Best's Disease and Its Effects on Vision

Vitelliform Macular Dystrophy or Best's Disease is a relatively rare eye condition that can lead to severe loss of central vision. Some basic information about how it affects an individual and what can be done about it, we will take a look in this article.

How does Best's disease affect the vision?

This condition affects the macula, which is part of the retina and responsible for our central vision. It affects vision, by causing the build up of lipofuscin in the macula. This build up eventually starts affecting nearby cells, leading to damage and impairing the central vision of the individual. Peripheral vision is generally not affected by it.

This condition is observed in people by having an eye exam as early as their childhood, even though it will start affecting them much later in life.. The disease is progressive, so it tends to get worse over time. It is a relatively rare condition, with the rate of people being affected being unknown.

When it comes to this condition, the vision of an individual can be affected in either one or both eyes. The progress of the condition in one eye can differ from the other eye.

What are the symptoms of Best's Disease?

The loss of central vision is one of the symptoms that may signal Best's Disease. Your eye specialist would be able to tell if that is the case. Sometimes it does take a very long time without the individual realising a change in their vision, which is due to the slow progression of this condition. Other signs that are observed include:

- Loss of sharpness

- Blurred vision

- Distorted vision

- It can affect colour perception

What causes Best's disease?

Best's disease is an autosomal dominant genetic condition which means it requires only one of the parents to pass down a faulty gene, to result in a chance to develop this condition. Either of the parents can pass down the gene. There is no known gender related risk, as males and females seem to have approximately similar rates of developing it.

Is there treatment for Best's disease?

As of now there is no treatment for this condition, however as advances in gene therapy are made, the future's looking brighter for people suffering from it. There, however, are treatments for other complications which may arise as a result of Best's disease.

In some situations new blood vessels can form in the eye which would leak and affect further the retina. This is, because they are underdeveloped and much more prone to eventually break, so they need to be monitored and handled if necessary. There are therapies available now, which can effectively deal with such blood vessels and reduce the risk of further damaging the vision of an individual.

Other than that, there are different visual aids that might be able to boost the remaining vision. Moral support and helping an individual re-adjust are important. Thankfully this condition does not progress too fast, so by the time its effects are severe a person can take many steps toward re-adjusting and there might be a good chance for a cure or at least reliable treatment appearing in the meantime.