'A Sickle In a Haystack': Can India Eliminate Sickle Cell Anaemia By 2047?

According to the latest National Family Health Survey(NFHS-5), anaemia afflicts over half the population of India.

(Photo: The Quint)

The big fact first: Anaemia afflicts over half the population of India, according to the latest National Family Health Survey(NFHS-5).

This has only worsened in recent times despite concerted efforts to tackle the problem. While the causes for these anaemias are many and layered, the programmes that address them are barely so.

An example of such an anaemia is Sickle Cell Disease (SCD). About a third of children afflicted with the disease die before reaching adulthood and a fifth before they even reach two years of age.

A genetic disease that causes the change in the Red Blood Cell (RBC) shape from a biconcave disc to a sickle (C-like) shape – Sickle Cell Disease has been catapulted into the mainstream conversation since the recent Union Budget announced a mission to eliminate Sickle Cell Anaemia (SCA) by 2047.

Since SCA is a genetic disease, elimination, completely stopping the transmission of a disease is hardly feasible.

At best, this can be viewed as an attempt to eliminate SCA as a public health problem or alleviate the suffering of people afflicted with it. More notably, the disease must be understood and addressed appropriately.

Nature Of the Disease Poses Daunting Challenge

Even then, the nature of the disease poses a daunting challenge for its diagnosis and therapy. Sickle Cell trait (HbS) is an autosomal recessive trait, which means:

• It is passed on irrespective of sex

• It does not manifest in carriers (HbSA) usually, that is, individuals who carry a single copy of the gene rarely show the symptoms of the disease.

Sickling of the RBCs causes the oxygen-carrying capacity of blood to decrease. This leads to tiredness, retardation of growth, increased infections and so on. It also hinders the flow of blood leading to blockages which further cause pain crises, and strokes, and may cause damage to several other organs.

Additionally, the increased destruction of sickled and damaged RBCs leads to lower haemoglobin and iron overload which can further lead to damage to organs like the spleen, liver, kidney and heart.

The Evolution of Sickle Cell Anaemia

Despite the suffering caused by Sickle Cell Disease, the evolution of this trait has been crucial for survival throughout history.

It has been established that SCD is more prevalent in regions where malaria is endemic and it is now understood that the sickle cell trait offered protection against especially lethal forms of malaria. This trait has evolved in distinct genetic backgrounds, thus it is a product of convergent evolution where the sickle cell trait offered a survival advantage to people amidst the epidemics of malaria throughout the world.

This can still be seen in the people SCA affects the most today. Most pockets of SCD are in malaria-endemic regions even within our own country.

If we look even closer, these malaria-endemic regions and SCA-affected populations often overlap. The worst afflicted are tribal regions. According to the Ministry of Tribal Affairs, 1 in 86 tribal people are affected by SCD.

This disproportionate burden of Sickle Cell Anaemia has been addressed by screening tribal populations where possible and the current announcement, too seems to focus on doing the same- screening, raising awareness, and providing genetic counselling.

While these are of some use, it is necessary to understand the cause of this high prevalence before targeting these interventions in tribal areas. Several studies show that malaria morbidity and mortality are higher in areas with high levels of undernutrition. This is one of the oft-ignored factors that explain the high prevalence of these diseases in tribal regions. Thus, a very important factor often aggravating both malaria mortality and thus SCA is nutrition.

The Futility of Iron Fortification

Ignoring the demand for dietary diversity, the government has started distributing iron-fortified rice mandatorily in the Anganwadis, Mid-day Meals and Public Distribution System.

The Prime Minister of India in his Independence day speech in 2021 announced the ambitious plan of the Government of India to supply fortified rice in all food schemes by 2024.

A pilot scheme was unfolded in 15 blocks of 15 districts to evaluate the coverage, provision and utilisation of fortified rice in reducing the targeted micronutrient deficiencies in different age and gender groups.

