The purpose of this paper was to evaluate life experience, including disease characteristics, daily-living activities, employment, interactions with health care professionals, and support services in patients diagnosed with Stargardt's macular dystrophy.
More than 200 patients with Stargardt's disease responded to a 68-question survey. Results were analyzed using SAS Statistical Analysis Package.
Of 203 responders, 142 (70%) were women. Early disease onset occurred in more than 60% of patients. Blurred vision (134, 66%) and glare (183, 90.1%) were the leading symptoms reported. Reading (116, 57.1%), driving (86, 42.4%), and recognizing faces (66, 32.5%) were daily-living activities most difficult to perform. Patients with early disease onset had worse vision at presentation (p = 0.001), faster progression of visual loss (p = 0.007), and were more often diagnosed with a non-physiological visual loss (p = 0.007). Patients with late disease onset had more difficulty with orientation and coping skills (p = 0.02). Sixty-five percent of evaluated adults (108 of 165) were employed.
Although the study illustrates that patients with Stargardt's disease can contribute and function well in contemporary society, issues related to depression, and availability and quality of health care, are still major concerns for this patient population. The study shows differences in progression of visual loss between patients, with early versus late disease onset indicating that age at onset and visual acuity at presentation might be two important factors influencing patient's visual prognosis. Finally, the study suggests parallels in psychological profiles between late age at onset Stargardt's disease and age-related macular degeneration patients.