Clinical Stream
A/Prof Amanda Piper

A/Prof Amanda Piper

Dept of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Camperdown Central Clinical School, Faculty of Medicine, University of Sydney

Senior physiotherapist and Manager, Home Ventilation Service, Royal Prince Alfred Hospital. Amanda has been involved in the use of NIV for people with respiratory muscle weakness for more than 25 years, and is recognised nationally and internationally for her expertise in the area. She is also heavily involved in the education and training of health care staff interested in NIV

Key Note Presentation: “Ventilatory Assistance Overnight: Why NIV in MND is a Good Thing


Respiratory failure is the major cause of hospitalisation and death in people with MND. Sleep disordered breathing produces abnormalities in nocturnal gas exchange and disrupted sleep 1,2 long before daytime dyspnea or hypercapnia occurs, and is considered the earliest sign of respiratory insufficiency. Non-invasive ventilation assists (NIV) in symptom management, maintains quality of life and increases survival 3.  Its use in MND is widely recommended by evidence-based guidelines 4,5 although data to support this position primarily arises from a single trial of subjects with orthopnea and respiratory muscle weakness or symptomatic hypercapnia3. Nevertheless, non-randomised and retrospective studies have consistently demonstrated favourable outcomes with NIV use. Improvements in sleep architecture and correction of sleep disordered breathing with NIV are achievable both acutely 2 and in the longer term 1, irrespective of bulbar function 1. However, in those individuals where NIV fails to correct nocturnal hypoxemia and/or upper airway obstruction survival is significantly worse than in those effectively treated 6-8. Improved sleep quality undoubtedly contributes to the improved quality of life seen 3, with beneficial effects on somnolence and fatigue consistently shown 9. Acutely, NIV reduces resting energy expenditure 10 which may contribute to a slowing of respiratory decline seen after therapy is introduced 11. Despite NIV providing symptomatic relief and extending meaningful survival, a significant number of patients will decline therapy 12, with psychological and cognitive issues influencing this decision 13. However, there is no evidence that NIV use adversely impacts on carer burden to any significant extent 14. Overall, NIV is a good thing in MND but the decision to undertake this pathway needs to be properly discussed, appropriately timed and carefully monitored to ensure therapy is meeting the needs of the individual.



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  2. Vrijsen B, et al (2015). J Clin Sleep Med 11:559–566
  3. Bourke SC et al (2006). Lancet Neurol 5:140–147
  4. Miller RG et al (2009). Neurology 73:1227–1233
  5. Andersen PM et al (2012). Eur J Neurol 19(3): 360-375.
  6. Georges M et al (2016). J Neurol Neurosurg Psychiatry 87(10): 1045-1050.
  7. Quaranta VN et al Neurodegener Dis. 2017;17(1):14-21.
  8. Gonzalez-Bermejo J et al (2013). Amyotroph Lateral Scler Frontotemporal Degener 14(5-6): 373-379.
  9. Hannan, L. M et al (2014). Respir Med 108(2): 229-243.
  10. Georges M et al (2014). BMC Pulm Med 14(1): 17.
  11. Carratu P et al (2009). Orphanet J Rare Dis 4(1): 10.
  12. Ando H et al (2015). Br J Health Psychol 20(2): 341-359.
  13. Martin NH et al (2014). Amyotroph Lateral Scler Frontotemporal Degener 15(5-6):376-87.
  14. Baxter SK et al (2013). J Palliat Med 2013 Dec;16(12):1602-9.