Proliferative sickle cell retinopathy is caused by sickle cell disease and leads to the growth of abnormal blood vessels in the eye. Proliferative sickle cell retinopathy (PSR) is a complication in the eyes that can happen in some people who have sickle cell disease. Sickle cell disease is an inherited disorder that affects how red blood cells work. PSR causes new, abnormal blood
Proliferative sickle cell retinopathy (PSR) is a complication in the eyes that can happen in some people who have sickle cell disease. Sickle cell disease is an inherited disorder that affects how red blood cells work. PSR causes new, abnormal blood vessels to grow and leak behind the light-sensitive tissue in the back of the eye called the retina. The leakage from these blood vessels can lead to vision loss.
About 10% of people with sickle cell disease will experience some sort of vision problem due to their condition.
This article will address symptoms, causes, diagnosis, and treatment for proliferative sickle cell retinopathy.
For many people, PSR does not have any symptoms. This is one reason why, starting at age 10, people with sickle cell disease are encouraged to set annual appointments with a retina specialist. This type of healthcare provider can check for signs of PSR.
When PSR does have symptoms, they include:
Proliferative sickle cell retinopathy is caused by sickle cell disease. Sickle cell disease is an inherited blood disorder that causes red blood cells to become hard and sticky. These red blood cells die sooner, causing a constant shortage of red blood cells in the body. Surviving red blood cells can cause blockages in the blood vessels.
These complications can cause your eyes to have less blood flow and, therefore, less oxygen is getting to them. The blockages can lead to the growth of irregular blood cells. These irregular blood cells leak blood behind the retina.
An eye doctor, such as a retina specialist, can use several tools to help diagnose PSR, including:
During an exam, your eye care specialist will check for other eye conditions with signs and symptoms that resemble proliferative sickle cell retinopathy. These include:
To help identify PSR, eye doctors often use what's called the Goldberg stages that describe what is happening to the eye. These range from stages 1 to 5.
Stage 5 proliferative sickle cell retinopathy, the most advanced form includes retinal detachment. This is what happens when the retina pulls away from the back of the eye.
A retinal detachment is a medical emergency.
The treatments available for proliferative sickle cell retinopathy are:
If there is only a small area of abnormal blood vessels, your eye doctor may choose to observe the area via regular eye exams and not offer any specific treatment. This is because these small areas of growth often go away on their own.
If you have sickle cell disease, it's important to maintain your treatments to help prevent further problems.
The prognosis (likely outcomes) for PSR will depend on the stage. It can cause vision loss, including total blindness, at its most advanced stages. This is another reason why annual eye exams are crucial when you have sickle cell disease.
Proliferative sickle cell retinopathy is an eye condition that is caused by sickle cell disease. It may not have any symptoms, but it can cause vision loss at its advanced stages. Eye doctors can diagnose PSR with several types of imaging. Treatment may include lasers, surgery, and the use of drugs injected into the eye.
PSR is just one way that sickle cell disease can affect the body. Maintain recommended checkups with your healthcare team to better manage your sickle cell disease. Consistent visits can help identify any emerging problems earlier, before they become advanced.
Having an eye condition that can cause vision loss is scary. Know that total vision loss is rare with PSR—about 10% of people with sickle cell disease experience vision problems, and a fraction of those with vision problems experience total vision loss. Detection is key, which is why it's important to get an annual eye exam and discuss prevention and treatment options with your eye doctor.