Global and African Sickle Cell Burden !!!

• 300,000 babies are born annually with sickle cell disease worldwide. 

• Over 75% of these births occur in Sub-Saharan Africa. 

• Nigeria alone accounts for over 30% of global cases or contributes nearly 150,000 SCD births per year — the highest burden in the world. 

Sickle cell disease is a hereditary blood disorder that affects the structure and function of red blood cells. People with this condition have red blood cells that mostly contain hemoglobin S, an abnormal form of hemoglobin. Hemoglobin is the protein in red blood cells responsible for carrying oxygen from the lungs to the rest of the body. Normally, red blood cells with hemoglobin A are round, soft, and flexible, allowing them to move easily through small blood vessels.

In sickle cell disease, the red blood cells become stiff and shaped like a crescent or sickle. These misshapen cells can’t pass through tiny blood vessels easily and tend to block the flow of blood. They also have a shorter lifespan, living only about 16 days compared to the normal 120 days, which leads to a constant shortage of red blood cells. This blockage and lack of oxygen-rich blood can damage body tissues and organs.

The damage caused by poor blood flow is what leads to most of the complications seen in sickle cell disease. These can include pain, organ damage, infections, and other serious health problems. While there is currently no universal cure, some people may be eligible for a bone marrow transplant that can potentially cure the disease. Most patients require lifelong treatment and care to manage symptoms and reduce the risk of complications.

Sickle cell trait (AS) is a hereditary condition where both hemoglobin A and hemoglobin S are present in a person’s red blood cells. It is not the same as sickle cell disease. Individuals with sickle cell trait are usually healthy and may be unaware that they carry the trait.

To determine your hemoglobin type, a simple blood test is performed, followed by a laboratory procedure called Hemoglobin Electrophoresis. This technique involves applying an electric charge to a solution of hemoglobin, causing different types of hemoglobin to move varying distances based on their composition. It effectively differentiates between normal hemoglobin (A), sickle hemoglobin (S), and other variants such as hemoglobin C, D, and E.

Sickle cell conditions are inherited from one’s parents in a manner similar to other genetic traits, such as blood type, hair color and texture, and eye color. The specific types of hemoglobin produced in a person’s red blood cells are determined by the hemoglobin genes passed down from each parent. Just like most other genes in the human body, hemoglobin genes are inherited in pairs—one gene from the mother and one from the father. The combination of these genes influences whether a person has normal hemoglobin, carries the sickle cell trait, or has sickle cell disease.

In many parts of Nigeria, when families experienced repeated loss of children, these unfortunate events were historically attributed to spiritual beings known as Ogbanje (among the Igbo) or Abiku (among the Yoruba)—children believed to be reincarnating spirits born only to die again and again. However, modern medical science shows that most of these cases were actually due to genetic blood disorders, especially sickle cell disease (SCD).

• Lack of a nationwide genotype policy .

• Misdiagnosis and stigma .

• Cultural silence on marriage/genotype compatibility .