What is Sickle Cell Anaemia?

The disorder affects the red blood cells which contain a special protein called haemoglobin (Hb for short). The function of haemoglobin is to carry oxygen from the lungs to all parts of the body.
People with Sickle Cell Anaemia have Sickle haemoglobin (HbS) which is different from the normal haemoglobin (HbA). When sickle haemoglobin gives up its oxygen to the tissues, it sticks together to form long rods inside the red blood cells making these cells rigid and sickle-shaped. Normal red blood cells can bend and flex easily.

Because of their shape, sickled red blood cells can't squeeze through small blood vessels as easily as the almost donut-shaped normal cells. This can lead to these small blood vessels getting blocked which then stops the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs.

Everyone has two copies of the gene for haemoglobin; one from their mother and one from their father. If one of these genes carries the instructions to make sickle haemoglobin (HbS) and the other carries the instructions to make normal haemoglobin (HbA) then the person has Sickle Cell Trait and is a carrier of the sickle haemoglobin gene. This means that this person has enough normal haemoglobin in their red blood cells to keep the cells flexible and they don't have the symptoms of the sickle cell disorders. They do however have to be careful when doing things where there is less oxygen than normal such as scuba diving, activities at high altitude and under general anaesthetics.

If both copies of the haemoglobin gene carry instructions to make sickle haemoglobin then this will be the only type of haemoglobin they can make and sickled cells can occur. These people have Sickle Cell Anaemia and can suffer from anaemia and severe pain. These severe attacks are known as Crises. Over time Sickle Cell sufferers can experience damage to organs such as liver, kidney, lungs, heart and spleen. Death can be a result.

Another problem is that red blood cells containing sickle haemoglobin do not live as long as the normal 120 days and this results in a chronic state of anaemia. In spite of this, a person with sickle cell disorder can attend school, college and work. People with sickle cell disorder need regular medical attention particularly before and after operations, dental extraction and during pregnancy. Many hospitals arrange follow-up appointments and it is advisable to discuss with the doctors questions concerning schooling, strenuous exercise, family planning, suitable types of employment and air travel. When a person is found to have a sickle cell disorder it is important that all members of the family be tested. They will not necessarily have sickle cell disorder but may be healthy carriers of a sickle cell trait.

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