Thalassemia _ Maldivian standpoint
By: (mustho)
Maldives is a country with a distinctive geography that portrays a model of a small island nation. The total landmass of the 1190 small islands of the country is less than one percent of its national territory. Population of the 200 inhabited islands varies considerably. The country's 2006 population census shows a total of 298, 842 people living in the country.
Origins of the Maldivians are not very certain. However, it is believed that the history goes as far back as BC 1000. Early settlers were travelers on the Silk Route, and from the Indus Valley Civilization. The Maldivians are innately warm, friendly and generous by nature, and anyone can easily establish a casual conversation with the Maldivians.
It is believed that the genetically inherited heterogeneous disease Thalassaemia perhaps been introduced to Maldives through traders routes, how ever there is no scientific evidence to prove this, whether the gene is due to a mutations in the midst of Maldivian population or due evolutionary changes of malarial endemics can be of research interests . The earliest case of Thalassaemia diagnosed goes back to early 1970s. During 1980 -1990 there was dramatic increase of Thalassaemia patients in the country, due to this special clinics for these patients were been made in the paediatric ward of the hospital. The impact of Thalassaemia and its social stigma has been recognized by the government and many experts from various countries have been brought in collaboration with WHO during 1980s. Ever since 1992 one of the leading NGO in the country ,Society for Health Education (SHE) working towards well being of Maldivians health has started one of its essential element, Thalassaemia Prevention Program, comprising ,health education , population screening and genetic counseling .During 1993 National Thalassaemia Program has been formulated and in December 1994 National Thalassaemia Centre has been inaugurated with 17 beded ward , blood banking facilities and a diagnostic laboratory services.
The enormous contribution of many people in both Government and NGOs across the country, thalassemia awareness has increased dramatically and now the word thalassemia is a house hold story amid all the alliance of communities. The combine effort of these institutions has screened thalassemia and other hemoglobinopathies over 30 % of the entire population in the archipelago using modern diagnostic methodologies, including hemoglobin electrophoresis, automatic cell counting and quantification of hemoglobin. Some collaborative studies has been done with International Thalassaemia Research Institutions abroad, such as molecular defect of Beta Thalassaemia in the Maldives has been studies and their prevalence has been identified (74% being IVS 1 5 G C ) ( Journal of Hemoglobin 1998, Fucheron et al ). This knowledge has contributed and paves the way to guiding principle for the prevention of thalassemia for the sustainable development of health sector in the country.
Around 18.1% of the Maldivian population is found to be carriers for hereditary blood disorder, beta thalassemia. This prevalence level leads to a birth of a thalassemic child (thalassemia major) for every 120 births. At the end of the year 2005, nearly 581 thalassemia majors were registered at the National Thalassaemia Center.
Due to the impact of emerging issues of thalassemia and its impact to the community directly and indirectly, a six year National Thalassaemia Program has been formulated, many areas have been highlighted and its on the way for implementations. This includes, population screening, thalassemia education, prenatal diagnosis (PND) and research. In near future PND can be done in Maldives with the establishment of molecular biology laboratory in the country. The couples at risk can be counseled and if they desire medical termination of pregnancy (MTP) can be recommended.
Screening for thalassemia, as well as treatment of thalassemics, is costly thing any where in the world as it the same in the Maldives. Thalassemic children require continuous and regular care and treatment to stay alive. They require monthly transfusions and treatment with the drug Desfeal, injected five times a week. The annual cost of treatment exceeds US$6,000. At present only, Bone Marrow Transplant (BMT) ensures permanent cure for Thalassemics. But the cost of this treatment ranks between US$30,000 and US$50,000. Due to the low income of average people, this costly treatment BMT cannot be afforded by many families. Since Maldives is a small country at the moment services such as allogenic BMT are not available and those who can afford has to go for neighboring countries for these treatments.
The perspective of Thalassaemia seems to be a successful story in Maldives, as it is integrating government and non governmental efforts to control thalassemia in Maldives. A look at the expectations therefore indicates the need for a more comprehensive incorporated program to strengthen the existing agenda for the prevention of thalassemia in Maldives.