照顧一位表皮溶解水皰症新生兒之傷口護理經驗

Nursing Experience with Providing Wound Care for a Newborn with Epidermolysis Bullosa

表皮溶解水皰症是侵犯皮膚的罕見染色體遺傳疾病。廣泛皮膚破損若缺乏適當的照顧，恐引發敗血症、危急生命。本文旨在探討一位新生兒疑似表皮溶解水皰症導致全身水皰與皮膚剝離佔體表面積30%之傷口護理經驗。筆者於2013年12月1日至2014年1月7日進行整體性評估發現：大範圍的皮膚與黏膜損傷，導致傷口困難照護，案嬰面臨極大痛楚。筆者與醫護團隊針對此罕見疾病新生兒之傷口照護進行文獻搜尋，並採團隊照護模式，依據文獻建議，提供個別性皮膚黏膜與傷口護理措施，減少疼痛、控制感染與促進傷口癒合。同時帶領主要照顧者漸進式接觸罕見疾病新生兒，降低其面臨照護罕見疾病之身心壓力，引導主動學習傷口照護與包紮技巧，使其於返家後持續提供傷口護理。建議未來醫護人員面對表皮溶解水皰症新生兒急性照護期需以文獻為佐，輔以跨團隊照護模式提供傷口護理與支持療法控制合併症發生。並需將社會可運用資源納入護理計畫中，減少罕見疾病為家庭帶來之衝擊。

Epidermolysis bullosa (EB) is a rare hereditary, chromosomal disease of the skin. Life-threatening septicemia may result if appropriate care is not provided to alleviate the extensive skin irritation that is the main symptom of this disease. This case report describes the experience of the author in nursing a wound area on a newborn that was suspected of being caused by EB. This wound area comprised blisters and peeling skin that covered 30% of the entire skin area of the infant. A holistic assessment conducted from December 1st, 2013 to January 7th, 2014 revealed that this large of an area of damage to the skin and mucosa considerably complicated the task of wound care and caused severe pain to the infant. In response to the special needs of this case, our medical team conducted a literature review of wound care for this rare disease. Based on the suggestions of previous empirical studies, nursing measures for the skin, mucosa, and wounds of the newborn were then administered through inter-team cooperation. These actions effectively reduced the pain, controlled the infection, and accelerated wound healing. In addition, progressive contact was used to guide the primary caregivers of the newborn, which alleviated their physical and psychological stresses effectively. The caregivers were educated systematically on wound care and guided to learn techniques for nursing and dressing wounds. Thus, these caregivers were better prepared to continue providing wound care at home. We suggest that healthcare professionals reference empirical studies when providing care to EB newborns during the acute-care period and provide wound care and supportive therapies to control the occurrence of complications using a multidisciplinary team-care model. In addition, social resources should be used effectively in nursing care plans to mitigate the effect of this rare disease on families.