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Dr. Anuradha Shrikhande, Dr. Dipty Jain, Dr. Shankar Khobragade and Dr. Vinky Rughwani, experts in sickle cell disease, discussed the different ways of sickle cell disease. The two-day hematology masterclass was organized by the Association of Indian Doctors of the Nagpur Academy of Pediatrics and the Association of Pathologists and Microbiologists of Vidarbha.
Speaking about the prevalence of the disease, Dr. Shrikhande said that sickle cell disease was a burning issue in Nagpur as well as in Maharashtra. According to the Indian Council of Medical Research's 1998 statistics, the prevalence of sickle cell disease in central India is 22-44%, and the situation has not changed much. "Most of those affected here come from lower socioeconomic groups, are less literate and have financial problems," she said.
In this disease, iron deposits take place in the joints, causing pain. A program to fight sickle cell disease has been put in place to prevent the next generation from contracting it. "Even today, tribal populations are unaware of the existence of the disease and are diagnosed accidentally during testing camps," said Dr. Shrikhande, adding that it was necessary to combine the diagnosis of sickle cell disease and thalassemia.
Sickle cell disease is an inherited autosomal recessive disorder, said Dr. Shankar Khobragade, speaking of a vaso-occlusive crisis, a common painful complication of sickle cell disease in adolescents and adults. "In this state, sickle cell cells stop blood flow and can lead to acute thoracic syndrome and cardiac death, apart from other factors," said Dr. Khobragade.
"Blood transfusion is not routinely needed in sickle cell patients," said Dr. Vinky Rughwani, pointing out that it resulted in a variety of side effects – infection, increased iron levels, and organ damage – in the body. framework of sickle cell disease. "Blood should only be given to a patient when his hemoglobin is less than 6 g and has acute weakness," he added.
Dr. Rughwani also highlighted the benefits of hydroxyurea, a drug used in sickle cell disease. "This increases the hemoglobin level, decreases the number of attacks and reduces the frequency of blood demands, among other things," said Dr. Rughwani.
"Out of 100 sickle cell patients, only about five will need a bone marrow transplant," said Dr. Dipty Jain, speaking about advances in the field of sickle cell disease treatment. "There is an 80% chance of successful transplantation of a compatible donor," she said, adding that the universal stem cell registry was able to identify donors with HLA antigens ( human leukocyte antigen) corresponded in all countries.
"Gene therapy helps correct the gene defect, but the technology will take longer to come to India," she said.
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