Avinash is nine years old and studies in Class III. He suffers from sleeplessness, finds it difficult to breathe, especially at night, and is unable to concentrate on studies. The result: he misses school and examinations.
Abinav, 12, a student of Class VII, is prone to anaemia and jaundice. He often gets bouts of vomiting and suffers from chronic diarrhoea.
Both kids are otherwise talkative, playful, emotional and are aware of their ‘incurable’ illness — both suffer from the dreaded genetic disease, sickle cell.
Apart from their illness, there’s one more thing in common: their father is Arjun Munda, the Chief Minister of Jharkhand. The duo are among Munda’s three sons.
Doctors say the disease cannot be cured. “Being aware of this fact, we feel miserable,” said Munda’s wife Meera with tears in her eyes as the Chief Minister tried to hide his emotions.
Human blood is formed of red blood cells, 70 per cent of which consists of haemoglobin. Normal red blood cells contain haemoglobin. In the sickle cell disease, the red blood cells contain sickle haemoglobin and assume the shape of a sickle, with sharp edges. Sickling decreases the cells’ flexibility and results in a risk of various complications.
“Each person inherits two genes for forming beta globin — one from each parent. If a person inherits only one abnormal gene, he/she is called a carrier of sickle haemoglobin. Such a person cannot have any normal haemoglobin and can make only sickle haemoglobin that reduces life span interspersed with painful crises,and constantly faces the threat of sudden death due to severe infections,” writes IC Verma, GP Das and Manjeet Kaur in their joint article, ‘Genetic and Other Health Problems of Tribal Communities’ in the book
Tribal Health in India.
Dr Deepa Chand, a US-based physician attached to the Akron Children’s Hospital in Ohio, who is treating Munda’s sons, thinks proper diagnosis can reduce mortality by preventing infections through immunisations. “This is possible only when the disease is detected early as it was inherited by the miss-match of the parents (blood),” says Dr Chand.
Sickle cell disease is generally considered to be rare but doctors are now reporting an increase in the number of such patients visiting state government-run hospitals here. At the state government-run RIMS hospital, about four per cent of tribal patients who came to its OPD last year suffered from this disease.
Though there is no recent survey to show its latest trend, an old study found that sickle cell disease was common among tribals in 72 districts of central, western, southern and eastern India.
“There are more than 35 tribal population groups showing a frequency of more than 19 per cent. It is estimated that approximately a staggering 50 lakh individuals are carriers (heterozygotes) among the tribals. A prevalence rate of up to 40 per cent of heterozygous form (sickle cell trait) has been reported in some tribes, ie., Adiyan of Kerala, Irula, Paniyan, Muluku Rumbha of Nilgiri Hills, Gonds of Raipur,” writes Salil Basu in his article Health Status of Tribal Women in India in the same book.
There is no research throwing light on why so many tribals suffered from this genetic disease. But in 1993, Basu emphasised the need for an easily accessible local medical centre — PHC — equipped with facility of blood transfusion and a blood bank in tribal inhabited areas across the country.
“We suffered a great deal due to the absence of medicare. In fact, when Abhinav was six months old, one of our doctors suspected the disease. But due to wrong medication, his kidney was damaged,” said Meera.
Munda, who has collected a dozen-odd books on genetics and other health problems of the tribal communities, has a preventive plan: Rs 20,000 as a cash incentive to every couple who ties the nuptial knot only after getting their blood groups matched. “We are going to introduce this reform in Jharkhand very soon,” said Munda.
What is sickle-cell disease?
Sickle-cell disease (SCD), or sickle-cell anaemia (or anemia; SCA) or drepanocytosis, is an autosomal recessive genetic blood disorder, with overdominance, characterized by red blood cells that assume an abnormal, rigid, sickle shape.
Sickling decreases the cells’ flexibility and results in a risk of various complications. The sickling occurs because of a mutation in the haemoglobin gene.
Life expectancy is shortened, with studies reporting an average life expectancy of 42 in males and 48 in females.
Sickle cell anemia is an inherited, lifelong disease. Usually presenting in childhood, it occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common.
The cells are stiff and sticky. They tend to block blood flow in the blood vessels of the limbs and organs. Blocked blood flow can cause pain, serious infections, and organ damage, especially in the lungs, kidneys, spleen, and brain.
The disease is the most common inherited blood disorder in the United States, affecting 70,000 to 80,000 Americans.
IT IS estimated to occur in 1 in 500 African Americans and 1 in 1,000 to 1,400 Hispanic Americans