Usher Syndrome is not just a vision loss problem, but also a hearing loss. In other words, you start to lose your hearing as well as your vision. This is something you inherit from both parents mutated genes. The specific eye problem associated with Usher Syndrome is Retinitis Pigmentosa (RP).
RP causes night blindness and impairs the peripheral vision. Peripheral vision is when you are looking straight ahead, and yet, you can see what is at your side. The reason for loss of peripheral vision and for night blindness is because your retina is degenerating or deteriorating.
The retina is a very sensitive tissue behind the eye that is needed for your vision. With RP, the scope of your vision narrows and this is known as tunnel vision because you can only see what is ahead of you.
This problem can appear at birth if it is a severe case of Usher Syndrome. Most cases though appear at the age of adolescence and the vision problems can be sudden or slowly deteriorate.
Today, Usher Syndrome is the top reason for blindness combined with deafness in the country. According to the statistics, 1 in every 4,000 get RP. The treatment of this condition normally starts with a nutritional therapy which can slow down the progress of this disease. Vitamin A and an omega-3 rich diet have been widely used to help manage this. However, much of this kind of therapy is limited by insufficient research and clinical study.
There are some precautions that must be taken when trying the Vitamin A therapy. For instance, if you are pregnant, do not even try high dose Vitamin A because there is the risk of birth defects. You also should never take more than the 15,000 IU recommended daily dose.
Retinal implants are also being studied as an alternative. Of course, the implants are artificial and to date, the results have shown some promise. There is also growing interest in gene therapy and retinal cell transplantation. Unfortunately, there is no cure at present for Usher Syndrome, but you can use sunglasses to protect your eyes, and visit the eye doctor regularly. If it will help, he will prescribe treatment for cataracts which is a condition wherein your retina starts to swell.
Most often, it is the children who suffer the most especially when RP progresses to a point where vision is down to a minimum or completely lost. There are many cases of young adults losing their vision before reaching the age of 20. Some lucky ones can maintain partial sight. There is no set rules about RP. The extent of the disease will depend on the genes inherited, which makes each case slightly different.
Couples who plan to have children can get genetic testing to determine if they carry the genes or not. This will allow them time to plan their family, and maybe even prepare them for the possibilities of having a child who will develop RP due to Usher Syndrome.
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