Health-Related Quality of Life of Children and Adolescents with Sickle Cell Disease in the Middle East and North Africa Region

Abstract

Sickle cell disease (SCD) can significantly impair the health-related quality of life (HRQOL) of children and adolescents. This review aimed to assess current evidence regarding the HRQOL of children and adolescents with SCD in the Middle East and North Africa region. A systematic search of various databases was conducted to identify relevant articles, including MEDLINE® (National Library of Medicine, Bethesda, Maryland, USA), Scopus® (Elsevier, Amsterdam, the Netherlands), Cumulative Index to Nursing and Allied Health Literature®, Masader (Oman Virtual Science Library, Muscat, Oman) and EBSCOhost (EBSCO Information Services, Ipswich, Massachusetts, USA). A total of 533 articles were identified; however, only 10 were eligible for inclusion in the final analysis. Results from these studies showed that children and adolescents with SCD had compromised HRQOL compared to their healthy peers, particularly in terms of physical, psychosocial, familial, financial and academic functioning. Therefore, interventions are necessary to improve overall HRQOL outcomes for this population. Keywords: Sickle Cell Disease; Health-Related Quality Of Life; Infants; Children; Adolescents; Middle East; North Africa.

Common chronic conditions in pediatric primary care

“…At an estimated prevalence rate of 2.6%, eastern Saudi Arabia accounts for the highest number of SCD cases in the Middle East. 56 Young children and adolescents are especially prone to long-term complications like vaso-occlusive crises, acute splenic sequestration episodes, hemolytic and aplastic crises, infarction, and necrosis. 56 G6PD deficiency is another type of inherited disease which causes hemolytic anemia and jaundice, including neonatal jaundice, on exposure to oxidative stress.…”

“…56 Young children and adolescents are especially prone to long-term complications like vaso-occlusive crises, acute splenic sequestration episodes, hemolytic and aplastic crises, infarction, and necrosis. 56 G6PD deficiency is another type of inherited disease which causes hemolytic anemia and jaundice, including neonatal jaundice, on exposure to oxidative stress. Although G6PD deficiency is an X-linked recessive trait with a high male predominance globally, data from Saudi Arabia have shown a more balanced gender distribution, hypothesized to arise from higher inactivation of normal Xchromosome in heterozygous females, owing to the higher rates of consanguinity.…”

The Impact of Sickle Cell Disease on Academic Performance among Affected Students

“…Sickle cell disease (SCD) is a genetically inherited hematological disorder that affects red blood cells (RBCs) and results in abnormal hemoglobin (Hb) [ 1 , 2 , 3 ]. Sickle cell anemia (SCA) is the most common and severe hereditary form of SCD [ 1 , 3 ].…”

“…Sickle cell disease (SCD) is a genetically inherited hematological disorder that affects red blood cells (RBCs) and results in abnormal hemoglobin (Hb) [ 1 , 2 , 3 ]. Sickle cell anemia (SCA) is the most common and severe hereditary form of SCD [ 1 , 3 ]. Affected individuals are more likely to experience painful episodes known as vaso-occlusive crisis (VOC), which are considered a hallmark of the disease and which occur due to occlusion of small blood vessels by abnormally shaped RBCs [ 1 ].…”

“…The Middle Eastern and North African regions have a higher prevalence of SCD than the rest of the world [ 3 ] due to multiple factors, such as consanguineous marriages among first-degree relatives, lack of effective screening programs, and malaria [ 5 , 6 ]. In Africa, it is estimated that about 12 to 15 million have SCD [ 7 ].…”