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.