Sickle Cell Anemia

Introduction

Sickle Cell Anemia is a disease that is affecting the lives of almost everyone, the trait is something that affects someone from birth and throughout their whole lives. According to the National Heart, Lung and Blood Institute “sickle cell disease affects 70,000–100,000 people in the United States. It mainly affects African Americans. The disease occurs in about 1 out of every 500 African American births. Sickle cell disease also affects Hispanic Americans, occurring in 1 out of every 36,000 births. Approximately 2 million Americans have the sickle cell trait. The condition occurs in about 1 in 12 African Americans. Sickle cell disease affects millions of people worldwide. Incidence is higher in people who come from Africa, South or Central America (Panama), the Caribbean islands, Mediterranean countries (Turkey, Greece, and Italy), India, and Saudi Arabia.” (Ic.galegroup.com). Based on this data that has been reported it shows that sickle cell disease is something that is extremely prevalent in today’s society. The disease is something that can easily affect anyone who has a heritage that is mentioned above.

The history of sickle cell disease is related to the sickle cell trait that is very prevalent in many African Americans whose ancestors lived in places where malaria is present. Those who have the sickle cell trait are immune to malaria, which is very prevalent in many African countries even to this day. Thousands of years ago, the sickle cell trait was formed when the people who contracted malaria would then have their blood cells mutated by the disease; therefore, if they survived the disease and had children, the trait was therefore passed down through generations. As slavery occurred, the populations were moved and immigrated to different areas of the earth which migrated the disease to reach all of the continents. According to Gale Databases “In each pregnancy of two parents who both have sickle cell trait, there is a 50% chance that the child will have the trait, a 25% chance that the child will have sickle cell disease, and a 25% chance that the child will have neither the trait nor the disease.” (ic.galegroup.com) Due to these facts, it is very easy for the sickle cell trait to be passed down throughout generations and reach new heights and various levels of the population. The Gale Encyclopedia explains it as “Humans normally make several types of the oxygen-carrying protein hemoglobin. An individual’s stage in development determines whether he or she makes primarily embryonic, fetal, or adult hemoglobins. All types of hemoglobin are made of three components: heme, alpha (or alpha-like) globin, and beta (or beta-like) globin. Sickle hemoglobin is the result of a genetic change in the beta globin component of normal adult hemoglobin. The beta globin gene is located on chromosome 11. The sickle cell form of the beta globin gene results from the substitution of a single DNA nucleotide, or genetic building-block. The change from adenine to thymine at codon (position) 6 of the beta globin gene leads to insertion of the amino acid valine—instead of glutamic acid—at this same position in the beta globin protein. As a result of this change, sickle hemoglobin has unique properties in comparison to the usual type of adult hemoglobin. Most individuals have two normal copies of the beta globin gene, which make normal beta globin that is incorporated into adult hemoglobin. Individuals who have sickle cell trait (called sickle cell carriers) have one normal beta globin gene and one sickle cell gene. These individuals make both the usual adult hemoglobin and sickle hemoglobin in roughly equal proportions, so they do not experience any health problems as a result of having the trait.” (www.ic.galegroup.com)

Although the sickle cell trait and gene is not deadly or effective in itself as many people do in fact have the trait, when humans develop sickle cell diseases is when it turns deadly. Sickle cell disease is when the red blood cells in a human carry oxygen like normal red blood cells throughout the body and relay oxygen; however, when they release the oxygen they implode and harden into rod like structures. When the red blood cells stiffen like explained, they can get stuck and block the rest of the red blood cells from releasing oxygen to areas that are needed, as pictured below. (https://www.nhlbi.nih.gov/sites/www.nhlbi.nih.gov/files/images/anemia.jpg)

According to The National Heart, Lung, and Blood Institute sickle disease does not occur fully in a patient’s life until usually later into their lifetime: “Most children with SCD are pain free between painful crises, but adolescents and adults may also suffer with chronic ongoing pain.”( https://www.nhlbi.nih.gov/health/health-topics/topics/sca/). The pain that comes with SCD is very often contracted in the organs when their cells don’t bring oxygen to which can after long term affects cause cancer and failure organs in addition to the pain that is occurring on a daily basis. The body fails to keep up because “Sickle cells can’t change shape easily, so they tend to burst apart or hemolyze. Normal red blood cells live about 90 to 120 days, but sickle cells last only 10 to 20 days. The body is always making new red blood cells to replace the old cells; however, in SCD the body may have trouble keeping up with how fast the cells are being destroyed. Because of this, the number of red blood cells is usually lower than normal. This condition, called anemia, can make a person have less energy.” (www.nhlbi.nih.gov) Sickle Cell disease is a lifelong effect on everyday life; today the average life lifespan of a patient with Sickle Cell Disease is up to 40-60 years which is almost triple what the lifespan 40 years ago being only on average 14 years.

Results

There have been many treatments that have been introduced to the medical field in order to delay the process of Sickle Cell Anemia. One of the treatments that is used today is blood transfusions, which are not to occur regularly but often to prevent the disease from accelerating. When the blood transfusions occur, the new blood that is put into the body is often filled with a healthy amount of blood cells that overpower the sickle cells to a certain extent. Over a long period of time, the sickle cells will eventually outrun the new blood cells because they are a constant from the patient’s body rather than the blood transfusion that is a shorter-term fix when it comes to a lifetime of disease. Another form of treatment that occurs and can sometimes cure patients is mostly used when it comes to younger patients who are suffering harshly from the effects is Bone Marrow Transplants. The CDC states “Bone marrow is a soft, fatty tissue inside the center of the bones where blood cells are made. A bone marrow or stem cell transplant is a procedure that takes healthy cells that form blood from one person—the donor—and puts them into someone whose bone marrow is not working properly. Bone marrow or stem cell transplants are very risky, and can have serious side effects, including death. For the transplant to work, the bone marrow must be a close match. Usually, the best donor is a brother or sister. Bone marrow or stem cell transplants are used only in cases of severe SCD for children who have minimal organ damage from the disease.” (www.cdc.gov) Conclusion

The conclusions that have occurred in research is that the Sickle Cell Trait itself is not something that necessarily causes death in all that have it. For instance, having the sickle cell trait and gene will allow the carrier to be immune to the malaria disease that is contracted through mosquitos through the blood stream. The Sickle Cell trait is something that can occur to be deadly when the person has two sickle cell genes in their DNA and 11th chromosome. The Sickle Cell disease is something that occurs when two people that have the Sickle Cell trait have children and then they can have the disease. One of the most problematic things that come with the disease is that it can occur later in life and develop at a time when the patient can be too old for a cure, and therefore all they can do is treat the disease. When a patient becomes too old, the disease can never be cured because of the cures which are stem cell treatment and bone marrow transplants are only are performed on younger individuals or children because their body has the means to fight off the complications that come with. Despite the research that has been conducted, there are much more information that is to be developed throughout the medical world and biological field; such as, developing a cure for those who develop the disease later in life.