Thalassemia as a challenge in India

Pages: 524-525
Aparna Singh, B.D. Bhatia, S. K. Upadhyay and Vineeta Gupta (Department of Pediatrics, Institute of Medical Sciences, B.H.U., Varanasi)

Thalassemia has become a worldwide clinical problem due to increasing immigration of ethnic groups with high prevalence of thalassemia. 30 million people carry thalassemia gene in the world of which approximately 15 million alone are in India. The carrier rate varies from 3 – 17 % in different ethnic groups. Over the past three decades, better medical facilities, regular blood transfusions and iron chelation have improved the quality of life of children with thalassemia and converted it from fatal to chronic disease with prolonged life. Thalassemia is an inherited autosomal recessive blood disease which is characterized by an imbalance in the production of alpha and beta polypeptide chains of haemoglobin. The goal of medical care is not only to achieve long term survival but also to achieve the best possible psychological, social and spiritual wellbeing. With increased life expectancy of these patients, complications keep arising such as transfusion related infections, allosensitization, iron- overload related cardiac, endocrine and liver disturbances, toxicities of iron chelators, hepatic involvement, neurological complications and psychological manifestations. These can manifest as anxiety, nervousness, irritability, demanding a lot of attention, depression, etc. in the child. This paper emphasizes the fact that these children may require something more than simple medical management and also psychological support. In short, beta thalassemia has a spectrum of varied manifestations and complications. We need to develop preventive strategies like pre- marital screening, genetic counselling, ante- natal diagnosis and educational programs for awareness. Apart from early diagnosis, there is also a need to increase medical facilities which is a great challenge facing India .This paper gives a brief overview of the disease and focuses on the behaviour of children with thalassemia. We have described their psychological symptoms and strongly recommend psychological interventions as part of effective management plan.