The Link Between Sickle Cell Disease And Vision

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The Link Between Sickle Cell Disease And Vision

September 2, 2018

Congress has designated September as “National Sickle Cell Awareness Month” to help focus attention on the need for research and treatment of sickle cell disease, an inherited condition that currently affects almost 100,000 Americans. This month and throughout the years many medical institutes and professionals take a moment to reflect on the importance of finding a cure for this genetic disorder. Sickle cell disease can affect many organs in the body, including the eyes.


Signs of sickle cell disease usually begin in early childhood. “Typically around the age of 10, children should have a comprehensive eye exam,” says Dr. Stewart Shofner. Studies show that sickle cell retinopathy can start around that age. “Annual eye exams are vital to monitor red blood vessel growth within the retina after the age of 20, but children may require more frequent visits."


What is Sickle Cell Disease?

Sickle cell disease (SCD) is a group of blood disorders typically inherited from a person's parents and is most common in people with Mediterranean or African ancestry. The most common type is known as sickle cell anemia (SCA). It results in an abnormality in the oxygen-carrying protein hemoglobin found in red blood cells. People with sickle cell develop crescent-shaped red blood cells when there is reduced oxygen levels. These sickled red blood cells become stiff and do not flow easily through small blood vessels. When the sickled cells block the flow of blood through the body, extreme pain occurs,due to blood-starved tissues. People with sickle cell have extreme anemia because of the abnormal hemoglobin in their blood cells. 


How is SCD Diagnosed?

According to the Mayo Clinic, a blood test can check for hemoglobin S — the defective form of hemoglobin that underlies sickle cell anemia. In the United States, this blood test is part of routine newborn screening done at the hospital. In adults, a blood sample is drawn from a vein in the arm. In young children and babies, the blood sample is usually collected from a finger or heel. The sample is then sent to a laboratory, where it's screened for hemoglobin S. If the screening test is positive, further tests will be done to determine whether one or two sickle cell genes are present.


How SCD Affects Your Eyes

Sickle cell disease can affect many organs in the body, including the eyes. While SCD can be diagnosed at birth, signs of sickle cell retinopathy may start as early as the age of 10 years. The clinical manifestations vary depending on the presence or absence of vaso-proliferative changes. One of the most common eye complications associated with sickle cell disease is a "sea fan-shaped frond" of new retinal blood vessel growth. The sea fan of vessels represents the eye's attempt to resupply the retina with oxygen.

The new blood vessels that grow are weak and can leak fluid and blood and can also create traction on the retina and possibly tear it loose. Patients with SCD may experience flashes, floaters or dark shadows, eye redness, which may be indicative of retinal traction or detachment. Additional signs may include the following:

  • Atrophy or weakening of the iris
  • New blood vessel growth  (neovascularization) of the iris
  • Snake-like shaped blood vessels
  • Retinal hemorrhage
  • Pigmentations in the retinal periphery
  • Glistening deposits in the retina
  • Salmon patch (orange-pink colored patches) in the retina
  • Retinal blood vessel occlusion


If you or a loved one have recently been diagnosed with Sickle Cell Disease, have experienced changes in your vision or if you can’t remember the last time you had an eye exam, contact Shofner Vision Center online to schedule a comprehensive exam or call (615) 340-4733. Our professional staff is dedicated to providing the best personalized vision care to our patients.


Learn more about Sickle Cell Awareness Month. The CDC offers the following online resources:

The Link Between Sickle Cell Disease And Vision

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