Department of Pediatrics and Child Health, Makerere University, Kampala, Uganda.
Department of Pediatrics and Child Health, Kamuzu Central Hospital, Lilongwe, Malawi.
Pediatr Blood Cancer. 2022 Oct;69(10):e29876. doi: 10.1002/pbc.29876. Epub 2022 Jul 20.
Sickle cell disease (SCD) is characterized by both acute and chronic complications that affect the daily lives of patients and lower their quality of life.
To describe the health-related quality of life (HRQoL) and the associated factors in children aged 8-17 years with SCD attending the pediatric hematology clinic at Kamuzu Central Hospital (KCH) in Lilongwe, Malawi.
A mixed-methods cross-sectional study was conducted at KCH. Patient data were collected with the aid of a standardized case report form. HRQoL was assessed using PedsQL™ Sickle Cell Disease Module by the child's report. Associations between HRQoL scores and independent variables were evaluated by a linear regression model. In-depth interviews were then carried out and the qualitative data were analyzed using content thematic analysis.
A hundred and sixty-three children with SCD were enrolled and 52.1% were females. Their median age was 11.2 ± 2.7 years. The mean global HRQoL score of the children was 62 ± 17.3. The highest scores were in the treatment domain (72.5 ± 15.1) while the lowest scores were in the emotions domain (55.2 ± 28.7). The mean pain score was 58.8 ± 16.3. The factors associated with low HRQoL scores were pain (β-coefficient -6.97 CI (-3.07,-15.58); p value .034) and low hemoglobin levels (β-coefficient 2.29 CI (.65-3.91); p value .006).
The HRQoL of this population is low. Pain and low hemoglobin were significantly associated with low HRQoL scores. Adequate treatment to control pain and increase the steady-state hemoglobin may improve the HRQoL of children with SCD. Interventions to address low emotional scores are recommended.
镰状细胞病（SCD）的特征是既有急性并发症也有慢性并发症，这影响了患者的日常生活并降低了他们的生活质量。
描述在马拉维利隆圭的卡姆祖中心医院（KCH）儿科血液科就诊的 8-17 岁 SCD 患儿的健康相关生活质量（HRQoL）及其相关因素。
在 KCH 进行了一项混合方法的横断面研究。通过标准化病例报告表收集患者数据。通过儿童报告使用 PedsQL™镰状细胞病模块评估 HRQoL。通过线性回归模型评估 HRQoL 评分与独立变量之间的关联。然后进行深入访谈，并使用内容主题分析对定性数据进行分析。
共纳入 163 名 SCD 患儿，其中 52.1%为女性。他们的中位年龄为 11.2 ± 2.7 岁。儿童的平均总体 HRQoL 评分为 62 ± 17.3。得分最高的是治疗领域（72.5 ± 15.1），得分最低的是情绪领域（55.2 ± 28.7）。平均疼痛评分为 58.8 ± 16.3。与低 HRQoL 评分相关的因素是疼痛（β 系数 -6.97 CI（-3.07，-15.58）；p 值.034）和低血红蛋白水平（β 系数 2.29 CI（.65-3.91）；p 值.006）。
该人群的 HRQoL 较低。疼痛和低血红蛋白与低 HRQoL 评分显著相关。充分治疗以控制疼痛和增加稳定状态的血红蛋白可能会提高 SCD 患儿的 HRQoL。建议采取干预措施来解决情绪评分低的问题。
