Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Sickle-cell disease or sickle-cell anaemia (or anemia) is a 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 other complications. Life expectancy is shortened, with studies reporting an average life expectancy of 42 and 48 years for males and females, respectively.[1]
Sickle-cell disease occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common. One-third of all aboriginal inhabitants of Sub-Saharan Africa carry the gene.[2] This is because those with only one of the two alleles of the sickle-cell disease are resistant to malaria, since an infestation of the malaria plasmodium is halted by the sickling of the cells which it infests. In areas where malaria is common, there is a survival value in carrying a single sickle-cell gene, AS heterozygotes.[3]
The prevalence of the disease in the United States is approximately 1 in 5,000, affecting mostly blacks according to National Institute of Health.
Sickle-cell anaemia is the name of a specific form of sickle-cell disease in which there is homozygosity for the mutation that causes HbS. Sickle-cell anaemia is also referred to as “HbSS,” “SS disease,” “haemoglobin S,” or permutations thereof. Other, rarer forms of sickle-cell disease include sickle-haemoglobin C disease (HbSC), sickle beta-plus-thalassaemia (HbS/ß+) and sickle beta-zero-thalassaemia (HbS/ß0). These other forms of sickle-cell disease are compound heterozygous states in which the person has only one copy of the mutation that causes HbS and one copy of another abnormal haemoglobin allele.
The term “disease” is applied since the inherited abnormality causes a pathological condition that can lead to death and severe complications. Not all inherited variants of haemoglobin are detrimental, a concept known as genetic polymorphism.
Sickle Cell Anemia usually occurs in Black children but sometimes occurs in Hispanic children. About one in five hundred Black children have it and about one in one thousand Hispanic children end up with sickle cell anemia.
Sickle-cell disease may lead to various acute and chronic complications, several of which are potentially lethal.
[tubepress mode=’tag’, tagValue=’Sickle-cell disease’]