Kender and Jarod went in for EUA (Exam Under Anesthesia) yesterday. The short story is both boys had angiograms and needed lasering, and Kender may need to go on an anti-VEGF drug.

Jarod's eyes showed new avascular areas of retina. These areas were near the periphery, along previous areas of laser treatment. Dr. Trese says that avascular retina tends to send pro-VEGF (vascular endothelial growth factor) signals in an attempt to correct the vascularization, and these excess signals then lead to VEGF-related problems elsewhere including proliferation of blood vessels and new exudates. So he recommended preventively treating these areas with laser, as he describes in Pediatric Retina. The new avascular areas are likely caused by growth of the eye; in a normal eye, growth would result in corresponding proliferation to maintain even coverage of blood vessels, but in the FEVR eye new blood vessels will not grow in the avascular portion of the retina. Dr. Trese expressed skepticism about Jarod's recent acuity measurements, only because of his age; I'll keep my own judgement, since I thought he was pretty cooperative during the exam. Angiogram photos showed that Jarod's left macula is indeed crossed by the primary retinal fold in that eye and is dragged out of shape; his right macula has some dragging as well.

We had Dr. Trese and Dr. Ho look at Kender's eyes before getting dilating drops; nobody had done this before, and I've been getting the distinct impression that nobody believed me about the persistent hyperdilation. They both verified my observation, and after angiogram Dr. Trese reported that Kender has uveal ectropion caused by proliferating blood vessels on the backside of the iris. In essence, the iris has rolled outward like a scroll, pushed by the extra blood vessels, and has now stiffened. This process is irreversible, and from what I can tell could be causing the glaucoma. This new proliferation demonstrates that this eye is still very active, and makes Kender a candidate for drug therapy. Exactly what drug is still to be determined; Dr. Trese indicated that he wanted to do some more research before seeing Kender in the office in 3 weeks to discuss options. MacuGen is one option, but another option that is newly on the table is Avastin, an anti-VEGF drug originally developed to starve cancer tumors of their blood supply. This drug is also effective at blocking the abnormal VEGF signals in FEVR eyes as well as in age-related macular degeneration. However, Dr. Trese expressed some concern about possible systemic side effects of using this drug in very young children. VEGF is important in other developing areas of the body, not just the eyes, and so long-term effects could be much more significant in young children than in seniors with AMD. To finish up yesterday, Kender also had some lasering in both eyes, for the same reason as Jarod.

I'll get the photos scanned and posted later.