On the other hand, the consumption of oily fish, in a diet similar to that which doctors recommend for age related macular degeneration, can be used to slow the progression of the disease.Advances in technology have brought devices that help Stargardt patients who are losing their vision maintain their independence. Low-vision aids can range from hand lenses to electronic devices and can allow those losing their vision to be able to carry out daily activities. Some patients may even opt for in-person services. Prognosis
The long-term prognosis for patients with Stargardt disease is widely variable and depends on the age of onset and genetic alleles.The majority of patients will progress to legal blindness, which means that central reading vision will be lost. However, perimetry and microperimetry studies indicate that the peripheral light sensitivity is preserved over a long time in a significant fraction of all patients (i.e., >50%). Stargardt disease has no impact on general health and life expectancy is normal. Some patients, usually those with the late-onset form, can maintain excellent visual acuities for extended periods and are therefore able to perform tasks such as reading or driving. Epidemiology
A 2017 prospective epidemiologic study that recruited 81 patients with STGD over 12 months reported an incidence of between 1 and 1.28 per 10 000 individuals. The median age of presentation was 27 years (range 5–64 years), most (90%) were symptomatic, with a median visual acuity of Snellen equivalent 20/66. History
Karl Stargardt (1875–1927) was a German ophthalmologist born in Berlin.
Since the advent of genetic testing, the picture has become more complex. What was thought to be one disease is, in fact, probably at least three different diseases, each related to a different genetic change. Therefore it is currently a little confusing to define what Stargardts disease is. Stargardt disease (STGD1) is caused by bi-allelic ABCA4 gene variants (i.e., autosomal recessive). Importantly, the exact genotype (i.e., combinations of both ABCA4 variants) is highly prognostic for the age of onset and disease progression.Autosomal-dominant Stargardt-like diseases were linked to genes such as PROM1 (STGD3) or ELOVL4 (STGD4) missense mutations play a role remains to be seen.The carrier frequency in the general population of ABCA4 alleles is 5 to 10%. Different combinations of ABCA4 genes will result in widely different age of onset and retinal pathology. The severity of the disease is inversely proportional to ABCA4 function and it is thought that ABCA4 related disease has a role to play in other diseases such as retinitis pigmentosa, cone-rod dystrophies and age-related macular degeneration (AMD). STGD1: By far the most common form of Stargardt disease is the recessive form caused by mutations in the ABCA4 gene. STGD4:  A rare dominant defect in the PROM1 gene. STGD3:  A rare dominant form of Stargardt disease caused by mutations in the ELOVL4 gene. Late-onset Stargardt disease is associated with missense mutations outside known functional domains of ABCA4. Pathophysiology
In STGD1, the genetic defect causes malfunction of the ATP-binding cassette transporter (ABCA4) protein of the visual phototransduction cycle.