Sickle Cell Anemia occurs in about 1 out of 500 African American births, and 1 out of 36,000 Hipic births. It is a lifelong disease, and sometime can be deadly. According to Dr. Whittaker, in our region there are abnormally higher cases of Sickle Cell Anemia, so I found it important to learn more about the disease, what cause it, what are the symptoms as well as the options of treatments for this disease. According to www. invista. com, it is believed that sickle cell disease occurs more often in people from parts of the world where “malaria” is or was common.
Also the coincident of people who carry the sickle cell trait are less likely to catch malaria make a lot of us wonder of these two diseases are associated. “In the 1940s, E. A. Beet, a British medical officer stationed in Northern Rhodesia (now Zimbabwe), observed that blood from malaria patients who had sickle cell trait had fewer malarial parasites than blood from patients without the trait. ” ( innvista) There are researchers have came up with several links between the two diseases. Malaria” is caused by a type of parasite that completes its life cycle in human’s red blood cell. This parasite enters the bloodstream with the aid of a female (Anopheles) mosquito. With individual who has Sickle Cell Anemia, the red blood cell break down when infected with this parasite disallow the development of the “malaria” parasite. There are others explanation such as: Hb S in Sickle Cell individual leads to the polymerization of hemoglobin and this prevent the parasite from ingest the hemoglobin which it needs for its life cycle, therefore the parasite get destroyed.
Also the parasites of “Malaria” need oxygen for their development however a patient with Sickle Cell Anemia can’t provide them just that, therefore they cannot continue their life cycle. There are still questions regarding the relationship between Sickle Cell Anemia and “malaria”, but the explanations offered are quite interested. Sickle Cell Anemia is an inherit disease. An individual who has two copies of the trait will inherit Sickle Cell Anemia from the parents.
Those people who only have one copy of this trait will become a carrier for Sickle Cell Anemia, and if their husband or wife also a carrier, the disease will keep passing on. “In Sickle Cell Anemia, the havoc caused by the abnormal hemoglobin s (HbS), results from a change in just one of the 146 amino acids in a beta chain of the globin molecule”( Marieb 642). It is amazing how one tiny change in our body can has such dramatic effect, but on the other hand they all possible; human’s body is extremely complex.
The disease has to do with the protein hemoglobin found in human red blood cell. A normal person’s red blood cell has a round, doughnut shape without the whole in the middle; however a sickled red blood cell will take on a crested moon shape, and these sickled cells are fragile and prone to rupture. The normal life p of a healthy red blood cell is about 120 days before spleen takes out the bad ones and the bone marrow replace them. The sickle cells will go bad in only about 10 to 20 days.
Imagine the spleen has to work extra hard to get rid of these bad blood cells, and with patients of sickle cell anemia about all of their hemoglobins are bad, therefore spleen has to take out all of them; that’s why these patients have to get their blood transfuse regularly. These sickled cells after got produced by red bone marrow can go back and forth between being normally shaped and sickle shaped until they eventually become sickle shaped permanently. And Instead of moving through the bloodstream easily like normal red blood cells, these sickle cells are very sticky and so they can clog blood vessels. These events interfere with oxygen delivery, leaving the victims gasping for air and extreme pain. Bone and chest pain are particularly severe, and infection and stroke are common sequels. ” ( Marieb 642). Sickle cell anemia produces a chronic anemia which may become life- threatening when hemolytic crises, which is the breakdown of red blood cells, which is when bone marrow fails to produce blood cells. These crises occur under condition of low oxygen, such as during intense exercises, masses of hemoglobin molecules in each red blood cell cut out its normal disk shape.
For some individual the “crises” can last hours, days or even weeks; some has “crises” once a year, for others they occur way more often. There are several symptoms that can be diagnosed as soon as infant is four months old. “The clinical course of sickle cell anemia does not follow a single pattern; some patients have mild symptoms, and some have very severe symptoms. The basic problem, however, is the same: the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood” (Bownas, Jennifer).
And they are: the period of pain (mentioned above), hand-foot syndrome ( babies with swollen hands and feet), jaundice (yellowish color found on the baby’s skin or/and in the white parts of their eyes), frequent infections, Delayed growth and puberty in children and often a slight build in adults. The slow rate of growth is caused by a shortage of red blood cells. There is also vision problem, because of the eyes not getting enough nourishment from circulating red blood cells, these types of damages can be serious enough to cause blindness if not discover on time.
