Sickle cell anemia affects 70,000 to 100,000 Americans and millions of people worldwide. It occurs especially in people whose ancestors are from sub-Saharan Africa, Spanish-speaking regions of the Western Hemisphere such as South America, Central America, and the Caribbean. This hereditary disease also occurs in Mediterranean countries such as Turkey, Greece, Italy, Saudi Arabia and India.
The disease provides protection against malaria in regions of the world where malaria is endemic or continuously present. Though several forms of sickle cell disease exist, the hemoglobin SS is the variant which affords the most protection against malaria. These patients have a survival advantage over individuals in malaria endemic parts of the world who do not have the sickle cell gene.
Patients with sickle cell disease will have symptoms of a crisis, but those with the trait do not generally experience symptoms. However, people with the trait may have problems with high altitudes such as when they climb mountains, fly, or reside in high altitude regions. Individuals with the trait may also encounter pain or other symptoms with scuba diving or when they become dehydrated.
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