Sickle cell anemia: clinical features and pathophysiology

Sickle Cell (Rough Draft)

Sickle cell anemia is a blood disease that causes badly formed red blood cells. The disease is genetec. Mostly people from Africa or other coutries around the Mediterraean Sea get it. In the United States, African-Americans are most likely to have it (Howard, 1995).

Red blood cells are the blood cells that carry oxygen throughout the body. When a person has sickle-cell anemia the hemoglobon in the cell is shaped wrong. Because of this the cells are not round but sickle-shaped, which is how the disease gets its name. Sometimes these cells stick together and interfere with blood flow. This is very painful but also causes medical problems. It can cause damage to organs and keep the body from receiving the oxygen it needs to function well. It also leaves the person likely to get lung infections (Howard, 1995).

Although sickle-cell anemia is genetec, both parents have to carry the gene for the child to get the disease. In the United States, one in every 12 African-Americans carries a gene for the disease, and about 1,000 babies are born each year with sickle cell anemia. One in twelve carriers is a high number, but there is a reason for it. If a person has only one gene, they have the "sickle cell trait." Having the trait rarely makes any problems, but makes it much less likely that the person can catch malaria, a blood disease spread by mosquitoes. Malaria is common in Africa, and those who had the sickle cell trait had good protection from this serious disease (Loupe, 199). Because of this, people with the sickle cell trait were more likely to survive and reproduce.

Unusual practices arose in Africa because of sickle cell anemia. In the disease, it can affect the growth of the hands, and many people with sickle cell anemia often have at least one finger that is shorter than it should be. It can also interfere with the growth of arms and legs. Unfortunately, children born with sickle cell pneumia sometimes die from pneumonia or other diseases because they aren't as able to fight the illness off (Loupe, 199).

The problem with the hemoglobon cell occurs when the cell releases its oxygen somewhere in the body. When the oxygen releases, it leaves a kind of bump on the surface of the cell that can catch on other cells, causing the small vessel to block as the deformed cells continut to catch on each other. When they lock together they become rigid and block further blood flow (Loupe, 199).

This transforms flexible, smooth, doughnut-shaped red blood cells into stiff, sticky, curved cells that clog small blood vessels. The circulatory traffic jam can lead to acute pain, organ damage, swollen or stunted hands and feet, and sometimes stroke due to blocked blood flow (Loupe, 199).

Sickle cell anemia can be treated but not cured. One treatment is the drug hydroxyurea, sometimes used for cancer treatment. In some people it prevents the blood cells from deforming so they won't clump together and interfere with blood flow. Unfortunately, not everyone with sickle cell anemia can take the drug (Howard, 1995). Doctors suggest that people with sickle cell anemia avoid cold climates, because cold temperatures also reduce circulation and can make the problems with sickle cell anemia worse (Howard, 1995). It is important for people with sickle cell anemia to eat a balanced diet and get plenty of liquids (Howard, 1995).

An interestng fact about sickle cell anemia is that the unborn child does not have defective blood cells. Fetal blood cells will not stick together and make problems (Howard, 1995). However, gradually, adult blood cells begin to form and the disease becomes visible. This fact makes researchers beleive they may eventually find more effective treatments for the disease. They may be able to find a way to increase fetal oxygen cells and reduce the ones likely to bend (Loupe, 199). One possibility might come from stem cell research. Researchers are also looking at genetec engineering with the hope that they can artificially make the body create red blood cells that will not sickle (Loupe, 199).