Impact of Retinitis Pigmentosa on Quality of Life, Mental Health, and Employment Among Young Adults

Impact of Retinitis Pigmentosa on Quality of Life, Mental Health, and Employment Among Young Adults

Accepted Manuscript Impact of Retinitis Pigmentosa on Quality of Life, Mental Health and Employment Among Young Adults Anne-Elisabeth Chaumet-Riffaud,...

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Accepted Manuscript Impact of Retinitis Pigmentosa on Quality of Life, Mental Health and Employment Among Young Adults Anne-Elisabeth Chaumet-Riffaud, Philippe Chaumet-Riffaud, Anaelle Cariou, Céline Devisme, Isabelle Audo, José-Alain Sahel, Saddek Mohand-Said PII:

S0002-9394(17)30076-4

DOI:

10.1016/j.ajo.2017.02.016

Reference:

AJOPHT 10052

To appear in:

American Journal of Ophthalmology

Received Date: 17 June 2016 Revised Date:

11 February 2017

Accepted Date: 15 February 2017

Please cite this article as: Chaumet-Riffaud A-E, Chaumet-Riffaud P, Cariou A, Devisme C, Audo I, Sahel J-A, Mohand-Said S, Impact of Retinitis Pigmentosa on Quality of Life, Mental Health and Employment Among Young Adults, American Journal of Ophthalmology (2017), doi: 10.1016/ j.ajo.2017.02.016. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

ACCEPTED MANUSCRIPT ABSTRACT • PURPOSE: To determine the relationship between visual function and quality of life, education, mental health and employment among young adults with Retinitis Pigmentosa (RP). • DESIGN: Cross-sectional study.

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• METHODS: Inclusion of 148 patients (mean age 38.2 +/- 7.1 yrs) diagnosed with RP, living in France. Quality of life was assessed using the National Eye Institute Visual Function Questionnaire (VFQ-25), mental state with the Hospital and Anxiety and Depression Scale (HADS) and employment with a specifically designed questionnaire.

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• RESULTS: Limited visual impairment was noted in 22.3%, low vision in 29.7% and legal blindness in 48.0%. There was a correlation between quality of life scores and residual visual field (P < .0001). Mental health scores were suggestive of anxiety in 36.5 % and depression in 15.5%. The rates did not increase with disability level (P = .738, P = .134). The percentage of subjects with higher education did not significantly decrease with disability level (P = .113). The employment rate did not significantly decrease with disability level (P = .276). It was lower in subjects reporting depression (P = .0414). Self-rated impact of RP on employment increased with disability level (P =.02642).

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• CONCLUSIONS: Our results differ from previous results showing lower education rates and employment rates in young adults with RP. Further research is warranted focusing on the impact of mental health, education, workplace conditions and employment aids on employment rate vs. age and education matched normally sighted controls to guide visual disability strategies in RP.

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IMPACT OF RETINITIS PIGMENTOSA ON QUALITY OF LIFE, MENTAL HEALTH AND EMPLOYMENT AMONG YOUNG ADULTS Impact of retinitis pigmentosa in young adults Corresponding Author CHAUMET-RIFFAUD Anne-Elisabeth 1 CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DGOS CIC 1423, 28 rue de Charenton, 75012 Paris, France Email :[email protected] ; [email protected] Tel number : +33 06 30 87 26 07

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CHAUMET-RIFFAUD Philippe 2 Service de Biophysique et Médecine nucléaire Hôpitaux universitaires Paris Sud, Bicêtre APHP Univ Paris Sud 78 rue du général Leclerc, Le Kremlin Bicêtre 94275 FRANCE

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CARIOU Anaelle CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DGOS CIC 1423, 28 rue de Charenton, 75012 Paris, France 1

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DEVISME Céline 1 CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DGOS CIC 1423, 28 rue de Charenton, 75012 Paris, France

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AUDO Isabelle 3 Sorbonne Universités, UPMC Univ Paris 06 UMR S968, INSERM U 968, CNRS UMR_7210, Institut de la Vision, 17 rue Moreau, 75012 Paris, France 1 CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DGOS CIC 1423, 28 rue de Charenton, 75012 Paris, France

