Stargardt disease is a form of juvenile macular dystrophy and is a rare inherited condition causing loss of central vision.
What is Stargardt disease?
Stargardt disease is a rare inherited condition affecting one in 8,000 to 10,000 people. In Stargardt’s the light-sensitive layer of cells in the macular region of the eye degenerate. The macular is the area at the back of the eye which is responsible for the fine detailed vision necessary for activities such as watching TV and reading.
Symptoms of the condition typically develop before the age of 20 and include:
- A loss of detailed vision and colour perception
- Wavy vision
- Blind spots
- Difficulty adapting to low light levels
Whilst people with Stargardt’s do not lose their peripheral (side) vision, many people will reach the point of severe vision loss.
Causes of Stargardt’s
The most common cause of the condition is genetic. There are three genes which have been associated with Stargardt’s: ELOVL4, PROM1 and ABCA4.
Treatments for Stargardt’s
UV-blocking sunglasses can offer some protection for remaining vision. At the moment, Stargardt’s is untreatable.
A number of novel interventions are currently under investigation, including stem cell therapies. Stem cells are a special type of cell which, when put under the right conditions, can develop into many other types of cell including those found in the macular. It is hoped that new cells derived from stem cells can be grown in a laboratory to be transplanted into the eye to replace areas of dead or non-functioning cells. Stem cells can be sourced from a number of places including blood, bone marrow, umbilical cord and fertilized egg cells.
Researchers are involved in Europe’s first ongoing stem cell trial for Stargardt’s. More research will need to be undertaken in the future to determine to what extent stem cell therapy might help improve vision for people with Stargardt’s.