SICKLE CELL DISORDER
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.