Sickle Cells Anemia is a disease with many complications, and all of them are dangerous for patient’s health. One of which is referred to as ERD (exercise-related death). This scenario happens when a patient with Sickle Cells Anemia attend into a lot of physical practice. “The possibility that previously healthy young people with sickle cell trait might suffer increased mortality from exercise was first suggested by observations of enlisted recruits in US Armed Forces basic training. A military trainee with Hb AS suffered exercise related hypernatremia during physical training in the field.
He only survived a critical illness that included acute renal failure because of dialysis” (Kark, John). And also during that single summer, there were already four deaths among recruiting at this area, all of them were black and had Sickle Cells Anemia. So John Kark, whom is the author of this article, was very positive about the significant risk associate between Sickle Cells and ERD. And I think this is reasonable, because the nature of Sickle Cell Anemia is to decrease dramatically the amount of red blood cell circulating in the human’s body, which will cause the lack of oxygen in multiple organs and muscles in the body.
And when an individual with Sickle Cell try so hard on these physical activities and keep pushing themselves forward, it’s hard to avoid damage to the body, which is very unfortunate. Another big complication with Sickle Cell Anemia is infections. Patients of this disease are extremely prone to infection due to their abnormal red blood cells. Recently the screening tests for Sickle Cell Anemia were required for newborns. Before this happen, 35% of infant with Sickle Cell died from infection.
The most common bacteria that usually attack these infants are treptococcus pneumonia and Haemophilus influenza which can cause pneumonia, blood infections, or meningitis). A death can occur to these infants only a few hours after the fever. Acute Chest Syndrome is also yet another dangerous complication of Sickle Cell Disease. It is the leading cause of illness among the Sickle Cell Anemia patients. “Pulmonary disease, manifested as the acute chest syndrome (ACS) is a common complication of sickle cell anemia.
It is the second most common cause of hospitalization in persons with sickle cell anemia and accounts for 25% of premature deaths. ” (Gladwin, Mark) ACS occurs when the lung tissues are starved for oxygen during a crisis. ACS can be caused from infection that lead us back to Sickle Cell Anemia, to blockage of blood vessels which also remind us of Sickle Cell disease, because of the sickle red blood cells with their crested moon shape, as mentioned earlier, instead of moving smoothly along the blood stream, these sickle cells are very sticky and tend to block the blood vessels.
There are a few symptoms that can be recognized such as: high fever, rapid breathing, wheezing or cough, and finally acute chest pain. As of today, the only potential cure of Sickle Cell Anemia is bone marrow transplant. However according to the mayo clinic’s website, it is extremely difficult to find a matching donor and also, the procedure is very risky and can be life threatening. So for treating Sickle Cell Anemia, there are more aims to reduce the pain of the “crises”. As a result, treatment for sickle cell anemia is usually aimed at avoiding crises, relieving symptoms and preventing complications” (Mayo Clinic Staff). Sickle Cell Anemia victims always have to take antibiotics because of their sensitivity, they are prone for infection. Blood transfusion is an option, with this procedure the patient will get supplied with healthy red blood cells from donors, but this can also be risky, because the new red blood cells also carry with them irons, this can cause excessive iron build up in patients’ body; so people who get blood transfusion also need others treatments to reduce iron level.
A prescription drug called Hydroxyurea is usually used to treat cancer can be use in the severe case of Sickle Cell Anemia for adults; it reduces the frequency of painful crises and may reduce the need for blood transfusions. When one experience “crises”, it can be helpful to have supplemental oxygen, which provides extra oxygen and help breathing easier. Doctors and scientists still in the lab to experiment some more possible cure for Sickle Cell Anemia patients, hopefully they will be successful in finding a complete ure. Sickle Cell Anemia is a serious lifelong disease. It is hard to live with it, and it is just as hard to live with a loved one that has Sickle Cell Anemia.
The “Health Education Research” of Oxford Journal has done a study on mothers who have children with Sickle Cells in Western Nigeria :“ Financial stress and disease factors were met with confrontation, while family sources of stress were either complained about, accepted or avoided. (Olley, Lydia) Oxford Journal also mentioned that less educated family members (especially the mothers) get in more stress than others “Higherlevels of stress were associated with less educated and older women” (Olley, Lydia). Hopefully in the near future, there will be more education, counseling about Sickle Cell Anemia to everyone, so we can take care of the patients better, and bring better attitude and hope toward them, because I believe they need that the most especially from a family member.
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