腎上腺腦白質退化症病童父母親的長期照護經驗及需求之探討

The Long-Term Care Experiences and Care Needs of Parents Caring for Children With Adrenoleukodystrophy

10.6224/JN.201902_66(1).05

馮瑞君(Jui-Chun FENG)；吳維紋(Wei-Wen WU)；卓妙如(Miao-Ju CHWO)；梁淑媛(Shu-Yuan LIANG)；鄭夙芬(Su-Fen CHENG)

腎上腺腦白質退化症 ； 父母親 ； 長期照護 ； 罕見疾病 ； adrenoleukodystrophy (ALD) ； parents ； long- term care ； rare disease

護理雜誌

66卷1期（2019 / 02 / 01）

27 - 37

繁體中文

背景：腎上腺腦白質退化症病童從發病至死亡過程，需要仰賴長期照顧，然現階段研究結果缺乏不同階段疾病歷程的照護經驗與需求之探討。目的：瞭解父母親照顧腎上腺腦白質退化症病童的長期照護經驗及需求。方法：採質性研究法，以立意取樣方式進行收案。運用半結構式深入訪談共訪7位父母親，以Colaizz法分析資料。結果：整體照護經驗可分為三階段：「發病初期到確立診斷期」、「生理功能快速惡化期」、「長期臥床到死亡期」，其長期照護經驗涵蓋5個主題：從混亂與無助到被迫接收、自責愧疚與擔憂、激發父母的韌性、把握當下面對未來、陪伴孩子無痛苦的走完人生。而照護需求則分別為三個主題：整合資源即時醫治、快速獲得訊息與支持，以及建立符合個人需求的長期照護。結論／實務應用：本文呈現腎上腺腦白質退化症病童父母親的長期照護經驗與需求。提供個別化的照顧與護理指導、電話諮詢，同時結合兒童罕見疾病個案管理師與兒童安寧共同照護團隊，適時給予居家安寧緩和療護，以協助父母親因應照護病童的需求。

Background: Children suffering from adrenoleukodystrophy (ALD) requires life-long care. Little is known about the care needs of parents of ALD children at different stages of their disease. Purpose: The purpose of this study was to understand the long-term care experiences and care needs of parents caring for their ALD children. Methods: A descriptive qualitative study with 7 in-depth interviews was conducted with parents. Results: The results of these care experiences were distinguished into three phases: ＂pathogenesis to diagnosis＂, ＂rapid deterioration of physiological functions＂, and ＂bedridden until the death＂. The long-term care experiences revealed five themes, including ＂chaos and helplessness to seek medical attention then being forced to accept＂, ＂self-accusation and guilt＂, ＂strengthening parents' toughness＂, ＂seizing the moment and facing the future＂, and ＂accompanying children through life without pain＂. Within the three phases, the care needs comprised the three themes of ＂integrating resources and providing immediate care＂, ＂obtaining information and support regarding ALD rapidly＂, and ＂establishing individualized long-term care＂. Conclusion/Implication for Practice: This study revealed the long-term care experiences and care needs of the parents of ALD children. Providing individualized care, nursing instruction, and telephone consultation as well as connecting case managers with the hospice-care team will help facilitate and meet the care needs of these parents.

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