What is sickle cell retinopathy?
Sickle cell retinopathy is an eye complication of sickle cell disease. It is caused by loss of blood supply to certain parts of the retina. This is because the red blood cells in sickle cell disease are sticky and less flexible. They can block the tiny blood vessels that supply nutrients and oxygen to keep the retina healthy.
Sickle cell disease is the name for a group of inherited conditions that affect the red blood cells. Sickle cell disease most commonly affects people of African or Caribbean descent. It is also seen commonly in people of Asian, Middle Eastern and Eastern Mediterranean descent. They are a serious and lifelong health condition, although many of the symptoms can be managed with treatment, which includes keeping well hydrated, reducing infection risks and medical treatments.
What causes sickle cell disease?
In sickle cell disease, there is a fault in the gene that controls how haemoglobin is formed. Haemoglobin is the protein responsible for carrying oxygen in red blood cells.
Sickle cell disease is inherited in an autosomal recessive pattern. This means that to have sickle cell disease, you need to inherit a copy of the faulty haemoglobin gene from both parents. If both parents have a faulty gene, then there is a 1 in 4 chance of inheriting the condition. If you inherit one faulty gene then you will be a ‘carrier’ of sickle cell disease, but not have the symptoms. A carrier is referred to as having the sickle cell trait.
Sickle cell retinopathy can affect the retina in different ways. The type that is most likely to affect sight is known as proliferative sickle cell retinopathy (PSR). Please see What is proliferative sickle cell retinopathy?
Further information can be found on RNIBs sickle cell retinopathy webpage.
