Study on the home nursing care for the patients with neuromuscular disease who have chronic and progressive respiratory disorder

[目的和方法]：随着包括医疗保险制度修订在内的家庭医疗支持体系的完善，需要在家庭护理中进行机械通气和氧疗的晚期呼吸系统疾病患者的数量正在增加。本研究旨在以非侵入性方法调查慢性或进展性呼吸疾病神经肌肉疾病患者呼吸障碍的进展，以及在护理过程中引入适当的医疗护理(包括呼吸机治疗和安全护理活动)的有效性。研究对象包括13例进行性肌营养不良症患者(Duchenne-10型，Fukuyama-3型)和10例肌萎缩侧索硬化症患者。对疾病进展过程中获得的时间进程数据进行调查，并与神经疾病专业护士和神经科医生进行讨论。[结果和讨论]：1.…建议及早掌握呼吸紊乱的情况，多做以下介绍。对于进行性肌营养不良患者，除了检查呼吸紊乱发作前的生命体征和主观症状外，定期使用呼吸仪测定潮气量(TV)和最大肺活量(Max VC)是有用的。当预计会出现呼吸紊乱的迹象时，定期通过说话咳嗽流量(PCF)来评估咳嗽力量，测定整夜睡眠时的经皮血氧饱和度(脉搏血氧饱和度)和经皮血二氧化碳分压是有用的。对于肌萎缩侧索硬化症患者，从发病初期就观察患者的主观症状，并用呼吸仪测定TV和最大VC是有用的。在注意到呼吸紊乱的初步迹象后，建议定期测定整夜睡眠时的经皮血氧饱和度(脉搏血氧饱和度)和经皮血二氧化碳分压。以上数据可作为家庭机械通气入门阶段护理方案的基础数据。2.在接受机械通气者中，定期测定经皮动脉血氧饱和度和经皮血二氧化碳分压作为改变机械通气机设置以应对呼吸紊乱的进一步进展的指标是有用的。3.家庭护理的关键是护理团队的人数应该合作，他们的活动应该有时间的安排，以便测量的值可以在治疗和护理计划中得到适当的解释和反映。对病人的支持也很重要，以提高他/她的自我管理能力。这些都是我们打算在未来执行的任务。较少

[Purpose and method] : Due to the improvement in home medical care support system including the amendment medical insurance system, the number of patients with advanced respiratory disorders who require mechanical ventilation and oxygen therapy in home nursing is on the increase. This study was conducted for the purpose of investigating by non-invasive method the progress of respiratory disorder in the neuro-muscular disease patients who have chronic or progressive respiratory disorder and the usefulness of introducing appropriate medical care including ventilatory therapy as well as safe nursing activities in performing the care. The subjects of study included 13 patients with progressive muscular dystrophy (Duchenne type - 10, Fukuyama type - 3) and 10 with amyotrophic lateral sclerosis. The time course data which were obtained during the progress of disease were investigated and discussed with nurses specialized in neurological diseases and neurologists.[Results and Discussion] : 1. … More The following is recommended to grasp the status of respiratory disorder in early stage. In the patients with progressive muscular dystrophy, it is useful to regularly determine the tidal volume (TV) and maximum vital capacity (MAX VC) using a respirometer (respirometry) in addition to checking the vital signs and subjective symptoms from the time before onset of respiratory disorder. When signs of respiratory disorder are expected to appear, it is useful to regularly assess the cough force by speak cough flow (PCF), determine the percutaneous blood oxygen saturation (pulse oximetry) during sleep all through the night and percutaneous blood carbon dioxide partial pressure. In the case of patients with amyotrophic lateral sclerosis, it is useful to observe subjective symptoms from the initial stage of onset and to determine TV and MAX VC by respirometer. After initial signs of respiratory disorder are noted, it is advisable to regularly determine the percutaneous blood oxygen saturation (pulse oximetry) during sleep all through the night and percutaneous blood carbon dioxide partial pressure. The above maybe used as the basic data for the nursing protocol during the introductory phase of home mechanical ventilation. 2. In those receiving mechanical ventilation, it is useful to regularly determine the percutaneous arterial blood oxygen saturation and percutaneous blood carbon dioxide partial pressure as indices for changing the ventilatory settings to cope with further progress in respiratory disorder. 3. It is essential for home nursing that the numbers of a nursing team should cooperate and that their activities should be planned with allowance for time so that the measured values may be appropriately interpreted and reflected in the treatment & nursing plan. Also important is the support to the patient to increase his/her self management ability. These are the tasks which we intend to perform in the future. Less