Health-related quality of life of children and adolescents with sickle cell disease

Children and adolescents with sickle cell disease (SCD) may experience physical symptoms and psychosocial problems compared to healthy peers. This includes lower academic achievement due to frequent absence from school, and increased illness or pain. Sociocultural factors may exacerbate psychosocial issues. These factors may include perceived stigmatisation, reluctance to disclose, cultural and religious beliefs, family dynamics and financial hardship. Thus, SCD is likely to impact on health-related quality of life (HRQL). HRQL is a broad, multidimensional concept of well-being which considers a person’s physical, social, emotional and school/work functioning. Scales such as the Pediatric Quality of Life Inventory (PedsQLTM) are often used to assess HRQL and can be completed by children or their parents by proxy. Child self-reports of HRQL are generally higher than parent proxy-reports, but children with SCD often report lower levels of HRQL compared to healthy peers. Lower HRQL in children with SCD has been linked to demographic indicators, such as being from a single-parent family, disease-related problems, like the frequency of vaso-occlusive crises, and psychosocial factors such as depressive symptoms. The limited qualitative research in this area suggests that pain, or attempts to prevent vaso-occlusive crises, may interfere with children’s lives and undermine HRQL. However, there may be little discrepancy between children’s perceived current and ideal selves suggesting they have normalised and adjusted to SCD. Problem-focused coping strategies including engaging in some health-related behaviours may be useful. Research has focused on Western countries and more research is needed in Sub-Saharan Africa, especially due to differences between nations in culture, economics, healthcare, education and employment which are all likely to impact HRQL.

This is an Accepted Manuscript of a book chapter published by Routledge in Sickle Cell Disease in Sub-Saharan Africa: Public Health Perspectives on April 30, 2024, available online: http://www.routledge.com/9781003467748 .
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