Tuberous sclerosis complex: Concerns and needs of patients and parents from the transitional period to adulthood
Tuberous sclerosis complex: Concerns and needs of patients and parents from the transitional period to adulthood
复制标题
DOI：
10.1016/j.yebeh.2018.03.012
复制
发表时间：
2018-06-01
影响因子：
2.6
通讯作者：
van Eeghen, Agnies
中科院分区：
文献类型：
--
作者：
Both, Pauline;ten Holt, Lyenne;van Eeghen, Agnies
简介：对于患有结节性硬化症 (TSC) 等罕见慢性疾病的年轻人及其父母来说，过渡到成年以及从儿科服务到成人医疗保健都是具有挑战性的过程。成人医疗保健系统通常不太以家庭为导向，缺乏多学科护理和 TSC 经验，这可能导致健康风险和发病率增加。患者驱动的护理需求数据对于优化对这一弱势患者群体的支持是必要的。目的：本研究的目的是探讨年轻 TSC 患者在医学、心理和社会经济领域的担忧和护理需求。方法：通过半结构化访谈对 16 名患者（中位年龄：21 岁；范围：17 至 30 岁）和 12 名家长进行定性研究。使用国际功能、残疾和健康分类 (ICF) 来组织关注点和护理需求。结果：主要关注点涉及精神和身体健康、参与、自我管理技能、计划生育和药物副作用。患者表示需要信息灵通、易于获取且关注患者整体（包括其家人）的多学科护理。父母报告压力水平较高。结论：当前的研究提供了以患者为导向的信息，为促进患有 TSC 的年轻人的护理（过渡）提出建议。除了癫痫发作、肿瘤生长和 TSC 相关神经精神疾病 (TAND) 之外，还需要更多关注针对过渡期、参与和环境因素的担忧和护理需求。成人医疗保健提供者应为 TSC 成人患者提供专业的多学科护理，包括关注父母的压力。 (C) 2018 年由爱思唯尔公司出版
Introduction: Transitioning into adulthood and from pediatric services to adult healthcare are both challenging processes for young adults with rare chronic disorders such as tuberous sclerosis complex (TSC) and their parents. Adult healthcare systems are often less family-oriented and lack multidisciplinary care and experience with TSC, which can result in increased health risks and morbidity. Patient-driven data on care needs are necessary to optimize support for this vulnerable patient group.Aim: The aim of this study was to explore the concerns and care needs of young adult patients with TSC in medical, psychological, and socioeconomical domains.Method: A qualitative study was performed using semistructured interviews with 16 patients (median age: 21 years; range: 17 to 30) and 12 parents. Concerns and care needs were organized using the International Classification of Functioning, Disability, and Health (ICF).Results: Main concerns involved mental and physical health, participation, self-management skills, family planning, and side effects of medications. Patients expressed the need for multidisciplinary care that is well-informed, easily accessible, and focused on the patient as a whole, including his/her family. Parents reported high stress levels.Conclusion: The current study provides patient-driven information, allowing recommendations to facilitate the (transition of) care for young adults with TSC. In addition to seizures, tumor growth, and TSC-associated neuropsychiatric disorders (TAND), more attention is needed for concerns and care needs specific to the transitional period, participation, and environmental factors. Adult healthcare providers should offer expert multidisciplinary care for adult patients with TSC, including attention for parental stress. (C) 2018 Published by Elsevier Inc.