FRATHEMO: Quality of Life and Adjustment Among Siblings of Children and Adolescents With Severe Hemophilia.
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 30
- 试验地点
- 1
- 主要终点
- Global quality of life
研究概览
简要总结
Severe hemophilia is a rare and chronic disease characterized by spontaneous bleedings from early childhood, which may lead to various complications especially in joints. The diagnosis of this disease, but also its long term care have an impact on the relatives of the affected persons, including the siblings who bear daily the cognitive, emotional and social impacts of the disease.
Studies conducted in the framework of pediatric chronic diseases showed that siblings of affected children presented a higher prevalence of physical and psychological troubles (emotional distress, behavioral disorders, etc.) than siblings who were not concerned by a disease. Few studies have been conducted in the framework of severe hemophilia, and to our knowledge, no study addresses this issue in France.
详细描述
-The assessment of the quality of life would allow assessing both positive and negative impacts, by exploring in a global manner the children and adolescents functioning in various domains (physical and psychological well-being, relations with parents and friends, school...).
Regarding the specific context of hemophilia:
- Few studies have been conducted on the experience of severe hemophilia of a young patient by the siblings, and to our knowledge, no studies have been conducted in France on this topic.
- There are numerous studies concerning the impact of chronic diseases such as diabetes or cancer of a child on the siblings, contrary to those concerning rare diseases. Hemophilia, a frequent disease among rare diseases, might be an interesting model in order to understand the impact of this type of disorder on the functioning of the siblings.
- Contrary to insufficiently treated hemophilia potentially leading to disability, well-treated hemophilia might be considered as an at-risk disorder. In this specific context, the representation of the disease by the siblings might be singular.
- Parents are the major actors of the surveillance of the affected young patient in the everyday life. The transfer of parental responsibilities to healthy siblings, and its impact on the fraternal relationships might therefore be increased. Gender might have a specific modulating role on these elements (risk of transmission in female and different representation of the social role).
- Results presented in the literature are reported from studies whose representativeness of the study sample (or sample size) is questionable. The cohort of the French National Network FranceCoag represents at an international level a rare exhaustive population of persons with severe hemophilia followed over time.
Objectives The main objective of this study is to understand the mechanisms involved in the quality of the siblings' functioning and interactions in the context of severe hemophilia, in order to be able to propose adapted support modes.
The secondary (and operational) objectives are:
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 8 Years 至 18 Years（Child, Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Sibling (brother, sister, half-brother or half-sister) of older children or adolescents included in the TRANSHEMO study, who lives in the same home)
- •Sibling aged 8-18 years old
- •Sibling affiliated to the French social security system
- •Sibling whose parents are not opposed to the participation in the study
排除标准
- •Sibling (brother, sister, half-brother or half-sister) of older children or adolescents who are not included in the TRANSHEMO study)
- •Sibling aged under 8 or over 18 years old
- •Sibling not affiliated to the French social security system
- •Sibling whose parents are opposed to the participation in the study
- •Sibling with difficulties reading and writing
- •Sibling with learning disabilities
结局指标
主要结局
Global quality of life
时间窗: one day
Kidscreen 10 Index, is a self-report measure, consisting of 10 items that are scored on a fivepoint scale, ranging from 1 (not at all/never) to 5 (extremely/ never)
次要结局
- Emotional adjustment(one day)
- Coping strategies use(one day)
- Social support(one day)