Sickle Cell Disease (SCD), a hereditary blood disorder, poses a significant health challenge in India, particularly among tribal populations. This condition leads to misshapen red blood cells that block blood flow, causing severe pain and complications.

According to the Ministry of Tribal Affairs, the sickle cell trait is present in 10% to 40% of tribal individuals in certain states, with higher prevalence in regions like Chhattisgarh, Maharashtra, Madhya Pradesh, Odisha, and Gujarat. Despite its widespread occurrence, limited awareness, delayed diagnosis, and inadequate access to healthcare services continue to impede timely treatment.

Interview with Dr. Gaurav Dixit

To delve deeper into the nuances of SCD and effective management strategies, Firstpost interviewed Dr. Gaurav Dixit, Associate Director of Haemato-oncology & Bone Marrow Transplant at Paras Health. Here are some key insights from the discussion:

Factors Contributing to SCD Prevalence

Dr. Dixit highlighted that genetic factors play a significant role in the prevalence of SCD among tribal communities in India. Limited access to early screening and healthcare services further exacerbates the impact of the disease.

Neurological Complications

Dr. Dixit emphasized that SCD can lead to severe neurological issues, especially in children, such as strokes and cerebral infarcts. Early detection through routine screening is crucial to prevent such complications.

Early Signs and Diagnosis

Key early signs of SCD in children include anaemia, jaundice, delayed growth, recurrent infections, and dactylitis. Diagnosis is typically confirmed through hemoglobin electrophoresis or newborn screening to differentiate SCD from other haemoglobinopathies.

Long-Term Organ Damage

Recurrent vaso-occlusive crises can result in chronic organ damage over time, affecting vital organs like the liver, kidneys, lungs, and spleen. Preventative care and crisis management are essential to limit long-term complications.

Common Complications in Adults

Adults with SCD often experience complications such as priapism, gallstones, chronic pain, strokes, and organ dysfunction. Comprehensive care is crucial to address both physical and psychosocial challenges.

Treatment Approaches

Hydroxyurea therapy has shown effectiveness in reducing pain episodes, hospitalizations, and the need for blood transfusions in SCD patients. Bone marrow transplantation is considered a curative option, particularly for severe cases with matched sibling donors.

Pain Management and Infection Prevention

Pain management in paediatric SCD patients involves hydration, NSAIDs, and opioids for severe cases. Infection prevention strategies include penicillin prophylaxis, vaccinations, and prompt fever treatment, along with parent education and regular monitoring.