Clinical features and health-related quality of life in children with sickle cell disease in Khartoum, Sudan.

INTRODUCTION: Sickle cell disease (SCD), a chronic hematologic disorder, is linked to higher morbidity and mortality rates. SCD is thought to impact 0.8% to 30% of Sudanese, making it one of the most common genetic diseases in the country. Patients with sickle cell disease may experience negative effects from the disease. In this study, children with SCD were evaluated at the selected hospitals in Khartoum state to assess their health-related quality of life (HRQOL). MATERIALS AND METHODS: A cross-sectional facility-based study was conducted at five governmental pediatric hospitals in Khartoum state and their referral clinics. A four-section questionnaire (sociodemographic information, clinical characteristics, barriers to accessing health care, and the standardized PedQL™ Generic Core Scale) was employed. Total coverage was used. The statistical software for social sciences (SPSS) was used to analyze the data and determine the correlation between the variables. RESULTS: We included 336 children with sickle cell disease, 191 (56.8%) males. Most participants were from Gaafar Ibnauf Specialized Hospital (n = 286, 85.1%). The majority were outpatients (77.4%), and 52.7% had a family history of the disease. Blood transfusions were required for 59.5% of patients, while 83.0% never needed exchange transfusions. The most common initial manifestation was painful crises (55.7%), while arthritis (49.4%) became a frequent symptom. The median total HRQOL score was 73.75 [57.50, 87.50]. The highest median HRQOL scores were in East Darfur, River Nile, and Central Darfur, while the lowest were in White Nile and Blue Nile. Factors significantly influencing HRQOL included hospital setting, number of affected siblings, rural residence, and father's employment. Linear regression showed that having more affected siblings was associated with higher HRQOL, while rural residence lowered HRQOL in older children. Father's employment improved HRQOL in younger children, while consanguinity had a negative impact. CONCLUSIONS: Children with SCD had poor HRQOL, with low scores across multiple HRQOL domains. Factors such as consanguinity and father's unemployment negatively affected patients' HRQOL. Interventions are required to address such factors, and to alleviate the impact of barriers to healthcare access.