Nagpur: Senior haematologist Dr Dilip Gohokar recalled how 30 years ago, three-to-four patients admitted with
sickle cell disease would die every week, and there wasn’t much that could be done about it. “With the advent of new techniques and medicines, the situation has changed,” he said while initiating the session ‘Public awareness for Sickle Cell Disease’ at the Haematology Master Class 2018 on Saturday.
Sickle cell experts Dr Anuradha Shrikhande, Dr Dipty Jain, Dr Shankar Khobragade and Dr Vinky Rughwani discussed different modalities of sickle cell disease. The two-day hematology masterclass has been organized by Association of Physicians of India, Academy of Paediatrics, Nagpur, and Vidarbha Association of Pathologists and Microbiologists.
Talking about the prevalence of the disease, Dr Shrikhande said that sickle cell is a burning issue in Nagpur as well as across Maharashtra. According to the 1998 statistics by Indian Council of Medical Research, prevalence of sickle cell in Central India is 22-44%, and the situation hasn’t changed much. “Here, most of the affected individuals come from lower socio-economic strata, are less literate and have financial problems,” she said.
In this disease, iron deposition takes place in the joints which causes pain. Sickle cell control programme has been put in place to prevent next generation from contracting it. “Even today, tribal population is unaware of the disease and they are diagnosed accidentally during screening camps,” said Dr Shrikhande, adding that there is a need to combine sickle cell and thalassaemia diagnosis.
Sickle cell anaemia is an inherited autosomal recessive disease, said Dr Shankar Khobragade, while speaking of vaso-occlusive crisis, a common painful complication of sickle cell anaemia in adolescents and adults. “In this condition, sickle cells stop blood flow and can cause acute chest syndrome and cardiac death, apart from other outcomes,” said Dr Khobragade.
“Blood transfusion is not regularly needed for sickle cell patients,” said Dr Vinky Rughwani, pointing out that it has various side-effects — infection, iron increase and spoiling of organs — in sickle cell disease. “Blood should be given to a patient only when his haemoglobin is less than 6gm and feels acute weakness,” he added.
Dr Rughwani also highlighted the benefits of hydroxyurea, a medication used in sickle-cell disease. “It increases haemoglobin, lessens the amount of attacks and reduces the frequency of requiring blood, among other things,” said Dr Rughwani.
“In 100 sickle cell patients, only about five will need bone marrow transplant,” said Dr Dipty Jain, while talking about the advances in the field of sickle cell treatment. “There is 80% chance of success with transplant from a matched donor,” she said, adding that universal stem cell registry helps identify donors with matching HLA (human leucocyte antigen) across countries.
“Gene therapy helps correct the defect in genes but the technology will require more time to come to India,” she said.