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SAHEL José-Alain 3 Sorbonne Universités, UPMC Univ Paris 06 UMR_S968, INSERM U_968, CNRS UMR_7210, Institut de la Vision, 17 rue Moreau, 75012 Paris, France 4 Fondation Ophtalmologique Adolphe de Rothschild Hospital, 29 rue Manin, 75019 Paris, France MOHAND-SAID Saddek Sorbonne Universités, UPMC Univ Paris 06 UMR S968, INSERM U 968, CNRS UMR_7210, Institut de la Vision, 17 rue Moreau, 75012 Paris, France 3

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CHNO des Quinze-Vingts, DHU Sight Restore, INSERM-DGOS CIC 1423, 28 rue de Charenton, 75012 Paris, France

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Rod-cone dystrophy also known as Retinitis Pigmentosa (RP) is a genetic disease affecting 1.6 million worldwide (1). It represents a leading cause of visual disability and blindness in subjects less than 60 years (2). It accounts for 25 to 30% of all visual disability cases and is the first cause of inherited blindness. Progressive bilateral degeneration of rod and cone photoreceptors leads to night blindness followed by significant loss of peripheral vision by young adulthood, and then to progressive loss of central vision, with legal blindness by the age of 40. The loss of peripheral vision will reduce the subjects’ mobility and ability to perform common tasks, thus leading to severe visual disability (3). Employability and employment sustainability may well be affected at the prime of life. Previous studies in RP have shown that visual acuity (VA) and visual field (VF) abnormalities are strong predictors of self-reported visual quality of life, anxiety and depression (4) (5) (6).

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Severe visual impairment is associated with lower employment rates, decreased productivity and wellness at work (7) (8). In the USA, the employment rate was estimated at 42% in subjects with severe visual impairment vs. 69.5% in subjects with normal vision; only 22% of 4 million Americans with vision loss are employed (9). In France, 29% with low vision or blindness were employed vs. 65% with mild impairment (10). Studies focused on RP also show lower education levels, lower income levels and a higher rate of unemployment by the age of 40 vs age paired controls (11) (12). These results suggest that an insufficient number of visually disabled workers benefit from disability aids, despite structured state funded disability policies in developed countries. However, there is limited research taking into account the impact of mental health and work conditions on employment sustainability in visually disabled individuals.

METHODS

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The objectives of this study were to determine the relationship between visual function and quality of life, mental health, education and employment among young adults with RP.

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The study was designed as a cross-sectional survey on a sample of patients followed at REFERET, a national reference centre for inherited retinal dystrophies, at 15-20 National Ophthalmology Hospital, Paris, France. The study was conducted in accordance with the tenets of the declaration of Helsinki and French regulations. It was approved prospectively by an Ethics committee in May 2012 (CPP Ile de France V, Paris, n°IDRCB 2006-A003 47-44). Patients gave their consent for data collection and statistical analyses. • COLLECTION OF DATA: Patients followed at REFERET are routinely monitored every 2 years and have access to a resident psychologist, social worker and occupational health physician. Eligible subjects were identified in the retinal dystrophy cohort and contacted sequentially by date of last visit. Inclusion criteria were: diagnosis of RP confirmed by clinical history, VF, fundus appearance, electroretinogram (ERG) and genetic testing, age 25 to 50 years,

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ACCEPTED MANUSCRIPT country of residence France, last examination less than 2 years back comprising VA, VF, quality of life (QoL) and mental health, and informed consent.

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Non-inclusion criteria were any other ocular disease including known complications of RP (cataract and macular oedema), sensory disability, chronic disease or handicap which may influence residual vision or employability. Visual disability rating was established based on the International Classification of Diseases (ICD-10) and International Council of Ophthalmology (ICO) standards (13).

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Vision-related QoL was assessed using the National Eye Institute Visual Function Scale (NEI-VFQ-25) (14) (15), and mental status using the Hospital Anxiety and Depression Scale (HADS) (16) (17). A 30-item employment status questionnaire, previously tested in a pilot study, was administered by an experienced psychologist or physician by phone or during face to face interviews. The employment assessment, conducted by an occupational health physician, required at least 12 consecutive months in the same position, based on current position or last position held in the last 5 years. It focused on occupational factors which may increase the risk of work accident for severely disabled individuals or their entourage, eg driving, and employment aids.

