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肌萎缩侧索硬化症的姑息治疗。

Palliative Care in Amyotrophic Lateral Sclerosis.

机构信息

Main Regional Center of Pain Relief and Supportive/Palliative Care (S.M.), La Maddalena Cancer Center, Palermo, Italy; Regional Home Care Program, SAMOT (S.M.), Palermo, Italy.

Department of Clinical Medical Sciences (L.A.H.), Yarmouk University, Irbid, Jordan.

出版信息

J Pain Symptom Manage. 2023 Oct;66(4):e485-e499. doi: 10.1016/j.jpainsymman.2023.06.029. Epub 2023 Jun 26.

Abstract

Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease of the motor neurons. Given the evolutive characteristics of this disease, palliative care principles should be a foundation of ALS care. A multidisciplinary medical intervention is of paramount importance in the different phases of disease. The involvement of the palliative care team improves quality of life and symptoms, and prognosis. Early initiation is of paramount importance to ensuring patient-centered care, when the patient has still the capability to communicate effectively and participate in his medical care. Advance care planning supports patients and family members in understanding and sharing their preferences according to their personal values and life goals regarding future medical treatment. The principal problems which require intensive supportive care include cognitive disturbances, psychological distress, pain, sialorrhrea, nutrition, and ventilatory support. Communication skills of health-care professionals are mandatory to manage the inevitability of death. Palliative sedation has peculiar aspects in this population, particularly with the decision of withdrawing ventilatory support.

摘要

肌萎缩侧索硬化症(ALS)是一种不可治愈的运动神经元退行性疾病。鉴于这种疾病的发展特征,姑息治疗原则应该是 ALS 护理的基础。多学科医疗干预在疾病的不同阶段都至关重要。姑息治疗团队的参与可以改善生活质量和症状,并改善预后。早期启动姑息治疗对于确保以患者为中心的护理至关重要,因为此时患者仍然能够有效地沟通并参与他的医疗护理。预先护理计划支持患者和家属根据个人价值观和生活目标理解和分享他们对未来治疗的偏好。需要强化支持性护理的主要问题包括认知障碍、心理困扰、疼痛、流涎、营养和通气支持。卫生保健专业人员的沟通技巧对于管理死亡的必然性是强制性的。在这种人群中,姑息镇静具有特殊的方面,特别是在决定撤回通气支持时。

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