What is Sickle Cell Anemia?

What is sickle cell disease?

Sickle-cell disease is a specific group of disorders that are rooted in the genes. It is caused by a particular type of hemoglobin which is "sickle hemoglobin" or Hgb S for short.

The forms that this disease comes in many types however in most of the forms, the red blood cells morph whenever deoxygenating happens because the abnormal sickle hemoglobin undergoes polymerization.

What this means is that the hemoglobin proteins stick to each other. What this does is that it essentially creates a rigid surface and it forms into a sickle-shape, thus the name of the disease.

This process is very harmful to the red blood cell membrane and eventually will cause the different cells to be stuck in blood vessels. What this does to the person is that it deprives the various downstream tissues of oxygen.

What this does is it eventually leads to ischemia and infarction which is the cause for organ damage. In the long run, it will lead to strokes and other complications.

This particular disease is chronic and lifelong so even though the individuals who have this disease lead lives normally, they will have periodic painful attacks from time to time. Because of this the life-expectancy is quite shortened.

Current statistics still do not have a count on the contemporary survival rate so that data is still up in the air waiting to be confirmed.

Who is at risk?

There are some studies that show that those who suffer from sickle-cell disease live to an average of 40 to 50 years with the average of males being 42 and the average for females being 48.

This type of disease is known to be much more common in specific parts of the world such as sub-Saharan Africa where malaria was once common.

However, it occurs in other ethnicities as well as it has claimed victims from different races as well. Because of this, those with one or two alleles of the sickle cell disease are resistant to the disease of malaria simply because the red blood cells are not conducive to the propagation of the sickle cell disease.

Complications

Complications that arise from this disease are painful vaso-occlusive crises, spenectomy infections, strokes and gallstones. There you have it, the introduction to Sickle-cell disease.

Be sure to learn more and don’t stop reading about the disease because it might come in hand one day when a relative or you yourself fall prey to this disturbing disease.