Living With Stargardt’s, Best Disease, and Juvenile Retinoschisis
If you have been told there is nothing that can be done for juvenile macular degeneration, give us a call! Our eye doctor has extensive experience using the latest technology to enable you to live life to its fullest.
What Is Juvenile Macular Degeneration?
Juvenile macular degeneration or JMD is the term used to refer to a few genetic conditions that cause deterioration of the macula and lead to central vision loss that is similar to age-related macular degeneration. The three types of JMD are Stargardt’s, Best Disease, and Juvenile Retinoschisis. JMD effects children, young adults, and adults, although in many cases vision loss only begins later in life. Because Stargardt’s, Best Disease and Juvenile Retinoschisis cause such extreme vision loss that can make it feel like there is no hope, it is recommended to visit an eye doctor that is well versed in the latest technology available for a JMD patient.
Stargardt's is the most common form of juvenile macular degeneration, as it generally causes sever vision loss of 20/100 to 20/400 by the time the patient is in their 30's and 40's. In most cases the vision loss will start slowly beginning to speed up rapidly as the patients vision deteriorates to 20/40. While there is currently no treatment option available for stargardts, there are many different options available that can significantly improve the independence and lifestyle of a patient with stargardt's. A person with this disease may benefit from telescope glasses or bioptic glasses which will allow the performance of tasks such as reading and driving.
Best Disease or Cest Vitelliform Macular Dystrophy is the second most common form of juvenile macular degeneration. The development of Best Disease occurs over 5 stages:
- Stage 1-3 exhibiting little or no vision vision loss
- Stage 4 shows similar or slightly worse vision then stage 1-3
- Stage 5 is clasified by vision from 20/30-20/200
- Stage 6 occurs in 20% of patients with vision of 20/200 or worse
Best Disease effects central vision and the patient generally retains side or peripheral vision. In many cases the disease does not effect both eyes the same way and many patients will retain vision better in one eye over the other. There is no current treatment for Best Disease, however significant improvements in quality of life can be achieved with task specific corrective lenses such as telescope glasses and CCTV magnifiers.
Juvenile retinoschisis effects mainly males and begins showing signs of vision loss between the age of 10 and 20 years old. In the more serious cases the patient may show signs of involuntary eye movement in infancy. Other early symptoms of juvenile retinoschisis include strabismus (cross eyed) and farsightedness. While vision loss begins in the teen years, vision is usually stabilized until the patient reaches their fifties in which case severe vision loss occurs with 50% of patients losing side/peripheral vision. Vision loss ranges between 20/30 and 20/200.
There are no known treatments for juvenile retinoschisis, however significant improvement to quality of life can be achieved with telescope glasses, vision aids, and computer software.