Session 6: Invasive, tracheostomy ventilation in MND

MND Statistics Death is almost always due to respiratory failure as a consequence of respiratory muscle weakness and/or repeated chest infections. ‘respiratory’ is written 97 times in the NICE 2016 Summary Guidance (383 uses in the full guidance) Burkhardt, C. et al 2017. Cause of Death in MND

What we already know about NIV in MND NIV improves survival with maintenance of, and improvement in, quality of life, significantly more than neuroprotective therapy (in those without severe bulbar dysfunction). In patients with severe bulbar impairment, NIV improves sleep-related symptoms, but is unlikely to offer a large survival advantage. Increasing non-RCT evidence and expert opinion that cough augmentation helps in MND. non-invasive ventilation is written 76 times and cough 16 times in the full NICE MND Guidance 2016.

Tracheostomy in MND Little in the literature about long term ventilation in MND via tracheostomy. What little there is shows a mortality advantage, improved QoL (for patient) and increased risk of sudden death. Not surprising, the 2016 NICE Guidance, doesn't give any advice on when, how or where to use it in the management of MND. In fact tracheostomy is mentioned zero times in the guidance.

Tracheostomy in MND Despite lack of evidence, long term tracheostomy ventilation is used in patients with MND. Inability to manage 24 hour ventilation with non-invasive interfaces. Could be argued that MPV and using a variety of interface could rule this out.

Why perform a tracheostomy in MND Severe bulbar involvement with inability to maintain adequate ventilation despite NIV and cough augmentation, particularly those with upper motor neuron/CNS bulbar hypertonicity. Bach et al 2004 suggests patients with ALS need ‘trachs’ when spO2 falls below 95% permanently despite optimal NIV and Cough Assist. 80% of these patients are trached or dead within 2 months.

Case Study 1 – Mr X Diagnosed with MND in 2010 aged 26 Commenced nocturnal NIV 2012 Trache 1st discussed in 2013 Vent dependent 2016 Emergency tracheostomy March 2016 Discharged home end of April 2016 Went to Wimbledon to watch the tennis that summer Back to work as a teacher later that year

Case Study 2 – Mrs Y Diagnosed with MND and early stage dementia in 2014 Emergency tracheostomy during diagnosis due to respiratory failure and increased oral secretion Weaned to a mini-trach and no ventilation, discharged home for follow up in clinic Admitted into ITU a few weeks later and trache reinserted Ventilator dependent and complex discharge process commenced Discharged to intermediate care Nov 2014 Discharged home May 2015 No ADRT or EHCP has been done

Evidence to support tracheostomy in MND Good 1-year survival rate in MND, respiratory problems being the main cause of hospitalisation but not of death. Sudden death was the main cause of death and only one patient died from respiratory causes.

Summary of pilot national audit (2017) around 9% of patients in specialist centres have TV for MND, a lot more than anecdotally reported. more men than women have TV 26 male v 12 female possibly younger patients, mean age 56 years: Range 26-78 appears to be safe and extends length of life sudden death is high around 45% mean inpatient stay is shorter than expected, particularly in electives most patients go home with skilled carer package more comprehensive audit to follow soon