Latest news with #GauravDixit
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First Post
21 hours ago
- Health
- First Post
Fighting a genetic enemy: How sickle cell disease quietly impacts childhood in India
Sickle cell disease affects thousands of Indian children, yet its most dangerous complications often go unnoticed. Experts tell Firstpost that what begins as a blood disorder can escalate into serious, chronic health problems if not diagnosed and managed early. read more Sickle cell disease (SCD) remains one of the most under-recognised genetic conditions affecting children in India with profound long-term effects on the brain, organs, growth and quality of life. While many associate the disease primarily with painful episodes, experts say the damage often begins quietly, early in life, and worsens over time without timely intervention. Firstpost spoke to leading paediatric and haematology experts — Dr. Shweta Bansal, Head - Paediatric Haemato Oncology & Paediatric BMT, Medical Oncology, Sir H.N. Reliance Foundation Hospital; Dr. Gaurav Dixit, Associate Director, Haemato-oncology & Bone Marrow Transplant at Paras Health, Gurugram; and Dr. Atique Ahemad, Newborn & Child Specialist, Saifee Hospital, Mumbai to understand the complex burden of this disease and how parents and physicians can intervene early. STORY CONTINUES BELOW THIS AD How sickle cell affects the brain — often without warning According to Dr. Shweta Bansal, sickle cell disease significantly increases the risk of both overt and silent strokes in children. 'Due to the sickle-shaped red blood cells, the brain's blood vessels can get blocked, depriving areas of the brain of oxygen. About 10% of children with SCD experience a stroke by age 14,' she said. Yet the bigger threat may be silent strokes which occur in nearly 1 in 4 school-age children with SCD. These don't show immediate symptoms but can impair memory, learning, attention and behaviour. 'We use Transcranial Doppler (TCD) ultrasounds to measure blood flow in the brain. If the velocity is high, it indicates stroke risk and we start preventive therapy. MRIs also help identify silent strokes,' Dr. Bansal added. Early signs and complications in children Dr. Atique Ahemad said that while infants with SCD may remain asymptomatic in the first 6–7 months due to protective fetal haemoglobin, symptoms emerge soon after. These include pallor, delayed growth, hand-foot swelling (dactylitis), and frequent pain episodes. 'Children often cry excessively due to pain. These crises can start by the age of six and affect various body parts, arms, legs, back and chest leading to long-term damage,' he says. Pain crises, or vaso-occlusive crises (VOCs), are particularly harmful. Dr. Gaurav Dixit said that each episode deprives vital organs of oxygen, progressively damaging the kidneys, brain, lungs, liver and heart. 'Frequent VOCs significantly increase the risk of chronic organ dysfunction, stroke, pulmonary hypertension, and early mortality,' he warned. How pain and infections are managed Pain in SCD isn't just distressing — it's also diagnostically challenging. 'There is no scan to quantify pain. The patient's words are the only reliable indicator,' Dr. Ahemad said, emphasising the need for compassionate and responsive care. Dr. Bansal outlines a two-pronged strategy: preventing crises and managing acute attacks. Preventive measures include hydration, avoiding extreme temperatures, and reducing physical or emotional stress. For mild pain, paracetamol or ibuprofen suffices but severe cases may require hospital-based morphine treatment. Preventing infections is equally critical, as SCD weakens the spleen. Dr. Bansal advises routine handwashing, safe food hygiene, vaccinations (including annual flu and pneumococcal vaccines), and daily penicillin till age 5 for high-risk children. 'With regular check-ups and support, these children can lead full lives,' she says. Hydroxyurea: A gamechanger in disease management One of the most effective tools in managing SCD is hydroxyurea. Dr. Bansal explains: 'It helps produce more fetal haemoglobin, reducing red cell sickling. This means 50–70% fewer pain crises, fewer transfusions, better school attendance and significantly extended life expectancy, even in rural areas.' Importantly, she said that hydroxyurea is safe for long-term use, including in children older than 9 months. Bone Marrow Transplant: A potential cure — for a few While hydroxyurea helps manage symptoms, a bone marrow transplant (BMT) remains the only proven cure. However, it is only an option for a select group of patients. 'Children below 16 with a perfectly matched sibling donor and severe SCD complications — like stroke, lung disease, organ damage, or frequent crises — are considered eligible,' said Dr. Bansal. BMT carries risks and is generally reserved for the most severely affected cases. Research is ongoing into alternatives such as half-matched donors and gene therapies. STORY CONTINUES BELOW THIS AD Hidden organ damage and long-term risks Dr. Dixit reiterates the toll VOCs take on the body over time. 'Organ systems, kidneys, heart, lungs and brain deteriorate silently with each crisis. Chronic kidney disease, pulmonary hypertension, and heart failure are major causes of reduced life expectancy in adult SCD patients,' he said. Dr. Ahemad lists stroke, acute chest syndrome, retinal damage, and splenic or hepatic sequestration among the most severe complications seen in adults. 'Long-term pain, bone necrosis and arthritis are common too. Early management of VOCs is key to preserving organ health and improving life quality,' he advised.
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First Post
14-06-2025
- Health
- First Post
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. STORY CONTINUES BELOW THIS AD 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.