Assessment of Cognitive Function and Quality of Life in Thalassemic Children at Sohag University Hospital

Thalassemia

Thalassemia syndromes are a heterogeneous group of single gene disorders, inherited in an autosomal recessive manner ,prevalent among all ethnic groups and in almost every country around the world . Once a child has been diagnosed as thalassemia, he has to take lifelong treatment , where cure is not attainable and treatment may be prolonged . It is a life-threatening and life-limiting condition that affects the patient clinically and psychologically, so Health-related Quality of Life (HRQOL) is likely to be an essential outcome for these patients. Quality of life in thalassemic children such as : Repeated visits to hospitals for regular blood transfusion, cost of chelation therapy, repeated laboratory tests for monitoring therapy and for early detection of any complications. also life-long costly therapy along with poor quality of life will have adverse impact on the family. A better understanding of the factors associated with HRQOL among children with thalassemia could have a direct effect on the development of more suitable clinical, counselling and social support programs to enhance treatment outcomes. Cognitive dysfunction was Reported either due to the disease or its treatment ,frequent school absences, frequent hospitalizations, and physical and social restrictions lead to cognitive dysfunction . This neurological involvement in thalassemic children is primarily silent, with subclinical manifestations that can only be detected by cognitive assessment tests.

Thalassemia syndromes are a heterogeneous group of single gene disorders, inherited in an autosomal recessive manner ,prevalent among all ethnic groups and in almost every country around the world . Thalassemias are genetic diseases characterized by a deficit in the production of hemoglobin, the protein contained in red blood cells that transports oxygen in the bloodstream. The disease is qualified as α-thalassemia or β-thalassemia depending on whether the anomaly results from a defect in the synthesis of the alpha (α) chains or beta (β) chains making up the protein. It is the most prevalent chronic hemolytic anemia in Egypt (85.1%), the carrier rate of thalassemia in 1000 normal subjects was found to be 9-10.2% . Once a child has been diagnosed as thalassemia, he has to take lifelong treatment , where cure is not attainable and treatment may be prolonged . It is a life-threatening and life-limiting condition that affects the patient clinically and psychologically, so Health-related Quality of Life (HRQOL) is likely to be an essential outcome for these patients. The principles of treatment have been well defined which include (a) maintaining hemoglobin between 9 and 10 g/dl by repeated packed cell transfusion (b) regular chelation therapy to maintain serum ferritin around 1000 ng/dl (c) preventing the development of complications of the disease or secondary to therapy (d) ensuring normal growth and development . This conventional treatment is required for life-long. The main objective of thalassemia life-long treatment is to maintain good quality of life as close to near normal life. there are many Factor that can affect Quality of life in thalassemic children such as : Repeated visits to hospitals for regular blood transfusion, cost of chelation therapy, repeated laboratory tests for monitoring therapy and for early detection of any complications. also life-long costly therapy along with poor quality of life will have adverse impact on the family. The assessment of HRQOL in children is essential for the provision of proper care, since it helps in identifying the impact of the disease and treatment on children . A better understanding of the factors associated with HRQOL among children with thalassemia could have a direct effect on the development of more suitable clinical, counselling and social support programs to enhance treatment outcomes. Cognitive dysfunction was Reported either due to the disease or its treatment ,frequent school absences, frequent hospitalizations, and physical and social restrictions lead to cognitive dysfunction . This neurological involvement in thalassemic children is primarily silent, with subclinical manifestations that can only be detected by cognitive assessment tests. Previous studies showed significantly different results between patients and controls regarding the intelligence quotient (IQ).

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