Juvenile macular degeneration is a group of rare inherited eye disorders that cause central vision loss in children and young adults. The most common form of the disorder is Stargardt disease. Other forms include Best’s disease and juvenile retinoschisis. There is no treatment and no cure for these diseases and they result in legal blindness. Here are four important facts about juvenile macular degeneration.
1: Juvenile macular degeneration is a genetic disorder passed from parent to child that affects adolescents and young adults in their 20s and 30s. There are different genes that pass these disorders from one generation to another. Some genes are considered recessive which means that both parents have to carry the gene in order for the child to inherit the disorder. Stargardt disease is a recessive gene. Others are what is called dominant genes meaning that only one parent has to carry the gene for the child to inherit the disorder. Best’s disease is a dominant gene. Juvenile retinoschisis is called an “X-linked disorder” and as a result affects mostly males. According to the American Academy of Ophthalmology, “The genetic mutation that causes the disease is found on the X chromosome, which males inherit from their mothers. (Fathers contribute the Y chromosome.)”
2: Juvenile macular degeneration affects the central field of vision. The disorder affects the macula, which is at the center of the retina, which controls the ability to see fine details in the central field of vision. As the disorder progresses, it impacts the central field of vision, making it blurry, wavy, distorted in other ways or clouded by a grey or dark circle. This vision loss makes it difficult to see objects in the central field of vision, read and drive. Juvenile macular degeneration doesn’t impact peripheral, or side, vision but it may adversely impact the ability to see colors. Each eye may be affected differently.
3: Upon examination, an ophthalmologist can see the presence of Stargardt disease in the eye. The disease may cause yellowish flecks in the center of the eye. Sometimes they create rings in the eye that extend outward from the center of the retina. The yellow flecks are caused by deposits of lipofuscin, a byproduct of cell activity in the eye. As it builds up it causes the yellow flecks to appear. When an ophthalmologist conducts a dilated eye examination of the retina he or she will be able to detect signs of the disease.
4: There is no treatment or cure for juvenile macular degeneration but advanced technologies can enhance low vision. A comprehensive vision exam will create a roadmap of remaining sight and assist ophthalmologists in determining which customized vision systems and technologies will best enhance remaining vision. Then microscope and telescope lenses, and wearable technologies like IrisVision can be used to allow the individual to fully participate in activities of daily living. These technologies vastly expand the lived environment for those with low vision.
It may be considered rare, but for those who live with the impact of juvenile macular degeneration it is life changing – and permanent. That is why we work every day to improve the lives of those living with low vision. We understand that it can be isolating. If you or a loved one has been told that nothing more can be done to improve your low vision, call us. We work with hundreds of patients who have been told the same thing and every day we use advanced technologies to improve their remaining sight. Call us so we can help you.