The Indian Council of Medical Research has invited Expressions of Interest for the joint development and commercialisation of low dose or pediatric oral formulation of hydroxyurea to treat sickle cell disease in children.

• India ranks first with the highest prevalence of sickle cell disease in South Asia, with over 20 million sickle cell affected individuals residing in the country.

About Sickle Cell Disease

Sickle cell anemia is a genetic blood disorder that affects the production of hemoglobin, the protein in red blood cells that carries oxygen throughout the body. 

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• Cause:  People with sickle cell anemia have abnormal hemoglobin levels that cause their red blood cells to become rigid and sticky and shaped like a crescent or sickle.
  • These abnormally shaped cells can get stuck in small blood vessels, clogging the small blood vessels 
  • Healthy red blood cells are soft, flexible, and round. 
• Symptoms: Anaemia, pain crisis, fatigue, jaundice, delayed growth, and increased susceptibility to infections.
• Occurrence: It is most commonly found in people of African descent, but it can also occur in people of Hispanic, Middle Eastern, and Mediterranean descent.
• Impact: The sickle cells also break down more quickly than healthy red blood cells leading to anemia which causes a constant shortage of red blood cells and increasing the risk of infection and death from conditions like stroke, heart problems, kidney problems, and pregnancy complications.
• Treatment: There’s no cure for sickle cell anemia. Early detection, management, and treatment of SCD enable the affected individuals to lead a longer life
• World Sickle Cell Day: It  is celebrated on June 19 to raise awareness about this genetic blood disorder and to educate people about how to manage their lifestyles.

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Hydroxyurea in Sickle Cell Disease

• Hydroxyurea is a myelosuppressive agent, is an effective drug for treating patients of sickle cell disease, and thalassemia as it keeps blood cells round and flexible, so as to ensure easy flow and better delivery of  oxygen to the body. 
• Function: Hydroxyurea helps the body make a type of hemoglobin that helps keep red blood cells round. So people who take hydroxyurea can have more energy, and less pain and anemia.
• Need: There is a need for pediatric formulation of HU (hydroxyurea), considering the alarming number of sickle cell disease cases in India and in view of the launch of the National Mission to eliminate Sickle Cell Anaemia/SCD (by 2047)
• In India: 
  • Most pharmaceutical companies in India market 500 mg capsules or 200 mg tablets of Hydroxyurea while the prescribed dose in children  is 10-15 mg per kilogram of body weight after two years of age. 

• • National Health Mission’s guidelines: As per it, the healthcare providers initiate hydroxyurea therapy to only symptomatic sickle cell disease patients among children both because of the lack of availability of paediatric doses as well as the fear of toxicity.
• Challenge:
  • Unavailability: It is not available in the suspension form for effective use in the case of paediatric patients.
  • A low dose treatment: Preparing it  is a challenge for service providers, as the capsule or tablet has to be broken down appropriately to be administered in accordance with body weight, thereby risking the efficacy available with measured doses.
  • Titration process: The titration of dose is difficult, and currently, is carried out by using a fraction of the broken capsules, which can lead to less accurate administration of the drug. 

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