In this study, we explored the lived experience of children with β-thalassemia major (β-TM). We considered children as experts on their experiences in contrast to the prevalent approach of asking parents or other adults about children's perspective. The sample consisted of 12 children, aged 8 to12 years. There were two stages to data collection. In stage one we employed two focus group discussions and two role-plays and analyzed the data thematically. This directly informed stage two, consisting of 12 in-depth interviews subjected to interpretative phenomenological analysis (IPA). From our findings we show that living with β-TM involves a continuous struggle between feelings of being different and strategies to minimize these differences to strive for normalcy. We suggest that understanding the experiences of living with β-TM from children's perspective can provide unique insights into their experiences which can fill the gap in the existing, predominantly adult-oriented, research on chronic illness.
KeywordsAsia, South / Southeast; children, illness and disease; coping and adaptation; developing countries; interpretative phenomenological analysis (IPA); illness and disease, experiences; lived experience Beta-thalassemias are a group of inherited hematological disorders caused by a mutation in the beta (β)-globin chain of hemoglobin. β-thalassemia major (β-TM) is the most severe type, which involves either a complete lack of β-globulin in the hemoglobin or abnormally low β-globulin production causing life-threatening anemia. It occurs worldwide but is more common in Mediterranean populations, the Middle East, Indian subcontinent, and Southeast Asia (Higgs, Thein, & Wood, 2001) and is usually diagnosed within the first year of life. If untreated or under-treated, it can lead to an enlarged spleen and/or liver, bone deformities, and spontaneous fractures. Managing β-TM requires patients to have lifelong access to regular blood transfusions, every two to four weeks. However, because β-TM is associated with increased iron absorption by the gastro-intestinal system, repeated transfusions cause iron overload, which can damage the heart, liver, and other organs (Higgs et al., 2001). Blood transfusions are therefore supported with daily use of chelating agents such as deferoxamine and deferiprone.Existing psychosocial research in β-TM suggests that lifelong blood transfusions and chelation pose major demands on children and their families. Previous studies have reported a higher incidence of depressive and anxiety disorders and an impaired general functioning in β-TM patients (e.g., Sadowski et al., 2002). Clinical complications have also been found to impact negatively on children's self-concept (e.g., Yalçan et al., 2007). Similarly, other researchers have In these conflicting previous studies, the researchers have mainly employed standardized measures of psychosocial functioning which might not be sufficiently sensitive to capture the meanings that children give to their experiences (Woodgate, 1998). Furtherm...