Key Findings
- Purpose
To assess adults’ knowledge of ocular complications associated with SCD and their reported eye-care practices, including screening and responses to acute visual symptoms. The study focuses on sickle cell retinopathy (SCR), a potentially vision-threatening complication, and examines whether patients understand the risks to vision, the potential for blindness, and the role of eye examinations in detecting retinal disease.
- Population (Model)
The study used an investigator-developed, structured 12-item questionnaire administered to adults with SCD attending routine outpatient visits at the University of Illinois Health Adult Sickle Cell Center. The questionnaire included seven knowledge questions, scored from 0 to 7, and five questions about reported access to and use of eye-care services. Participants were eligible if they were at least 18 years old and had SCD; people experiencing an acute vaso-occlusive crisis during the clinic visit and those who declined participation were excluded. A total of 64 participants completed the survey. The study did not collect demographic information such as age, gender, or SCD genotype, preventing subgroup analyses by these characteristics.
- Headline Result
- Overall knowledge of ocular complications was limited, with a mean score of 2.7 out of 7 (SD 1.27; 95% confidence interval [CI], 2.39–3.01). Although 61% recognized that SCD can affect vision and 63% knew blindness may be avoidable, only 41% recognized that SCD can cause blindness. Just 25% correctly identified that SCD-related blindness is not curable.
- Among all 64 participants, 28% identified the emergency department as the first point of care for sudden vision problems, and 33% for new-onset floaters.
- Regarding reported eye-care practices, 55% said they attended annual ophthalmology visits, while 25% reported visits every 2–5 years, 13% attended only when symptomatic, and 8% were unsure. Although 56% reported having an established ophthalmologist, among all 64 participants, 58% believed that refraction (testing for glasses) constituted a full eye examination; only 14% recognized that this was inadequate, and 28% were unsure.
- Participants with an established ophthalmologist (n = 36) had a higher mean knowledge score than those without one (n = 20): 2.97 versus 2.30, a mean difference of 0.67 (P = .063). Participants who were unsure whether they had an ophthalmologist (n = 8) were excluded from this subgroup analysis.
- Why It Matters
The findings identify a gap between reported engagement with eye care and understanding of the examinations needed to detect SCD-related retinal disease. In particular, patients may attend appointments for corrective lenses without realizing that refraction testing alone does not assess the retina for sickle cell retinopathy. This distinction matters because retinal complications may progress without obvious symptoms, while some acute complications require urgent assessment. The low proportion of participants who recognized the need for emergency evaluation of sudden visual symptoms is another clinically relevant finding. Hematology teams may have opportunities during routine visits to reinforce which symptoms require immediate attention and where patients should seek care. The study also found that participants with an established ophthalmologist tended to have higher knowledge scores, although the difference was not statistically significant and cannot establish that ophthalmology access improves knowledge.
- Evidence Gaps & Limitations
The study included 64 adults from a single clinical setting, limiting the generalizability of its findings. Because age, gender, and genotype were not collected, the researchers could not determine whether knowledge or reported screening practices differed across demographic or clinical subgroups. Information on disease-modifying therapies was also unavailable. The questionnaire was investigator-developed, and the study assessed knowledge and reported practices rather than verifying screening records, examination findings, or subsequent clinical outcomes. Self-reported annual visits therefore do not establish that participants received a comprehensive dilated fundus examination. The survey also cannot establish whether knowledge gaps caused delayed care or whether education or referral interventions would improve screening adherence or visual outcomes.
Source: Journal of Sickle Cell Disease- “Blind Spots: Patient Knowledge and Practices around Ocular Complications in Sickle Cell Disease”
Regulatory & Guideline Watch
The article discusses existing recommendations for routine ophthalmologic surveillance in SCD, including dilated retinal examination, and emphasizes prompt evaluation of sudden visual changes. The study does not introduce new screening intervals or establish a new clinical guideline. For clinical teams, the findings support consideration of practical steps during routine hematology visits: asking whether patients have had a retinal screening examination, clarifying the difference between refraction and a dilated fundus examination, and explaining where to seek urgent assessment for sudden vision loss or new floaters. These are practice considerations, not interventions tested by this survey.