Hilton T, Orr R D, Perkin R M, Ashwal S
Department of Nursing, Loma Linda University School of Medicine, California 92350.
Pediatr Neurol. 1993 May-Jun;9(3):165-77. doi: 10.1016/0887-8994(93)90080-v.
End of life care for patients with Duchenne muscular dystrophy (DMD) has become increasingly complex because of new technologies, changes in medical personnel over periods of time, emergence of home health care systems, and increasing patient and family autonomy in decision-making. In this review, we discuss the medical problems, particularly respiratory and cardiac failure, faced by DMD patients. Current concepts concerning the evaluation and options for treatment of these problems are presented as well as the ethical issues involved in the care of the DMD patient. These issues include the medical indications for treatment, patient preferences, quality-of-life issues, and contextual features related to legal, institutional, religious, geographic, cultural, social, and financial factors. We also present our experience at Loma Linda University Medical Center over the past 10 years in the development of a home mechanical ventilation program for DMD patients and an algorithm for the evaluation of these patients. Many patients with DMD do well on long-term ventilation, but some find that their quality of life is less than desirable and choose to discontinue this method of life-prolongation. Many of these new options are very expensive, making the decision to use them a difficult one. Ultimately, these are societal issues that require clear reflection on matters of resource allocation that should be performed by health care professionals, citizens, and health planners.
由于新技术的出现、医疗人员随时间的变化、家庭医疗保健系统的兴起以及患者和家庭在决策方面日益增强的自主权，杜氏肌营养不良症（DMD）患者的临终关怀变得越来越复杂。在这篇综述中，我们讨论了DMD患者面临的医疗问题，特别是呼吸和心力衰竭。介绍了当前关于这些问题的评估和治疗选择的概念，以及DMD患者护理中涉及的伦理问题。这些问题包括治疗的医学指征、患者偏好、生活质量问题，以及与法律、机构、宗教、地理、文化、社会和财务因素相关的背景特征。我们还介绍了洛马林达大学医学中心在过去10年中为DMD患者制定家庭机械通气计划和评估这些患者的算法方面的经验。许多DMD患者在长期通气方面情况良好，但有些患者发现他们的生活质量不尽如人意，选择停止这种延长生命的方法。许多这些新选择非常昂贵，这使得决定是否使用它们变得困难。最终，这些都是社会问题，需要医疗保健专业人员、公民和卫生规划者对资源分配问题进行清晰的思考。
