Sickle Cell Anemia

First discovered in 1904 when a black student from Grenada became ill while attending Chicago College of Dental Surgery. This was the beginning of the medical professions recognition of Sickle Cell Anemia. However, there is evidence that Sickle Cell Anemia has been around for centuries some evidence dating back as far as 1670.

In order to contract Sickle Cell Anemia one must have a special type of hemoglobin called sickle hemoglobin. Hemoglobin itself is a substance in the red blood cell that carries oxygen from the lungs throughout the body. Red blood cells with normal hemoglobin remain round after releasing oxygen where as, sickle hemoglobin assumes a sickled shape. When sickle hemoglobin releases oxygen molecules tend to join together this causes the hemoglobin to form rods in turn alters the shape of the red blood cell itself. These red blood cells have two characteristics that are not helpful, cells are rigid and fragile. Normally, red blood cells are round, soft and pliable. This is so they may move through small blood vessels. However, the red blood cells of someone with Sickle Cell Anemia will not move through these vessels, this is due to their rigidity, plug the blood vessels and occasionally obstructing the flow of blood. This usually causes tissue and/or organ damage. The normal life span of red blood cells is 120 days, but one of someone with Sickle Cell Anemia is only six to thirty days and the bone marrow cannot make replacement cells fast enough to replace those that are destroyed. The result is a constant anemia (smaller than normal quantity of red blood cells and hemoglobin).

Sickle Cell Anemia is determined by genes that govern what type of hemoglobin will be produced, thus it is a genetic disease. The type of hemoglobin one has is determined by two hemoglobin genes, one from each parent, only those who inherit sickle hemoglobin gene from both parents have Sickle Cell Anemia. Practically, all of the hemoglobin in Sickle Cell Anemia patients are sickled and rod formation readily occurs in many cells when oxygen is released to the tissues and organs.

There are three types of sickle cell disease: sickle cell-hemoglobin C disease, which is when an individual inherits a gene of sickle hemoglobin from one parent and for hemoglobin C abnormal hemoglobin) form the other. The other is sickle cell-Beta+ and Beta and thalassemia disease, condition in which the individual has inherited a gene of sickle hemoglobin from one parent and a gene of thalassemia from the other. This gene, thalassemia, decreases or prevents the production of normal hemoglobin. These three diseases are usually milder in severity than Sickle Cell Anemia. Inheritance of one gene from one parent results in sickle cell trait; this is when only about one-half of the hemoglobin is sickle. This is too small amount for rod formation and sickling to occur. Thus, sickle cell trait is a carrier form and not a disease.

In the United States, sickle cell condition occurs primarily in African-Americans. Approximately one in twelve African Americans are born with sickle cell trait and one in six hundred with Sickle Cell Anemia. Rarely, do any of the conditions occur in other ethnic groups, but more so in those with African ancestry, Puerto Ricans and Cubans for example.

There are many problems that occur with Sickle Cell Anemia but the primary problem is recurrent, unpredictable pain attacks that may be severe enough to require narcotics and hospitalization. The pain most frequently occurs in the extremities, abdomen, and chest. Additional problems include anemia; a lowered tolerance for exercise; the plugging of blood vessels and acute infection in the lungs: painful swelling of hands and feet in infancy; sudden pooling of blood in the spleen in infancy; susceptibility to overwhelming pneumococcal infections; leg ulcers; breakdown of the head of the femur; they tend to tire more easily; growth retardation; delayed onset of puberty; prolonged, painful erections of the penis; gallstones; and strokes.

Individuals with Sickle Cell Anemia may also have to cope with a number of psychosocial stresses that include a lifetime of unpredictable occurrences of pain, growth retardation, delayed onset of puberty, repeated hospitalizations (more frequently for pain management), unpredictable interruption of important activities, unavailability of a cure, decreased life expectancy, limited job opportunities, and uncertainties about the desirability of marriage and having children. It should also be realized that no Sickle Cell Anemia patient experiences all of these physical and/or psychosocial problems.

When trying to manage Sickle Cell Anemia one has to encompass both the physical and psychosocial aspects of the disease. Though they try continually, physicians have not been able to prevent cells from sickling, convert sickle cells to normal red blood cell shape, give sickle cells a normal life span, prevent sickle cells from plugging blood vessels, or unplug blood vessels that are blocked by sickled cells. Basically the only thing doctors can do is treat the health problems when they occur and efforts to minimize physical