ABSTRACT
Negative pressure ventilation in restrictive disorders 176
Negative pressure ventilation versus non-invasive ventilation 177
Paediatric negative pressure ventilation 178
Domiciliary non-invasive ventilation 178
Effects of NIV on physiological outcomes, morbidity and quality of life 179
NIV combined with long-term oxygen therapy 180
Paediatric non-invasive ventilation 180
Mechanisms of action of NIV in restrictive disorders 181
Compliance with non-invasive ventilation 183
Pregnancy and scoliosis/neuromuscular disease 184
NIV during exercise and pulmonary rehabilitation 188
T-IPPV in restrictive disorders 189
Quality of life in NIV versus T-IPPV users 189
References 190
In this chapter the outcome of domiciliary non-invasive ventilation (NIV) in restrictive disorders is discussed. Use of NIV in specific situations such as during pregnancy and pulmonary rehabilitation is also considered. In all these areas case series data (sometimes extensive) are now available, but there have been few randomized controlled studies in patients with chest wall or neuromuscular disease. As patients with ventilatory failure due to restrictive ventilatory disorders were originally treated with negative pressure ventilation and T-IPPV (tracheostomy-intermittent positive pressure ventilation), use of these techniques and NIV will be compared and contrasted. It is important to note that these findings should be interpreted bearing in mind that ventilatory modes have evolved over the last few decades, there has been a gradual change in the indications and patient selection for ventilatory support, and a growing public debate on the ethics of life-saving therapies and the quality of life that results.
