What is stargardt disease?

Stargardt macular dystrophy (pronounced star-gaarts ma-kyoo-luh di-struh-fee) is an inherited eye condition that affects your macula. It is an inherited condition, caused by a fault in the ABCA4 gene. It is sometimes referred to as juvenile macular dystrophy due to the fact that it can first appear in childhood. However, Startgardt disease can also begin in young adults and late adulthood.

Stargardt causes a wasting of a central area of the retina called the macula. This area is eventually surrounded by a ring of white or yellow spots. An important layer of the retina, the retinal pigment epithelium (RPE), is also affected by an accumulation of 'waste' material called lipofuscin.

Stargardt disease will make your vision unclear or blurry at first. Things may sometimes appear distorted or wavy. You may have problems with your central, detailed vision which can make activities such as reading and recognising faces difficult. Your colour perception may also be affected. If you've had Stargardt disease for a number of years then you may have a blank patch in the centre of your vision. Stargardt disease doesn't usually affect other parts of your retina so your peripheral vision (side vision) is not normally affected.

Stargardt disease can also cause problems with light, such as glare and difficulties adapting to changing light conditions. Some people who have lost a significant amount of vision due to Stargardt or another eye condition may experience visual hallucinations – they may see shapes, colours or figures that aren’t really there. For further information see I've started losing my sight and think I am seeing things that aren't really there. What should I do?

Stargardt disease can also cause problems with light, such as glare and difficulties adapting to changing light conditions.

Unfortunately at the moment there is no treatment for Stargardt disease. Research and developments into gene therapy and stem cells is very active and it's hoped that this may lead to treatments becoming available at some stage in the future.

Another area of research is looking at medications that can reduce the amount of vitamin A by-product that builds up in the eyes of those with Stargardt disease.

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