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Sickle-cell disease (SCD), or sickle-cell anaemia (SCA) ordrepanocytosis, is a hereditary blood disorder, characterized by red blood cells that assume an abnormal, rigid, sickle shape. Sickling decreases the cells' flexibility and results in a risk of various life-threatening complications. This sickling occurs because of a mutation in the haemoglobin gene. Individuals with one copy of the mutant gene produce a mixture of both normal and abnormal haemoglobin. This is an example of incomplete dominance.Life expectancy is shortened. In 1994, in the US, the average life expectancy of persons with this condition was estimated to be 42 years in males and 48 years in females,[1] but today, thanks to better management of the disease, patients can live into their 70s or beyond.[2]
Sickle-cell disease occurs more commonly among people whose ancestors lived in tropical and sub-tropical sub-saharan regions where malaria is or was common. Where malaria is common, carrying a single sickle-cell allele(sickle cell trait) confers a selective advantage—in other words, being aheterozygote is advantageous. Specifically, humans with one of the twoalleles of sickle-cell disease show less severe symptoms when infected with malaria.[3]
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