2例儿童晚发型庞贝病患者酶替代治疗的观察与护理

Observation and nursing of enzyme replacement therapy in children with late onset Pompe disease

目的总结我院2021年11月收治的2例儿童晚发型庞贝病(late-onset pompe disease,LOPD)患者进行酶替代治疗的效果和护理经验。护理重点为充分评估病情、用药观察和护理、呼吸和骨骼肌功能锻炼、高蛋白和低碳水化合物饮食、个性化心理支持、出院宣教和随访。2例患者规范用药25次,未发生输注相关不良反应,体重指数增加,临床症状好转。应重视儿童LOPD行酶替代治疗患者的护理,针对用药、功能锻炼、饮食和心理等方面制定个性化治疗与护理方案,可有效促进患者健康、提高生活质量。

Objective To summarize the effectiveness and nursing experience of enzyme replacement therapy in children with late-onset pompe disease(LOPD),and provide reference for clinical practice on November 2021.Care focused on adequate evaluation,medication observation and care,respiratory and skeletal muscle function exercise,high protein and low carbohydrate diets,personalized psychological support,and discharge education and follow-up.Two patients received standard medication 25 times without any infusion related adverse reactions,increased body mass index,and improved clinical symptoms.The care of children with LOPD undergoing enzyme replacement therapy should be emphasized,and personalized treatment and nursing plans for medication,functional exercise,diet,and psychology can be developed,so as to effectively promote patient health and improve quality of life.