Sickle Cell Anemia
A sickle cell disorders as a type of common blood disorder related to hereditary factors. This disorder affects the shape of the oxygen-carrying red blood cells which results in the slow movement of these cells, or altogether blocking of the blood vessel. Although there is no cure for this disorder the sickle cell anemia treatment can help to relieve the painful and troubling symptoms.
The red blood cells have hemoglobin in them, which is a protein that carries oxygen in the blood. Normally, the red blood cells are flexible in nature and round in shape which allows them to flow freely throughout the body in the blood vessels and deliver oxygen to the various parts of the body.
The sickle cell disease causes these red blood cells to change their form into a sickle-shape (crescent). These mutated (changed) red blood cells are liable to break apart easily and cause anemia. The lifespan of sickle-cells is also shorter (10-20 days). These cells can also collect together and stick to the walls of the blood vessels which blocks the flow of blood. This condition of blood (oxygen) deprivation may give rise to intense pain and permanent damage to the brain, lungs, heart, liver, kidneys, spleen and bones.
Development of a sudden and severe pain is a medical emergency known as ‘acute sickle cell crisis’ and is suspected to be brought upon by infection and dehydration.
Causes of Sickle Cell Anemia
Sickle cell disease is a hereditary blood disorder and occurs when the person’s hemoglobin-related gene becomes abnormal and causes the production of sickle hemoglobin. When this sickle hemoglobin releases oxygen it begins to form together into hard rod shapes which cause damage to the red blood cells and also changes their shape. The sickle red blood cells are responsible for causing the signs and symptoms of sickle cell anemia.
As this is a hereditary disease children tend to be born (congenital) with it and is not contagious. This disease is the result of the child inheriting one sickle cell hemoglobin gene from each parent.
Signs and Symptoms of Sickle Cell Anemia
Commonly seen signs and symptoms of sickle cell anemia are not seen in infants often and are mostly seen after the baby is at least four months old. These are signs and symptoms of sickle cell anemia:
- Anemia – As the sickle cells are fragile and weak they tend to break apart easily and die, hence depriving you of a considerable amount of red blood cells. As the number of healthy oxygen-carrying red blood cells in the body begins to get low the different parts of the body are deprived of oxygen for energy this results in a feeling of fatigue-causing anemia.
- Erratic pain – Episodes of pain (crises) are one of the major signs of sickle cell anemia. This is caused when the steady accumulation of sickle-shaped red blood cells begins to affect the blood flow to the smaller blood vessels that are situated in your joints, abdomen and chest. Pain is also felt in the bones and may vary in intensity and time, from a few hours to a few weeks. Severe type of crises requires immediate medical attention and hospitalization.
- Hand-foot Syndrome – Babies often show swollen hands and feet as the first signs of sickle cell anemia. The swelling is caused when the sickle-shaped red blood vessels block the blood flow out of the hands and feet.
- Frequent infections – Sickle cells can damage the spleen and affect the body’s infection-fighting abilities. This makes you more vulnerable to even the smallest of infections. Vaccinations and antibiotics are initially used for babies to prevent life-threatening infections such as pneumonia.
- Vision problems – People having sickle cell anemia tend to start losing their normal vision gradually. This happens when the tiny blood vessels in the eye are deprived of blood due to being blocked by sickle-shaped red blood cells.
Diagnosing Sickle Cell Anemia
A blood test is the most suitable diagnostic test to determine the presence of sickle cell anemia in the body. This test checks for the defective form of hemoglobin (hemoglobin S) that underlies the sickle cell anemia.
In case the screening blood test is positive the doctor may recommend several additional tests to confirm the initial diagnosis, such as:
- Microscopic inspection – The sample red blood cells are observed under a powerful microscope to determine the extent of the disease in the body. A low red blood cell count indicates towards the presence of anemia.
Sickle Cell Anemia Treatment
Sickle cell anemia is mostly successfully treated with a stem cell (bone marrow) transplant procedure but finding a suitable donor is a difficult task as well as the high mortality rate associated with it.
Hence, the main aim of the sickle cell anemia treatment is to avoid crises, relieve symptoms and prevent additional complications from arising. Sickle cell anemia treatment requires frequent and regular visits to the doctor for a red blood cell count and to monitor your general health. The treatment procedure may include painkillers, antibiotics, blood transfusions and supplemental oxygen as well as bone marrow transplant.