Haemoglobinopathies

Sickle cell Syndromes are among the most common genetic disorders in the UK and worldwide.

Sickle Cell Syndromes are haemoglobinopathies i.e disorders of the oxygen carrying haemoglobin in the blood.
Haemoglobinopathies are a group of disorders of haemoglobin that are:

- outosomal i.e. genetic involving a non sexual chromosome
- recessive i.e. normally only passed on if both parents have the full syndrome or trait
- inherited i.e. not contagious

The most common Haemoglobinopathies are Sickle Cell Syndrome and Thalassaemia. Sometimes called sickle cell disease or sickle cell disorder, although The Sickle Cell Centre prefers the term syndrome to disorder or disease just as many other inherited conditions such as haemophilia, Cystic fibrosis or Down's syndrome do not have the word disease with all its negative connotations, attached to them.
There are milder and more severe forms of both Sickle Cell Syndrome and Thalassaemia and it is possible for one person to carry both the sickle cell and thalaasemia genes at the same time.

In the UK, the haemoglobinopathies mainly, but not exclusively. affect minority groups. But no specific haemoglobinopathy is confined to a single group (Department of Healt, 1993).

The trait for alpha thalassaemia occurs in people of Chinese, Vietnamese, Greek, Middle Eastern and Cypriot heritages (Anionwu, 1993; Department of health)

The trait for beta thalasemia occurs in varying rates among people of Mediterranean, Southern European, South Asian, chinese, Vietnamese and African Caribbean heritages. The gene rarely occurs in peple of white British Heritage (Department of Health 1993)

In britain the two main groups affected by Thalasaemia are the Cypriot and South Asian Communities.

Sickle Cell Statistics & Figures (Merton Sickle Cell & Thalassaemia Group)
There are no actual national or local figures to represent the number of people who suffer from Sickle Cell and Thalassaemia in the UK . However carrier frequencies can be used with census records to calculate estimates. We would like you to note that these figures are only minimums.

National Carrier Frequencies of Sickle Cell & Thalassaemia in the UK

In Britain 1 in every 300 people are considered at risk of having Sickle Cell Anaemia.
In Britain 1 in 10 people are considered ‘at risk' of having the Sickle Cell Trait.
(figures from ‘Sickle Cell in Merton' research report, 1996)
In Britain 1 in 100 people are considered ‘at risk' of having the Thalassaemia Trait.

As an organisation supporting these suffers we have no doubt that these figures do not do justice to the actual number of people who exist and need support. (Merton Sickle Cell & Thalassaemia Group)