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By Anosh Malekar With the spotlight on lifestyle diseases like diabetes and hypertension, traditional illnesses like sickle cell disease, which affects tribals all across India, are not receiving the attention they deserve
 Devjibhai Rathwa and his family have sickle cell anaemia but do not know the exact nature of the ailment | “I am pleased to lend my voice to the effort… The more people understand the disease, the better we can respond,” United Nations Secretary-General Ban Ki-Moon said recently urging governments, civil society and all other partners to do their bit to improve the quality of life of people with sickle cell anaemia and enable them to live full, productive lives. Ban Ki-Moon’s message on the occasion of the first World Sickle Cell Anaemia Awareness Day, celebrated on June 19, 2009, is an important step in dealing with the disease, an inherited condition that affects hundreds of thousands of babies born each year, mostly in low and middle-income countries. On December 18, 2008, the UN General Assembly adopted a resolution calling for the recognition of sickle cell anaemia as a public health problem and “one of the world’s foremost genetic diseases”. It also called for member states and the UN system to raise awareness about sickle cell anaemia on June 19 every year at the national and international level. The resolution has five major components: recognise sickle cell anaemia as a major health problem, raise public awareness to eliminate harmful prejudices associated with the disease, support health systems and primary healthcare delivery, encourage member states in which sickle cell anaemia is a public health problem to establish national programmes and specialised centres for treatment, and invite international organisations and civil society to support the efforts. Few in India know or care about sickle cell disease, which is largely confined to the adivasi (tribal) hinterlands of the country, from the northeast, across central India, Gujarat, Maharashtra, northern Tamil Nadu, and Kerala. In the new millennium, alarm bells are ringing louder than ever over diseases such as HIV/AIDS, diabetes, obesity, hypertension and heart disease. Sickle cell disease, unlike HIV/AIDS or the more recent swine flu, does not threaten to spill out from the hinterlands to cities, continents and beyond, through easy road and air travel. It originated and remained separately in Central Africa, India and western Saudi Arabia, sometimes travelling with the affected, as in the case of Afro-Americans. The disease is a group of inherited red blood cell disorders known to the peoples of Central Africa for hundreds of years. Clinical manifestations of sickle cell anaemia in India seem to be milder than in Africa. The genetic disease produces its symptoms by causing the human body to synthesise a different type of haemoglobin, called haemoglobin S. Normal haemoglobin, or haemoglobin A, and most of its variants, dissolve in the fluid within the red blood cells. But sickle cell haemoglobin does not dissolve unless it is carrying oxygen. Normal red blood cells are round like doughnuts, and they move through small blood tubes in the body to deliver oxygen from the lungs to the rest of the body. Sickled red blood cells become hard, sticky and comma-shaped, and clog the flow in the small blood tubes. This causes pain, damage and a low blood count, or anaemia. Among India’s tribals, prevalence varies widely. The Genetic Atlas of Indian Tribes, published in the 1970s, states that some adivasi communities lack the sickle cell gene entirely, while in others more than 40% of individuals tested carried the sickle cell trait; however many of these figures are not definitive as the sample sizes involved were quite small. My first encounter with people afflicted with the disease was a little over five years ago while on a visit to Bhasha Research and Publication Centre’s Adivasi Academy at Tejgadh in Gujarat, some 100 km east of Baroda, closer to the Madhya Pradesh border. There I met Devjibhai Rathwa and his family. At first glance, their poor physique appeared a natural fallout of years of sleeping on an empty stomach. Devji’s family comprised daughter Kokila, barely five, who kept complaining of abdominal pain instead of playing with her older brother, Dinesh. Their young mother, Sancharben, was at her wits’ end because the youngest child, Arjun, wouldn’t stop crying. The three-year-old had a painful left ear. Sancharben herself was anaemic and suffered from frequent joint pains. The eldest child, Surekha, a shy eight-year-old, was somehow managing the kitchen and the daily chores. Devjibhai, who was in his early-30s, appeared stunned by the bizarre health problems. He did not know the exact nature of the ailment.  