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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.