Faculty of Medicine, University of Lisbon, Avenida Professor Egas Moniz, Lisboa, 1649-028, Portugal.
North Lisboa Hospital Centre, Santa Maria Hospital, Lisboa, Portugal.
BMC Palliat Care. 2024 Apr 16;23(1):100. doi: 10.1186/s12904-024-01429-z.
Breathlessness is a prevalent symptom affecting the quality of life (QOL) of Amyotrophic Lateral Sclerosis (ALS) patients. This systematic review explored the interventions for controlling breathlessness in ALS patients, emphasizing palliative care (PALC), non-invasive ventilation (NIV), opioids, and non-pharmacological strategies.
A comprehensive search of PubMed, Cochrane Library, and Web of Science databases was conducted. Eligibility criteria encompassed adults with ALS or motor neuron disease experiencing breathlessness. Outcomes included QOL and symptom control. Study designs comprised qualitative studies, cohort studies, and randomized controlled trials.
Eight studies were included, most exhibiting low bias risk, comprising one randomized controlled trial, three cohort studies, two comparative retrospective studies, and two qualitative studies (interviews). Most studies originated from Europe, with one from the United States of America. The participants totaled 3423, with ALS patients constituting 95.6%. PALC consultations significantly improved symptom assessment, advance care planning, and discussions about goals of care. NIV demonstrated efficacy in managing breathlessness, with considerations for device limitations. Opioids were effective, though predominantly studied in non-ALS patients. Non-pharmacological strategies varied in efficacy among patients.
The findings underscore the need for individualized approaches in managing breathlessness in ALS. PALC, NIV, opioids, and non-pharmacological strategies each play a role, with unique considerations. Further research, especially ALS-specific self-management studies, is warranted.
呼吸困难是一种普遍存在的症状，会影响肌萎缩侧索硬化症（ALS）患者的生活质量（QOL）。本系统评价探讨了控制 ALS 患者呼吸困难的干预措施，重点关注姑息治疗（PALC）、无创通气（NIV）、阿片类药物和非药物策略。
对 PubMed、Cochrane 图书馆和 Web of Science 数据库进行了全面检索。纳入标准为患有 ALS 或运动神经元病并出现呼吸困难的成年人。结局包括 QOL 和症状控制。研究设计包括定性研究、队列研究和随机对照试验。
纳入了 8 项研究，大多数研究的偏倚风险较低，包括 1 项随机对照试验、3 项队列研究、2 项比较回顾性研究和 2 项定性研究（访谈）。大多数研究来自欧洲，其中一项来自美国。参与者总计 3423 人，其中 ALS 患者占 95.6%。PALC 咨询显著改善了症状评估、预先护理计划和对护理目标的讨论。NIV 在管理呼吸困难方面有效，但需要考虑设备限制。阿片类药物有效，但主要在非 ALS 患者中进行了研究。非药物策略在患者中的疗效各异。
研究结果强调了在 ALS 患者中管理呼吸困难需要个体化方法。PALC、NIV、阿片类药物和非药物策略各有作用，需要考虑独特的因素。需要进一步研究，特别是针对 ALS 患者的自我管理研究。
