脊髓性肌萎缩症家庭康复与护理的研究进展

Recent research on home rehabilitation and nursing for spinal muscular atrophy

摘要:

脊髓性肌萎缩症（spinal muscular atrophy, SMA）是一种常染色体隐性遗传的神经肌肉疾病。随着疾病修正治疗的出现，SMA的预后得以显著改善，也引起了人们对家庭康复与护理的重视，长程规范的家庭康复与护理可延缓SMA疾病进展，提升患儿及照顾者的心理健康和生活质量。该文综述了SMA患者家庭康复的目标、基本的功能训练方法、呼吸管理、营养管理以及相关心理健康问题，强调在康复过程中获得适当家庭康复与护理支持的重要性。

Abstract:

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disorder. With the emergence of disease-modifying therapies, the prognosis of SMA has significantly improved, drawing increased attention to the importance of home rehabilitation and nursing management. Long-term, standardized home rehabilitation and nursing can delay the progression of SMA, enhance the psychological well-being, and improve the quality of life of both patients and caregivers. This article provides an overview of the goals of home rehabilitation, basic functional training methods, respiratory management, and nutritional management for SMA patients, as well as psychological health issues, emphasizing the significance of obtaining appropriate home rehabilitation and support during the care process.