Abstract
BackgroundThalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. Whilst substantial progress has been made towards the improvement of Health related quality of life (HRQoL) in western countries, scarce evidence-based data exists on HRQol of thalassemia children and adolescents living in developing countries.MethodsWe studied 60 thalassemia children from Middle Eastern countries with a median age of 10 years (range 5 to 17 years). HRQoL was assessed with the Pediatric Quality of Life Inventory (PedsQL) 4.0. The Questionnaire was completed at baseline by all patients and their parents. The agreement between child-self and parent-proxy HRQoL reports and the relationship between HRQoL profiles and socio-demographic and clinical factors were investigated.ResultsThe scores of parents were generally lower than those of their children for Emotional Functioning (mean 75 vs 85; p = 0.002), Psychosocial Health Summary (mean 70.3 vs 79.1; p = 0.015) and the Total Summary Score (mean 74.3 vs 77.7 p = 0.047). HRQoL was not associated with ferritin levels, hepatomegaly or frequency of transfusions or iron chelation therapy. Multivariate analysis showed that a delayed start of iron chelation had a negative impact on total PedsQL scores of both children (p = 0.046) and their parents (p = 0.007).ConclusionsThe PedsQL 4.0 is a useful tool for the measurement of HRQoL in pediatric thalassemia patients. This study shows that delayed start of iron chelation has a negative impact on children’s HRQoL.
Highlights
Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia
Patients and features From November 2007 to August 2008, we performed a cross-sectional study on a group of 60 thalassemia children and their parents, all originally coming from countries of the Middle East (i.e. Kurdistan, Palestine, Libya, Iraq and Syria)
Despite the fact that betathalassemia major was diagnosed at a median age of 8 months and that blood transfusions were started at a median age of 11.5 months, there was a significant delay in the start of iron chelation therapy, reaching a median of 49 months after birth
Summary
Thalassemia is a common disorder worldwide with a predominant incidence in Mediterranean countries, North Africa, the Middle East, India, Central Asia, and Southeast Asia. The introduction of new drugs capable of reducing the accumulation of iron in body organs, the heart, Beta thalassemia major is a serious life-limiting and potentially life-threatening condition that causes substantial disruption in education and social activities. Children often need to miss school because of hospital appointments or admissions for regular monthly blood transfusion and/or treatment of complications. Their self identity is compromised and they become increasingly dependent upon others [4]. Patients and their families are conscious of the disabling nature of the condition and chronic therapy is a constant reminder that they are “different”, making it practically impossible to lead a normal life. The psychosocial burden affects many aspects of the patient’s life such as education, free-time, physical activities, skills, capabilities and family adjustment, the effects of which often result in anxiety, isolation and depression
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