Sickle Cell Disease (SCD)

Sickle Cell Disease, SCD, is a genetic disorder of the haemoglobin (oxygen carrying capacity of blood) due to a single amino acid substitution with valine replacing glutamic acid. In low oxygen state affected red blood cells become deformed changing from a doughnut-shape to a sickle-like rigid form that blocks blood follow to vital organs, thus causing tissue damage. This is responsible for generalised pain, end organ damage among other features.

SCD is a debilitating condition and symptoms begin as early as five-six months of life such as generalised body pain, infections and stroke. The risk of death is highest during the first three years of life. Simple cost-effective measures such as early diagnosis, health promotion and preventive therapies instituted early will improve survival and quality of life. Simple cost effective prophylaxis including penicillin, antimalarial therapy, folic acid and fluid intake can be introduced early in childhood.

Key Facts About Sickle Cell Disease (SCD)

  1. SCD is one the most common inherited disorder in the world with over 75% of all newborn in sub Saharan Africa
  2. About 5% of the world’s population carry the trait for haemoglobin disorders, 300,000 new births with SCD per year
  3. Nigeria has the highest prevalence of SCD in the world and > 100,000 annual births with the disorder. It is estimated that only 50% of children with SCD live past the age of 10 years compared to over 99% surviving into adulthood in Europe and USA.
  4. SCD is a public health problem that demands a well-orchestrated strategy for the benefit of the less privileged in Society.
  5. It is projected that by 2050 three countries namely – Democratic Republic of Congo, Nigeria and India in the absence of control programme will see the number of sickle cell anaemia patients increase by 100% except specific action with regards to control. 
  6. Simple preventive measures such as penicillin prophylaxis and malarial prevention will reduce the risk of complications such as infection and improve survival and overall well-being 
  7. Hydroxyurea therapy reduces painful crises, blood transfusion requirement and hospitalisation and safe in children and adults. It requires regular blood monitoring to ensure that patients adhere to therapy and safety of the medication

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