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RESULTS

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• STATISTICAL ANALYSES: Unless indicated otherwise, results are given as mean ± SD in the text. Comparisons were established with French age adjusted socioeconomic and employment statistics collected by the French institute of statistics and economic surveys INSEE in France (18) (19), as there was no control group. For categorical variables, data were analysed using the Pearson’s χ2 test. For quantitative variables, comparisons between ICD score groups were done with the independent-samples t test or ANOVA test. Kruskal Wallis test served for comparisons with variables not fulfilling the normality assumption of a one-way ANOVA. Statistical significance was defined as a P value < 0.05 (twotailed test). Data were analysed using SPSS version 20.0 (IBM Corp, Armonk, New-York,USA).

The study included 148 subjects diagnosed with RP, 70 male and 78 female, mean age 38.2 ± 7.1 years, last followed at REFERET between April 2013 and November 2015. • VISUAL FUNCTION AND DISABILITY STATUS (Tables 1 & 2): The diagnosis of RP was confirmed in all subjects, Usher syndrome type 2 with fully compensated hearing deficit was recorded in 16.2% (24/148). It was established before the age of 25 in 50 % of the cases: 18.2% (27/148) were aged 15 or less. Over five years had elapsed since diagnosis in 67.6 % (100/148). As expected, time since diagnosis increased with the level of visual disability (P<.0001, Kruskal Wallis test).

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ACCEPTED MANUSCRIPT Median Best Corrected Visual Acuity (BCVA) was 0.63 (range: Positive Light Perception- 1.6) and binocular visual field radius (VF) III-4-e 12° (range: 0° - 90°). Visual acuity (VA) was generally well preserved with near normal vision range (BCVA >= 0.32) in 82.4 % (122/148). Severe constriction of the visual field (VF <= 40° or less) was noted in 76.3% (113/148). Subje cts with BCVA less than 0.32 had VF radius less than 20°, except in 2 cases . Eighty percent (119/148) did not meet legal requirements for driving in France.

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Based on the ICD score (Table 2), 22.3% had limited visual impairment (BCVA above 0.3 and VF above 40°), 29.7% were rated low v ision (VF between 10° and 40°or BCVA between 0.05 and 0.3), and 48.0% were le gally blind (VF 10° or less or BCVA less than 0.05). The distribution by ICD score did not differ between male and female subjects (P = .328).

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More subjects rated blind (42.3%) required visual aids, such as low vision rehabilitation, a white cane or guide dog (P = .0650). Mental health counselling was recorded in 9.5% (14 /148). The rate of mental health counselling tended to increase with disability level but did not reach significance (P =.109).

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More subjects rated blind were granted visually disabled worker status (73.2%; P =.000153) or were the beneficiary of a disability pension (35.2%, P =.000024). Fifty percent in the low vision group (22/44) were reluctant to request official recognition of their handicap.

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• SELF-RATED QUALITY OF LIFE AND MENTAL STATUS: Visual quality of life (VFQ-25) scores were lower in the study sample, with a mean composite score of 58.1 ± 17.9 vs. reported values in the 80-100 range in normally sighted healthy subjects (20). There was a correlation between total score and VA (P < .0001), total score and VF (P < .0001), composite score and VA (P < .0001), composite score and VF (P < .0001) (figure 1). Mental state self-rated scores (HADS) were suggestive of anxiety in 36.5 % (54/148) and depression in 15.5% (23/148) vs. reported values of 9 % and 2% in healthy controls (16). Anxiety scores did not increase with disability level (P=.738). Depression scores tended to increase with disability level but did not reach significance (P =.134).

