About Sickle Cell Anemia

Sickle Cell Anemia is an inherited blood disorder, which mainly affects people of Afro-Caribbean origin, and some people from the Mediterranean region, Arabia and Asia. It's estimated that there are some 12,000 people with the condition in the UK - of which 1 in 4 are West African, 1 in 10 are Afro-Caribbean, 1 in 50 are Asian and 1 in 100 are Northern Greek. The aim and mission is to raise awareness of the condition and encourage Black and Asian communities to get screened.

What causes sickle cell anemia?

Within our blood vessels are red blood cells (which are shaped like doughnuts). These red blood cells contain a protein called hemoglobin (HbA). The function of hemoglobin is to carry oxygen from the lungs to all parts of our body. Sickle Cell Anemia occurs when the Hba is replaced by sickle hemoglobin. When you have sickle hemoglobin, your red blood cells become sickle-shaped, therefore, they cannot pass through very small blood vessels. This causes blockage, resulting in lack of oxygen to parts of the body, which in turn results in pain, and/or damage to vital organs.

How does a person get sickle cell disease?

Sickle Cell Anemia is not 'catching'. We all have two copies of the gene for HbA, one inherited from our mother and one inherited from our father. Now, if one parent carries the HbS gene then sickle cell trait can occur in their baby. This means that the child is a carrier of the sickle cell gene, but has enough HbA to keep their red blood cells flexible. So the child will not have the symptoms of Sickle Cell Anemia. Very occasionally they have to be careful when doing things that demand less oxygen than normal, for example scuba diving. If both parents carry the trait then the child may be born with the full Sickle Cell Disease with the symptoms as described below. There are other types of hemoglobin such as HbC and beta thalassaemia, that when combined with sickle hemoglobin cause sickling disorders. Consequently, there are different forms of Sickle Cell Disorder, which occur in different ethnic groups.

The symptoms of sickle cell disease.

Symptoms include fatigue, breathlessness, bone and joint pain, weakness and susceptibility to infections. People affected with Sickle Cell Anemia also experience what's known as 'crisis'. These are severe attacks, which can encompass the aforementioned symptoms as well as jaundice, fever and vomiting. A crisis usually leads to hospitalization, and in some cases a blood transfusion is needed. Repeated crisis can lead to liver, lung, kidney damage and other medical conditions.

Managing sickle cell disease and anemia.

There is no cure for the disease but daily medication and regular hospital check-ups keep Sickle Cell Anemia manageable. People with the condition are able to go to school, college or work but, of course, may have to take time off for periods of illness. Like with many other conditions, it's a matter of being aware of the 'limitations' of the disease and taking precautions. So, for instance, avoiding strenuous and emotional stress, drinking plenty of fluids to avoid dehydration and being able to recognize the onset of illness is important.