At present, fortified rice distribution has been scaled up in Jharkhand at the state level in all food schemes without the release of findings from the nationwide pilot and pushed to the unsuspecting citizens who have not given their prior consent. With a large population of Adivasi communities who are also the most affected by adverse health conditions and blood disorders, this is highly unethical and harmful. Studies from various countries have shown that iron-fortified rice has failed to improve the condition of anaemia.

The Right to Food Campaign and ASHA (Alliance for Sustainable and Holistic Agriculture) conducted two fact-findings in Jharkhand and Chhattisgarh in May and June 2022 respectively.

The reports of both states raised grave concerns regarding the public health and food security of the most marginalised section of our society. Through these reports, various public health experts and citizens expressed concerns about the contraindication of fortified rice in sickle cell anaemia and thalassemia patients.

With the poor public health infrastructure across the country, there would be many undiagnosed patients of these particular diseases as hardly any screening is done at local levels.

The FSSAI (Fortification of Foods) regulation, 2018 recognises this risk and warns patients about the danger of consuming iron-fortified rice. Fortified rice worsens the risk of iron overload in the body, which can cause organ damage.

The high burden of sickle cell anaemia and thalassemia falls disproportionately on the poor and marginalised which largely depend on the Public Distribution System for their staple diet.

The rice fortification program in India has ignored the screening and protection of those patients for whom fortified rice is contraindicated. They are being coerced to consume iron-fortified rice with no alternatives available at the PDS shop. This is a violation of the National Food Security Act since it denies the right to choice of food.

The limited entitlement to wheat and rice, and lack of a diversified food basket in terms of non-vegetarian diet including eggs, millets, pulses and oil within PDS, Anganwadis and Midday meals paints a disheartening picture in the fight against the different forms of malnutrition for it cannot be solved with silver bullets.

The Way Ahead

Further inspection of the tools proposed for the Sickle Cell Control Programme also brings forth the ethical issues associated with genetic counselling. Often, marginalised communities have to suffer the eugenic tendencies of the more privileged sections of society.

Although the guideline is to merely raise awareness and not force the parents to abstain from having biological children, the power differential between the healthcare provider and the laypersons, especially those from marginalised communities, makes this an exercise in coercion if the history of birth control is any indicator.

The pain crises of SCA can be addressed through the use of the drug hydroxyurea. Other than this, blood transfusions, provision of necessary vaccines(other than the universal vaccines) and in recent times, gene therapy are some of the ways in which SCA can be treated. Out of these, only gene therapy can definitively cure SCA, currently, this is an expensive and difficult procedure which is not feasible at a mass scale.

There is a need to place Sickle Cell Anaemia in its larger context. The programme mission does attempt to do this, however, this requires more than lip service. In a country where tribal areas are still underserved in terms of primary health care, and disproportionately affected by malaria, first, access to care must be provided. The screening tools and subsequent cards that the government plans to provide people living with Sickle Cell Anaemia will only be as beneficial as the functionality of its most remote PHC.

Technocentric solutions like Iron fortification and Iron Folic Acid supplementation too are inept at addressing various types of anaemias. Nutritional anaemias, too, can only be partially addressed through such short-term measures.

Ensuring the availability of safe and sufficient food to all, especially vulnerable groups is essential for addressing the multiple causes of anaemia(not only SCA). We cannot eliminate the disease through counselling and medication on the one hand while providing potentially harmful foodgrains and allowing malnutrition to exist on the other.

Discussion about Sickle Cell Anaemia must address the dearth of health infrastructure, indiscriminate food fortification, inadequate ration and the epidemiological and social realities of the tribal population that is most susceptible to this disease. One can only hope that the thrust of this programme will eventually lead to the redressal of all of these problems, too.

(Dr Shivangi Shankar is a Medical Doctor currently studying Public Health. She writes about mental health, doctor-patient relationships, caregiver health, awareness regarding diseases, issues concerning healthcare workers and public health in India.

Raj Shekhar is a coordinator at the National Secretariat of the Right to Food Campaign as well as a researcher. He has led and participated in fact-finding exercises on fortified rice distribution in Jharkhand and Chattisgarh.)