The sickle that cuts deep

This genetic disease affects the ability of red blood corpuscles (RBCs),which acquire the shape of a sickle, to carry oxygen in the body. This leadsto increased viscosity of blood and blocking of the small vessels, resultingin devastating pain. The pain can originate in virtually any organ, joint orbone in the body and can strike any time. The disease carries the risk ofdebilitating fatigue, blindness, organ damage, stroke and a life-span ofjust about 30 years.

The disease’s symptoms include anaemia, hand foot syndrome and infection.The usual disorders are vaso-occlusive crises (severe pain in almost allorgans), haemolitic crises (yellow eyes, hepatitis infection), aplasticcrises (production of RBCs stops) and the deadliest of disorders,segestration crises (blood goes to spleen). There are two types of sicklecells: AS (a carrier of the disease trait who does not suffer) and SS (thesufferer).

The disorder was first reported in India by Lehman and Cutbush and Dunlopand Muzumdar in 1952. According to figures provided by the Sickle CellSociety of India (SCSI), SCD is found in almost 12 Indian states and oneUnion Territory. About 300 population groups were studied, and theprevalence rate was found to be staggering a 0-44 per cent, with the highestbeing 22.5 to 44.4 per cent in central India.

The study also dispelled the belief that SCD is confined to Scheduled Tribesand lower castes only. Rather, the gene is widely prevalent in India,passing through different castes and communities. In Maharashtra alone, morethan 16 districts have been affected. As per the Tribal Research Project ofBJ Medical College, Pune, out of 15 lakh carriers in the tribal districts ofGadchiroli and Dhulia, 1.5 lakh people are suffering from SCD. Yet,successive governments have failed to understand its seriousness, despitethe fact that another disease in the same category of haemoglobonopathiesthalassaemia gets all treatment facilities.

In the US on the other hand, where the occurrence is as low as one person inevery 400, the government has passed a legislation making it mandatory tocarry out SCD detection tests on newly-borns. Patients are treated rightfrom their birth to the age of ten years, and a follow-up action isinitiated if necessary. A separate budgetary provision has also been madefor this purpose.

In sharp contrast, countless Indians are not even aware of this disorder.However, manifestation of the symptoms differs region-wise, says Dr DiptiJain, Associate Professor, Government Medical College (GMC), Nagpur, who hasbeen treating SCD patients for the last 20 years.

President of the Sickle Cell Society of India (SCSI), ST Ramteke, who worksin rural areas, says, “The disease is mostly found in the poorer classes.These people cannot go to health centres, hence the government should reachthem by conducting mass surveys in the affected parts. But the mostimportant thing is to create awareness.” SCSI, in association with theregional centre of the Anthropological Survey of India, has undertaken aproject to identify the SCD in Nagpur.

Dr Jain says those who come to her have little inkling about the disorder.“They are shattered when they learn about the disease, but then they followmedical advice to minimise suffering, though a complete cure is in nobody’shands.” According to her, the disease is diagnosed very late in most of thecases because we lack awareness regarding the neonatal, pre- andpost-pregnancy screenings.

Dr Jain informs that there are ways to minimise the suffering. A regularintake of folic acid tablets reduces the trauma considerably and improvesthe life span, but there is only one way to stop the malady’s furtherspread: avoid marriages between the carriers and sufferers. Even if theymarry and conceive, says Dr Jain, the birth of affected children must beavoided by using pre-natal diagnostic techniques (termination of suchpregnancies is approved under the Regulation and Prevention of Misuse Act1994).

However, Ramteke says it is very hard to convince people from rural andtribal areas not to arrange marriages in this manner. It is also difficultto convince the upper castes that SCD is confined to specific castes only.Dr Dilip Arjune, a Nagpur-based pathologist, says he has investigated SCD inupper castes like Brahmins, Telis and Kunbis. “The upper castes think thatSCD can never strike them. But the myth has been broken. Now they shouldcome forward to get themselves tested,” he says.

What’s needed is an area-specific and exclusive control programme in theaffected regions. It is also necessary to inform medical practitioners whohave failed to adequately guide patients. Every primary health centre shouldhave diagnostic facilities and a genetic laboratory that should beaccessible and free of cost for the poor. Immunisation programmes foraffected children is also a must.

Ramteke decries the discrimination between SCD and thalassaemia patients,who get free blood for transfusion from the state government, and can alsoavail of concessions in rail fare. This, despite the fact that there aremore than 4,000 SCD patients, compared to 100 suffering from thalassaemia(which has medically been proved to be found only in communities who havetheir roots in business and trade activities) in Nagpur alone, says Ramteke.The Indian Council of Medical Research (ICMR) had long ago selected GMCNagpur for the SCD project. However, its pediatrics wards cannot even supplya regular quota of folic acid tablets. Dr Jain pays from her pocket to buy amonthly quota of folic acid tablets. “It’s my little contribution to acause I feel strongly for,” she shrugs.

However, all is not lost. ICMR has allotted major projects to Nagpur,Sambalpur, Valsad and Kotagiri in the Nilgiris. The Nagpur project isexclusively for the tribals of Ghadchiroli and Yavatmal districts. Based inHemalkasa, where Dr Prakash Amtes’s Lokbiradari is already serving thetribals, the project is managed by Dr Dipti Jain.