Oman College of Health Sciences, Muscat, Oman.
University of California Los Angeles, Los Angeles CA, US.
Nurs Child Young People. 2023 Jul 6;35(4):22-27. doi: 10.7748/ncyp.2023.e1448. Epub 2023 Jan 9.
Sickle cell disease is an inherited haematological condition with life-threatening consequences. It can affect all aspects of the lives of children with the condition, including biopsychosocial and cognitive aspects. These children tend to have a low health-related quality of life (HRQoL).
To identify factors associated with HRQoL in Omani children with sickle cell disease.
The study was a secondary analysis of data from a randomised controlled trial conducted with 72 parent-and-child dyads who were recruited from two tertiary hospitals in Oman. The aim of the original study was to examine the effects of an educational programme on the knowledge and self-efficacy of parents of children with sickle cell disease. As part of that study, parents and children completed two questionnaires on HRQoL, one generic and one specific to sickle cell disease.
Parents' knowledge of sickle cell disease, parents' self-efficacy in managing their child's symptoms, parents' age, children's age and treatment with hydroxyurea were found to affect children's HRQoL.
Healthcare providers need to include biopsychosocial and cognitive aspects of HRQoL in their assessments of children with sickle cell disease. Programmes designed to enhance parents' and children's knowledge and self-efficacy, as well as measures designed to ensure that children receive treatment with hydroxyurea, are likely to improve the HRQoL of children with sickle cell disease.
镰状细胞病是一种遗传性血液病，可导致危及生命的后果。它会影响患病儿童生活的各个方面，包括心理社会和认知方面。这些孩子往往生活质量相关健康状况（HRQoL）较差。
确定与阿曼镰状细胞病儿童 HRQoL 相关的因素。
该研究是对在阿曼两家三级医院招募的 72 对父母-儿童进行的随机对照试验数据的二次分析。原始研究的目的是检查教育计划对镰状细胞病儿童父母的知识和自我效能的影响。作为该研究的一部分，父母和孩子完成了两份关于 HRQoL 的问卷，一份是通用的，一份是针对镰状细胞病的。
发现父母对镰状细胞病的了解、父母管理孩子症状的自我效能、父母年龄、孩子年龄和羟基脲治疗与孩子的 HRQoL 有关。
医疗保健提供者需要在评估镰状细胞病儿童时纳入 HRQoL 的心理社会和认知方面。旨在增强父母和孩子的知识和自我效能的计划，以及旨在确保孩子接受羟基脲治疗的措施，可能会改善镰状细胞病儿童的 HRQoL。
