Summary.
RP
DISEASE
Overall, this study demonstrates the systematic differences in severity and progression seen among different genetic subtypes of RP. It further demonstrates how the genetic cause of disease, and the 30-Hz ERG implicit time, can have significant predictive power for a patient’s rate of progression. We hope that the lasting contribution of this historical data set will be in helping RP patients and their doctors better understand the severity and expected progression of their disease. Future work may include methods of providing these estimate and predictions in a more accessible format and with refined specificity. Additional endpoints (including visual acuity, visual field, rod-dominant ERG responses) and their progression rates could be evaluated among genetic subgroups as well and may complement data from ongoing prospective natural history studies with additional outcomes such as full-field stimulus threshold testing (