Patient-Reported Social Impact of Molecularly Confirmed Macular Dystrophies and Cone-Rod Dystrophies

患者报告的经分子确诊的黄斑营养不良和锥杆细胞营养不良的社会影响

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Abstract

Objectives: To identify patient-reported key disease-related challenges of macular and cone-rod dystrophies (MDs/CRDs) in a large consecutive cohort of individuals with molecularly confirmed diagnoses. Methods: Out of the 281 patients contacted, 194 (69.0%; 55.2% female) responded to an anonymized survey exploring the effects of MD/CRD on vocational training, professional careers, social participation, family life, personal well-being, and experience with ophthalmologic care. Results: While vocational training was generally less affected, professional careers were frequently disrupted, with 20.6% of patients aged ≥ 50 retiring early. A majority (54.7%) reported feeling restricted in public life. Financial constraints were noted by 20%. A negative impact on familial life (12.3%) was less frequently reported compared to anxiety (74.2%) and depression (15.8%). Diagnostic delays (≥2 years) were common (34.2%), along with a notable rate of initial misdiagnoses (22.1%). The lack of adequate psychological support was a major complaint in professional care. Conclusions: Compared to a previous study in retinitis pigmentosa, MD/CRD patients reported differing patterns of burden, especially in early retirement and family impact. Our findings underscore the need for ophthalmic and social care providers to accelerate the diagnostic process and enhance access to financial assistance and psychological support as key areas to improve patient care.

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