照顧一位初診斷龐貝氏症病人首次氣切之護理經驗

Nursing experience of caring a newly diagnosed Pompe Disease patient undergoing first tracheostomy

本文為一位18歲女性初診斷龐貝氏症並經歷首次氣切之護理過程。筆者於2023年8月30日至9月19日的護理時間，藉由觀察、陪伴、筆談的方式與病人進行溝通、身體評估及病歷查閱等進行整體性評估，確立病人有呼吸道清除功能失效、活動無耐力和身體心像改變等健康問題。經由團隊照護改善病人健康問題：一、指導病人及照顧者執行胸腔照護、肺部復原及返家照顧技能等個別性照護技巧；二、物理治療師及護理師與病人共同訂定復健計畫，使病人恢復活動功能；三、護理師透過主動關心、傾聽、陪伴，與病人逐步建立信任關係，給予病人鼓勵使其能以正向思考面對身體心像改變之問題。此病人為罕見疾病合併肌力改變及呼吸衰竭等問題，對病人及照顧者皆面臨極大挑戰，透過出院準備病人管理師協同出院準備，醫師與護理師共同擬定照護計畫，以利病人及早復能並提升生活品質。

This article details the nursing process for an 18-year-old female newly diagnosed with Pompe disease following her initial tracheotomy. During the care period from August 30 to September 19, 2023, a holistic assessment—incorporating observation, written communication, and physical examination—identified several critical health challenges: ineffective airway clearance, reduced activity endurance, and a disturbed body image. To address these, a collaborative care plan was implemented, focusing on several key pillars: first, providing the patient and caregivers with essential skills in chest physiotherapy and lung rehabilitation; second, coordinating with physical therapists to initiate muscle strength training for functional recovery; and third, fostering a trusting therapeutic relationship through active listening and companionship to support the patient's psychological adaptation to her altered body image. Given that Pompe disease is a rare condition characterized by progressive muscle weakness and respiratory failure, this case highlights the necessity of a coordinated discharge plan and a multidisciplinary approach to facilitate recovery and improve the long-term quality of life for both the patient and her family.