Sickle cell disease is a blood disorder caused by an inherited genetic mutation. 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 sickle 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. Hemoglobin is a protein present in red blood cells that help to carry oxygen throughout the body.
Sickle Cell and Your Eyes
Sickle cell disease can affect many organs in the body, including the eyes. Eye signs can include the following:
- Eye redness caused by comma-shaped blood vessels
- Subconjunctival hemorrhage
- 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
- Angioid streaks
- Retinal blood vessel occlusion
- Retinal neovascularization
- Retinal detachment
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. This sounds like a good thing, but the new blood vessels that grow are weak and can leak fluid and blood. They can also create traction on the retina and possibly tear it loose.
When sickle cell disease worsens in the eye, it becomes proliferative sickle cell retinopathy. This worsening can create severe hemorrhages in the eye and retinal detachment. A retinal specialist’s goal is to prevent or eliminate retinal neovascularization before it gets to the point of destruction.
Annual Eye Exams
Comprehensive eye examinations should start no later than 10 years of age. Studies show that sickle cell retinopathy can start around that age. The eye exam should be a thorough dilated retina exam. Follow-up exams will be dependent on the findings. Typically, if the exam appears normal, it should be repeated annually.