Sickle Cell Anemia and Vision | National Sickle Cell Month

Sickle cell anemia is a rare inherited condition that affects the blood cells in the body. The disease largely affects people with ancestors from sub-Saharan Africa, Spanish-speaking regions in the Western Hemisphere, Saudi Arabia, India, and Mediterranean countries. According to the Centers for Disease Control and Prevention, in the United States, sickle cell anemia affects about 100,000 people.

September is National Sickle Cells Month. The article below takes a look at what sickle cell is and how it can affect the eyes. 
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What is Sickle Cell Anemia? 

Sickle cell anemia is a disorder in which the red blood cells that carry oxygen to the body are unhealthy. Normally, red blood cells are round and able to travel through the blood vessels easily, but in people that have sickle cell disease, their red blood cells are shaped abnormally like a crescent moon or a sickle, which is where the disorder got its name.

Because of the abnormal shape, the cells block blood flow to parts of the body. According to the Mayo Clinic, typical symptoms of sickle cell anemia include:

• Fatigue
• Swelling of the hands and feet
• Episodes of pain in the bones, joints, and chest
• Frequent infections 

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How Can Sickle Cell Anemia Affect the Eyes? 

Sickle cell anemia can also affect the eyes. The small blood vessels can become blocked due to the abnormally shaped red blood cells. The blockage can develop in any part of the eye, but the retina is where it occurs most often.

Inflammation and decreased oxygen to the retina can develop. When the retina becomes damaged, it can lead to sickle cell retinopathy.

Sickle cell retinopathy causes thinning of the retina and abnormal blood vessel growth. The combination of damage to the retina can cause vision loss. Permanent blindness may also occur. Damage may develop slowly or suddenly.  

Some risk factors for developing retinal damage if you have sickle cell disease include the following:

• Being older than 35
• Male gender
• Low white blood cell count
• Having lung disease
• High hemoglobin levels

Some people that have retinal involvement due to sickle cell anemia do not notice any symptoms, but for others, symptoms may include:

• Sudden floaters
• Blind spots
• Flashes of light
• Loss of side vision 

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Treatment 

Currently, there is no cure for sickle cell anemia, but there are treatments that can help decrease the risk of complications and symptoms.

Treatment for people with sickle cell disease may include stem cell therapy, red blood cell transfusions, and medication. One drug used is hydroxyurea. This medication helps increase a type of hemoglobin that helps carry oxygen to the cells. The Food and Drug Administration also approved two additional drugs to treat sickle cell anemia, including Voxelotor and Crizanlizumab.

To help protect vision, it is important for people with sickle cell anemia to have regular eye exams. Identifying any changes in vision early may help decrease the risk of vision loss.

When small areas of abnormal blood vessels are detected, they may disappear without treatment, but it is critical to monitor the vessels for changes.

In some instances, sickle cell retinopathy develops, and treatment is needed. The type of treatment recommended may depend on the extent of symptoms and damage to the retina. Possible treatment includes:

Laser treatment: If a large area of blood vessels is blocked, laser treatment can help decrease further development of abnormal blood vessels.

Medications: Certain medications are available that prevent the formation of additional abnormal blood vessels. The drugs are injected directly into the eye.

Surgery: If the condition becomes serious and the retina detaches, surgery is needed to prevent blindness.

If you have any questions about how to maintain good eye health with sickle cell anemia or would like to ask if an appointment with one of our eye doctors would be appropriate at this time, call our office at 508-746-8600.