What is sickle cell retinopathy?

Sickle cell disease is a genetic hereditary hemolytic anemia that occurs in black people. It occurs in only one to two percent of blacks but the sickle cell trait is seen in eight to ten percent of the black population. It has historically thought to have been confined to those of a black race, however, it also occurs in Northern Greece, Saudi Arabia, Turkey, Southern Italy and the Mediterranean. Patients with sickle cell disease experience sudden liver and spleen enlargement, organ pain, vascular occlusion and the function of marrow production can cease.

The vascular occlusion can happen in the conjunctiva, iris, retina and choroid. Proliferative sickle cell retinopathy is the most severe ocular change of the disease. There are five medical stages and three distinct stages to this part of the disorder. It is important that treatment be performed before the patient reaches the last stage.

In the first stage, the arteriolar occlude and show as dark red vessels that change to a silver wire looking vessel. In the second stage, the eye has tried to adjust and blood is diverted to venules that are next to the artrioles. In the third stage the eye continues to try to adjust to the new formation of vessels and create neovascular tufts often referred to as ‘sea fans’. As they grow they will acquire a fibrotic tissue envelope that can pull on the vitreous, causing a retinal break. There are surgical procedures that can be performed at this level, but they are highly risky and the patient may not regain their site. Only moderate a moderate increase in eye pressure can put a patient at risk.

There are usually no symptoms in the beginning stages of sickle cell retinopathy. Once any symptoms begin to show the damage has already occurred. The only action for early treatment is to have regular examinations by an Opthalmologist, beginning at around age seven. The doctor will put a few drops into the eyes to dilate or open up the eye vessels. The doctor can then observe the vessels using a specific medical device designed for that purpose. There is only a little discomfort after the test involving light sensitivity. It is suggested that sun glasses be worn or simply stay out of the daylight for an hour or so after the test. If sickle cell disease is within the family, it is advised that the testing be done as early as twenty months of age. The discovery is more common in those patients within the fifteen to thirty age group.

The Sickle Cell Disease of America organization is a non profit organization that promotes awareness, education and advocacy and assists in pursuing a cure for sickle cell disease. Up to date medical reports and information can be found to assist in quality of life and health for those patients and their families that suffer from sickle cell disease and the medical problems associated with the debilitating disorder.

The information supplied in this article is not to be considered as medical advice and is for educational purposes only.