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• LEVEL OF EDUCATION AND EMPLOYMENT STATUS (Table 2): The percentage of subjects with higher education (completed at least a 2-year university course) tended to decrease with disability level but did not reach significance (P =.113). It was 56.3 % in the blind group vs 42.3 % in French age paired statistics (18). Four percent (6/148) had to quit school because of their illness. The rate did not increase with disability level (P = 0.941). The employment rate tended to decrease with disability level but did not reach significance (P = .276). At inclusion, it was 67.6% in the blind group and 79.5 % in the low vision group vs. 80.2% in French age-paired estimates (25-49 years) (19). All subjects had worked in the last 5 years for at least 12 consecutive months, except for three blind patients who never worked. The employment rate was significantly higher in subjects with higher education (P= .00127). It was lower in subjects reporting a depressive state (P = .0414). It did not significantly differ between male and female patients (P = .328).

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• OCCUPATIONAL ASSESSMENT AND EMPLOYMENT AIDS (Table 3): Subjects rated blind were more likely to hold a desk job: 55.9 % (38/68) had a management, office or administration position (P=.0242). Over 70 % of the subjects had a long term contract (106/145) in a company with over 20 employees (103/145). Median seniority was 7.5 years in the same position. It did not vary with disability level (P = .341). Occupational factors which may increase the risk of work accident for severely disabled individuals or their fellow workers were recorded in 75.0% rated low vision vs 48.5% rated blind (P =.0204). Their position entailed regular driving or out-of-the office assignments in 45.5 % (20/44) rated low vision. Thirty-two percent (14/44) in the low vision group drove to work, 75.0% (51/68) in the blind group used public transports. Twenty percent (9/44) in the low vision group used dangerous equipment or chemical products on a daily basis. Self-rated impact on employment, in terms of job security, professional recognition and compensation, increased with disability level (P = .0264).

DISCUSSION

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The percentage informing their employer of their handicap tended to increase with disability level but did not reach significance (P = .078): it was 52.3 % in the low vision group. Over 40% (59/145) were not in a position to request employment aids: 31/145 had not disclosed their handicap and 28/145 were self– employed. The rate benefiting from employment aids was 22.7% in the low vision group vs 54.4% in the blind group (P = .0000141). Individuals relied on colleagues for assistance, e.g. 29.4 % in the blind group. Only 1.5 % (2/145) had an aid for commuting to work. Nearly 45% (64/145) requested information relative to disabled worker benefits, redundancy, transport and employment aids. Thirtytwo percent (47/145) planned to request technical aids or a job change after the study.

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Visual acuity and especially residual visual field were strong predictors of selfrated visual QoL in RP, in line with published data (4) (20) (21). Our results corroborate previous findings showing severe functional limitations for a residual visual field below 10° radius, near-normal orientat ion and mobility in the 10° to 30° range and normal abilities above 40° (5) (13). Subjects adapt to progressive visual field loss with visual aids, rehabilitation and develop vision substitution skills (20) (22). Lower QoL scores in the blind subset suggest that vision substitution skills may not be sufficient to deal with social interactions, at an advanced stage of the disease. Our results suggest that RP is associated with a higher prevalence of anxious or depressive states than normally-sighted individuals. Mental health is a determinant of visual quality of life, with lower QoL scores in depressed vs. nondepressed RP subjects (12) (23) (24) (25). Adjustment to visual impairment is a protracted process which involves acceptance, integrating functional implications and developing adaptive skills (12). The longer the duration of perceived vision loss, the less it is associated with emotional distress, which could be explained by adjustment to the diagnosis (4).

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ACCEPTED MANUSCRIPT Our results did not support previous results indicating that fewer subjects with RP achieve higher education than normally sighted individuals (7) (11). This may be related to the exclusion of subjects with non-compensated syndromic RP (11).

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To our knowledge, there is limited information to evaluate the impact of visual disability level on employment in RP (26). Lower employment rates were noted in subjects with non-syndromic RP vs controls (61.49 % vs 83.92 % at age 40) in a Danish study (11). In our study sample, the employment rate did not significantly decrease with disability level. Our findings could partially be explained by a higher level of education than in the general population (18) (27) and seniority in large organizations (27) (28). The employment rate of disabled workers is significantly higher in large organizations than in small companies (27). Large companies are more likely to implement company-sponsored policies in favor of disabled workers than small companies (27) (28). Disabled workers still employed have greater seniority and a more qualified job than those not employed (27).