Devjibhai's younger children with identification cards that help doctors and health workers make a quick diagnosis | “Kokila has sickle cell disease, inherited from Sancharben who carries a sickle cell trait,” explained Rajendra Varia, a health worker with Bhasha, who accompanied me. “The other family members need to undergo blood tests. Arjun may have inherited the red blood cell disorder. It is typical to adivasis. It is painful and could kill.” The statistics are frightening. Data collected by Bhasha reveal 30% frequency of the sickle cell trait in the Rathwa community of Chhota Udepur taluka. Approximately 2 million adivasis from Gujarat’s total 7 million population carry the sickle cell trait in their blood. Two to 4% of infants were born with sickle cell disease at the beginning of this millennium. A more recent survey by the Indian Council of Medical Research said 30% of children with sickle cell anaemia in south Gujarat die by the age of 14. “Tribals, even their children, do not (normally) cry. The sickle cell pain alone makes them cry,” observed Ganesh Devy, the Baroda-based writer-activist who founded Bhasha to work among tribals. “The pain can be relieved. But what about the right to have a complete blood cell like every other human being? It should be a cause for alarm if social conditions start making them incomplete or fragmented.” Unrelieved poverty, lack of education facilities, and a non-existent healthcare system are seen to be the traditional villains. Devy suggested I visit Melghat in my home state Maharashtra while reminding me that when the Korku children were dying of hunger a few decades ago, the Maharashtra government had appointed a committee of doctors who declared it was sickle cell anaemia. “The issue is whether history is right or medical science is right,” said Devy. “I am not sure if medical science is absolutely right. Otherwise, completely unrelated adivasi groups could not have been the victims of the incomplete cell disease.” It is possible that malnutrition for generations has imposed this condition of perpetual anaemia on the adivasis. Can getting a square meal be treated as a basic human right? Or does India merely ignore 9 crore adivasi citizens as being genetically defective, Devy asked. The latter seems to be true. Even today, cautious governments have not made public the exact figure of those affected by sickle cell disease. “Identification of people with sickle cell disease is essential. It needs to be treated vigorously and quickly otherwise it could lead to death,” said Dr R Z Patel who spent three decades researching the disease at the department of pathology, Baroda Medical College. “Identification is easy without laboratory tests. Three symptoms -- jaundice, a swollen spleen, and intense joint pain -- are sure signs.” Anaemia, or a low haemoglobin count, is one medical problem caused by sickle cell disease. Red blood cells that sickle have a shorter life expectancy, resulting in the release of haemoglobin remnants into the blood, which causes jaundice. However, sickle cell anaemia is not as big a problem as the acute, sometimes recurrent ‘crisis’ associated with the disease. On average, red blood cells spend one to two seconds in major arteries, one second in micro-circulation and 15 seconds returning to the lungs through veins. In the micro-circulation phase they pass through very narrow blood vessels where healthy red blood cells must fold in half to negotiate. Under certain conditions -- high concentrations of haemoglobin S due to dehydration, lack of sufficient oxygen or other stresses -- red blood cells sickle whilst in micro-circulation. When this happens they tend to get stuck, blocking the red blood cells behind them. This causes loss of large amounts of oxygen, which leads to further sickling of red blood cells. This runaway logjam is called a sickle cell ‘crisis’, a very painful experience because it deprives tissues downstream of oxygen. In early childhood, the small bones of the hands and feet are common sites for the crisis, and in later life the large bones, often causing degeneration of the joints. Blockage crisis may also occur in the central nervous system, causing strokes. Repeated sickling inside the spleen damages it, limiting the body’s ability to respond to bacterial infections. In young children, the spleen sometimes enlarges rapidly, absorbing a large fraction of the body’s blood and causing death by shock. A type of potentially deadly lung crisis known as ‘acute chest syndrome’ may be caused by infection, blood vessel blockage, or a combination of both. “Several treatments exist,” said Dr Patel. “Routine prevention of fever, dehydration, acidosis, cold exposure, and lack of oxygen. Sufferers need to be advised against working in the hot sun. Administration of folic acid helps produce red blood cells.” Unfortunately, Dr Patel added, there are not enough doctors, government or private, to advise the Rathwas in Chhota Udepur. Many tribals die unattended in their hamlets, due to lack of medical attention. “Poor people’s diseases are also poor,” said Dr Devy. In fact, the prevalence of sickle cell disease is one reason the insurance sector is wary of selling their products to the tribals of Chhota Udepur. Whenever there was a crisis, Devjibhai’s family would have to trek 11 km to Bhasha’s health clinic in Tejgadh. The Bhasha clinic functioned on weekends, with three doctors from Baroda volunteering to treat patients. Bhasha’s