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Sickle cell disease is a hereditary blood condition that is passed on from parent to child. The disease results in abnormally shaped red blood cells, which become crescent shaped or sickle shaped instead to their normal disk shape. This abnormal form causes many complications for the individual, the most severe of which is called Sickle Cell Crisis.

The cause of a Sickle Cell Crisis goes back to the faulty gene that the offspring inherited from his or her parent. This gene, which codes for a specific chain of the hemoglobin protein, is mutated and results in a dysfunctional form of hemoglobin. The hemoglobin protein is responsible for binding to oxygen and carrying it throughout the body, but in these mutated versions, the protein cannot properly hold on to oxygen. This oxygen deficiency distorts the entire blood cell, twisting it into the sickle shape and making it more rigid. When these distorted blood cells try to pass through capillaries (very narrow blood vessels), the crescent-shaped cells hook together and clog up the blood vessel, reducing or entirely halting blood flow. The bodily tissues downstream of the blockage now become oxygen starved and the individual experiences extreme pain, the defining characteristic of a sickle cell crisis.

Though a sickle cell crisis can occur in any individual with sickle cell disease, many individuals live perfectly fine for extended periods of time without suffering from a sickle cell crisis. This is because these attacks only occur under certain circumstances. Individuals who are suffering from extreme stress, dehydration, infection or even pregnancy are more likely to have a sickle cell crisis than others. Also, if a person already experiences a partial blockage in a blood vessel, due to fat deposits on the blood vessel wall, their chances of encountering a sickle cell crisis are very significant.

Luckily, treatments for a sickle cell crisis do exist. One of the simplest self-treatments is to drink plenty of water. This causes the blood to be more fluid and thus allowing it to flow through narrow blood vessels to easily. In some cases, fluids are administered intravenously to quickly elevate blood fluidity. Another treatment that has been shown to be effective is blood transfusion. By giving the individual a unit of normal-shaped red blood cells, they are better able to feed their body tissues with the oxygen they need. Less effective treatments include pain-killers to numb the pain associated with the oxygen deprived tissue, or antibiotics to reduce inflammation in hopes of opening up narrow blood vessel.

Paradoxically, there are benefits to having sickle cell disease. A type of parasite, called Malaria, lives in the blood stream of mammals, feeding off their blood cells. Malaria, however, cannot survive in the blood stream of sickle cell individuals due to their abnormal blood cells. As a result, these individuals are effectively immune to malaria. Natural selection has thus resulted in a high prevalence of sickle cell disease in areas of the world where malaria is common, such as Africa. In fact, about 1 in 370 African Americans is born with the disease.

Sickle cell crisis is a very dangerous and potentially fatal reality of the sickle cell disease. However, with improved medications and treatment options, the disease can be reasonably well managed.

Learn more about this author, Brad Norman.
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