Qualify of life and palliation predict survival in patients with chronic alveolar hypoventilation and nocturnal ventilatory support.

OBJECTIVES: Non-invasive positive pressure ventilation (NPPV) improves health-related quality of life (HRQL) in patients with chronic alveolar hypoventilation (CAH). We studied the prognostic impact of HRQL on survival in relation to clinical factors. PATIENTS: Forty-four patients with CAH due to post-polio (12), scoliosis (11), post-tb (17) or other diagnoses (4) who received nocturnal NPPV were prospectively studied during 6-10 years. MEASUREMENTS: Blood gases and HRQL were analysed at baseline and after 9 months and after 8 years. HRQL was evaluated with measures of functioning (SIP), emotional well-being (HADS and MACL), and global QL. RESULTS: Blood gases and HRQL measures improved during NPPV. The overall 5-year survival rate was 73%. In multivariate survival analysis, a diagnosis of post-polio and low baseline SIP physical index scores, indicating low levels of physical dysfunction, predicted longer survival (P = 0.02, respectively). Similarly, palliation of physical dysfunction and preserved or improved global QL by 9 months were associated with longer overall survival (P = 0.009 and P = 0.001, respectively; multivariate Cox regression). CONCLUSION: 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.

Impact of home mechanical ventilation on health-related quality of life in patients with chronic alveolar hypoventilation: a prospective study.

BACKGROUND: Nocturnal ventilatory support by nasal positive pressure ventilation (NPPV) is an established treatment method in patients with chronic alveolar hypoventilation (CAH). The knowledge about its long-term effects on health-related quality of life (HRQL) is limited. METHODS: In a prospective, longitudinal, single-strand study, patients with CAH caused by non-COPD conditions, consecutively recruited among referral patients in three Swedish university hospital pulmonary departments, were examined at baseline and after 9 months (n = 35) and 8 years (n = 11) on NPPV treatment. Both volume pre-set and pressure pre-set ventilators were used. Patients completed a battery of condition-specific and generic HRQL questionnaires at baseline and follow-up. Spirometry and blood gases were measured. Compliance with treatment, side effects and patient satisfaction were evaluated. RESULTS: After 9 months of NPPV, improvements were seen primarily not only in sleep-related domains, but also in emotional behaviour, ambulation and sleep/rest functioning as measured with the Sickness Impact Profile (SIP). Improvements in sleep-related symptoms were related to effectiveness in ventilation, evaluated by morning PaCO(2), and remained by 8 years. Mental well-being was stable over time, while emotional distress improved by 8 years. Satisfaction with treatment was high in spite of frequent side effects. CONCLUSION: 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.