July is Sickle Cell Awareness Month. Sickle cell anaemia is an inherited, genetic blood disorder in which red blood cells, which carry oxygen around the body, become sickle-shaped and therefore the red blood cells can’t squeeze through small blood vessels as easily as normal cells. Small blood vessels become blocked, stopping the oxygen from getting through to where it is needed. This in turn can lead to severe pain and damage to organs.
- Sickle cell anaemia is an autosomal recessive condition.
- ‘Sickle cell ’ describes a group of disorders of haemoglobin, which are caused by an inherited mutation in the beta globin gene.
- Sickle cell anaemia (SCA) is the commonest of the sickle cell diseases and is defined by the presence of the abnormal haemoglobin HbS. This results from having two copies of the beta globin gene containing the mutation for HbS. The other forms of sickle cell anaemia result from inheriting a mutation for HbS in one gene and another abnormal beta globin chain variant in the second beta globin gene.
- SCA is characterised by episodes of pain, chronic haemolytic anaemia and severe infections,usually beginning in early childhood.
- Sickle cell anaemia is common in people of African, Mediterranean, Middle Eastern, and Indian ancestry and in people from the Caribbean and parts of Central and South America. Sickle cell carriers are particularly common in areas of the world where malaria is common as the sickle trait would seem to offer some protection.
- The highest incidence worldwide is in West Africa where 1 in 4 of the population are carriers
Living with sickle
Find out what it is like living with SCA: ‘Tony’ and ‘Patrick’ share their personal experiences of living with this condition on the Telling Stories website. Mike also has Sickle Cell Anaemia and talks about his experiences as an accredited Expert Patient Programme Facilitator and Regional Care Advisor for the Sickle Cell Society.