The most common and serious complications of sickle cell disease are anemia, pain, fatigue, and organ failure. Today there are many alternatives and opportunities that a sickle cell patient may consider. One outlined in this paper is the Hydroxyurea method. Sickle Cell Anemia Sickle Cell Anemia takes place in the bloodstream of the body. The 'Sickle' reference is in relation to the disordered shape red blood cells take.
This "sickled cell," which resembles a crescent moon, can't continue to glide through the small blood vessels as usual. Instead, it gets stuck in the tiny blood vessels, blocking the flow of blood and causing pain. The spleen, an organ on the left side of the abdomen across from the liver, is responsible for filtering the blood for infections and other abnormalities. Normal red blood cells can change shape and squeeze through this filter, but sickled cells get stuck and cannot recirculate through the body, so that the number of circulating blood cells goes down. Meanwhile, the bone marrow, where red blood cells are made, pitches in by making more cells.
Sickle cell disease (SCD) is a genetic disorder, characterized by defected hemoglobin (Hb) synthesis with production of an abnormal form, known as sickle hemoglobin (HbS). Under low oxygen concentration or increased body oxygen demand, red blood cells that are sickle and rigid shaped fail to pass smoothly through capillaries, with multiple clotting and thrombosis. Eventually, this leads to frequent hemolytic crisis and progressive organ damage. Transfusion is the key therapy in SCD. However, transfusion has been associated with many side effects in SCD patients, mainly iron overload and alloimmunization that cause severe hemolytic transfusion reactions.
Tissue that does not receive a normal blood flow eventually becomes damaged. This is what causes the complications of sickle cell disease. There are several types of sickle cell disease. The most common are: Sickle Cell Anemia (SS), Sickle-Hemoglobin C Disease (SC)Sickle Beta-Plus Thalassemia and Sickle Beta-Zero Thalassemia. Sickle Cell trait (AS) is an inherited condition in which both hemoglobin A and S are made in the red blood cells, there are always more A than S. Sickle cell trait is not a type of sickle cell disease.
Normal hemoglobin looks like little dots, where hemoglobin S forms stiff strands which makes it into its shape. The sickle or crescent shape is harmful because it will get lodged in the blood vessels and block blood flow. Blocked blood flow results in pain and serious organ damage. Anemia is a condition where a person would have a lower amount of red blood cells than normal. Red blood cells are made in the spongy bone marrow inside the larger bones in the body.
These large numbers of immature or incompletely developed cells do not function like healthy red blood cells leading to oxygen deficiency throughout the organs and tissues of the body. Since these cells are underdeveloped they also have a shorter life expectancy. Pernicious anemia has been studied by many different researchers, starting in the late 17th century. Between 1876 and 1877 William Osler and William Gardner studied a patient they suspected of pernicious anemia. Their findings were that the patient had the following symptoms: numbness in the fingers, hands, and forearms, the gastric mucosa was atrophic, and high levels of red bloods cells containing nuclei were found in the bone marrow.
However, in sickle cell disease the red cells become rigid and sticky. They are shaped like sickles or crescent moons. Because these cells are irregular, they can get stuck in small blood vessels. The small blood vessels slow or block blood flow. Without enough red blood cells in circulation a person's body cannot get the adequate amounts of oxygen needed to feel energized.
Sickle cells contain abnormal hemoglobin due to a mutation in the hemoglobin chain. This type of hemoglobin is known as sickle hemoglobin or hemoglobin S. Sickle hemoglobin is what distorts red blood cells into a sickle shape. Sickle cells are sticky and less elastic than normal red blood cells. They are not able to pass through the blood as easily so in result they tend to block blood flow in blood vessels. Blocked blood flow causes pain, organ damage, and increases the risk for infections.
In sickle-cell anemia the altered shape of the red blood cells prevent them from passing through blood vessels and this will result in an inadequate amount of oxygen being delivered to tissues in the body (Peachley, n.d.). Sickle cell anemia was discovered because of gel electrophoresis, which is a method that evaluates the charges of the hemoglobin. Gel electrophoresis, the inheritance pattern, and hemoglobin
In the capillaries, the sticky, rigid, odd-shaped cells create a logjam. This causes a block in blood flow and deprives body tissues of their necessary oxygen. Without oxygen, cells begin to die and tissues suffer damage. These episodes are painful for the patient, but they are not permanent. Sickled red cells eventually regain their normal round shape when they are re-oxygenated and re-hydrated.