What is Sickle Cell Anemia?
Sickle cell anemia (also known as sickle cell disease) is a disorder of the blood caused by an inherited abnormal hemoglobin (the oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted (sickled) red blood cells.
It is a major genetic disease in most countries in Sub-Saharan Africa with prevalence ranging between20% and 30%; and in some parts of Uganda it is as high as 45%.
The sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture, anemia is the result. This condition is referred to as sickle cell anemia.
According to WHO, Northern Uganda has the highest prevalence of Sickle cell trait in the country standing at 18.6%.
What causes Sickle Cell Anemia?
Sickle cell anemia is one of the most common inherited blood anemias. It is inherited as an autosomal (meaning that the gene is not linked to a sex chromosome) recessive condition. This means that the gene can be passed on from a parent carrying it to male and female children. In order for sickle cell anemia to occur, a sickle cell gene must be inherited from both the mother and the father, so that the child has two sickle cell genes.
How is Sickle Cell disorder transmitted?
The inheritance of just one sickle gene is called sickle cell trait or the “carrier” state. Sickle cell trait does not cause sickle cell anemia. Persons with sickle cell trait usually do not have many symptoms of disease and have hospitalization rates and life expectancies identical to unaffected people. When two carriers of sickle cell trait mate, their offspring have a one in four chance of having sickle cell anemia.
Can it be prevented?
While personal risk factors for developing sickle cell anemia are out of your control, it is possible to avoid passing the disease down genetically. For instance, a genetic counselor can advise you if you plan to have a child and carry the sickle cell trait. Some options, like in vitro fertilization, may decrease the chance that two people with the sickle cell trait will pass the disease down genetically.
In conclusion, while Sickle Cell Disorder is a major public health concern in Uganda, the interventions that are currently in place are not sufficient to address the current burden. Furthermore, systematic screening for SCD using a simple blood test is seldom done; and diagnosis is often made when a child presents with severe complications.
