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Background: Noninvasive ventilation (NIV) reduces mortality and improves some aspects of quality of life (QoL) in ALS. However, concerns remain that progressive disability may negate these benefits and unnecessarily burden caregivers. Methods: Thirty- nine patients requiring NIV were offered treatment. Twenty- six were established on NIV, but 13 declined or could not tolerate NIV. Fifteen patients without respiratory muscle weakness (RMW) but with similar ALS severity and age were studied in parallel. Caregivers of 21 NIV, 7 untreated, and 10 patients without RMW participated. Patients and caregivers had detailed QoL measurements for 12 months. NIV patients underwent cognitive testing before and after treatment. Results: RMW correlated with lower QoL. The median survival of untreated patients (18 days; 95% CI 11 to 25 days) was shorter than for NIV patients (298 days; 95% CI 192 to 404 days) and non- RMW patients (370 days; 95% CI 278 to 462 days; log rank test 2 df = 81, p = 0.00001). A wide range of QoL measures improved within 1 month of starting NIV, and improvements were maintained for 12 months. QoL of non- RMW patients declined as RMW progressed. Caregivers of NIV and non- RMW patients showed similar increases in burden, but NIV patient caregivers developed a deterioration in the Short Form- 36 Vitality score. No improvements were found on measures of learning and recall in the NIV patients. Conclusions: Respiratory muscle weakness has a greater impact on quality of life (QoL) than overall ALS severity. Noninvasive ventilation (NIV) improves QoL despite ALS progression. NIV has no impact on most aspects of caregiver QoL and does not significantly increase caregiver burden or ess.
However, concerns remain that progressive disability may negate these benefits and unnecessarily burden caregivers. Methods: Thirty- nine patients require NIV were delivered treatment Twenty-six were established on NIV, but 13 declined or could not be tolerated NIV. Fifteen patients without respiratory muscle weakness (RMW) but with similar ALS severity and age were studied in parallel. Caregivers of 21 NIV, 7 untreated, and 10 patients Patients with caregivers had detailed QoL measurements for 12 months. NIV patients underwent cognitive testing before and after treatment. Results: RMW correlated with lower QoL. The median survival of untreated patients (18 days; 95% CI 11 to 25 days ) were shorter than for NIV patients (298 days; 95% CI 192 to 404 days) and non-RMW patients (370 days; 95% CI 278 to 462 days; log rank test 2 df = 81, p = 0.00001). A wide range of QoL measures improved within 1 month of starting NIV, and improvements were held for 12 months. QoL of non-RMW patients declined as RMW progressed. Caregivers of NIV and non- RMW patients showed similar increases in burden, but NIV patient caregivers developed a deterioration in the Short Form- 36 Vitality score. No improvements were found on measures of learning and recall in the NIV patients. Conclusions: Respiratory muscle weakness has a greater impact on quality of life (QoL) than overall ALS severity. Noninvasive ventilation (NIV) improves QoL despite ALS progression. NIV has no impact on most aspects of caregiver QoL and does not significantly increase caregiver burden or ess.