What is Sickle Cell Disease?
Sickle cell disease (SCD) is one of the most common genetic disorders in the world affecting persons in Africa, Asia, the Middle East, the Mediterranean region, Europe, Central, South and North America and the Caribbean. Researchers have theorised that the sickle cell trait resulted from a natural mutation in the body’s fight against malaria, as SCD is very prevalent in all regions of the world with a long history of malaria.
SCD is a hereditary blood disorder caused by an abnormality which affects red blood cells. In Jamaica, 10 per cent of the population, or approximately 270,000 persons, carry the sickle cell trait and are at risk of having a child with SCD. Another five per cent of the population or approximately 135,000 Jamaicans carry other abnormal haemoglobin traits such as the C gene and the thalassaemia gene, which combine with the sickle cell trait to give rise to other forms of sickle cell disease such as SC disease, SB+ thalassaemia and SB0 thalassaemia. Based on the aforementioned numbers, 405,000 Jamaicans (15 per cent of the population) are at risk of having a child with some form of sickle cell disease.
The incidence of SCD in Jamaica is relatively high, as one in every one hundred and fifty (150) children born has a form of sickle cell disease, compared to one in every 375 African-Americans born in the United States. Approximately 300 children are born with SCD every year in Jamaica.
The main symptoms of this disease include:
- Painful crises (bouts of moderate to severe bone pain)
- Bone damage (especially the hip joint)
- Painful leg ulcers (sores around the ankles)
- Increased susceptibility to infections, especially pneumonia and meningitis
- Enlargement of the spleen
- Kidney disease
- Sickle retinopathy, which can cause loss of vision and strokes. This occurs in seven per cent of children with SS disease, the most severe form of SCD.