Sickle Cell Anemia and Systemic Complications

In a post from MD Magazine, a video from the HCPLive Network depicts a conversation between a number of sickle cell anemia experts about different clinical presentations and complications associated with the illness. More specifically, they discuss some of the more common symptoms as well as some that are less frequently documented. The conversation highlights the variability of the manner in which sickle cell anemia can affect patients.

About Sickle Cell Anemia

Sickle cell anemia is a genetic, inherited disorder of the blood. This disorder is characterized by an abnormality affecting hemoglobin, which is found in red blood cells and is responsible for carrying oxygen. The abnormality causes blood cells to lose their typical circular shape and instead take on an elongated, sickle-like appearance. This is caused by a genetic mutation that may have arisen as a defense against malaria, although this benefit only occurs in people with sickle cell trait, not the disease. Symptoms begin to appear at around six months old and include swelling of the hands and feet, stroke, bacterial infections, and acute episodes of severe pain termed sickle cell crisis. Severity of disease varies, but these attacks can result in serious declines in health and organ damage. Treatment is mostly symptomatic, but bone marrow transplant has been curative in children. The disease most frequently affects people of African ancestry. Life expectancy is between 40 and 60. To learn more about sickle cell anemia, click here.

Sickle Cell Crisis

Perhaps the most common problem experienced by patients is sickle cell crisis, also known as vaso-occlusive crisis. These distressing episodes of acute pain are the result of micro-blockages caused by the misshapen blood cells. While this is one of the most characteristic features of the disease, not every patient will have them, and there are a variety of symptoms that only a small percentage of patients may experience.

Other Complications and Risks

As an example, pulmonary arterial hypertension (high blood pressure in the lung arteries) is a problem for about six percent of patients. This is associated with more severe disease and an increased risk of death. The spleen is also affected in this illness. In most cases, the spleen dies, with 90 percent of patients losing it in childhood. This increases infection risk. In other cases, the spleen becomes enlarged, which is a potential emergency.

Microcirculation blockages can put many organs at risk, such as the kidneys. Patients are also at a greater risk of strokes, and these factors can progress in severity even when a patient is relatively comfortable and unaffected by pain. Neurocognitive effects can also be a major factor, especially as patients get older.


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