一位LAMP2基因缺損致心肌病變病人的護理經驗

Experience nursing of a case with LAMP2 mutation of cardiomyopathy

陳汶儀(Wen-Yi Chen)；林小閔(Shiau-Min Lin)；黃任瑩(Jen-Ying, Huang)；李榮真(Jung-Chen Lee)

LAMP2基因缺損 ； Danon disease ； 不確定感 ； 心肌病變 ； LAMP2 mutations ； Danon disease ； Uncertainty ； Cardiomyopathy

嘉基護理

21卷1期（2021 / 06 / 01）

73 - 83

繁體中文

本文描述一位48歲女性因LAMP2基因缺損致心肌病變的護理經驗；照護期間於2018年6月13日～6月20日。筆者使用生理、心理、社會及靈性層面進行整體性評估，經由觀察、會談、傾聽及身體評估等方式，蒐集個案主、客觀資料，確認個案住院中的健康問題有：活動無耐力、不確定感及知識增進準備度／與居家自我照顧有關之護理問題。筆者提供疾病相關照護認知及調整日常生活飲食型態，活動無耐力方面則加強運動的認知及方式，並衛教不可隨意停藥及有心衰竭症狀時須立即就醫之重要性；面對疾病造成的不確定感，適時給予正向鼓勵、關懷，協助病人及家族成員進行基因檢測，重燃對未來的希望並配合相關治療，適應疾病後的新生活；強化個案自我照顧的能力，獲得最佳身心調適。由於國內尚無此類疾病之報告，因此引發探討的動機，期望藉此個案的經驗分享，提供護理人員認識此種罕見疾病之需求及照護。

This report described a nursing experience of 48 years old woman for cardiomyopathy produced by LAMP2 mutations. The nursing period lasted from June 13, 2018 to June 20, 2018, through case manager interviews, observations, listening, and visits, and by using assessment guidelines for physical, psychological, social, and spiritual functioning to frame the nursing-related problems of the patient, including activity intolerance, uncertainty in illness theory, and knowledge enhanced, readiness for cardiomyopathy nursing care. During the treatment period, we educated family and patient about the disease process, complications of disease process, information on medications, need for weighing daily, and when it is appropriate to call doctor. The uncertainty in illness theory, we were given positive encouragement and care at the appropriate time and assist the patient and family members for genetic testing. The process continued to enhance the patient's selfcare skills through continuous tracking over the telephone so that she could proactively undergo treatment and achieve the best possible physical and mental adjustment. We wish to share the nursing experience as a reference for peers when facing similar cases.

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