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題 名 | 運用助咳機於嬰兒型龐貝氏症病童之呼吸照護經驗=A Case Report of Using CoughAssist on Infantile Pompe Disease |
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作 者 | 鄭靖雯; 林鳳卿; 邱銘煌; 陸嘉貞; | 書刊名 | 呼吸治療雜誌 |
卷 期 | 13:2 2014.07[民103.07] |
頁 次 | 頁49-57 |
分類號 | 415.415 |
關鍵詞 | 嬰兒型龐貝氏症; 呼吸器依賴; 助咳機; Infantile Pompe disease; Mechanical ventilator dependent; CoughAssist; |
語 文 | 中文(Chinese) |
中文摘要 | 龐貝氏症是不可逆代謝性神經肌肉疾病,1932年Johannes Pompe醫生發現一名死於肺炎七個月大女嬰不僅肺部問題,更有心臟肥大及大量肝醣堆積於心肌與骨骼肌。造成呼吸肌與橫膈肌等無力導致呼吸道清潔功能失效,使痰液蓄積、肺炎、低血氧與肺塌陷等併發症產生,臨床上需呼吸器輔助及適時肺復原介入,避免進行式肌肉無力導致呼吸衰竭最後死亡憾事發生。近年許多文獻一致認為最有效清除痰液方式為助咳機(CoughAssist),CoughAssist是藉由正壓快速轉換負壓的機轉加強痰液清除,可降低病人入急診率、加護病房住院率、住院時間及死亡率,同時維持病人生命與生活品質。本病童因患有龐貝氏症,導致肌肉無力與呼吸衰竭,已完成氣切造廔口並以雙壓型正壓呼吸器支持通氣,但仍因數次反覆肺炎入院,藉CoughAssist執行有效咳嗽協助與促進痰液排除,協同照顧者協助病童胸腔物理治療與抽痰後,明顯減少肺炎再入院治療情形。國內對於龐貝氏症相關治療經驗豐富,但未見龐貝氏症與呼吸器及CoughAssist的重要性深入探討,希望由本個案呼吸照護經驗分享,提供呼吸治療師協助神經肌肉疾病病人有效呼吸治療與增進其生活品質。 |
英文摘要 | Pompe disease is an irreversible metabolic neuromuscular disease. In 1932, Dr. Joannes Pompe discovered a seven-month old girl who had died of not only pulmonary problem, but also cardiomegaly and accumulation of amount glycogen in cardiac and skeletal muscle. The common symptom is respiratory muscle and diaphragm weakness which will further make impaired airway clearance. The complication includes sputum accumulation, pneumonia, hypoxemia, and lung collapse. So, it is necessary that required mechanical ventilator assistance and invent pulmonary rehabilitation clinically to avoid death of respiratory failure results from muscle weakness. Much recent research mentions that the most effective way to manage secretion is CoughAssist. It applies positive pressure to airway, followed by quickly change due to negative pressure. This will assist patient remove secretion effectively and reduce admission of emergency, ICU rate, length of hospitalization and mortality rate. Simultaneously, it will maintain patients' lives and quality of life. This patient was diagnosis of Pompe disease. Because of respiratory failure by muscle weakness, he used bi-level positive airway pressure (BiPAP) and performed tracheostomy. However, he entered the hospital repeatedly due to recurrent pneumonia. Therefore, we used CoughAssist to assist his cough. In addition, daily pulmonary rehabilitation and suction by his family made his readmission case better. By this case, we will like to discuss the importance of mechanical ventilator and CoughAssist in infantile Pompe disease. |
本系統中英文摘要資訊取自各篇刊載內容。