International Medical Journal Vol. 25, No. 4, pp. 211 - 214 , August 2018 INTERNAL MEDICINE A Descriptive Study on Quality of Life among Adolescents with Beta-Thalassemia Major in the Maldives Shanooha Mansoor 1) , Zahiruddin Othman 2) , Azizah Othman 2) , Maruzairi Husain 2) ABSTRACT Background: The Maldives has the highest prevalence of thalassemia in the world. However, there is little research done on the psychosocial aspects of this illness. Objectives: This study aimed to examine health related quality of life (HRQOL) among adolescents with beta-thalassemia major attending the National Thalassemia and Other Hemoglobinopathies Centre (NTC), Maldives Blood Services, Maldives. Thus, appropriate recommendation could be proposed. Methods: A total of 81 adolescents (mean age 15.7 years) with beta-thalassemia major were engaged. HRQOL was assessed using the Pediatric Quality of Life Inventory (PedsQL). Other relevant information was gathered through interview or medical record. Results: The HRQOL was reduced. The mean for physical, emotional, social, school and psychosocial HRQOL was 80.50, 72.30, 88.18, 76.44, and 78.96, respectively. The total HRQOL was 79.50 and this was lower in females (75.29) compared to males (83.29). Ferritin levels of 1,001-2,499 μg/l and > 2,500 μg/l were noted in 34.2% and 53.9%, respectively. Good compliance was reported in 55% of participants. Conclusions: Adolescents with beta-thalassemia major in the Maldives have reduced HRQOL. The high ferritin level could reduce the HRQOL and hence an attempt should be made towards lowering ferritin and improving compliance to chelation treatments. The gender difference in HRQOL signifies the need for more attention to the female patients and for areas of improvement to be explored. KEY WORDS thalassemia, quality of life, ferritin, iron overload, psychosocial Received on July 27, 2017 and accepted on August 2, 2017 1) Psychiatry Department, Indira Gandhi Memorial Hospital, Male Maldives 2) School of Medical Sciences, Universiti Sains Malaysia 16150 Kubang Kerian, Kelantan, Malaysia Correspondence to: Zahiruddin Othman (e-mail: zahirkb@usm.my) 211 INTRODUCTION The Republic of Maldives is an island nation of 350,000 people located in the lndian Ocean. It consists of 1900 islands, of which 200 are inhabited. It has one of the highest incidences of thalassemia and has the world's highest concentration of carriers at 16-18% of the popula- tion 1) thought to be due to prevalent consanguineous marriages in the past 2) . A total of 670 cases have been registered in the National Thalassemia and Other Hemoglobinopathies Centre. It is estimated that more than 30% of the population has been screened to date and the high percentage of carriers lead to 1 in every 120 child being born is a thalas- semia major child 3) . Poor uptake of screening for the condition is one of the main reasons for this high number of new cases 4) . The Maldivian government identified thalassemia as a national problem in the early 1990s, and took initiative involving awareness, education, the prevention and treatment programs. 4) Though thalassemia has been identified as an issue of national concern, the infrastructure still lacks the psychological support this group of adolescents need. The lack of awareness of this important aspect is highlighted by a number of patients as well as family members who were noted to have psychologi- cal and coping issues, among those met at the daycare centre and the NTC institute. Of note was the unintentional omission of this aspect of the disease in any of the support booklets or meetings carried out. Beta-thalassemia major patients require regular blood transfusions which lead to iron overload and its complications. In addition, chronic hypoxia due to anemia further increases the toxicity of iron deposition on various organs. 5) Studies have found that high levels of ferritin are associated with diabetes mellitus, thyroid and parathyroid disorders as well as hypogonadism, with diabetes being the most common 6-9) . Certain complications, especially those related to growth and other metabolic changes like delayed menses, has a negative impact on the thalassemia children. It has been highlighted that children often endured teasing due to their short-stature, and facies as well as "dark" skin colour leading to lower self-esteem, feeling stressed up and anxious 10) . Most studies have highlighted that adherence to subcutaneous iron chelation therapy was poor and the reasons were side effects, finding it difficult and burdensome as well as not wanting to do it 10) . Other studies have found older age, age at the start of treatment and emotional distress to be associated with low levels of adherence 11) . Chronic illness and its treatment are associated with lower health related quality of life (HRQOL) 12) . Similarly, studies had demonstrated HRQOL is significant- ly affected in children with beta-thalassemia major on regular transfu- sion across all age groups, gender and socio-economic classes and also in their caregivers 13-16) . Thus, improving on patients and maternal educa- tion is crucial as these factors are recognized as independent predictor of better HRQOL 17) . At present the Maldives has a good program in place for the medi- C 2018 Japan Health Sciences University & Japan International Cultural Exchange Foundation