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:

Sickled and normal cells that would be found in a patient with Sickle Cell Anaemia

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.


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News

The Times - Sickle cell disease is spreading through the UK

22/09/2008 00:00
  Pamela Gyebi-Ababio, 18, first realised she had sickle cell disease when she started primary school. “I was always cold and had to wear socks and tights and I remember the head teacher picking on me for this in front of the whole school. It was horrid and I cried. As a result my parents...

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BBC - Calls to treat Sickle Cell better

20/05/2008 00:50
Treatment of sickle cell anaemia is compromised by health workers' lack of knowledge, a report warns.    The first national survey of the disease found seriously ill patients were not offered support from sufficiently experienced staff. Patients' use of painkillers was not effectively...

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The Guardian - Scientists raise hope for sickle cell patients

10/01/2006 09:28
  Scientists have developed a technique using embryonic stem cells to treat the genetic abnormality that causes sickle cell anaemia. Sickle cell anaemia is one of the most common genetic disorders worldwide and primarily affects people of African, Mediterranean, Middle Eastern and Indian...

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