health activists trudged the back roads to identify affected people. “We fanned out across 10 villages in 2001, collecting blood samples of 1,000. Thirty-three had the disease while 258 had the trait. In 2003, 666 samples were collected out of which 15 had the disease and 168 the trait. Four had died in the interval,” said Varia. The health workers provided simple tips for survival: drink twice as much water as other people, avoid being out-of-breath, avoid too hot or too cold weather conditions, and generally try not to become too stressed either physically or emotionally. More than a year ago, Gujarat Chief Minister Narendra Modi announced a grant of Rs 24.76 crore as part of the state government’s plan to check the spread of sickle cell anaemia in Gujarat. But the situation on the ground remains the same, as reported by The Indian Express on June 19, 2009. There is an acute shortage of counsellors and technicians in the 11 tribal districts populated by different adivasi groups like the Bhils, Rathwas, and Naykas. Vadodara DEO Dr Vijay Birla said: “There are three sensitive zones in our district -- Chhota Udepur, Kwant and Pavi Jetpur. But there is just one counsellor in this region. No one is willing to work in rural areas.” Last year, the state health department advised tribals to get their blood tested for traces of the genetic disease before they got married. “We are doing work. We counsel and also spread awareness on how to avoid sickle cell anaemia. For those who have no risk, we give a white card, while for those at risk we give them yellow cards. Patients affected by this disease can match their cards,” Dr P B Prajapati, nodal officer of the sickle cell anaemia project, said. For its part, Bhasha has been spreading awareness about sickle cell disease through its network in Chhota Udepur. People in the hamlets are aware of its existence, but most still do not know how to prevent it from spreading. “Every time a person dies people go by the external symptoms because sickle cell disease can mimic many other afflictions. We need to stress that the ‘person died of sickle cell disease’, to spread awareness,” Varia said. Individuals like Sancharben and Kokila are being issued identification cards according to their genetic type so that their condition is known to doctors and health workers for quick diagnosis. The biggest challenge is informing individuals who carry the sickle cell gene of the range of consequences of their future reproductive choices. That could prevent more instances of this painful, life-threatening disease. It required genetic screening of marriageable boys and girls among the Rathwas. Dr Patel volunteered to screen only suspected cases of sickle cell anaemia, which meant people without external symptoms would have to depend on private laboratories -- a costly affair given tribal household incomes. Dr Patel, who is now retired, said: “We tried in Gujarat during 2003-2004 to stop marriages between patients with sickle cell anaemia and also to ensure early diagnosis. We suggested this to the state government too. However, nothing worked out.” The state government is charged with not doing enough to sustain projects related to sickle cell anaemia research at the Baroda Medical College (BMC), one of the six medical colleges in Gujarat that began research in the field years ago. The college’s expertise even won it the Indian Council of Medical Research (ICMR) project in 2000-2005. The aim of the project was to check the prevalence of congenital diseases such as thalassemia and sickle cell anaemia among 10,000 youths and expectant mothers. Away from Gujarat, sickle cell anaemia has attained alarming proportions in Chhattisgarh too, with nearly 35 lakh people suffering from the disease. Though all 18 districts are affected, the prevalence is alarmingly high in 10 districts. Surveys by the state Red Cross Society and hospital records indicate nearly 20% of the tribal population and 17% of the scheduled caste population has the disease. In 2004, the state government set up a Centre for Genetic Diseases and Molecular Biology, and launched ‘Project Sickle’ to prevent the spread of the disease. A door-to-door survey to identify carriers and directly affected children in the age-group 3-15 was conducted in Raipur district, in 2007. These steps though have had limited success. In Orissa, a Sickle Cell Disease Research Centre is functioning at Sardar Raja Medical College in Jaring, Kalahandi district. The state has witnessed growing infant and maternal mortality rates mostly among specific tribes in Kalahandi, Koraput, Balangir, Sambalpur, Sundargarh, Keonjhar, Boudh, Kandhamal, Mayurbhanj, Dhenkanal and Ganjam districts. Maharashtra has set up a committee to look into the growing prevalence of sickle cell anaemia in the state, and to prepare a plan to check the spread of the disease. The move comes in the wake of a recent study in 19 districts by the Nagpur-based Indira Gandhi Medical College and Hospital which found that almost 10% of the state’s population was affected and that most of the victims were tribals. Infochange News & Features, June 2009 |
