One of the major functions of the blood is to carry oxygen from the lungs to the tissues. To accomplish this, the blood relies on a protein called hemoglobin, which binds to oxygen at the lungs and releases it at the cells. Red blood cells are filled with hemoglobin, making them the blood's oxygen-carrying cells. Sickle cell anemia is a genetic disease in which the genetic material, or DNA, carries mistaken information about how to make hemoglobin, resulting in faulty hemoglobin production in the body. Many body cells are affected by the disease.
Red Blood Cells
Of all of the cells negatively impacted by sickle cell anemia, perhaps the most obvious are the red blood cells. Correctly formed hemoglobin assembles into normal red blood cells, which have a biconcave shape, says Dr. Gary Thibodeau in his book, "Anatomy and Physiology." Malformed hemoglobin produced by individuals with sickle cell anemia packs into red blood cells incorrectly, resulting in oddly shaped cells. Red blood cells of sickle cell patients are shaped like quarter moons, or sickles, hence the name of the disease. These misshapen blood cells can't carry oxygen as effectively as normal red blood cells and have a tendency to stick to each other and form clots.
Body Organ Cells
The cells of the body organs are nearly all effected by sickle cell anemia. According to the Mayo Clinic, the tendency of sickled red blood cells to cluster and form clots means that they block blood flow to organs, particularly flow through narrow vessels. This leads to reduced organ function and severe pain. The liver, which receives a significant amount of blood flow because it's responsible for detoxifying blood, is particularly affected, as are the kidneys, which filter blood. The spleen, too, is often subject to organ damage, because it is a highly vascular organ, meaning that significant quantities of blood pass through it on a regular basis.
Brain Cells
A stroke is a serious event resulting from the formation of a blood clot in one of the vessels that supplies blood to the brain. After a clot forms, blood can't reach the part of the brain fed by that vessel, leading to brain cell death and loss of function. Since sickle cell anemia patients have the tendency to form blood clots much more frequently than those with normally shaped red blood cells, they're particularly susceptible to stroke and brain cell damage, according to a 2001 article published in the journal Hematology. The authors note that headache and loss of consciousness, dizziness and confusion often mark the onset of a stroke event. They further note that stroke and brain cell damage are so common in sickle cell patients that when a patient with sickle cell presents to a physician with altered neurological capabilities, the physician should assume that the patient has had a stroke unless evidence strongly suggests otherwise.
References
- "Anatomy and Physiology"; Gary Thibodeau, Ph.D.; 2007
- Mayo Clinic: Sickle Cell Anemia
- "Hematology"; Sickle Cell and the Brain; Robert Adams, M.D. et al; 2001
