Quality of life in patients with chronic alveolar hypoventilation.

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.

Home mechanical ventilation in Sweden, with reference to Danish experiences. Swedish Society of Chest Medicine.

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.

Ventilatory response to CO2 re-breathing before and after nocturnal nasal intermittent positive pressure ventilation in patients with chronic alveolar hypoventilation.

Long-term nocturnal nasal intermittent positive pressure ventilation (NIPPV) has beneficial effects on daytime PaCO2 in patients with chronic alveolar hypoventilation. Our aim was to investigate if these beneficial effects are related to improved respiratory drive as measured by ventilatory response to CO2. In 17 hypoventilated patients (mean age 62 years) we obtained daytime arterial blood gases, nocturnal transcutaneous oxygen saturation, nocturnal transcutaneous PaCO2 ventilatory response to CO2 re-breathing, spirometry and indices of respiratory muscle strength before and after 9 months of NIPPV. Patients served as their own controls. After 9 months of NIPPV day-time PaCO2 decreased from 7.1 kPa to 6.3 kPa, (P<0.001) and PaO2 increased from 8.1 kPa to 9.3 kPa, (P<0.01). The changes in morning and daytime PaCO2 and in nocturnal transcutaneous oxygen saturation were significantly correlated to the changes in several variables derived from the ventilatory response to CO2 re-breathing. In patients with substantial improvement in daytime PaCO2 we found significant improvements in ventilatory response to CO2 re-breathing. The present study confirms the beneficial effect of long-term NIPPV on daytime arterial blood gases. The results are consistent with the hypothesis that the improvement of daytime PaCO2 is related to improved respiratory drive observed after NIPPV.

Treatment of sleep apnoea with continuous positive airway pressure in children with craniofacial malformations.

We conducted sleep studies in a consecutive series of 21 children with craniofacial malformations and suspected obstructive sleep apnoea. Thirteen had sleep apnoea, and one patient with a tracheostomy could not be tested. Twelve children were admitted for initiation of treatment with nasal continuous positive airway pressure (nCPAP). Ten patients accepted nCPAP, two did not. Seven children use home treatment successfully. Obstructive sleep apnoea in most children is completely eliminated with nCPAP. The treatment is safe, effective, and leads to a reduction in the number of operations done for children with craniofacial malformations. Aesthetic operations can be delayed until facial growth is complete.

[Ventilator treatment at home--experiences with old and new methods]. / Ventilatorbehandling i hemmet--erfarenheter av gamla och nya metoder.

Nocturnal ventilation in respiratory insufficiency due to neuromuscular disease and/or thoracic deformity leads to improvement in the quality of life and daytime arterial blood gases, and also in survival. Several methods for nocturnal ventilation are now available. In this paper we report our experiences of treatment with nocturnal ventilation with various methods in 26 patients in Gothenburg. We conclude that respiratory insufficiency due to neuromuscular disease and/or primary treatment for thoracic deformity should be nocturnal positive pressure ventilation via a nasal mask. If this treatment fails positive pressure ventilation should be administered via special mouth pieces or via tracheostoma.