Prognosis
RP is a slowly progressive disease, which manifests over years. There is no cure for RP, and most treatments have been shown to only modestly slow the degeneration of the disease. While the age of onset is quite variable, the rate of degeneration once it begins seems similar across the different forms of the disease. Visual field loss begins in the mid-periphery and then expands to the periphery and centrally. Patients tend to lose about 50% of their remaining visual field every 5 years.[64] Good central visual acuity is often maintained even when visual fields have diminished to small tunnel fields. An abrupt loss of central acuity can result from the development of cystoid macular oedema and should be brought to the attention of the patient's physician. Many patients with advanced disease will eventually lose central acuity as the macular cones degenerate, but some patients will maintain central vision until the end of their life. Complete visual loss is rare with <0.5% of patients in one study measuring no light perception.[65]
The ability to drive is a common issue. Guidelines for driving vary from state to state, as well as by country. In general, measurements of central visual acuity and visual fields are incorporated in most guidelines. A consultation with a low-vision consultant is often helpful for maximising remaining vision. Additionally, many patients with RP will eventually be entitled to register as legally blind, entitling them to specific benefits and protection under the law.
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