Thalassaemia is the name given to a blood condition that affects what is known as haemoglobin within the blood. Sickle Cell disorder is another condition which is closely related.

 

Thalassaemia affects people mainly from Asia, Africa, the Far East, sub continent, India, Middle East and the Mediterranean.  In the UK members of the Asian community affected by Thalassaemia come from all the main religious groups that span Pakistan, India and Bangladesh. 

 

There are two forms of the disorder:

 

  • Thalassaemia Major (A +Thal and A oThal)
  • Thalassaemia Carrier (also known as Thalassaemia Minor or Thalassaemia Trait)

 

Thalassaemia Major is a serious blood disorder where the person with the condition is unable to produce enough red blood cells within their blood.  The seriousness of the disorder can greatly affect those who are not treated.  The chances of an untreated child’s survival beyond eight years of age is very slim. 

 

Thalassaemia Carriers are completely healthy and may not know they are carriers unless they have a special blood test carried out.   Thalassaemia is carried by*:

 

  • 1 in 7 to 1 in 10 Gujeratis
  • 1 in 10 Sindis
  • 1 in 20 South Indians
  • 1 in 25 Pakistanis
  • 1 in 15 to 1 in 30 for Punjabis and Bangladeshis

 

 

In some instances healthcare experts misdiagnose a carrier as having Iron Deficiency Anaemia.  This is due to the fact that the red blood cells of the carrier are smaller than those of a non carrier and less able to carry as much iron.

 

It is important that an individual takes an actual blood test to determine whether they are a Thalassaemia carrier. 

 

An individual carrier who plans a family with a non carrier will not risk their child inheriting the disorder.  The child however has a 50% chance of becoming a carrier. 

 

However there is a 25% likelihood that a child born to parents who are both Thalassaemia carriers will inherit Thalassaemia major.

 

A child with the condition will begin to show symptoms from three months old.  These symptoms include:

 

  • Paleness
  • Restlessness
  • Lack of appetite
  • Vomiting

 

Treatment for Thalassaemia Major does exist.  It involves regular blood transfusions in hospitals every month followed by injections or taking tablets.

 

After each transfusion the red blood cells in the new blood are broken down slowly over a period of four months.  The iron from the red blood cells remain and if not removed can build up and damage the liver, heart, and other parts of the body.

 

Removing the iron involves use of drugs including ‘Desferal’ or ‘EXJADE’.  ‘Desferal’ is taken as a daily injection and works by picking up the iron, carrying it out in the person’s urine.  The alternative drug ‘EXJADE’ comes in tablet form and is new to the market.  Described as the ‘once-daily oral breakthrough that simplifies iron chelation’ EXJADE performs the same job as ‘Desferal’ and may be more convenient for those with the disorder.  Please review the related link, or speak to your GP for more information,

 

Most people who receive both types of treatment grow normally into their teens and lead fairly healthy lives in which they are able to work and marry, with an increasing number able to have children.

 

Bone marrow transplant treatment also exists, although the results may not be successful.  In cases where the transplant is a success the individual will no longer require treatment.

 

People with Thalassaemia are born with the condition.  It is not contagious and can only be inherited. 

 

Research continues into other treatments for those with the disorder.  A simple blood test known as ‘electrophoresis’ is available through your GP and can tell you whether a person has Thalassaemia major or is a carrier of Thalassaemia.  For more information regarding ‘EXJADE’ visit the related`link or speak to your GP.

 

Please contact us or view our list of frequently asked questions for further information.

 

*Source: Thalassaemia Society (No date) ‘Why you need to know about Beta Thalassaemia’, Leaflet, United Kingdom Thalassaemia Society

 

Related Links:

 

ExJade

Thalassaemia Society