16 June 2022, Bengaluru: Sickle cell disease (SCD), an inherited blood disorder, continues to remain a public health challenge in India. Characterized by repeated episodes of debilitating pain, it can lead to serious health complications including pneumonia, bloodstream infections, stroke, and acute & chronic pain. India is estimated to have the second-highest burden of SCD after Nigeria[i]- it is estimated that there may be 18 million SCT and 1.4 million SCD patients among the tribal population[ii].
According to the Ministry of Tribal Affairs (MoTA), 1 in 86 births among the scheduled tribe has SCD. Recognizing this rising burden, MoTA has set up a Sickle Cell Disease Support Corner to bridge the gap between patients and health care services in tribal areas. In Karnataka, the state government has launched steps to reduce the state’s anemia burden, including the establishment of screening centers in high-density areas. The state has also been proactive in leveraging the Union Ministry of Health and Family Welfare’s central initiatives to subsidize the treatment of such rare diseases. Private organizations and medical schools have also helped raise awareness of the disease and have given technical training to doctors on how to effectively diagnose and treat SCD.
Dr. Meera Varadarajan, Clinical Haematologist, and Haemato-oncologist at Victoria Hospital, Bengaluru shared, “Today, we are dealing with a sickness that can be effectively eradicated with persistent efforts. If certain rules are put in place, we will be able to not only detect carriers early but provide counselling and prevent the disease from passing from one generation to the next. While the central government and state are increasing the number of screenings, there is still a need for increased awareness about the condition. A simple blood test can determine the markers in the hemoglobin that underpins the sickle cell anemia indicators. And while this blood test can be made a part of the routine for newborn screening, the same test can be taken for adults as well, preferably before marriage.”
Vaso-occlusive crises (VOC) are a common complication of sickle cell disease and occur when blood vessels in a part of the body become blocked by sickled red blood cells. VOC may lead to excruciating pain that might last up to a week, anemia, organ damage, and early mortality. It is difficult to predict the frequency of these pain episodes and it varies from patient to patient. VOC has been seen to have a large influence on the mortality rate of patients. Anemia, impaired spleen function, and secondary infections like pneumococcal sepsis are some of the other complications that patients might suffer from. SCD also has a significant impact on pregnant women who often suffer from anemia.
To effectively manage SCD, hydroxyurea is provided to patients. Other therapies include the prescription of antibiotics to combat infections and vitamin supplements to help generate red blood cells.
Timely detection, screening, and appropriate treatment are the biggest challenges in tackling SCD. On the need for a comprehensive model of care, Gautam Dongre, Secretary, National Alliance of Sickle Cell Organizations, NASCO says, “We need to re-think the model of care for SCD. In my experience of dealing with patients, proper and timely treatment has led to normal life expectancy and almost 70-80% enhanced life quality for patients. Timely, comprehensive, and appropriate treatment is of utmost importance. For this, we need to ensure last-mile connectivity and delivery of healthcare models in the remotest parts of the country and actively establish centers of excellence to meet advanced care requirements. While the Ministry of Health and Ministry of Tribal has taken active measures at a central and state level, emphasis needs to be put on skilling of healthcare professionals and other medical personnel so that they can identify SCD and accordingly recommend treatment.”
SCD management requires a comprehensive care model and a multi-disciplinary approach where all stakeholders in the ecosystem come together and put in efforts and resources to meet the needs of sickle cell patients in India. To tackle SCD as a health issue, India drafted a national-level policy in 2018 that outlines guidelines on the prevention and control of hemoglobinopathies that included sickle cell disease along with hemophilia and thalassemia. The policy is yet to be implemented and the government is working with various stakeholders to work on a revised version. Once it is implemented at the national level, it needs to be adopted at the state level.
