Sickle cell disease is an inherited condition that affects every organ and system of the body. It is mainly characterized by sickle or C-shaped erythrocytes--red blood cells. Normal red blood cells are round or doughnut-shaped with an indentation instead of a hole in the middle. In sickle cell anemia, the red blood cells become deformed due to defective hemoglobin production by the body. Hemoglobin is the vital protein inside red blood cells that binds to oxygen. Sickle cell disease is a lifelong disease that cannot be prevented or cured, however its symptoms can vary between individuals as outlined by a study published in the "Journal of Occupational Medicine."
Anemia
The primary characteristic of sickle cell disease is the deformity to the shape and function of the red blood cells produced by the body. Healthy red blood cells can survive for up to 120 days, while fragile sickle cells live for only 10 to 20 days. According to the National Heart, Lung and Blood Institute this causes a shortage of oxygen-carrying red blood cells because the bone marrow cannot replace red blood cells as quickly as they break down. This causes anemia with symptoms such as chronic fatigue, dizziness, weakness and increased heart rate.
Blood Vessel Damage
The University of Maryland Medical Center lists blocked or narrowed blood vessels as a characteristic of sickle cell anemia. Unlike healthy red blood cells that remain smooth and spherical, sickle cells become stiff and have difficulty flowing through narrow and divergent blood vessels, due to their sickled shape. Sickle cells also become sticky causing them to clump together to form blood clots or stick to the walls of blood vessels causing stenosis or narrowing. This can result in heart disease, stroke and characteristic symptoms of sickle cell anemia such as pain and swelling of the hands and feet. Decreased blood flow can also lead to slowed development, growth, immune response and healing of the skin and organs.
Spleen Damage
The University of Maryland Medical Center notes that spleen damage in individuals with sickle cell anemia proves so common, that most children with this disease do not have a functioning spleen or undergo surgery to remove the spleen by the age of eight. The spleen can become damaged, enlarged and cause severe pain due to trapped sickle cells in a condition called splenic sequestration or pooling. This can cause a dramatic decrease in hemoglobin in the body that can lead to death if left untreated. In many individuals, the best treatment is removal of the spleen.
