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Introduction
Motor neuron disease (MND) is a progressive neurodegenerative disorder that usually leads to death within 2-4 years. Ventilatory failure is the most common cause of death, caused in varying degrees by respiratory muscle weakness, unsafe swallowing and poor cough. 1 Non-invasive positive pressure ventilation (NIV) can alleviate symptoms, improve quality of life 2 and prolong survival in MND patients, 2 3 particularly in those without severe bulbar dysfunction. However, the initial symptoms of respiratory failure may be subtle and difficult to detect. 1 Some patients experience a rapid and unexpected decline in respiratory function, frequently in the context of respiratory tract infection, and may be invasively ventilated, even before a diagnosis of MND is established. 4 5
Case series have reported that weaning from invasive ventilation can be difficult, independence from invasive ventilation is rarely achieved, and prognosis is poor. 4 5 Therefore, an emphasis has been placed on the palliation of symptoms. 5 In contrast, the active management of these patients including attempted weaning from invasive ventilation and treatment of associated complications, such as intercurrent infection with physiotherapy, antibiotics and bronchial toilet using bronchoscopy has not been reported.
In this article, the outcomes of 30 patients with MND, referred to a specialist weaning service, who were intubated and ventilated for acute respiratory failure are presented. The aim was to examine whether differences in outcomes justify elective NIV being considered best practice 2 while intubated patients are considered for a more palliative approach due to their reported prognosis. 5
Methods
A retrospective case note review was performed on all patients with a diagnosis of MND referred to a specialist weaning centre (Respiratory Support and Sleep Centre (RSSC), Papworth Hospital) between 1 January 1992 and 31 December 2007. The diagnosis of MND was confirmed by a consultant neurologist and appropriate investigations including electrophysiology. The outcomes of patients invasively ventilated and referred for weaning were compared with those of patients referred for consideration of elective NIV during the same time period. In addition, patients with a known diagnosis of MND prior to intubation were compared with those without a diagnosis at intubation.
In those invasively ventilated, the demographics, duration of symptoms, duration of tracheostomy, length of stay at referring Intensive Care Unit (ICU) and RSSC, ventilation...