BEAT THE BEST! - A non-profit awareness and support group for those who wish to learn more, link to, and support further research and awareness of Best's Disease currently rare in the United States and beyond but gaining test cases as the years pass. Please join me in involving yourself in learning about, supporting a cure and research, and getting others to do the same. Together we can BEAT THE BEST!
-- About Best's --
Best's disease is a somewhat rare type of vision condition where a small yolk-like mass effects vision and progresses through stages, ultimately leading to blindness for some. My youngest brother and I were born with Best Disease and I find it essential to gain support and understanding for this condition and its research. Our family researcher on the topic (Dr.Ed Stone - University of Iowa / Carver Lab) has been an excellent pioneer when it comes to BEST'S and so I'm just looking to get the word out about it and see what type of support we can pull together.
-- Definition as found on Best.org --
Best disease, also known as vitelliform macular dystrophy, is an hereditary form of progressive macular dystrophy first identified in 1905. The macula is a small area of about 1mm in diameter in the center of the retina, at the back of the eye where there is a concentration of cone cells. There are 6-7 million cone cells in the retina, the highest concentration of which is in the fovea, a tiny area in the centre of the macula. Best disease is characterised clinically by the classical feature of a round or oval yellow subretinal macular deposit. The yellow material is gradually resorbed over time, leaving an area of RPE atrophy and often subretinal fibrosis. The flash electroretinogram (ERG) shows a normal response but the electrooculogram (EOG) shows a very reduced or absent light rise indicating that there is widespread dysfunction of the retinal pigment ephithelium (RPE). Also, histopathology of donated eyes from patients with Best disease has shown accumulation of lipofuscin throughout the RPE. Best disease can is usually identified between 3 and 15 years of age. Group & Individual interest and support for this group is greatly welcome! If you joined, please spread the invite out to other friends or similar support groups. Spread the wealth of knowledge of conditions similarly looking for cures and awareness.
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I will be looking for ways and means of both gaining awareness for this condition as well as different venues of gaining support for genetic and BEST cure research. Any suggestions, stories, and comments are always welcome!
