慢性神経疾患におけるケア

抄録

We discuss the following aspects in the care of amyotrophic lateral sclerosis (ALS) and multiple system atrophy : key points of the care, maintaining the quality of life (QOL) of patients and their family, selection and decision–making for medical treatments and their planning, transitions to home care, and the end–of–life care. In order to sustain the QOL, it is important to have home care supported by the collaboration of experts in various fields and to have advance care planning (ACP) from an earlier stage of disease and close discussions on the plans of treatments and of coping the end–of–life stage, respecting the patients' will. We consider it necessary to explain, at a satisfactory level, merits and demerits to the patients in deciding medical treatments, especially enteral nutrition and tracheostomy positive pressure ventilation, and also, particularly in the case of ALS, to mention to them the risk of totally locked–in state and frontotemporal lobar degeneration. During transition periods to home care, they should be referred to primary care doctors who are available at any time as soon as possible, and it should be explained not to call an ambulance without consideration if they prefer not to receive life–prolonging treatments. The most important at the end–of–life stage is to ensure the patients and their family to have a sense of ease by placing the highest priority on pain palliation.