Sickle cell anemia is the most usual form of SCD or also known as sickle cell disease. This is considered as a serious disease wherein the body produces red blood cells in the shape of a sickle hence the name sickle cell anemia. The shape of the red blood cells running through the blood is that of a crescent which is abnormal compared to the disc-shape, almost like a donut just with the absence of the hole at the center. They easily move around inside the blood vessels. The hemoglobin is the iron-rich protein found within the red blood cells which are responsible for carrying oxygen all throughout the body.
The sickle cell holds irregular hemoglobin as well which are known as sickle hemoglobin or hemoglobin S. The sickle hemoglobin is the one giving the red blood cells the shape of a crescent. The sickle cells are also sticky and stiff and they are capable of blocking the flow of blood within the blood vessels of the organs and limbs. If the flow of blood is being blocked then it will result to further complications within the body many of which are serious such as organ damage and severe infections.
What is sickle cell anemia?
The sickle cell anemia is one of the forms of anemia. Anemia is a condition associated with blood wherein the number of red blood cells is lower than normal. Anemia also happens when the red blood cells do not have enough hemoglobin or the shape and size of these cells are irregular.
The organ responsible for manufacturing the red blood cells is the bone marrow. This is the spongy marrow located within the large bones. The bone marrow is also responsible in making new red blood cells to replace the old ones from the blood. The normal lifespan of the red blood cells is about 120 days within the bloodstream. They are responsible for carrying oxygen to the entire body and at the same time expelling carbon dioxide or the waste product out of the body.
When it comes to sickle cell anemia, the amount of red blood cells flowing in the blood is lower than normal because the sickle cells do not live up to the expected lifespan of the red blood cells. The usual lifespan of the sickle cells lasts between ten to twenty days only. The spongy marrow or bone marrow cannot keep up in replacing the ones that died. This results to reduced quantity of erythrocytes (red blood cells) in the blood.
Sickle cell anemia is a genetic disorder. Oftentimes anemia is only diagnosed after the child is being diagnosed with it. The disease lasts a lifetime so it needs constant monitoring by the doctor. The hereditary trait is possible only if there are two genes which come from each parent that is responsible for making sickle hemoglobin. Those who have inherited the sickle hemoglobin from a parent with the SCD and a normal parent possess the condition known as sickle cell trait. This is quite distinct from sickle cell anemia. People with the sickle cell trait do not have the disease but they are capable of passing the gene to their children.
The sickle cell disease varies from one person to another, of which some of them have long-term or chronic fatigue or pain, and sometimes tiredness. The disease can be managed, but there is no sure-fire way to cure it.
Causes
Since it is an inherited trait, only those who have both parents with these genes are affected.
Symptoms of Sickle Cell Anemia
The symptoms vary from person to person as well. They range from mild signs to severe ones where there is often a need to be brought to hospital for emergency treatments. They are usually present after birth but some symptoms are not identifiable until the infants reach the age of four months. The symptoms that link to sickle cell anemia are the following:
• Fatigue or the feeling of weakness or tiredness.
• Headache
• Dizziness
• Coldness in the feet and hands
• Paler appearance like being drained out of blood
• Jaundice – some patients with anemia are often mistaken to have HIV because of jaundice or the yellowish color of the whites in the eyes and the skin.
• Shortness of breath
There are other symptoms of anemia where the patient feels pain such as sudden pain that often attacks the lungs, joints, abdomen and bones. The pain felt from sickle cell anemia can be acute, which is highly common; or chronic, such as in cases where there is organ damage. The pain ranges from mild to intense ones and usually lasts for hours. However, the pain could take as long as a few weeks or more. Other people with the sickle cell disease have chronic pain, particularly in the bones. The pain usually lasts for weeks to months which can be intolerable and drains both the physical and mental health. Chronic pain affects daily activities since it impedes the person from moving normally.
Since sickle cell disease affects various areas of the body. It sometimes causes a wide array of complications such as:
• Splenic crisis
• Hand-foot syndrome
• Infections
• Pulmonary hypertension
• Delayed puberty and slowed growth
• Acute chest syndrome
• Eye problems
• Stroke
• Gallstones
• Priapism
• Leg ulcers
• Failures of multiple organs
Treatment and prevention of sickle cell anemia
Like anemia which has no definite cure, it is also the same with sickle cell anemia. Although treatments are available in order to lessen the complications and live life normally. The goal of the treatments is to prevent complications such as stroke, organ failures and infections. Constant monitoring by the doctor is imperative for this disease.
Mild pains are treated with over-the-counter medications coupled with heating pads, drinking plenty of fluids and rest. Severe pain needs medical attention. Worst cases require emergency treatment or an extended hospital stay for proper monitoring.
Severe sickle cell anemia is treated with medicine known as hydroxyurea which stimulates the body in creating fetal hemoglobin or hemoglobin F. This is a type of hemoglobin found in newborn babies. This helps prevent the red blood cells to form into sickles and improves the body’s chances of not developing anemia.