What is sickle cell anemia?
Sickle cell anemia is an inherited blood disorder characterized by abnormally shaped red blood cells that block the flow of blood. This is a genetic disorder which affects the structure of hemoglobin.
This disease is not contagious like a cold or cough so it cannot pass from one person to another. This disease is inherited, which means it is passed by genes from parents to their children.
The distorted red blood cells are prone to rupture, resulting in increased destruction. Obstruction of blood vessels by sickle-shaped cells may damage the organ and cause pain.
It is more common among Africans and African-Americans.
There is no complete cure for sickle cell. Treatment strategies focus on managing the symptoms.
Sickle hemoglobin is not like normal hemoglobin. They can form stiff rods within these red cells. These sickle shaped cells are not flexible like normal red cells, and can stick to blood vessel walls. This causes blockage, leading to slowing down or stopping the flow of blood. This in turn affects the supply of oxygen to nearby tissues.
This lack of oxygen in the tissue can cause severe panic attacks and pain. These attacks and pain occur without any warning sign. To get these treated the person would need to go to the hospital.
These sickle shaped red blood cells undergo premature death since they cannot function properly due to their shape. This leads to anemia. Sickle cells damage the spleen which is an important organ for fighting infections in the body.
Symptoms
All symptoms of sickle cell anemia are caused by the abnormally shaped red blood cells that block the flow of blood in blood vessels.
Those people affected with sickle cell anemia start to show signs of the disease during the first year of life. This is usually around five months of age. The symptoms and complications of sickle cell anemia vary for each individual and also can range from mild to severe.
The main symptoms of sickle cell anemia are:
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Anemia
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Pain crises
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Swelling in hands and feet
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Frequent infections
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Delayed growth
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Vision problems
Diagnosis
Sickle cell anemia is diagnosed by blood test. The diagnostic blood test checks for the presence of hemoglobin S, the abnormal hemoglobin produced in sickle cell anemia.
It is this hemoglobin S that causes distorted red blood cells. Additional tests are recommended to identify the amount of sickle cells.
Blood tests can be conducted on newborns as well as adults or older children. There are two types of sickle cell anemia that are genetically transmitted. In some cases, where both the parents are carriers of hemoglobin S, the child has a risk of developing sickle cell anemia or even.
It is important that the child is diagnosed early with the disease. Couples are urged to undergo prenatal testing if it is found that the parents are at risk of passing sickle cell anemia to their child.
Treatment
Sickle cell anemia is a disease that worsens over time. Treatment of sickle cell anemia is developed around symptom management and preventing complications to lengthen the lives of those who have this condition. Treatment options can vary for each person depending on symptoms and severity of the disease.
Medications, blood transfusions, supplemental oxygen, and bone marrow transplant are the common methods used in controlling symptoms of anemia.
One can live a normal life if sickle cell anemia is detected early. Late diagnosis can make treatment more painful and debilitating.
Antibiotics are prescribed to prevent infections. Vaccinations during childhood are also important in preventing infections.
Prevention
As sickle cell anemia is an inherited disease, genetic counseling is the only way to prevent having a child with the mutations.
Genetic counseling helps to understand the risk of having a child with this condition.
Risks and Complications
Sickle cell anemia may lead to a number of complications such as:
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Stroke
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Acute chest syndrome
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Organ damage
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Pulmonary hypertension or high blood pressure in the lungs
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Vision problems or blindness
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Skin ulcers
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Gallstones
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Blockage of blood flow to the lungs leading to acute chest syndrome
