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Sickle Cell & Thalassaemia DisordersSickle cell and thalassaemia disorders are both blood disorders (haemoglobinopathies) affecting haemoglobin (the oxygen-carrying substance in red blood cells). Sickle cell disorders predominantly affect people whose ancestry is African/Caribbean and Middle Eastern. Thalassaemia predominantly affects people from Asian, Mediterranean and Middle Eastern countries. Inheritance of affected genes from both parents may result in severe haemoglobin disorders (e.g. Sickle cell anaemia or thalassaemia major). What is thalassaemia?Thalassaemia is an inherited condition of the blood, which affects the production of haemoglobin - an oxygen-carrying substance that gives blood its red colour.
There are many forms of thalassaemia, among which Beta-Thalassaemia
Major is the commonest. It is a life threatening condition.
Children who are born with Beta-Thalassaemia Major cannot make enough
haemoglobin in their blood. As a result, they become severely
anaemic and need blood transfusion every four weeks and regular
injections for their life.
People who are of African, Caribbean and Mediterranean origins are at 'risk' of sickle cell disorders. People who are of Asian, Mediterranean, Middle-Eastern and African origins are at 'risk' of thalassaemia. Caucasian people are also affected, but this is less common.
If both parents have sickle cell or thalassaemia trait, there is a one in four chance (25%) that in each pregnancy the child will have sickle cell or thalassaemia disorder. The Sickle Cell & Thalassaemia Support Project provides genetic
counselling which gives individuals the opportunity to
discuss the several options for making informed decisions.
This is a carrier state. Having the trait is not an illness and this will not develop into the disorder. Individuals with sickle cell or thalassaemia are usually healthy people. Individuals carrying the trait usually do not know they are carriers. People with sickle cell trait should lead a normal life, however it is important that you inform your GP, dentist or other health professionals if you are undergoing general anaesthesia during an operation. People with Beta-Thalassaemia have smaller red blood cells than normal. Although this does not usually cause a problem, it can be confused with iron deficiency anaemia.
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