Home ventilation in progressive disorders, quadriplegia and palliative non-invasive ventilation A K Simonds
Amyotrophic lateral sclerosis/motor neurone disease 193
Spina biﬁda 204
Respiratory failure, often exacerbated by a chest infection, is the terminal event in many patients with progressive neuromuscular disease. Motor neurone disease (MND) (or amyotrophic lateral sclerosis, ALS), the most prevalent of these disorders, and other progressive conditions such as Duchenne muscular dystrophy and spinal muscular atrophy are considered here. It is clear that assisted ventilation may extend the life of patients and alter the natural history of these disorders, however the effects of the intervention on quality of life have not been systematically studied and data are mainly derived from uncontrolled series. Conditions such as quadriplegia due to cervical cord injury and spina biﬁda comprise another area where the use of non-invasive ventilatory (NIV) support is growing but has not yet been well evaluated. Some information on prognostic factors and the outcome of NIV is, however, available. The possible indications for NIV and the practical challenges that are associated will be considered.