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What is Sickle Cell Anemia? Know Symptoms, Causes, and More

What is Sickle Cell Anemia? Know Symptoms, Causes, and More

Sickle cell anemia is a chronic hereditary blood cell disorder. Sickle cell anemia also called sickle cell disease (SCD), is a genetic disorder mainly involving red blood cells (RBCs). Typically, RBCs have a disc-like shape, which provides them the flexibility to move from the narrowest and smallest blood vessels. However, with sickle cell anemia, the RBCs of a person get an abnormal crescent shape that looks like a sickle. This causes the red blood cells to be rigid, sticky, and prone to being trapped in the small blood vessels. Sickle cell anemia is a condition that ultimately blocks blood from reaching various parts of the body. This action ultimately becomes a cause of low oxygen levels in the blood. Therefore breathing difficulties, tissue damage, and pain are some common symptoms of sickle cell anemia. Till now, there is no sure shot cure discovered for sickle cell disease. However, some medicines and natural treatments, such as Himalayan shilajit resin, are beneficial to relieve pain and other complications related to the disease.

Sickle cell disease is an autosomal recessive health condition. This means a person has to have two copies of a gene to have sickle cell anemia. Only one copy of a gene results in sickle cell trait, which is different from sickle cell anemia. This condition is not very severe as it provides people with a healthy oxygen level and sufficient normal hemoglobin to prevent the sickling of their red blood cells. The people who have sickle cell trait have fewer chances of developing sickle cell disease. It happens in sporadic cases; however, sickle cell traits can also get genetically transferred, like sickle cell anemia. 

Who is Prone to Sickle cell Anemia?

If both the parents have sickle cell trait, then chances are very high that their children will have sickle cell anemia. Hemoglobin electrophoresis is a type of blood test which helps determine if the child has sickle cell anemia. It also reveals the type of sickle cell disease. The carriers of sickle cell disease are mostly the people who live in regions that have categorized malaria as an endemic. Most of them belong to Africa, the Mediterranean, India, Saudi Arabia, etc. 

Types of Sickle Cell Anemia

Hemoglobin SS Disease

Hemoglobin SS disease is the most common among the types of sickle cell anemia. The body formulates it when it inherits the copies of the hemoglobin S gene from both the parents. This creates hemoglobin protein known as Hb SS. This is the most severe form of sickle cell disease; hence, individuals with this health issue often experience the worst symptoms. 

Hemoglobin SC Disease

Hemoglobin SC disease is the second in the list of the most common category of sickle cell anemia. This complication occurs when an individual inherits the Hb S gene from one parent and the Hb C gene from another one. People with Hb SC have identical symptoms to individuals who have Hb SS sickle cell anemia. However, the Hb SC anemia is less severe.

Hemoglobin SB+ (beta) Thalassemia

Hemoglobin SB+ (beta) thalassemia interrupts the production of the beta-globin gene. The size of the red blood cell reduces in people with Hemoglobin SB+ (beta) because the body creates fewer beta proteins. If inherited along with the Hb S gene, the patient will also have hemoglobin S beta-thalassemia. Symptoms of this sickle cell anemia are not as severe as the other ones.

Hemoglobin SB 0 (Beta-zero) Thalassemia

Sickle beta-zero thalassemia is the fourth category of sickle cell anemia. It also involves the production of the beta-globin gene. It has alike symptoms to Hb SS anemia. But, sometimes the symptoms of Hemoglobin SB beta zero thalassemias are more severe than the rest of sickle cell anemia. It is also associated with a poor prognosis.

Also See: Symptoms of Iron Deficiency Anemia

Symptoms of Sickle Cell Anemia

Symptoms of sickle cell anemia appear 5-6 months after the birth of an infant. The symptoms of the disease majorly depend upon the type of anemia. They may vary in people with sickle cell disease and change as per the treatment and surroundings. Below listed are some major symptoms of sickle cell anemia- 

Anemia

Sickle cell disease makes the cells break and destroy easily, leaving the body with very few red blood cells. Red blood cells generally live for 120 days before they get renewed. But sickle cells usually just die in 10 to 20 days, lacking red blood cells in the body. This condition of lack of red blood cells is called anemia, and it is a major symptom of sickle cell anemia. Lack of red blood cells also causes a lack of oxygen in the body which ultimately causes fatigue and other similar health concerns. 

