacity, maximal expiratory pressure (MEP), maximal inspiratory pressure (MIP) and sniff nasal inspiratory pressure (SNIP)) and outcomes were recorded. RESULTS: For patients with diaphragmatic palsy, mean±sd seated and supine vital capacity pre-noninvasive ventilation (NIV) were reduced at 1.7±1.2 L and 1.1±0.9 L, respectively, with a mean±sd postural fall in vital capacity of 42±0.16%. The mean MEP/MIP and MEP/SNIP ratios for diaphragmatic palsy were 3 and 3.5, respectively. After a year of treatment with NIV, mean±sd upright and supine vital capacity had increased to 2.1±0.9 L and 1.8±1 L, respectively, and the mean±sd fall in vital capacity from sitting to supine reduced to 29±0.17%. MEP/MIP and MEP/SNIP ratios reduced to 2.6 and 2.9, respectively, from the pre-NIV values. The values of postural fall in vital capacity correlated (p<0.05) with MEP/MIP and MEP/SNIP ratio (r2=0.86 and r2=0.7, respectively). CONCLUSION: Tests of respiratory muscle strength are valuable in the diagnostic workup of patients with unexplained dyspnoea. A triad of 1) orthopnoea, with 2) normal lung imaging and 3) MEP/MIP and/or MEP/SNIP ratio ≥2.7 points towards isolated diaphragmatic palsy. This needs to be confirmed by prospective studies.

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.

What's in a name: How do junior doctors address their consultants?

INTRODUCTION: Evidence has suggested that when senior doctors are less approachable, junior staff are less likely to raise safety issues. There are limited existing data on whether the name by which junior doctors address their seniors reflects approachability and if this varies between grade and specialties. METHODS: An online survey was conducted in a large teaching hospital. Respondents were asked about their use of first names when addressing consultants and whether they felt this reflected their perceptions of the consultants' approachability. RESULTS: Four-hundred and twenty-three responses were received from a cohort of approximately 800 junior doctors. Of these, 410 were included in this analysis. Respondents came from 57 different subspecialties and all years of training. Overall, junior doctors addressed 43% of consultants by their first name; 71% of junior doctors perceived these consultants to be more approachable. There were significant differences in the results between grades and specialty of junior doctor. CONCLUSION: Throughout all specialties, the majority of junior doctors consider the consultants that they address informally to be more approachable.

High Variability in Sepsis Guidelines in UK: Why Does It Matter?

It is recommended that developers of Point Of Care Tests (POCTs) assess the care pathway of the patient population of interest in order to understand if the POCT fits within the pathway and has the potential to improve it. If the variation of the pathway across potential hospitals is large, then it is likely that the evaluation of effectiveness is harder and the route towards large-scale takes adoption longer. Evaluating care pathways can be a time-consuming activity when conducted through clinical audits or interviews with healthcare professionals. We have developed a more rapid methodology which extrapolates the care pathway from local hospital guidelines and assesses their variation. Sepsis kills 46,000 people per year in the UK with societal costs of up to £10 billion. Therefore, there is a clinical need for an optimized pathway. By applying our method in this field, we were able to assess the variation in current hospital guidelines for sepsis and infer the potential impact this may have on the evidence development on innovations in this applications. We obtained 15 local sepsis guidelines. Two independent reviewers extracted: use of the national early warning score (NEWS), signs and risk factors informing the decision to prescribe antibiotics, and the number of decisional steps up to this point. Considerable variation was observed in all the variables, which is likely to have an impact on future clinical and economic evaluations and adoption of POCT for the identification of patients with sepsis.

Requested withdrawal of mechanical ventilation in six patients with motor neuron disease.

OBJECTIVES: Mechanical ventilation (MV) has been shown to improve survival and quality of life in motor neuron disease (MND). However, during the progression of MND, there may come a point when MV is no longer felt appropriate. Association of Palliative Medicine Guidelines have been recently published to help clinicians withdraw MV at the request of patients with MND in a safe and compassionate manner to ensure that symptoms of distress and dyspnoea are minimised. METHODS: In this report, we discuss the palliative and ventilatory management of six ventilator-dependent patients with MND who had requested the withdrawal of MV as part of their end-of-life care. RESULTS: We have withdrawn MV from six patients with MND at their request and our practice has been influenced by the Association of Palliative Medicine Guidelines. CONCLUSION: Withdrawal of MV in MND at a patient's request is challenging but is also a fundamental responsibility of healthcare teams. We discuss the lessons we have learnt which will influence our practice and help other teams in the future.

Management of lercanidipine overdose with hyperinsulinaemic euglycaemia therapy: case report.

This case report describes the first reported overdose of the dihydropyridine calcium channel blocker (CCB) lercanidipine. A 49 yr old male presented to the Emergency Department 3 hrs after the ingestion of 560 mg of lercanidipine. In the department he had a witnessed seizure within 15 minutes of arrival attributed to the overdose. Following immediate recovery of consciousness after the seizure, he had refractory hypotension and bradycardia which failed to respond to fluid resuscitation, glucagon therapy, and intravenous calcium. He went on to require vasopressor support with noradrenaline and was treated with high dose insulin therapy which was successful in achieving cardiovascular stability. Vasopressor therapy was no longer required within one half life of lercanidipine, and the total stay on intensive care was one day before transfer to a ward.Calcium channel blocker overdose is an uncommon but life-threatening overdose. Treatment for severe toxicity is similar to b-blocker overdose. Hypotension is treated with intravenous fluid therapy, intravenous calcium and possibly glucagon with vasopressor or inotropic support as required. Atropine is used to attempt reversal of bradycardia. High doses of intravenous insulin with intravenous dextrose as required (hyperinsulinaemic euglycaemia or HIET), has also been successfully reported. Experimental animal data suggests that HIET is of benefit and potentially superior to fluid therapy, calcium, glucagon and potentially vasopressor therapy. HIET effectively and sustainably reverses hypotension, bradycardia and improves myocardial contractility and metabolism. Current advice in calcium channel blocker overdose is to begin therapy early in toxicity, starting with a 1.0 IU/kg insulin bolus followed by an infusion of 0.5 IU/kg/hr of insulin and dextrose as required titrated to clinical response.