Bipap improves survival and rate of pulmonary function decline in patients with ALS

• Published in: Journal of the neurological sciences Vol. 164; no. 1; pp. 82 - 88
• Main Authors: Kleopa, Kleopas A., Sherman, Michael, Neal, Bettie, Romano, Gary J., Heiman-Patterson, Terry
• Format: Journal Article
• Language: English
• Published: Shannon Elsevier B.V 15.03.1999; Elsevier Science
• Subjects: Adult; Aged; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - complications; Amyotrophic Lateral Sclerosis - mortality; Amyotrophic Lateral Sclerosis - physiopathology; Biological and medical sciences; Bipap; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Humans; Intermittent Positive-Pressure Ventilation - methods; Intermittent Positive-Pressure Ventilation - mortality; Lung - physiopathology; Male; Medical sciences; Middle Aged; Neurology; Noninvasive ventilatory support; Respiratory insufficiency; Respiratory Insufficiency - etiology; Respiratory Insufficiency - mortality; Respiratory Insufficiency - physiopathology; Respiratory Insufficiency - therapy; Retrospective Studies; Survival Rate; Noninvasive ventilatory support; Amyotrophic lateral sclerosis; Respiratory insufficiency; Bipap; Human; Nervous system diseases; Treatment; Positive pressure; Intermittent; Central nervous system disease; Degenerative disease; Artificial ventilation; Spinal cord disease; Survival
• ISSN: 0022-510X; 1878-5883
• DOI: 10.1016/S0022-510X(99)00045-3

Amyotrophic Lateral Sclerosis (ALS) is a progressive motor neuron disease that frequently causes death within five years of diagnosis. The majority of deaths are due to pulmonary complications resulting from respiratory muscle weakness and bulbar involvement. A promising respiratory intervention is the recently introduced bi-level intermittent positive pressure (Bipap), which is a noninvasive ventilator modality shown to reduce the work of breathing and improve not only gas exchange, but also exercise tolerance and sleep quality. The aim of this study was to assess the utility of Bipap in prolonging survival in ALS. We retrospectively analyzed the results of Bipap use in 122 patients followed at Hahnemann University. All patients in this study were offered Bipap when their forced vital capacity (FVC) dropped below 50% of predicted value. Group 1 (n=38) accepted Bipap and used it more than 4 h/day. Group 2 (n=32) did not tolerate Bipap well and used it less than 4 h/day. Group 3 (n=52) refused to try Bipap. There was a statistically significant improvement in survival from initiation of Bipap in Group 1 (14.2 months) compared to Group 2 (7.0 months, P=0.002) or 3 (4.6 months, P<0.001) respectively. Furthermore, when the slope of vital capacity decline was examined, the group that used Bipap more than 4 h/day had slower decline in vital capacity (−3.5% change/month) compared to Group 2 (−5.9% change/month, P=0.02) and Group 3 (−8.3% change/month, P<0.001). We conclude that Bipap can significantly prolong survival and slow the decline of FVC in ALS. Our results suggest that all patients with ALS be offered Bipap when their FVC drops below 50%, at the onset of dyspnea, or when a rapid drop in %FVC is noted.