Sickle Cell Disease

Sickle Cell Disease – Group of Inherited Red Blood Cell Ailment

Sickle cell disease – SCD is considered as a group of inherited red blood cell ailment wherein the red blood cells tend to get hard and sticky and look like a C-shaped farm tool known as a sickle. SCD is a serious disorder wherein the body makes sickle shaped red blood cells in the shape of the sickle while normal red blood cells are disc shaped which look like doughnuts without holes in the centre.

They tend to move with ease in the blood vessels and contain an iron rich protein known as haemoglobin and this protein carries the oxygen from the lungs to the various parts of the body. Sickle cells comprise of abnormal haemoglobin known as sickle haemoglobin which causes the cells to develop a sickle or crescent shape.

In the case of this disorder, the abnormally shaped red blood cells get caught in small blood vessels, blocking the flow of blood and oxygen to organs in the body. These obstructions tend to cause repeated incidents of severe pain, serious infections, organ damage and even stroke.

These episodes are known as sickle cell crisis or vaso-occlusive crisis and they could last from a few minutes to several months though on an average most of them could last for five to seven days.

Abnormal Blood Cells – Shorter Lifespan 

The abnormal blood cells also tend to have a shorter lifespan and cannot be replaced as quickly as the normal blood cells which lead to shortage of red blood cells called anaemia. Sickle cell anaemia is due to mutation in the gene which instructs the body to develop haemoglobin and this gene is inherited.

To obtain sickle cell anaemia, the person has to inherit the defective gene from both the parents. If the gene is inherited from one of the parent, the sickle cell strait – SCT and it is likely that the blood will have some sickle cells and they will be able to produce normal haemoglobin without any symptoms.

However, they could be carrier of sickle cell anaemia and could pass the abnormal gene to their children. If two individuals with the sickle cell trait tend to have a child, there could be a possibility of one in four chance that the child could be born with sickle cell anaemia.

Those who inherit one sickle cell gene and one normal gene have sickle cell trait – SCT and people with SCT frequently do not have any of the symptoms of sickle cell disease – SCD

Symptoms 

Sickle cell anaemia is diagnosed with a simple blood test wherein the blood is checked for defective haemoglobin. A small portion of defective haemoglobin can indicate if the person has the sickle cell trait but not sickle cell anaemia and a high level can indicate sickle cell anaemia.

To reduce the risk of sickle cell crisis, one should cultivate a simple lifestyle of consuming plenty of fluids.At times, a person suffering from sickle cell anaemia continues to experience pain inspite of taking precautions for which medication is recommended to get relieve.

Due to the risk of potentially life threatening complications, it is essential to check out for any symptoms or signs where the health of a person suffering from this condition could suddenly get worse which include a high temperature of 38C or above, breathing difficulties or severe pain that cannot be controlled even with medication that are available over the counter.