Living with sickle cell disease can be risky and challenging, but l have a belief that it makes us some of the supereminent people on the planet. The first time l learned l carry the sickle cell characteristic was in a biological class at senior high school. I couldn't believe what the educator was saying. I could relate to the symptoms she gave on sickling. Immediately, I left the class at a nearby hospital to run a test. They run a hemoglobin electrophoresis test on me and it turned out to be positive. Instantly, I became bitter towards anyone around me particularly my parents. I lost interest in moving on in the world. I lost my reasons for living. I thought l was going to perish because of how the disorder was explained in class. I started to live anyhow until hope gripped me.
The type of sickle cell disease I have is called hemoglobin sickle cell(Hb SC). Although, it's an easy-going type of sickle cell disorder. l still have pain episodes. pain crisis can happen at any moment. It's uncertain. I can just start hurting all of a sudden and the only way I can term a pain crisis is to weigh it to be rheumatism, but it's worse than it. I use to wrap myself whenever I have a pain crisis and l could barely do the little things such as bathing, eating, and strolling for myself.
Sickle cell is a continual inherited disease dominating millions of humanity worldwide. Generally, in Ghana, as in most parts of tropical Africa, sufferers have limited access to quality clinical, health, social, educational, and psychological scrutiny, which is discouraging. Well, this manuscript focuses on bridging the gaps in health qualities, with precise emphasis on the health status of children with sickle cell illness, especially in the unsophisticated sectors in Ghana.
A CLOSE LOOK AT SICKLE CELL DISEASE.
Sickle cell is an inherited condition existing from inception, that makes red blood cells switch shape like a crescent moon and result in discomfort and other health dangers. In sickle cell disease, the cells become thick, rigid, sticky, and can get stuck in tiny blood vessels, which barricade the flow of blood and oxygen to parts of the body.
WHAT CAUSES SICKLE CELL DISEASE?
Sickle cell disease is stimulated by a change in the gene responsible for the globin of normal adult hemoglobin A, dominating the production of sickle hemoglobin. In other words, sickle cell disease is inherited when a child obtains two sickle cell genes one from each parent. Also, if only one parent passes the sickle cell genes to the child, that youngster will have the cell trait. With one normal hemoglobin gene and one impaired form of the gene. However, people with the sickle characteristic make both normal hemoglobin and sickle cell hemoglobin.
TYPES OF SICKLE CELL DISEASE.
The subsequent are the most common types of sickle cell disease.
Hemoglobin S Disease(sickle cell trait); this happens when the hemoglobin "S" gene is inherited only from one parent and a normal hemoglobin gene A is inherited from the other, that person will carry the sickle cell trait. But people who have sickle cell trait are mostly healthy. Only infrequent do people who have sickle cell trait have difficulties similar I those seen in individuals who have sickle cell anemia. However, people who have sickle cell trait are carries of an imperfect hemoglobin S gene, so they pass it on to their children. In other words, those with sickle cell trait have a combination of the two types. The difference in electrophoretic mobility is possibly due to a distinct number of ionization amino acid residues in the protein distribution of hemoglobin and that these changes in the molecular arrangement are responsible for the sickling process.
The image above denotes how the hemoglobin S gene is inherited.
Hemoglobin SC disease; is abnormal hemoglobin in which glutamic acid residue at the sixth position of the beta-globin chain is replaced with a lysine residue due to a degree of a mutation in the HBB(beta hemoglobin) gene. It develops sickle cell trait but not the disease. It causes only mild sickling of the red blood cells. Thus, It is the least dangerous among sickle cell traits producing hemoglobin such as HbS and HBO. It occurs when one inherits the HbC gene from one parent and HbS from the other parent.
Hemoglobin SS disease; This is the most common type of sickle. In this type of sickle cell disease, individuals who possess this form of sickle cell disorder inherit two sickle cell genes S from both mother and father. This forms hemoglobin known as HbSS. Similarly, affected individuals have a different form of the metalloprotein(a protein containing a metal atom) hemoglobin in their blood.
Lastly, Hemoglobin SE, SD, SO; are inherited disorders affecting the part of the blood that carries oxygen known as hemoglobin. People with HbSE, HbSD, HbSO have hemoglobin that doesn't work efficiently. Individuals usually develop in early adulthood. They may entail mild anemia, broadened spleen, reduced blood flow to the joints, and unbearable attacks due to blocked blood vessels. Many people with this circumstance have no symptoms. HbSE, HbSO, HbSD are caused by hereditary changes(DNA variants). In the HBB(Beta-globin) gene that influences it to not work correctly. Also, it is inherited in an autosomal recessive pattern. Many people with these disorders do not need treatment.
SYMPTOMS OF SICKLE CELL DISEASE.
Signs and symptoms of sickle cell disease usually happen around the smooth of age. They change over time and vary from person to person. Moreover, episodes of pains, swelling hands, and feet, delayed growth, vision problems, anemia, and regular infections are some of the indications and symptoms of sickle cell disease.
To start with, delayed growth; red blood cells provide the body with the nutrients and oxygen required for growth. However, a shortage of healthy red blood cells can hinder the growth of infants and children. Children with sickle cell disease grow short and skinny.
Furthermore, periodic infections; infection is the invasion of an organism's body tissues by disease-causing agents, their multiplication and consequence of host tissues to the infectious agents, and the toxins they produce. Sickle cells most times damages the spleen which fights invading germs in the blood, exposing the sickler the more to infections. Children with sickle cell anemia are prone to pneumonia.
Lastly, episodes of pain; victims of sickle cell disease develop when sickle-shaped red blood cells block the flow of the blood through blood vessels to your chest, abdomen, joints, and even your bones.
COMPLICATIONS AND MANAGEMENT OF SICKLE CELL DISEASE.
Sickle cell disease is associated with a lot of risk factors and complications. On a more serious note, sickle cell anemia can lead to a host of complications including blindness, organ damage, stroke, leg ulcers, hypertension, gestation complications, and more. Throwing light on gestation complications, sickle cell anemia can enhance the risk of miscarriage, premature delivery, and having low birth weight babies. It can also increase the risk of blood clots during pregnancy. However, sickle cell anemia most commonly affects Africans.
On the whole, the management of sickle cell anemia is usually geared toward preventing pain episodes and ridding symptoms. Also, treatments might include medications and blood transfusions which cost. For some youngsters, a stem cell transplant also known as a bone marrow transplant might ease the disease.
In a nutshell, like a sickle cell patient, find something that grounds you and gives you hope. Do not look at your condition as a death penalty. Do not let go of your ambitions and do not let sickle cell disease control you. You should have control of sickle cell disease. Well, mine wasn't a sad saga after all. Why? because I defeated death. So, you can also come out of it strong and healthy.
Lambda Stories Newsletter
Join the newsletter to receive the latest updates in your inbox.