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Dr explains: Why sickle cell disease disproportionately affects India's tribal communities
Sickle Cell disease disproportionately affects India's tribal populations, with complications impacting both children and adults. Firstpost brings out experts' view on early signs, treatments and strategies for managing pain and infection in sickle cell patients. read more
Sickle Cell Disease (SCD), a hereditary blood disorder, remains a serious public health challenge in India, especially among tribal communities. The disease causes abnormally shaped red blood cells that obstruct blood flow, leading to severe pain and complications.
According to the Ministry of Tribal Affairs, nearly 10% to 40% of the tribal population in certain states carries the sickle cell trait. The burden is highest in states like Chhattisgarh, Maharashtra, Madhya Pradesh, Odisha and Gujarat. Despite its high prevalence, limited awareness, delayed diagnosis, and poor access to care continue to hinder timely treatment.
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To gain a deeper understanding of the disease and explore effective management approaches, Firstpost spoke with Dr. Gaurav Dixit, Associate Director of Haemato-oncology & Bone Marrow Transplant at Paras Health to shed light on the key issues surrounding SCD in India.
What factors contribute to the prevalence of sickle cell disease in tribal populations?
Dr Dixit: Tribal communities in India are indeed hit particularly hard by sickle cell disease (SCD), especially in states like Gujarat, Madhya Pradesh, Odisha, Maharashtra, and Chhattisgarh. The sickle cell gene is prevalent in these populations due to genetic factors. The disease's impact is further exacerbated by limited access to early screening and adequate healthcare.
How does sickle cell disease affect the brain?
Dr Dixit: Serious neurological issues, such as overt strokes and silent cerebral infarcts, can result from SCD, particularly in children. These complications occur when sickled red blood cells block blood vessels in the brain. Therefore, early detection and prevention through routine screening using transcranial Doppler ultrasounds is crucial.
What are the early clinical signs that help differentiate sickle cell disease from other haemoglobinopathies in children?
Dr Dixit: Key early indicators include anaemia, jaundice, delayed growth, recurrent infections, and dactylitis (painful swelling of hands and feet). A definitive diagnosis is made using hemoglobin electrophoresis or newborn screening, which helps differentiate SCD from other hemoglobinopathies, such as thalassemia.
How do recurrent vaso-occlusive crises affect long-term organ function in sickle cell patients?
Repeated vaso-occlusive crises can lead to chronic damage in vital organs such as the liver, kidneys, lungs, and spleen. Over time, patients may develop complications like pulmonary hypertension, chronic kidney disease, or avascular necrosis. Preventative care and effective crisis management are essential in limiting long-term damage.
What are the most common complications you observe in adult patients with sickle cell disease in India?
Dr Dixit: Adults with SCD often experience complications like priapism, gallstones, leg ulcers, chronic pain, strokes, and organ dysfunction, particularly in the kidneys and lungs. Many also suffer from psychosocial challenges, which underscores the need for comprehensive and multidisciplinary care.
How effective is hydroxyurea therapy in reducing hospitalisations and improving life expectancy in sickle cell patients?
Dr Dixit: Hydroxyurea has significantly reduced the frequency of pain episodes, hospitalisations and the need for blood transfusions. Early initiation and regular monitoring with hydroxyurea therapy can improve both life expectancy and overall quality of life in patients.
Could you explain the role of bone marrow transplantation in managing sickle cell disease? Who qualifies for it?
Dr Dixit: Bone marrow or stem cell transplantation is currently the only curative treatment for SCD. It is most effective when performed early in life and with a matched sibling donor. However, due to donor scarcity, it is generally reserved for patients with severe disease and frequent complications.
How do you approach pain management and infection prevention in paediatric sickle cell patients?
Dr Dixit: Pain management starts with hydration and NSAIDs, escalating to opioids in severe cases. For infection prevention, we rely on early childhood penicillin prophylaxis, timely vaccinations, and prompt treatment of fevers. Parent education and regular monitoring also play a crucial role in managing paediatric cases.