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Our results suggest that subjects rated low vision may require an assessment of working conditions at each visit to ensure they comply with safety regulations, eg stop driving or using dangerous machinery or products. Risk management measures and employment aids may contribute to employment sustainability (29) but there is limited information to evaluate their impact in visual disability (26).

CONCLUSIONS

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The main strengths of this study were standardized data collection and methodology taking into account visual function, quality of life, mental health and employment factors. Limitations are related to small sample size, restricted geographic scope and lack of an age and education matched normally sighted control group.

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Our results differ from previous results showing lower education rates and employment rates in young adults with RP. Further research is warranted focusing on the impact of mental health, education, workplace conditions and employment aids on employment rate vs. age and education matched normally sighted controls to guide visual disability strategies in RP.

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ACCEPTED MANUSCRIPT Acknowledgments / disclosure

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All authors have completed and submitted the International Committee of Medical Journal Editors (ICMJE) form for disclosure of potential conflicts of interest and none were reported. Contributions of authors: design of the study (AEC, SMS); conduct of the study (AEC); collection and management of data (AC, CD, IA, SMS); analysis and interpretation of the data, (PC, AEC, SMS); preparation of manuscript (AEC, PC); review or approval of manuscript (JAS, SMS). Funding

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This project was supported by Foundation Fighting Blindness (FFB) FFB center grant [C-CMM-0907-0428-INSERM04], [C-CL-0912-0600-INSERM01]; Ville de Paris and Région Ile-de-France; LABEX LIFESENSES [reference ANR-10-LABX65].

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21. Wright SE, Mc Carty C, Burgess M, Keeffe JE. Vision impairment and handicap: the RVIB Employment Survey. Aust N Z J Ophthalmol. 1999; 27(3-4):204–7. 22. Hayeems RZ, Geller G, Finkelstein D, Faden RR. How patients experience progressive loss of visual function: a model of adjustment using qualitative methods. Br J Ophthalmol. 2005;89(5):615-20. 23. Szlyk JP, Seiple W, Fishman GA, Alexander KR, Grover S, Mahler CL. Perceived and actual performance of daily tasks: Relationship to visual function tests in individuals with retinitis pigmentosa. Ophthalmology. 2001;108(1):65-75. 24. Zhang X, Bullard KM, Cotch MF et al. Association between depression and functional vision loss in persons 20 years of age or older in the United States, NHANES 2005-2008. JAMA Ophthalmol. 2013; 131(5):573-81. 25. Nyman SR, Gosney MA, Victor CR. Psychosocial impact of visual impairment in working-age adults. Br J Ophthalmol. 2010; 94(11):1427-31. 26. Zambelli-Weiner A, Friedman DS. Building a basis for action: Enhancing public health surveillance of vision impairment and eye health in the United States. Am J Ophthalmol. 2012;154(6 Suppl):S8-S22. 27. Barhoumi M, Ruault M, Valat E. Les accords au titre de l’obligation d’emploi des travailleurs handicapés: quelles incidences sur l’emploi et les actions menées par les établissements? DARES Analyses n°065, November 9, 2016. Available at http://dares.travail-emploi.gouv.fr/IMG/pdf/2016065v2.pdf. Accessed February 9, 2017 28. Chabanon L. L’obligation d’emploi des travaileurs handicapés: comment les établissements s’en acquittentt-ils? DARES Analyses n°064, November 5, 2016. Available at http://dares.travailemploi.gouv.fr/IMG/pdf/2016-064.pdf. Accessed February 9, 2017 29. American Diabetes Association, Anderson JE, Greene MA et al. Diabetes and Employment. Diabetes Care. 2012;35(1):S94-8.

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ACCEPTED MANUSCRIPT Figures

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FIGURE 1 Scatterplot showing a significant association between the NEI-VFQ composite score and binocular visual field III-4 (R2 = 0.158, P <0.01; Pearson’s correlation coefficient 0.398). The black line indicates the best-fit linear regression line.

