The normal shape of red blood cells is round, but in sickle cell, aproximately 80-100% of red blood cells are sickle or 'C' shaped and rigid and stiff, causing them to clump together, blocking blood vessels and causes severe episodes of pain. This abnormality in the haemaglobin means that oxygen is not able to be carried through the blood properly to the organs.
This blockage is known as a vaso-occlusive 'crisis'. This in turn may result in organ damage and can cause death if left untreated.
Crisis pain can last minutes, to hours, to days/weeks and in some instances has been known to last months at a time. Although there are other forms of crisis, this is the most commonly experienced.
The severity and length of this pain varies from individual to individual and can depend on the site of pain on the body and other physiological and environmental factors.
Due to oxygen levels within the red blood cells being depleted, anaemia and chronic fatigue is another presentation individuals with this disorder suffer.
As this is a very brief overview, further information can be obtained from the Sickle Cell Society by clicking the link as follows: