A REPORT ON NEW FINDINGS IN VIEW OF REVISING ON HOW TO PREVENT MAJOR THALASSEMIA AND SICKLE CELL ANEMIA IN KHUZESTAN PROVINCE

Thalassemia is one of the most common genetic diseases in Iran, especially in Khuzestan province. It is classified into 4 groups: minima, minor, intermediate and major. It has a complex genetic structure with more than 170 different gene mutations types of inheritance. All of which are in the form of autosomal recessive. Due to the high prevalence of -Thalassemia minor (nearly 10%), in addition to the presence of sickle cell and -thal minor genes in Khuzestan province, it is necessary to collect and designing a comprehensive direction in order to provide birth control measures in new wedding couples as well as gene-carrying couples. Prudently, it is clear enough that prevention of birth a newborn affected by major thalassemic or similar diseases not only is an important measure in the pathway towards promotion in the quality of community health services, but also decreases the profound financial burden forced on health and treatment system. The specific prevailing condition in this province, where the presence of different ethnic and tribal families (Arabic, Sadat and Ghanavati) as well as Mahshahr, Hendijan population in whom there is an abundance of sickle cell anemia and -gene, making the present ministerial circular not to be comprehensive enough for this province and which dose not take account as appropriate actions that facilitate limiting the spreading of this disease among this population. This study was conducted on 162 couple-to-be volunteers from Arab families in Dasht-Azadegan and Khoramshahr, 3.63% of couples in Dasht –Azadegan and 10.57% of couples in Khoramshahr were carriers for sickle cell gene. However, despite the fact that among these 84.21% had normal RBC indices (MCV and MCH), 1-hour sickle prep test was positive in all. In addition, among 25 couple-to-be volunteers in Ahwaz who had near normal RBC indices (MCV and MCH), showed to suffer from silent -thalassemia after complementary tests.It is worth noting that in Khuzestan province, some cases of Hb j, C, and D genes are present too, which due to their low prevalence are not included in presen preventive direction. In planning the new proposal, the special prevailing condition in Khozestan was specifically considered and this may bring about better utilization of the health resources in this province, both in terms of cost-effectiveness and cost benefits which are the aims of all specialties and authorities involved.