In spite of the improvements in care and the emergence of disease-modifying treatments, Duchenne muscular dystrophy (DMD) remains a life-limiting disease of adolescence and (young) adulthood. Palliative care approaches and principles should be integrated from the point of diagnosis and implemented throughout the lifespan. A nationwide cross-sectional survey based on a mixed-method-design of qualitative and quantitative research approaches evaluated the structural implementation and perception of palliative care for DMD in Germany. Data analyses revealed that palliative care was predominantly provided at the primary care level by pediatricians, general practitioners and specialized multi-professional outpatient structures. The majority of patients did not utilize the scopes of specialized palliative structures. Simultaneously, insufficiently treated complex symptoms, emergent and elective hospitalizations and barriers in transitioning into adult care presented a considerable burden. A collaborative integrated model with a close cooperation of patients, families and care providers is proposed involving task areas and interfaces complementing primary and specialized palliative care (1) management of complex symptoms, (2) crisis support, (3) intermittent relief of the strain for caregivers, (4) coordination of care, (5) advance care planning and (6) end-of-life care. Specialized palliative care should be used as an "add-on" approach in time of need rather than as a prognosis or disease stage.

中文翻译：

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Duchenne 肌营养不良症初级和专业姑息治疗的任务和界面——患者的观点。

尽管护理有所改善并出现了改善疾病的治疗方法，但杜氏肌营养不良症 (DMD) 仍然是青春期和（年轻）成年期的一种限制生命的疾病。姑息治疗的方法和原则应该从诊断的角度进行整合，并在整个生命周期中实施。一项基于定性和定量研究方法的混合方法设计的全国横断面调查评估了德国 DMD 姑息治疗的结构实施和感知。数据分析显示，姑息治疗主要由儿科医生、全科医生和专业的多专业门诊机构在初级保健级别提供。大多数患者没有使用专门的姑息结构的范围。同时，复杂症状得不到充分治疗、急诊和择期住院以及过渡到成人护理的障碍是相当大的负担。提出了一种患者、家属和护理提供者密切合作的协作集成模型，涉及补充初级和专业姑息治疗的任务领域和接口 (1) 复杂症状的管理，(2) 危机支持，(3) 间歇性缓解压力对于护理人员，(4) 护理协调，(5) 预先护理计划和 (6) 临终护理。专业姑息治疗应在需要时用作“附加”方法，而不是用作预后或疾病阶段。提出了一种患者、家属和护理提供者密切合作的协作集成模型，涉及补充初级和专业姑息治疗的任务领域和接口 (1) 复杂症状的管理，(2) 危机支持，(3) 间歇性缓解压力对于护理人员，(4) 护理协调，(5) 预先护理计划和 (6) 临终护理。专业姑息治疗应在需要时用作“附加”方法，而不是用作预后或疾病阶段。提出了一种患者、家属和护理提供者密切合作的协作集成模型，涉及补充初级和专业姑息治疗的任务领域和接口 (1) 复杂症状的管理，(2) 危机支持，(3) 间歇性缓解压力对于护理人员，(4) 护理协调，(5) 预先护理计划和 (6) 临终护理。专业姑息治疗应在需要时用作“附加”方法，而不是用作预后或疾病阶段。(5) 预先护理计划和 (6) 临终关怀。专业姑息治疗应在需要时用作“附加”方法，而不是用作预后或疾病阶段。(5) 预先护理计划和 (6) 临终关怀。专业姑息治疗应在需要时用作“附加”方法，而不是用作预后或疾病阶段。