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A new anaemic syndrome
Jyothi, 8, had dropped out of school, when she was in Std. IV. It is not another case of poverty or illiteracy forcing the parents to withdraw her from school. She was unable to sit for long hours in the classroom as she was severely anaemic. She used to suffer from recurring fever, vomitings and jaundice. This had forced her parents to take the hard option.
That was in 1990. The disease was later identified as `sickle cell anaemia'. Jyothi's haemoglobin (Hb) content which was a mere 5.4 grams on March 28, 1990, increased remarkably after treatment and she responded positively to the drugs and her Hb levels were maintained.
She underwent transfusion on three occasions in the last 12 years. She looks normal now though she has not undergone blood transfusion for over three years now. "But for an occasional weakness, I feel absolutely normal,", she says.
Jyothi is not an isolated case, there are hundreds of others, especially in the tribal areas, many of whom are not even aware of the problem. The daughter of a leading advocate in the city died of sickle cell anaemia at the age of two-and-a-half years. His second daughter has shown 90 per cent recovery with the administration of drugs, says S. Muralikrishna, a homoeopath, who has been treating such cases for the past 12 years.
The hereditary disease, which does not have a cure and was earlier believed to have been confined to tribals, is now affecting other communities as well. The affected persons suffer from severe anaemia, growth retardation, frequent jaundice, enlargement of the spleen, recurring fever and swelling of the feet and hands, says P. Veeraraju of the Department of Human Genetics, Andhra University.
"Blood transfusion is the only solution to cure this disease. The number of transfusions to be made depends on the severity of the disease. The genetic counselling centre run by the department is getting hundreds of cases from the three districts of Srikakulam, Vizianagaram and Visakhapatnam besides parts of Orissa and Madhya Pradesh every year", he says.
Unfortunately, a blood test by conventional methods may not be sufficient to detect the occurrence of sickle shaped cells in the blood. While the disease affected persons normally die in their twenties, the carriers can lead normal lives without even being aware of their problem. An electrophoresis test can help in the detection of the disease, avers Prof. Veerraju.
The carriers will, however, have problems in doing rigorous exercises or when having to go at high altitudes, where the oxygen will be less. When carriers of the disease marry, 25 per cent of their progeny are likely to be affected, says M. Ramesh, a faculty member in the department, who has done considerable research in the subject.
Though there are drugs to improve the transportation of oxygen by increasing the foetal haemoglobin in the blood, the drugs can only work to a certain extent. The other solution is bone marrow transplantation, which is currely available only at the Cancer Research Institute, Mumbai.
Gene therapy which is seen as the best solution to treat the disease is still in the stage of clinical trials, he says.
The genetic counselling centre conducts electrophoresis through random selection of individuals to isolate the sickle cell gene. If one of the members is affected, the others in the family would be screened. The carriers are advised against going to high altitudes as they would have difficulty in breathing, says Ramesh.
A soldier from Srikakulam district, who was posted at Kargil during the war, experienced breathlessness at high altitudes. He was sent back to his home town and later referred to the genetic counselling centre which confirmed him as a carrier of the disease and advised him against going to high altitudes, he recalls.
B.M.G.
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