Abstract

In Reply. —We very much appreciate Dr MacKay's interest in our article on color vision defects in patients with sickle cell disease. However, we would like to respond to her two comments. First, Dr MacKay argues that blue-yellow color vision defects are not unexpected in patients with sickle cell anemia who have peripheral retinopathy. We looked for, but could not demonstrate in our patients, any substantial association between the presence of a color vision defect and the severity of the peripheral signs of sickle cell retinopathy. This lack of association suggested to us that these retinal changes were probably too peripheral to have affected a substantial number of blue cones. We also searched for evidence of more posteriorly located signs of sickle cell retinopathy, but could not find any. Thus, we noted that, based on these retinal findings, the presence of such color vision defects was unexpected. Second, Dr MacKay

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