Recurrent Pain

Recurrent episodes of pain, also known as pain crises, are a significant symptom of sickle cell anemia. The pain grows when the blood flow gets blocked in the tiny blood vessels by the sickle-shaped, which has to reach various body parts such as the abdomen, chest, and joints. The pain caused by sickle cell anemia can even get inside the bones. The pain can vary in intensity and can last from a few hours to a few weeks. Some people face little pain crises, and some get extreme pain that they have to rush to a hospital. Some adolescents with sickle cell anemia also have intense pain, resulting from causes like joint and bone damage, ulcers, or some other reason.

Swelling of Hands and Feet

The blockage of red blood cells causes swelling of hands and feet in a person having sickle cell anemia.

Vulnerability to Infection

Sickle cell anemia can damage the spleen, leaving the patient more prone to infections. Doctors commonly advise children and infants with sickle cell anemia antibiotics and vaccination to prevent possibly life-threatening infections, like pneumonia.

Delay in Growth or Puberty

Red blood cells provide the body with essential nutrients and oxygen needed for growth. A shortage of healthy red blood cells causing sickle cell anemia also results in stagnant growth in infants and children and even delays puberty in teenagers.

Lack of Vision

Small blood vessels that supply eyes vision can get plugged with sickle cell anemia. This can ruin the retina (the part where the eyes process the visual images) and lead to vision issues.

Also See: Home Remedies for Joint Pain

Causes of Sickle Cell Anemia

A mutation in the gene causes sickle cell anemia which produces an iron-rich compound that inhibits the red color of the blood. This same activity also enables red blood cells, with the help of hemoglobin, to transfer oxygen from the lungs to the rest parts of the body. In sickle cell anemia, unusual or abnormal hemoglobin production causes the mishappening of red blood cells and makes them rigid and sticky. If the child gets affected by sickle cell anemia, it is evident that both the parents’ defective genes have been transmitted in the child’s body. 

If only one parent transfers the sickle cell gene to the child, then there are chances of getting sickle cell trait. With one defective gene and one normal hemoglobin gene, individuals with sickle cell traits create both sickle cell hemoglobin and normal hemoglobin. Their blood might contain sickle cells, but they generally do not experience intense symptoms. The parents become carriers of sickle cell disease and become capable of passing the defective genes to their children. 

Sickle cell anemia can lead to many complications as well. These include:

Stroke

Sickle cell anemia can block the blood flow to a part of the brain. Signs of a stroke include weakness or numbness of your arms and legs, seizures, sudden speech difficulties, problem in concentration, and loss of consciousness. Immediate medical attention is required if a sickle cell anemia patient faces a stroke.

Acute Chest Syndrome

A lung infection or sickle cell anemia that blocks blood vessels in the lungs can cause life-threatening complications, like fever, chest pain, and difficulty breathing. In such situations, sickle cell anemia patients require emergency medical treatment.

Pulmonary Hypertension

Individuals with sickle cell anemia can experience high blood pressure followed by shortness of breath. This complication generally affects adults. Fatigue and breathing issues are common symptoms of this sickle cell anemia which may prove fatal.

Organ Damage

Sickle cells block the blood flow to several organs and deprive the organs of blood and oxygen. This is why blood gets chronically low in people with sickle cell anemia. This lack of oxygen blood can damage nerves such as the liver, kidneys, and spleen.

Blindness

Sickle cell anemia can block oxygen to the tiny blood vessels of the eyes. Over time, this can damage eyesight and may even lead to blindness.

The treatment of sickle cell anemia is very subjective. It completely depends upon the current health condition of the patient and measures prescribed by the individual’s doctor. The general treatment of sickle cell anemia involves rehydration, pain relievers, treatment of underlying infections, and in some cases it may demand blood transfusions and supplemental oxygen.

Also See: Best Natural Ways To Increase Oxygen in The Blood

FAQs About Sickle Cell Anemia

Q.1 How common is sickle cell anemia?

Ans. SCD affects roughly about 100,000 Americans. Sickle cell anemia occurs among approximately 1 out of every 365 Black or African-American births. It occurs among 1 out of every 16,300 Hispanic-American babies. The Sickle cell trait even affects Asians, Hispanics, Caucasians, and people from the middle east. 

Q1. Is sickle cell anemia genetic?

Ans. An infant born with sickle cell disease inherits defective genes from both parents. When both mother and father have the genetic defect, there is a 25 percent chance of the child being born with sickle cell disease.

Q.3 Is sickle cell anemia dangerous?

Ans. Yes. The interruption of blood flow can prevent oxygen from reaching several parts of the body. This can damage nerves and organs such as kidneys, liver, heart, et. and can be fatal. 

Q.3 Can white people get sickle cell anemia?

Ans. The disease is an inherited blood disorder. 1 million to 3 million Americans get affected by sickle cell anemia. 

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