Managing sickle cell during pregnancy, childhood

WORLD Sickle Cell Day was celebrated on June 19, and Sickle Cell Awareness Month in September. 

What is sickle cell disease?

According to the Sickle Cell Support Foundation of Jamaica, it 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 the disease is very prevalent in all regions of the world with a long history of malaria. Sickle cell disease 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 people, carry the sickle cell trait and are at risk of having a child with sickle cell disease.

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 sickle cell disease in Jamaica is relatively high, as one in every 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 sickle cell disease 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)

•Jaundice

•Lethargy

•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 sickle cell disease.

The US Centers for Disease Control and Prevention says a woman with sickle cell disease is more likely to have problems during pregnancy that can affect her health and the health of the unborn baby, than a woman without it.

During pregnancy the disease can become more severe, and pain episodes can occur more often. A pregnant woman with sickle cell disease is at a higher risk of preterm labour, having a low birth weight baby, or other complications.

However, with early prenatal care and careful monitoring throughout pregnancy, a woman with sickle cell disease can have a healthy pregnancy.Sickle cell disease is recessive, which means that both parents must pass on the sickle cell gene for a child to be born with sickle cell disease.

During pregnancy there is a test to find out if the baby will have sickle cell disease, the sickle cell trait, or neither one. The test is usually done after the second month of pregnancy.

Women who have the sickle cell trait can also have a healthy pregnancy. Children with sickle cell disease can live full lives and enjoy most of the activities that other people do.

Good, quality medical care from doctors and nurses who know a lot about the disease can help prevent some serious problems. Regular health check-ups with a primary care doctor can help prevent some serious problems.

Note that common illnesses, like the flu, can quickly become dangerous for a child with sickle cell disease. The best defence is to take simple steps to help prevent infections