Continuing Education Activity
The World Health Organization estimates that about 7 percent of the world’s population are carriers of hemoglobinopathies and about 300,000 to 400,000 babies are born every year with a severe form of hemoglobinopathy. Hemoglobinopathies are genetic disorders characterized by either abnormal hemoglobin, as in sickle cell disease, or insufficient production of hemoglobin chains, as in thalassemia. Proliferative sickle cell retinopathy is the most serious vision-threatening complication of sickle cell disease and is reportedly seen in 0.5 percent of patients with HbSS disease, the severe variant of sickle cell disease and about 2.5 percent of patients with HbSC disease, a less severe variant of sickle cell disease. The frequency of ocular involvement in patients with beta-thalassemia ranges from 41.3 to 85 percent, according to studies. This activity describes the causes, pathophysiology, and presentation of hemoglobinopathy-associated retinopathy and highlights the role of the interprofessional team in the care of affected patients.
- Describe diseases associated with retinopathies.
- Describe sickle cell retinopathy.
- Describe the forms of retinopathy seen in patients with thalassemia major and intermedia.
- Members of the interprofessional team should ensure that patients with hemoglobinopathies are referred to ophthalmologists for assessment of visual function; these patients need close monitoring for visual deficits.