What is Sickle Cell Disease?
Sickle cell disease is the most common genetic disease, affecting about 400,000 newborns each year. It is caused by a mutation in the gene that codes for the hemoglobin protein that carries oxygen in the blood stream. Sickle hemoglobin tends to clump into a sickle shape when it gives up its oxygen molecule to the tissues it supplies. When this occurs, the red blood cells lose their flexibility and tend to block the small blood vessels in the body. The retina in your eye is like the film inside a camera. The retina “takes the picture” of objects you look at and sends the message to the brain. The retina is a living tissue, which requires blood supplied by tiny vessels. These blood vessels may be damaged in people with sickle cell disease.
Who is at risk of eye problems in sickle cell disease?
Although more extensive blockage of retinal blood vessels occurs in sickle cell disease, more severe complications (bleeding and retinal detachment) occur in people with a combination of sickle hemoglobin and hemoglobin C (called Hemoglobin SC disease). Some studies suggest men are more likely than women to have loss of vision.
What are the visual symptoms of sickle cell disease?
Blurring of vision may occur if excess damage occurs to the retinal blood vessels. Floaters can look like tiny dots or cobwebs moving about in your vision. They may be due to bleeding from the retina into the central gel of the eye. Retinal detachment may cause a dark shadow to appear off to the side (in the peripheral visual field) and may progress to total loss of vision. Pain is rare and may be due to high pressure in the eye (neovascular glaucoma).
What treatment is available?
There is no cure, but treatment may improve vision or keep the vision from worsening. If bleeding occurs inside the eye due to blocked blood vessels, floaters are seen by the patient. Medicine injections may help recover vision and may be applied without pain in most cases. Laser may stabilize or improve the vision. In some situations, surgery is required. The vision may not return to normal following treatment as there may be some permanent damage to the retina. The earlier retinal problems are found, the better the outcome of treatment. Therefore, annual exams are important and it is critical to report new floaters without delay.
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