Mustfa N, Walsh E, Bryant V, Lyall R A, Addington-Hall J, Goldstein L H, Donaldson N, Polkey M I, Moxham J, Leigh P N
Respiratory Muscle Laboratory, Guy's, King's, and St Thomas' School of Medicine, King's College Hospital, UK.
Neurology. 2006 Apr 25;66(8):1211-7. doi: 10.1212/01.wnl.0000208957.88534.11.
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.
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.
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.
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 stress.
无创通气（NIV）可降低肌萎缩侧索硬化症（ALS）患者的死亡率，并改善其生活质量（QoL）的某些方面。然而，人们仍然担心进行性残疾可能会抵消这些益处，并给护理人员带来不必要的负担。
为39名需要NIV的患者提供治疗。26名患者开始接受NIV治疗，但13名患者拒绝或无法耐受NIV。同时对15名无呼吸肌无力（RMW）但ALS严重程度和年龄相似的患者进行了研究。21名接受NIV治疗的患者、7名未接受治疗的患者以及10名无RMW的患者的护理人员参与了研究。患者和护理人员进行了为期12个月的详细QoL测量。接受NIV治疗的患者在治疗前后进行了认知测试。
RMW与较低的QoL相关。未接受治疗的患者的中位生存期（18天；95%置信区间11至25天）短于接受NIV治疗的患者（298天；95%置信区间192至404天）和无RMW的患者（370天；95%置信区间278至462天；对数秩检验[2自由度]=81，p = 0.00001）。在开始NIV治疗后的1个月内，多种QoL指标得到改善，并持续改善了12个月。随着RMW的进展，无RMW患者的QoL下降。接受NIV治疗的患者和无RMW患者的护理人员负担增加相似，但接受NIV治疗的患者的护理人员在简短健康调查问卷36项活力量表评分上出现恶化。在接受NIV治疗的患者中，学习和记忆指标未发现改善。
呼吸肌无力对生活质量（QoL）的影响大于ALS的总体严重程度。尽管ALS病情进展，无创通气（NIV）仍可改善QoL。NIV对护理人员QoL的大多数方面没有影响，也不会显著增加护理人员的负担或压力。
