chapter  14
18 Pages

Home non-invasive ventilation in restrictive disorders and stable neuromuscular disease A K Simonds

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.