Inherited Retinal Dystrophy

Inherited retinal dystrophies are chronic and disabling disorders of visual function. The most common retinal dystrophy in Australia is the family of Retinitis Pigmentosa diseases that affect the retina, the light-sensitive part of the eye.

The retina consists of millions of light-sensitive photoreceptor cells, which transmit light to the brain via the optic nerve. Retinitis Pigmentosa (RP) causes some photoreceptor cells to fade gradually and die, losing the ability to transmit visual messages to the brain. RP usually begins to affect people in their teenage years.

Onset of inherited retinal dystrophies is painless, bilateral and typically progressive. Most people experience gradual peripheral vision loss (or “tunnel vision”), and difficulties with poor illumination and night vision. Central vision is usually unaffected, so the person may still be able to read. However, it can also deteriorate to cause total blindness.

Present treatment is aimed at minimising disease progression, thereby prolonging visual function. Recently attempts have been made in animal models to transplant retinal tissue, insert microelectrode arrays or use gene therapy to regain some of the already lost visual function.

Functional implications of RP include:

  • Having difficulty noticing objects at ground level, head height and above
  • Tripping over or bumping into objects
  • Being unable to differentiate between the footpath and road
  • Having difficulty walking on uneven ground
  • Being particularly sensitive to glare and light
  • Having difficulty adjusting to lighting changes (e.g. at dusk or sunrise)
  • Having a limited view of objects
  • Seeing only a portion of images.

For further information, visit Retina Australia.

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