What is Sickle Cell?


Sickle Cell is a genetic disease found in the red blood cells of a sickler. The hemoglobin are molecules which carry oxygen inside the red blood cell. Hemoglobin becomes misshaped with someone who has sickle cell. This causes the red blood cell to form a sickled shape, giving the sickle cell disease its name.

 

 

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Where is it Primarily Located?

​Sickle Cell is primarily found in Sub-Saharan Africa. This region has struggled with malaria, a sickness attained through mosquito bites. Untreated this sickness can be fatal. The sickle cell trait protects against mortality from malaria. This has allowed children with the sickle cell trait to survive and pass down the gene. However, children without this trait are susceptible to malaria and have a greater risk of dying. This has allowed the sickler population to become endemic in this region of the world. In Nigeria, where sickle cell disease is most prevalent, there is an average of 45,000-90,000 babies born with sickle cell disease each year, in which many of them die premature deaths.

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How is it Inherited?

To obtain the sickle cell disease, an allele for that trait must already be present in both parents of the child. If there is a man and a woman who are both negative for the sickle cell gene, then their child will not be a carrier or obtain the disease. If the man or woman in the relationship is a carrier for this trait, and his/her partner is clean, then there is a 50% chance that their child would be a carrier and not a sickler. If the man or woman has sickle cell and his/her partner is healthy, then they can only have children who are carriers of the disease. If both the man and woman carry the disease, then there is a 25% chance that they will have a healthy child, a 50% chance that they will have a carrier, and a 25% chance that they will have a sickler child. If a man or woman has the sickle cell disease, and his/her partner is a carrier, 50% of their children will either be sickle cell carriers or have the sickle cell disease. Finally, if both man and woman in a relationship have the sickle cell disease, then all their offspring will be guaranteed to be sicklers. Even if one does not have the sickle cell disease, it can be spread to offspring in a relationship with a sickler.

Symptoms

Those who are only carriers of sickle cell disease, usually do not show any symptoms, and if so, they are minimal. Death comes to victims of sickle cell from the effects of the disease on other organs and not from the sickling of the cell itself. The sickled shape of the red blood cell causes blockages in the blood stream and/or rupture of the red blood cell. This ultimately results in a decreased red blood cell count, which leads to less oxygen being dispersed throughout the body. This causes symptoms such as discoloration of the eye, thick mucus, severe pain, and eventually organ failure, which can lead to death.

Current Research

Currently there is not a feasible cure for the sickle cell disease. The most popular drug for coping with the symptoms of sickle cell and to help prolong life expectancy is hydroxyurea. While scientists have made great improvements towards prolonging life expectancy from the age of around 13 to now 40’s and 50’s, there is still no cure for this disease. Places in Sub-Saharan Africa where sickle cell is most common, are mostly developing countries. Consequently most of the clinics in these countries do not have the money to pay for drugs like hydroxyurea, which are essential to the wellbeing of a sickler’s health. Currently, research is being done on the transfer of bone marrow as a potential sickle cell cure, however it is quite expensive and not very accessible to the general public.