Measurements of health-related quality of life (HRQL) have not been reported in patients with chronic alveolar hypoventilation (CAH) before starting home mechanical ventilation. The purpose of this study was to investigate quality of life in a population of such patients.Forty-four consecutive patients with CAH due to previous polio, scoliosis, healed pulmonary tuberculosis or neuromuscular disease answered a battery of condition specific and generic (Sickness Impact Profile, Hospital Anxiety and Depression scale, Mood Adjective Check List) self-report questionnaires. Spirometry, arterial blood gases and overnight oxygen saturation were measured.Patients with untreated CAH had significantly impaired HRQL compared to historical data from a healthy reference population. Sleep-related problems were frequent. Age, underlying disease, and standard bicarbonate correlated significantly with HRQL measures, albeit with modest levels of explained variance (8-37%).Patients with chronic alveolar hypoventilation due to neuromuscular or restrictive chest wall disorders had severely impaired health-related quality of life. Age, the underlying disease and severity of hypoventilation are each related to the health-related quality of life decrements. Health-related quality of life measurements add important information to traditional clinical observations.

The Swedish Society of Chest Medicine has started a national register of patients on home mechanical ventilation, to establish reliable national prevalence data and to accurately document patient and treatment characteristics to enable a scientific evaluation of this treatment. In this first collection of retrospective register data, covering patients on home mechanical ventilation at the register start on 1 January 1996, we found 541 patients, corresponding to 6.1/100000 inhabitants, using home mechanical ventilation. Non-invasive ventilation, night-time ventilation and volume controlled ventilation dominated. We found four diagnosis categories of approximately equal size, namely post-polio, chest wall deformities, neuromuscular diseases and 'other diseases'. The age distribution was bimodal, with one small peak in the 20-29 year group and a large peak in the 60-69 year group. A survey of Danish patients on home mechanical ventilation showed that they were considerably younger and that almost half of them suffered from neuromuscular diseases. Further work will be done to follow the situation in Sweden and in Denmark to elucidate the obvious differences in the selection of patients for home mechanical ventilation.

NPPV improves HRQL, particularly in condition-specific areas. Improvements are related to effectiveness in ventilation. Side effects are common, but compliance is good and patient satisfaction is high.

Seventy-three percent of patients treated for CAH with NPPV survived more than 5 years. Diagnosis and self-rated physical functioning at pre-treatment were related to survival, as were major improvements in physical functioning and global QL during NPPV.