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Sickle Cell Anaemia and Thalassaemia  news

Sickle Cell Anaemia

Sickle Cell Anaemia is an hereditary blood disorder which is brought on by both parents having the Sickle gene in their blood. There are different types of Sickle Cell, including SS and SC Disease.

Haemoglobin is the pigment that transports oxygen in the blood. It is present in the red blood cells and it is this which gives blood its red colour. There are many different types of haemoglobin. Haemoglobin S, or sickle haemoglobin, is responsible for Sickle Cell Anaemia.

Red blood cells with normal haemoglobin are able to retain their shape when they release oxygen into the body. However, when sickle haemoglobin releases oxygen, the shape of the red blood cell changes into a sickle-shape. Red blood cells that contain normal haemoglobin are flexible and it is easy for them to flow through narrow blood vessels. Sickled red blood cells find it difficult to get through these narrow blood vessels, and so cause sickling. Sickling or 'crisis' are words to describe when 'sickled' red blood cells are trapped in veins and cause severe pain. Sickle cell anaemia is a life-threatening illness, for which there is no current cure.

Many factors can cause a sickle cell crisis. The main reasons include:

a) lack of fluids (dehydration)
b) lack of oxygen
c) cold temperatures for prolonged periods of time
d) alcohol and other 'drugs'
e) over exertion and tiredness, resulting in a struggle for the body to operate its basic functions adequately
f) stress, worry and anticipation

Taking into consideration the points above, the following precautions can be observed to help prevent a crisis:

a) ensuring that you are well hydrated
b) maintain warmth
c) ensuring frequent rest, not allowing the body to be over-exerted for long periods of time
d) drugs and other toxins kept to a minimum
e) stressful situations are managed and avoided where possible

Treatments for sickle cell include blood transfusions and medicines, including Hydroxyurea. People with Sickle Cell have to take several medicines to help manage the ongoing pain and painful episodes when they arise.

For more information on thalassaemia see - further reading

Thalassaemia

Thalassaemia is an hereditary blood disorder which can be brought on when both parents of the child have an abnormal haemoglobin molecule. There are different types, including Alpha and Beta thalassaemia.

Haemoglobin is made out of different parts. The main parts are called 'alpha chains' and 'beta chains' which are put together to make the haemoglobin molecule. In thalassaemia, part of the haemoglobin is faulty - usually either the alpha chains or the beta chains. This means that some of the haemoglobin does not work properly.

Symptoms of beta thalassaemia include:

a) fatigue and weakness
b) shortness of breath
c) a yellow discoloration of the skin (jaundice)
d) enlarged spleen
e) bones and liver complications

Treatment for thalassaemia includes blood transfusions and chelation therapy. Regular transfusions are necessary to to help prevent the complications associated with thalassaemia. A side effect of having regular transfusions is a build of iron in the blood which is dangerous and life threatening. Iron chelation therapy is necessary to help reduce the iron levels in the blood.What causes a Thalassaemia crisis

For more information on thalassaemia see - further reading