Sickle Cell Disease
Throughout the world, approximately 300 million individuals are heterozygous for a mutation in the β-globin gene, meaning they carry the sickle cell trait (Key, Connes, & Derebail, 2015). Of those 300 million individuals, 100,000 Americans are affected by sickle cell disease, making it the most common inherited blood disorder. Every single cell in our body needs oxygen to function efficiently, and the role of hemoglobin in red blood cells is to transport oxygen from the lungs to the rest of the body (“Sickle Cell Disease”, 2016). In a normal individual, the red blood cells have a disc-like shape that grant it flexibility and allow it to move through the smallest of vessels. However, as the name suggests, individuals with …show more content…
Today, the life expectancy for an individual with sickle cell disease is approximately 20-60 years, whereas back in the 70s, life expectancy was about 14 years (Lanszkron, Carroll, & …show more content…
Although this is the only known cure for the disease, it is often not viable because only less than 14% of sickle cell patients have a healthy compatible sibling donor. More than 90% of individuals who have had the transplants have a six year cure from the disease. Transplants with compatible, but unrelated individuals are not common because the risk of rejection is high (Hoban, Orkin, & Bauer, 2016). Because the current known cure for sickle cell disease is not definitive and often not possible for some, people have learned to manage the disorder using hydroxyurea. Hydroxyurea is a medication that works by increasing the levels of fetal hemoglobin in the blood, and decreasing the concentration of hemoglobin S, which is responsible for the abnormal sickling of red blood cells (Segal, Strouse, Beach, et al., 2008). Hydroxyurea has been proven to help adults have less pain episodes and acute chest syndrome, and it has helped with anemia, all of which are common complications of sickle cell disease. Another way to manage sickle cell disease is through red blood cell transfusion. Transfusion increases the healthy red blood cells and decreases obstructions in blood vessels, which in turn improves blood flow and oxygenation to organs and tissues (“Sickle Cell Disease”,
Throughout the world, approximately 300 million individuals are heterozygous for a mutation in the β-globin gene, meaning they carry the sickle cell trait (Key, Connes, & Derebail, 2015). Of those 300 million individuals, 100,000 Americans are affected by sickle cell disease, making it the most common inherited blood disorder. Every single cell in our body needs oxygen to function efficiently, and the role of hemoglobin in red blood cells is to transport oxygen from the lungs to the rest of the body (“Sickle Cell Disease”, 2016). In a normal individual, the red blood cells have a disc-like shape that grant it flexibility and allow it to move through the smallest of vessels. However, as the name suggests, individuals with …show more content…
Today, the life expectancy for an individual with sickle cell disease is approximately 20-60 years, whereas back in the 70s, life expectancy was about 14 years (Lanszkron, Carroll, & …show more content…
Although this is the only known cure for the disease, it is often not viable because only less than 14% of sickle cell patients have a healthy compatible sibling donor. More than 90% of individuals who have had the transplants have a six year cure from the disease. Transplants with compatible, but unrelated individuals are not common because the risk of rejection is high (Hoban, Orkin, & Bauer, 2016). Because the current known cure for sickle cell disease is not definitive and often not possible for some, people have learned to manage the disorder using hydroxyurea. Hydroxyurea is a medication that works by increasing the levels of fetal hemoglobin in the blood, and decreasing the concentration of hemoglobin S, which is responsible for the abnormal sickling of red blood cells (Segal, Strouse, Beach, et al., 2008). Hydroxyurea has been proven to help adults have less pain episodes and acute chest syndrome, and it has helped with anemia, all of which are common complications of sickle cell disease. Another way to manage sickle cell disease is through red blood cell transfusion. Transfusion increases the healthy red blood cells and decreases obstructions in blood vessels, which in turn improves blood flow and oxygenation to organs and tissues (“Sickle Cell Disease”,