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Retinitis Pigmentosa type, n (%)

124 (83.8) 24 (16.2)

Retinitis Pigmentosa, median, range

Age at diagnosis Time since diagnosis

24.6 (3-50) 10.2 (0-40)

Visual function, median, range

Visual acuity (BCVA) Binocular VF-III-4-e (radius)

0.63 (PLP-1.6) 12° (0-90°) 33 (22.3) 44 (29.7) 71 (48.0)

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Non syndromic RP Syndromic RP

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Table 1 Description of the Retinitis Pigmentosa study population : visual characteristics, quality of life and mental health ratings (n = 148)

ICD Visual impairment score n (%)

Visual disability status, n (%)

Mean HADS(b) (SD)

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Mean NEI-VFQ-25 (a) (SD)

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Visual aids, n (%)

Limited visual impairment Low vision Blindness At least one visual aid

49 (33.1)

Disabled worker status Disability allocation

82 (55.4) 29 (19.6)

VFQ total score VFQ composite score

57.8 (17.1) 58.1 (17.9)

HADS Anxiety score HADS Depression score

9.3 (4.2) 5.3 (3.9)

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(a) NEI-VFQ-25: National Eye Institute Visual Function Questionnaire – 25 items (b) HADS: Hospital Anxiety and Depression Scale

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Table 2 Impact of visual handicap level on socio-professional criteria in the Retinitis Pigmentosa study population (n = 148) Mild

Low vision

Blind

deficiency

P Value Pearson’s

n = 44

At least one visual aid n (%)

7 (21.2)

12 (27.3)

Disabled worker status n (%)

13 (39.3)

17 (38.6)

Disability pension n (%)

1 (3.0)

3 (6.8)

Higher education n (%)

25 (75.8)

24 (54.5)

Quit school due to RP n (%)

1 (3.0)

Employed in the past n (%)

33 (100.0)

Employed at inclusion n (%)

26 (78.8)

n = 71

χ2 test

30 (42.3)

0.065

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n = 33

0.000153

25 (35.2)

0.000024

40 (56.3)

0.113

2 (4.5)

3 (4.2)

0.941

44 (100.0)

68 (95.8)

0.190

48 (67.6)

0.276

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52 (73.2)

35 (79.5)

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The handicap level is evaluated according to the International Classification of Diseases (ICD-10) and International Council of Ophthalmology (ICO) standards

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Low

deficiency

vision

n = 33

n = 44

n = 68a

χ2 test

Companies > 20 employeesb n (%)

20 (60.6)

30 (68.2)

53 (77.9)

0.174

Long term contract n (%)

22 (66.7)

34 (77.3)

50 (73.5)

0.580

Seniority over 5 years n (%)

18 (54.5)

27 (61.4)

47 (69.1)

0.341

Office position n (%)

10 (30.3)

16 (36.4)

38 (55.9)

0.0242

At least 1 risk factord n (%)

20 (60.6)

33 (75.0)

33 (48.5)

0.0204

Employer informed n (%)

12 (36.4)

23 (52.3)

41 (60.3)

0.0780

Occupational health informed n (%)

17 (51.5)

26 (59.0)

43 (63.2)

0.531

Occupational health support n (%)

4 (12.1)

12 (27.3)

24 (35.3)

0.0504

Employment aids n (%)

5 (15.2)

10 (22.7)

37 (54.4)

0.000141

3 (9.1)

8 (18.2)

20 (29.4)

0.538

17 (51.5)

30 (68.2)

53 (77.9)

0.02642

12 (36.4)

19 (43.2)

33 (48.5)

0.625

8 (24.2)

15 (34.1)

24 (35.3)

0.517

Assistance from colleagues n (%) RP impact on employment n (%) e

Request for information n (%) Employment change planned n (%) a Three

P Value

SC

RI PT

Pearson’s

M AN U

c

Blind

subjects had never worked and were excluded from the analysis. In France, since July 1987, companies with over 20 employees are required to employ at least 6% disabled workers or contribute to a state disability fund c Other occupations: installation, maintenance and repair (n = 33), trade (n = 20), health and social care (n = 15), education (n = 11). d Occupational risk factors: regular out-of-office assignments (n = 54) or driving (n = 34), daily use of chemical or biological agents (n = 25), or complex machinery or equipment (n = 24) e Requests for information centered on visual disability recognition process, disabled worker status benefits, redundancy, transportation & technical aids

AC C

EP

TE D

b

AC C

EP

TE D

M AN U

SC

RI PT

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