Sickle cell disease is a disorder that causes red blood cells to become sickled (banana-shaped), as well as sticky and rigid. These sickle cells can block blood flow in small blood vessels of the body. The hemoglobin in blood cells carries oxygen to all areas of the body. Sickle cells can block the small blood vessels in the eye depriving the eye of oxygen and causing damage. This is called sickle retinopathy (SR).
What are some of the symptoms of SR?
Retinopathy (re ti NAH poh thee) is a disorder that can continue to get worse. It can occur in one (1) or both eyes. Early damage does not usually affect the person’s ability to see. Retinopathy can go undetected for years without an eye exam by a trained eye doctor (ophthalmologist). The eye doctors in the St. Jude Eye Clinic have been treating people with sickle cell disease for many years, and they are familiar with SR. Sickle retinopathy can progress to severe proliferative sickle cell retinopathy (PSR). PSR can cause bleeding into the
eye or detachment of the retina. It can lead to vision changes and, in rare cases, blindness.
Who develops SR?
Anybody with sickle cell disease can develop SR, but it is more common in people with sickle-hemoglobin C disease (SC) and sickle beta plus (Sβ+) thalassemia. Up to a third of children with SC and Sβ+ thalassemia disease may develop sickle retinopathy.
What is the best way to manage SR?
Periodic eye exams are needed. They allow the eye doctor to see if the patient has retinopathy in one or both eyes and follow the progression of the SR. If SR does progress to severe PSR, the eye doctor can provide treatment for PSR to prevent vision loss. Blindness is rare when a patient makes routine visits to a trained eye doctor. If left untreated, 10 percent of people with SR may develop vision problems1.
A person with sickle cell disease should begin having eye exams at 9 or 10 years old. The exams will be repeated annually for patients with sickle hemoglobin C disease and every other year for those with other types of sickle cell disease. If there are any signs of sickle retinopathy, exams will be scheduled more often by the St. Jude Eye Clinic. It is very important that you show up for St. Jude Eye Clinic appointments on the recommended schedule even if your child already sees an eye doctor for glasses. The retinopathy exam must be done by an eye doctor who regularly treats patients with sickle cell disease.
If you have questions or want to learn more about sickle retinopathy, talk with your child’s Hematology team.
1 Leveziel N, Bsatuji-Garin S, Lalloum F, Querques G, Benlian P, Binaghi M, Coscas G, Soubrane G, Bachir D, Galacteros F, Souied EH. Clinical and Laboratory Factors Associated with the Severity of Proliferative Sickle Cell Retinopathy in Patients with Sickle Cell Hemoglobin C (SC) and Homozygous Sickle Cell (SS) Disease. Medicine (Baltimore), 2011 Hov; 90(6); 372-378.
This document is not intended to take the place of the care and attention of your personal physician or other professional medical services. Our aim is to promote active participation in your care and treatment by providing information and education. Questions about individual health concerns or specific treatment options should be discussed with your physician.
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