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EMU Dr. Fazıl Küçük Medicine Faculty Releases a Statmement on Thalassemia Disease

EMU Dr. Fazıl Küçük Medicine Faculty Releases a Statmement on Thalassemia Disease

Eastern Mediterranean University (EMU), Dr. Fazıl Küçük Medicine Faculty Academic Member Assoc. Prof. Dr. Tayfur Toptaş released valuable information about Thalassemia Disease, which reads as follows: 

"Thalassemia and other hemoglobin diseases are important health problems creating emotional, psychological and economic burdens on millions of people. There have been major developments in the formation mechanism and treatment of thalassemia. However, the difficulty and cost of the treatment have not changed. In economically disadvantaged communities, awareness about disease is still insufficient and access to treatment is difficult. Regarding this, it is extremely important to understand how important the prevention of the disease is.

If no precautions are taken, an average of birth of 60,000 children with beta thalassemia and 240,000 children with sickle cell anemia are expected to be born each year. This is an optimistic calculation. The real expectation is far above this. It is observed that most of these children cannot survive long term if their anemia, which is defined as the low blood red cells carrying oxygen to the blood tissues, is deep in these children, if they encounter treatment and disease related problems and if they do not receive adequate treatment. Therefore; getting appropriate treatment, access to health centers are important for long-term survival and good quality of life. Especially, in the last 25 years, the rates of thalassemia births have been significantly reduced by health education, widespread screening and genetic counseling programs around the world, particularly in countries within the Mediterranean basin.

Red blood cells that carry oxygen to tissues and organs have a structure called hemoglobin. The function of this structure is to connect oxygen. For this reason, impairment in this structure reduces the oxygen carrying capacity of red blood cells. In addition to this, if the hemoglobin structure is impaired, red blood cells are broken into pieces either in the bone marrow or in the spleen. Thalassemia is an inherited disorder that characterized by impairments or deficiencies in the construction of protein building chains involved in hemoglobin structure. Individuals with  this disease may have to take red blood suspensions regularly and consistently, according to the degree of genetic susceptibility. Some patients may not have blood-borne needs, even if they have anemia. The most severe form of thalassemia syndrome is beta thalassemia major. In these patients, anemia is prominently severe, accompanied by other problems involving many organs.

 

Some of these problems include the growth and developmental delays in children, the formation of new focuses that cause blood formation in tissues and organs outside the bone marrow and problems related to them (such as severe pain, impingement on organs, impaired organ functioning, etc.), spleen overgrowth, infections, problems related to the regular blood donations, cardiac dysrhythmia, cardiac insufficiency, iron overload, cirrhosis, retardation in sexual development, retardation in bone development, osteoporosis, vascular occlusions, psychological problems, heart problems, diabetes, and infection from blood products.

If these patients are not properly treated, their expected life span is between 5 and 10 years. The most important issue about the disease is prevention of the disease. For this purpose, screening of carriers and genetic counseling are important factors in the creation of a standard policy. The treatment methods of the disease are blood transfusion policies according to international standards and the removal of iron from the body. It is also very important for patients to be monitored in terms of the other side effects mentioned above. In short, through a conscious society, genetic diseases as such will diminish. "

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Published Date
June 6, 2018