June 2022, Ahmedabad: 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 – it is estimated that there may be 18 million SCT and 1.4 million SCD patients among the tribal population . Moreover, before reaching the age of two, 20% of tribal children with sickle cell disease die, and 30% of children die before reaching adulthood .
Gujarat is one of the hubsfor the diseaseand it is most common in areas with a significant tribal population or where the disease runs in the family.The districts which have a significant endemicity are Chhota Udepur, Dahod, Banaskantha, Bharuch, Panchmahal, Mahisagar, and Narmada.This segment of the population has not seen widespread migration hence the prevalence of SCD is still concentrated in these districts.With a tribal population of roughly a million people, it is estimated that 9 lakh people have the sickle cell trait (carrier), with 70,000 people suffering from sickle cell disease.
In response to the state’s high disease burden, Dr. Anil Khatri, Paediatrician, Jeevandeep Hospital, Ahmedabad said, “For early diagnosis and treatment to be provided, the most important aspect is patient education & awareness so that they can seek timely help. The treatment usually consists of adequate hydration, pain-relieving medications, and administering drugs to decrease the incidence of pain crises. If not treated promptly, acute crises can result in the death of the suffering patient.” In terms of improving the healthcare system, he recommended, “Reduction in the SCD burden can be achieved by bringing about a paradigm shift in the training of healthcare workers and paramedical staff, introducing counselors for guidance, improving hospital infrastructure, laboratory facilities, blood banks, and providing transport facilities.”
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. Dr. Anil Khatrishared, “It is difficult to predict the frequency of these pain episodes and it varies from patient to patient. Stroke, acute chest syndrome, pulmonary hypertension, avascular necrosis of the hip joint, splenic sequestration, blindness, and other problems may occur because of SCD.”
It is incumbent upon the State Government to identify priorities and establish a ground force for effectively achieving the desired results with a definite timeline. All stakeholders including governments, medical experts, the pharmaceutical sector, NGOs, and patient groups, must work together to combat SCD. While the Gujarat government has always been sensitive to the cause, and various initiatives have been undertaken and made it a Model State in terms of the Sickle Cell Program, it would be prudent to make every possible treatment option available to all the state’s Sickle Cell Disease patients.
To effectively manage this condition, 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, NASCOsays, “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.”
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.