年轻的XLH患者报告了支持性护理计划的经验.
Anya Rothenbuhler1, Iva Gueorguieva2, Lydia Lichtenberger-Geslin3
1AP-HP, INSERM, Endocrinology and Diabetes for Children, Physiologie et Physiopathologie Endocriniennes, Reference Center for Rare Disorders of Calcium and Phosphate Metabolism, Filière OSCAR, and Platform of Expertise for Rare Disorders, ERN for Rare Endocrine Disorders and ERN BOND, Bicêtre Paris Saclay Hospital, Le Kremlin-Bicêtre, France.
Patient preference and adherence
|June 16, 2023
概括
该aXess计划改善了X链接低血症 (XLH) 患者的生活质量. 这项患者支持计划通过教育和持续的随访,增强了患者的福祉.
科学领域:
- 医学遗传学 医学遗传学
- 罕见疾病 罕见疾病
- 患者支持计划 患者支持计划
背景情况:
- 与X相关的低酸血症 (XLH) 是一种罕见的遗传性疾病,导致酸盐浪费和骨矿化受损.
- 由于其慢性和多方面的性质,XLH显著影响患者的生活质量.
研究的目的:
- 评估aXess患者支持计划 (PSP) 在改善XLH患者生活质量的有效性.
- 评估患者对aXess计划的满意度.
主要方法:
- 一项为期12个月的前性研究,涉及59名加入aXess计划的XLH患者.
- 参与者定期通过电话与护士联系,以协调治疗,监测坚持和进行激励访谈.
- 儿科生活质量库存 (PQLI) 在基线,6个月和12个月被管理.
主要成果:
- 大多数患者在12个月前在生活质量的各个方面都报告了显著的改善.
- 平均PQLI得分从入学时的75.6增加到第12个月的85.4 (p<0.05).
- 患者对该计划的满意度很高,第6个月的平均得分为9.8,第12个月的平均得分为9.2.
结论:
- 该aXess计划表明,有潜力提高患有 XLH 等慢性疾病的患者的生活质量.
- 计划的关键组成部分包括患者教育,治疗坚持支持,激励面试和频繁的随访.
- 该计划有效地将家庭环境管理与整体疾病护理相结合,促进患者,家庭和护理人员之间的合作.
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