Sickle cell disease is genetic condition of the red blood cells that results when a person inherits two sickle cell traits from his parents. The hemoglobin part of the red blood cell is defective and does not function appropriately. Hemoglobin allows the body to carry oxygen from the lungs around the body and to remove carbon dioxide. This results in red blood cells dying ahead of their normal cycle, a condition known as anemia, which deprives the body of oxygen.
Pain (Vaso-occlusive Crisis)
Episodes of pain experienced throughout the body are classic symptoms of sickle cell disease. This pain is also known as a vaso-occlusive crisis. This occurs when a person is involved in strenuous activity that increases the body's demand for oxygen. The sickled red blood cells, which are not able to bind the normal oxygen content, are unable to meet that requirement. Pain can also occur because of obstruction caused by the deformed red blood cells as they pass through the blood stream. The pain happens in intervals and is sharp and intense. It is mostly felt in the bones, as red blood cells are made in the bone marrow. When this pain happens in the lungs, it is known as acute chest syndrome, which can be life-threatening.
Spleen Damage and Infections
Children and adults with sickle cell disease are susceptible to infections. This is especially crucial for newborns, whose immune systems are not fully developed. This is also because of the damage done to the spleen, which, although it initially enlarges because of trapped red blood cells within, eventually shrinks completely. According to Children's Hospital Boston, most patients will end up with a permanently non-functional spleen.
Aneurysms and Strokes
The brain requires the most amount of oxygen in the body. With sickle cell disease, the short supply of oxygen supplied to the brain eventually causes a stroke. In sickle cell patients, blood vessels, which carry red blood cells around the body, are also prone to rupturing and tearing; this is known as an aneurysm. Multiple cases of aneurysms can also lead to a stroke.
Other Complications
Oxygen deprivation can affect the kidneys, resulting in infections. Because the kidneys acts as the body's filter, this affects urination and blood gas levels. Urine output is decreased, and urination is frequent, while blood gas levels build up and can be toxic to the body.
The liver is also affected, because it works together with the kidneys, and it can get enlarged. Gallstones are a common occurrence and cause pain in the abdomen, especially after eating.
Priapism, the condition where males maintain extended and painful erections, is commonly seen in males with sickle cell disease. This can ultimately lead to erectile dysfunction.
