Overview
Sickle Cell Anaemia is an Autosomal Recessive disorder of red blood cells, characterised by a disorder in the beta chains of haemoglobin.
Sickle Cell Anaemia is the most common form of a group of diseases celled Sickle Cell disease. (See “Other forms of Sickle cell disease” for details.)
When Oxygen levels lower in the blood or oxygen dissociates from Haemoglobin the cells “sickle” and become abnormal, rigid, and banana shaped. Pictured below is a selection of sickled cells:
These sickled cells have a reduced life expectancy and break down in 20 days compared to the 120 day normal life span. This causes the Anaemia, as blood cells cannot be produced fast enough.
Sickle Cell Anaemia causes 100000 deaths a year and 12,500 people in the UK have been diagnosed with this disorder.
Life expectancy in the Western world is 42 for men and 48 for females.
Cause of death includes bacterial infection, strokes or bleeding into the brain and kidney, heart or liver failure.
Sickle Cell Anaemia is characterised by conditions associated with Anaemia (low blood volume) and painful moments. (See Symptoms page for details)
This disorder has no definitive widely available cure but many drugs have been developed to alleviate symptoms.
The disease is most prevalent in African and African-Americans due to Malaria heterozygous advantage. (see Genetics section)
Sickle cells were first discovered in the early 1900s by many different scientists but it wasn’t until 1949 that Linus Pauling discovered that abnormal Haemoglobin was the root of the problem.