Stargardts DiseaseStargardts disease is the most common form of juvenile macular degeneration. The changes in vision are usually noticed before the age of 20. 
Juvenile Macular DegenerationThis genetic form of
macular dystrophy
results in a dying of the photoreceptor cells in the
macula
which provide us with our detailed and central vision.
1) they do not know that they are carriers and 2) the parents do not have the disease themselves. The responsible gene, ABCR, has recently been identified which will be useful for future gene therapy. There also is a form of Stargardt's that is inherited from a dominant gene. Detection and Diagnosis
Initially, the child will complain of some vision loss, but JMD may not be detected during an eye exam and the macula may appear normal. This often causes delayed diagnosis or a lack of validation of the child's complaints of vision problems.Later, as Stargardts progresses, and it will, a
fluorescein angiogram
will show changes in the
choroid layer,
a layer of blood vessels that feeds the macula. Eventually small yellowish-white flecks (fundus flavimaculatus) appear in the peripheral retina.
Macular Dystrophy Symptoms
Symptoms of Stargardt's are:√ Blurred central vision √ Distorted vision √ Loss of visual acuity √ Small blind spots; objects may disappear and reappear causing a come and go effect. √ Difficult time adapting to the dark from the light √ Difficulty seeing in dim lighting √ Increased light sensitivity √ Color vision declines as the disease progresses √ Loss of depth perception
Stargardt's Treatment
According to conventional medicine, there currently is no treatment for Stargardts. There are things that can be done to help protect your eyes like wearing sunglasses and wide brimmed hats. To read more about choosing the right sunglasses click here:
Sunglasses
Supplementing with Vitamin A is contraindicated for those with Stargardt's Disease. It is believed that the gene mutations lead to abnormal synthesis of the vitamin in the eyes, resulting in increased loss of vision. Because People with Stargardt’s Disease are missing an essential enzyme, they are unable to get rid of the waste products from metabolized Vitamin A. The build up of these waste products leads to the death of retinal cells, so supplementing with Vitamin A should be avoided. Also, certain foods should be restricted that are high in Vitamin A.
Nutrient Recommendations from Natural Eye Care
Low Vision RehabilitationTo maximize the existing sight of a patient and to learn tips for living independently, it is best to utilize the services of a low vision rehabilitation specialist.This specialist can assess the needs of an individual and make recommendations for visual aids to help a person at home, at school or at work. Click here to read more:
Low Vision Rehabilitation
Visual Aids for Macular DystrophyThere are many visual aids that can allow your child or young adult continue to be successful in school or at a job.Become aware of what these are so that when the need arises you will be prepared to help your child achieve independence. Click here to read more about visual aids:
Vision Aids for Macular Degeneration
Stargardt's Research1. DHA Supplementation in Patients With STGD3Currently at the University of Utah in Salt Lake, a clinical trial is going on that is looking at dietary intake of omega-3 fatty acids. This study is still on going, however, they are not recruiting any new participants. The stated purpose is: "We have found that biomarkers of long-term elevated dietary intake of omega-3 fatty acids such as DHA and EPA are inversely associated with severity of disease phenotype in Dominantly Inherited Stargardt's Disease (STGD3)STGD3 patients. Therefore, the purpose of this study is to follow STGD3 patients as they supplement their diets with DHA/EPA." I will update you when the study results are posted. 2. Saffron Supplementation in Stargardt's Disease (STARSAF02) "The aim of this research is to investigate the influence of short-term Saffron supplementation on retinal function in STG/FF patients carrying ABCR mutations. The macular cone-mediated electroretinogram (ERG) in response to high-frequency flicker (focal flicker ERG) will be employed as the main outcome variable." Contact: Benedetto Falsini, MD 0039063015 ext 4929 bfalsini@rm.unicatt.it Rome, Italy 3. Stem Cell Therapy Advanced Cell Technology received FDA approval to begin Phase I clinical trials using stem cells for the treatment of Stargardt's Macular Dystrophy. The clinical trial site is Jules Stein Eye Institute, UCLA School of Medicine in Los Angeles, California, United States. A press release from February 2011 highlighted a recent patent issued to ACT for their "proprietary single-blastomere technology that provides a non-destructive alternative for deriving human embryonic stem cell (hESC) lines." In other words, the embryos are not affected or destroyed by this new technology. When the trial sites have been determined I will report on it here. It is a Phase I/II study which means they are looking mostly at the safety of a subretinal injection of human embryonic stem cell derived retinal pigment epithelium cells. At this time so only 12 patients will be enrolled. To be eligible a patient must be over 18 and have advanced Stargardt's macular dystrophy. To learn more about this study go to the National Institute of Health's Clinical Trial website.
Macular Dystrophy Stem Cell Study
A company called Oxford BioMedica is working on a gene therapy treatment that replaces the defective ABCA4 gene that causes Stargardt disease by using their LentiVector® technology to deliver a corrected version of the gene into the cells of the retina. According to Oxford BioMedica, "The generation of a functional protein from the corrected gene has the potential to reduce or even halt the ongoing retinal degeneration." Go from Stargardts Disease to Juvenile Macular Degeneration
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