Methods

The medical records of all patients with the diagnosis of Stargardt disease seen by one of the authors (G.A.F.) were reviewed. Patients with documented VA of 20/70 or worse in both eyes either before or up to the age of 22 years were included in the study. All participants who were 21 years of age or older were interviewed.

Of the 545 patients with Stargardt disease who were examined in our clinic, 109 fulfilled the inclusion criteria stated above. We were able to contact 33 subjects via either a phone interview or during a follow-up eye examination. In 2 cases where our patients were deceased, the information regarding only the educational levels was acquired by a first-degree relative after obtaining his or her consent. The purpose of the study was explained to all subjects and participants were interviewed by one of the authors (A.A. or A.T.). All patients were asked the same group of 14 questions. The information that was obtained by the questionnaire included: patient demographics, the highest level of education attained, possible future educational plans, current occupational status, the use of low-vision aids during school years, and the presence of any other medical condition, including an attention deficit disorder, that could possibly interfere with education.

In cases where only a high school degree was obtained, patients were asked to report whether their visual impairment or other reasons (financial or lack of interest) were responsible for not pursuing further education. Subjects were asked subjectively to rate the perception of their visual function during the period of their education (poor, fair, good), the perception of their quality of life (QOL; poor, good, excellent), and whether they had a sense of accomplishment (yes, no, unsure). Additional information was collected regarding the level of education attained by the patients’ parents, the financial status of the family during the period of education, and whether any additional financial support (financial aid, scholarships, grants) was received. For the purpose of data analysis, the financial status of the family was defined as lower economic class for those households with an income of less than $40 000, middle economic class (household income $40 000 to $80 000), and upper economic class (household income more than $80 000).

The Spearman rank correlation coefficient (ρ) was used to evaluate the relationship between the financial status of the patient’s family and the highest level of education attained and between the patient’s subjective perception of visual function during the period of education and the patient’s subjective perception of his or her quality of life.

Results

Of the 33 patients included, 15 were males and 18 were females. The age range of the patients was 21 to 60 years, with a mean age of 32.7 years, (standard deviation, ± 11.6 years). Twenty-four patients were white, 5 were black, 2 were Asian, and 2 were Hispanic. The mean age at diagnosis was 14.3 years.

An analysis of the best-corrected visual acuity documented up to and including the age of 22 years, as measured on a Snellen projection chart, showed that 10 patients (30.3%) had visual acuity better than 20/200 in the better-seeing eye. The remaining 23 patients (69.7%) were classified as legally blind using the criteria of central visual acuity as 20/200 or worse in the better eye. Analytically, the best-corrected visual acuity of the study population ranged from 20/70 to 20/80 in 8 subjects, 20/100 to 20/160 in 2 subjects, and 20/200 to 20/400 in 23 subjects.

Thirty-two of the 33 patients completed high school, whereas 1 completed seventh grade. Of the 32 patients who obtained a high school degree, 5 (15.6%) did not pursue any further education and 27 (84.4%) proceeded for further education. For the 5 patients who obtained only a high school degree, 2 reported that the main reason for not pursuing any further studies was their visual condition and the remaining 3 reported that the reason was not related to vision.

Five individuals (15.6%) obtained an associate’s degree defined as a certificate granted upon completion of 2 years of education beyond high school (1 additional patient was enrolled for a bachelor’s degree after attaining an associate’s degree). Fifteen individuals (46.9%) obtained a bachelor’s degree, defined as the successful completion of 4 years of full-time undergraduate study or the equivalent. Three individuals (9.4%) were enrolled in college at the time of the interview: 1 patient was in the first year, and 2 patients were in their second year. Four additional patients (12.5%) had attended but did not complete a college program. In these 4 patients, the discontinuation of education was related to the visual impairment in 1 patient, partly related to the visual impairment in 1 patient, and unrelated to vision in 2 additional patients. Seven patients (21.9%) pursued a graduate degree, with 6 patients acquiring a master’s degree and 1 patient acquiring a doctoral degree. The Table summarizes these findings.

There was no statistically significant relationship between the age at which patients reached a clinically significant level of low vision (defined as 20/70 or less in their better-seeing eye) and their level of education (ρ = −0.06; P = .76). For the subjects with only a high school degree, the average age at which low vision was recorded was 15.5 years. For the subjects who attended college, the average age at which low vision was recorded was 15.1 years. For the subjects with education beyond a bachelor’s degree, the average age at which low vision was recorded was 14.7 years. These average ages are all within 1 year, indicating that the age at which patients reached a clinically significant low vision was not related to their educational levels.

Two patients included had significant medical disorders that had the potential to interfere with their studies: 1 was affected with severe Crohn disease and 1 was affected with a bipolar psychiatric disorder. Among these 2 patients, 1 completed a master’s degree, whereas the other discontinued high school.

Regarding the use of low-vision aids during school years, 26 patients (78.8%) used low-vision aids, most commonly magnifiers, a closed-circuit television, or both. Seven patients (21.2%) did not use any low-vision aids during their educational years. In the 26 patients who did use aids, the educational degrees attained were distributed among all the various academic levels; 3 patients obtained only a high school degree, 7 obtained a postgraduate degree, and 13 were either enrolled or had completed a college degree. Similar findings were obtained in the 7 patients who did not use low-vision aids: 2 of the patients completed only high school, 3 completed either a bachelor’s or associate’s degree, and 2 patients had discontinued college.

Assessment of patients’ self-perceptions of visual function during the period of their education yielded the following results: 15 patients reported a perception of poor visual function, fair visual function was reported by 14 patients, and good visual function was reported by 2 patients. Additionally, patients subjectively were asked to assess the perception of their quality of life: 9 patients described their overall quality of life as excellent; 20 described it as good; 1 described it as poor; and 1 patient was unable to give a direct answer. The patients’ self-reported perception of visual function during school years was correlated significantly with the self-reported perception of quality of life (ρ = 0.53; P = .002).

The economic status of the patients’ families was found to be lower class (n = 16), middle class (n = 7), and upper class (n = 9). All patients who attained only a high school degree belonged to the lower economic status group, and similarly, most of the patients who attained a degree beyond a bachelor’s were found to be in the same lower economic group (5 of 7). Thus, the financial status of a patient’s family and the highest level of education attained were not significantly correlated (ρ = 0.11; P = .56) Among the 31 patients, 18 received financial support from sources other than their families (grants, scholarships, student loans from disability services, etc.) during their educational years, whereas 13 did not receive any financial support apart from that from their families.

At the time of the interview, 20 patients were currently employed; 13 patients were employed full time and 7 patients held a part-time position. Ten patients currently were unemployed, and 1 was retired. Of the 10 patients who were unemployed, 6 reported that it was related either partly or entirely to their visual impairment, whereas 4 reported that it was unrelated to their vision.