Sickle cell disease (SCD) is a genetic disorder that affects the shape and function of red blood cells. People with SCD have abnormal hemoglobin, the protein that carries oxygen in the blood. This causes the red blood cells to become rigid and sickle-shaped, blocking the blood flow and damaging the organs.
SCD is a major health issue in India, especially among the tribal communities. According to the data, India has the second highest number of SCD cases in the world, after Nigeria. About 1 in 86 births in India suffer from SCD, and about 75% of them are from the states with high tribal population, such as Maharashtra, Madhya Pradesh, Chhattisgarh and Odisha.
SCD can cause severe complications, such as infections, anemia, pain crises, stroke, and organ failure. It can also lead to early mortality and morbidity. A recent study estimated that the life expectancy of adults with SCD in India is 54 years, which is 20 years shorter than that of adults without SCD.
How is SCD diagnosed and treated in India?
SCD can be diagnosed by a simple blood test that detects the presence of abnormal hemoglobin. However, many people with SCD in India are unaware of their condition or do not have access to proper diagnosis and treatment. Dr Rahul Bhargava, Principal Director & Chief BMT, Fortis Memorial Research Institute, Gurugram, said that the fear of a large number of the tribal patients slipping through the cracks is real.
The treatment options for SCD include medication, blood transfusions, and bone marrow transplantation. However, these are costly and not widely available in rural areas. Moreover, there is a lack of awareness and education about SCD among the patients and the health care providers. Dr Bhargava said that many kids with SCD suffer from growth retardation and drop out of school due to pain crises.
He also said that SCD causes a huge economic and productivity loss to the country, as well as personal loss to the patients and their families. He urged the government and the industry to work together to ensure the safety and benefits of SCD patients.
What is the link between SCD and malaria?
One of the reasons why SCD is prevalent in some regions of India is the historical association with malaria. Malaria is a parasitic infection that affects the red blood cells and can be fatal if not treated. However, people with sickle cell trait (SCT), who have one normal and one abnormal hemoglobin gene, have a lower risk of getting malaria. This is because the parasite cannot survive well in sickle-shaped red blood cells.
Therefore, SCT may have offered a survival advantage to people living in malaria-endemic areas over thousands of years. This explains why the areas with the highest malaria rates also have the highest rates of SCT and SCD.
However, this advantage comes at a cost. People with SCT can pass on their abnormal gene to their children, who may inherit two copies of it and develop SCD. This is why it is important to screen people for SCT and provide genetic counseling to prevent or reduce the incidence of SCD.
What is India’s plan to eliminate SCD by 2047?
In view of the increasing burden of SCD in India, the Union Health Ministry has announced a plan to eliminate the disease by 2047, as part of its National Health Policy 2023-2030. The plan includes creating awareness, universal screening and counseling for about 7 million people between the ages of 0 and 40 years in the affected tribal areas through a joint effort between central ministries and state governments.
The plan also aims to provide comprehensive care and support to SCD patients, including free medication, blood transfusions, bone marrow transplantation, and social welfare schemes. The plan also envisages setting up specialized centers for SCD management and research in various states.
The plan is expected to cost about Rs 1,000 crore over five years and will be funded by both central and state governments. The plan hopes to reduce the prevalence of SCD from 1.2% to less than 0.5% by 2047.