Long-term home-based invasive ventilation in patients with motor neurone disease / amyotrophic lateral sclerosis (MND/ALS) remains rare in the UK. We describe a case of an MND/ALS patient who was treated with long-term invasive ventilation at home but subsequently requested its withdrawal despite a seemingly stable period of his illness. We also discuss the impact of the delivery of this treatment and its withdrawal on his carers, primary healthcare team, community trust managers and specialist palliative care team.
BorasioGDRogersAVoltzR. Palliative medicine in non-malignant neurological disorders. In: DoyleDHanksGChernyNI (eds) Oxford Textbook of Palliative Medicine, Third Edition. Oxford: Oxford University Press, 2004.
2.
LeighPNRay-ChaudhuriK. Motor neurone disease. J Neurol Neurosurg Psychiatry1994; 57: 886–896.
3.
NIV numbers 2008. The MND Association (personal communication).
4.
NICE. The use of non-invasive ventilation in the management of motor neurone disease. NICE clinical guideline – draft for pre-publication check. NICE, May 2010.
5.
SivakEDCordascoEMGipsonWT. Pulmonary mechanical ventilation at home: a reasonable and less expensive alterative. Respir Care1983; 28: 42–49.
6.
OppenheimerEA. Decision-making in the respiratory care of amyotrophic lateral sclerosis: should home mechanical ventilation be used?Palliat Med1993; 7(suppl 2): 49–64.
7.
Jackson-KokuGOliverDFisherJ. Tracheostomy ventilation in motor neurone disease. Eur J Palliat Care2008; 15: 63–67.
8.
Department of Constitutional Affairs. Mental Capacity Act 2005. Code of Practice. Department of Constitutional Affairs.
9.
OliverD. Ventilation in motor neurone disease: difficult decisions in difficult circumstances. Amyotroph Lateral Scler Other Motor Neuron Disord2004; 5: 6–8.
10.
BrodyHCampbellMLFaber-LangendoenK. Withdrawing intensive life-sustaining treatment – recommendations for compassionate clinical management. New Engl J Med1997; 336: 652–657.
11.
BorasioGDVoltzR. Discontinuation of mechanical ventilation in patients with amyotrophic lateral sclerosis. J Neurol1998; 245: 717–722.
12.
HeffernanCJenkinsonCHolmesT. Management of respiration in MND/ALS patients: An evidence based review. Amyotroph Lateral Scler2006; 7: 5–15.
13.
ChernyNIRadbruchL. The Board of the European Association for Palliative Care. European Association for Palliative Care (EAPC) recommended framework for the use of sedation in palliative care. Palliat Med2009; 23: 581–593.
Kaub-WittemerDSteinbuchelNWasnerM. Quality of life and psychological issues in ventilated patients with amyotrophic lateral sclerosis and their caregivers. J Pain Symptom Manage2003; 4: 890–896.
