ABSTRACT

Retinitis pigmentosa (RP) is one of a large group of inherited retinal dystrophies characterized by slow progressive retinal degeneration. It affects about 1:3500 people in the population.

Problem

The diagnosis of RP has serious implications and so the assessment needs to be accurate. Although there is no treatment that as yet can alter the underlying retinal degenerative process, the patient should have access to: advice on visual prognosis, genetic counselling, visual rehabilitation and low vision assessment, registration for visual disability and social support services. Many patients are also keen to know about information on progress in RP research. There are many RP-associated syndromes which require specific management (e.g. Refsum’s disease and a phytanic acid-free diet).