腎上腺腦白質退化症之居家營養照護－個案報告

個案為16歲男性，2014年底前因注意力不集中、過動，至醫學中心求診，診斷為遺傳性罕見疾病「腎上腺腦白質退化症(Adrenoleukodystrophy, ALD)」，依醫囑每日飲用25ml羅倫佐的油(Lorenzo's Oil)，並接受飲食衛教控制脂質攝取。隨病情進展，個案退化快速，逐漸喪失咀嚼、吞嚥困難、視力、聽力、語言等功能，ADL(activity of daily living) 評為0 分。2015 年9 月透過社服單位轉介，提供居家醫療照護整合計畫。進食部分經藍鵲團隊語言治療師評估咀嚼吞嚥功能，可維持經口進食，營養師評估營養需求及飲食製備規劃，以舒適餵食(comfort feeding only)為前提。個案經由藍鵲團隊提供整體照護及適當的營養攝取，截至2017年2月為止BMI(BMI, body mass index) 由17.2kg/m^2提升至19.4 kg/m^2。

關鍵字

腎上腺腦白質退化症；吞嚥困難；舒適餵食

並列摘要

A 16 year -old boy was diagnosed with adrenoleukodystrophy in 2014 and under treatment of Lorenzo’s Oil 25ml/day and restrained dietary fat intake. However, with the progress of the disease, his health degraded quickly, gradually lost chewing, dysphagia, vision, hearing, language and other functions. His ADL score was 0. In September 2015, he was referred to the Social Service Department to be cared under the home health care integration program. The language therapist evaluated his chewing swallow function and found that he could still eat orally. The dietitian evaluated his nutritional requirement and planned his diet based on the premise of comfort feeding. Through the total care and appropriate nutritional intake provided by the medical team, his BMI increased from 17.2kg/m^2 to 19.4 kg/m^2 in February 2017.

並列關鍵字

Adrenoleukodystrophy ； Dysphagia ； comfort feeding only

國際替代計量

腎上腺腦白質退化症之居家營養照護－個案報告

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