照護一位狄喬治症合併發展遲緩病童之護理經驗

Nursing Experience in caring for a developmentally delayed child with DiGeorge disease

廖桂敏(Kuei-Min, Liao) ； 洪嘉玟(Chia-Wen, Hung)

《馬偕護理雜誌》 16卷1期 (2022/01) Pp. 72-83

https://doi.org/10.29415/JMKN.202201_16(1).0007

摘要

本文係照護一位狄喬治症的罕病病童合併發展遲緩之護理經驗，筆者於2018年12月21日至12月30日運用系統性身體評估及家庭評估，確立個案有營養失衡：少於身體需要、危險性發展延遲；主要照顧者方面有家庭因應失能等護理問題。照護過程中，筆者與醫療專業團隊合作，改善個案嘔吐問題，營養師制訂個別化的生酮飲食，提供營養照護，維持個案生理所需；並依據發展評估，召開家庭會議，介入早期療育知識及會診兒童心智科及復健科，提供早療服務及復健運動，促進個案的發展治療；運用同理心傾聽、關懷支持家屬，鼓勵家屬表達心裡感受及對疾病治療的看法，建立正確疾病知識，並提供罕病基金會資訊及病友的照護經驗分享，正向激勵家屬共同參與照護過程，建構家庭支持系統，增進家屬照護信心，落實以家庭為中心的整體性照護理念，使個案獲得良好的照護品質，達到最佳適應狀態。因罕病的相關文獻及照護經驗少，建議院方能定期召開案例討論會或相關研習會，並藉此個案的經驗分享，可促進兒科護理人員照顧類似病童之參考。

關鍵字

狄喬治症 ； 發展遲緩 ； 生酮飲食

並列摘要

The article describes the experience of caring for a child with both developmental delays and DiGeorge disease. From December 21 to December 30, 2018, the author determined through assessment that the pediatric patient had a nutritional imbalance due to a lower nutritional intake than physiologically required, dangerous developmental delays, and coping issues in-home care. The author collaborated with a team of medical professionals to resolve the child's vomiting problem. Dietitians formulated and offered an individualized ketogenic diet to improve the nutritional status based on a developmental assessment. We scheduled a family meeting to intervene to enhance knowledge of the disease, treatment and provide early childhood intervention and rehabilitation exercise. Health-care professionals provide empathy by listening to and supporting family members and assessing their understanding of illness and treatment. To provide good quality of care for pediatric patients and achieve optimal adaptation for the entire family by sharing care experiences, providing information about the Rare Disease Foundation and family support groups, building family support systems, and increasing the confidence of primary caregivers in their care.

並列關鍵字

DiGeorge disease ； developmental delay ； ketogenic diet

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