Practice Patterns and Challenges in Managing Inherited Retinal Diseases Across Asia-Pacific: A Survey from the APIED Network.

PURPOSE: The objective of this paper is to shed light on the current landscape of genotyping practices, phenotyping practices and availability of essential vision rehabilitation management for inherited retinal diseases (IRD) in the Asia-Pacific (APAC) Region. METHODS: The 62-item questionnaire was distributed electronically via email. The questions covered five domains: (1) structure of the IRD service and registry/database; (2) genotyping practices; (3) genetic counselling; (4) deep phenotyping practices; (5) low-vision rehabilitation services. RESULTS: The survey was completed by 36 of 45 centres in twelve countries and regions in APAC. Among these centres, 42 % reported managing more than 1000 patients. Notably, 39 % of centres lack an IRD database or registry, and 44 % of centres have tested less than one-quarter of their IRD patients. The majority of centres (67 %) do not have genetic counsellors. While there was consistency in the imaging-based investigations, there was marked heterogeneity for functional testing using electrophysiology and formal perimetry. Only 34 % of centres confirmed the availability of access to low-vision assistive devices. CONCLUSIONS: This study reveals several critical gaps in managing IRDs in the APAC region. These include the lack of IRD database/registry in one-third of centres, a substantial proportion of patients remaining genetically undiagnosed, and limited availability of genetic counsellors. The findings also underscore a need to harmonise investigations for evaluating retinal function and identify areas for improvement in the provision of low-vision rehabilitation services.

Low vision aids provision in an urban setting in Germany between 2014 and 2017: a regional population based study with healthcare claims data.

PURPOSE: Little is known about the utilization of low vision services (LVS) in Germany. To understand which persons and how often these services would be utilized, this study aimed to investigate low vision aids (LVAs) provision in an urban setting and to describe user characteristics and trends in their characteristics. METHODS: A retrospective study based on a population-based healthcare claims database in Cologne (N = ~ 500,000), Germany. The study population comprised individuals, who were continuously insured at four large statutory health insurers and who redeemed a prescription for visual aids or aids for blindness between January 2014 and December 2017. We examined their socio-demographic and clinical characteristics. Trends in characteristics were examined with logistic and linear regression models over time. RESULTS: Out of ~ 500,000 persons, 781 unique individuals (~ 0.2%) redeemed an LVA prescription. They were mainly female (68.7%), 60 years or older (75.3%) and had macular degeneration (50.6%) and/or glaucoma (25.9%). In the working-age subgroup, 33.8% were employed. Visual aids were most often prescribed (74.1%) and of all types of LVAs, individuals most commonly redeemed a prescription for magnifiers (35.8%), screen readers (34.3%) and/or canes (17.1%). Of the entire study population, 75.4% received their prescription from an ophthalmologist, 5.3% from a general practitioner and 7.1% from other medical specialists. Significant trends in characteristics of individuals who redeemed an LVA prescription were not found. CONCLUSIONS: Between 2014 and 2017, 781 individuals in Cologne redeemed an LVA prescription. They had characteristics which mostly can be explained by the epidemiology of VI. Results indicate that individuals that redeemed LVAs have a magnification requirement of ≥ 1.5-fold and ≥ 6-fold. Furthermore, next to ophthalmologists, general practitioners and other medical specialists seem to play a role in LVA provision as well, which should be taken into account by policy makers when planning interventions for increasing LVS provision. Our findings provide a starting point to examine LVS provision in Germany.

A retrospective big data study using healthcare insurance claims to investigate the role of comorbidities in receiving low vision services.

Introduction: The aim was to examine the association between physical and mental comorbidity with receiving low vision services (LVS). Methods: A retrospective study based on Dutch claims data of health insurers was performed. We retrieved data (2015-2018) of patients (≥18 years) with eye diseases causing severe vision loss who received LVS at Dutch rehabilitation organizations in 2018 (target group) and patients who did not receive LVS, but who received ophthalmic medical specialist care for glaucoma, macular, diabetic retinal and/or retinal diseases in 2018 (reference group). For examining the association between the patients' comorbidities and receiving LVS, multivariable logistic regression was used. The relative quality of five different models was assessed with the Akaike Information Criterion (AIC). Results: The study population consisted of 574,262 patients, of which 8,766 in the target group and 565,496 in the reference group. Physical comorbidity was found in 83% and 14% had mental comorbidity. After adjustment for all assumed confounders, both physical and mental comorbidity remained significantly associated with receiving LVS. In the adjusted model, which also included both comorbidity variables, the best relative quality was found to describe the association between mental and physical comorbidity and receiving LVS. Conclusions: Mental comorbidity seemed to be independently associated with receiving LVS, implying that the odds for receiving a LVS referral are higher in patients who are vulnerable to mental comorbidity. Physical comorbidity was independently associated, however, the association with receiving LVS might not be that meaningful in terms of policy implications. Providing mental healthcare interventions for people with VI seems warranted.

Neurovisual rehabilitation in multiple sclerosis: Why a close integration of low-vision rehabilitation and neuropsychological rehabilitation may be effective for visual complaints.

OBJECTIVE: The quality of life of people with multiple sclerosis (MS) is often affected by visual complaints. A previous study suggested that visual complaints are not likely to be related to specific visual functions, but by a global decline of cognitive and visual functioning. In this study, we further explore this hypothesis, by investigating the relation between visual functions and global cognitive functioning, aiming to provide recommendations for rehabilitation for visual complaints. DESIGN: Cross-sectional study. SETTING: A rehabilitation centre for partially sighted and blind people and a MS centre at a university hospital. PARTICIPANTS: 102 people with MS. MAIN MEASURE: Correlations between assessments of visual functions (acuity, contrast sensitivity, visual field, smooth pursuit and saccades) and composite scores of a neuropsychological assessment (tests with a visual component and without a visual component). RESULTS: All composite scores correlated with visual acuity, contrast sensitivity and the sensitivity of the monocular field, but not with smooth pursuit and saccades. Similar patterns were found in various subgroups. Results showed that visual functions that related to visual complaints correlated with a diffuse decline of global cognitive functioning and that visual and cognitive functioning may decline concurrently in people with MS. CONCLUSIONS: Visual complaints may occur as a result of a diffuse decline of the integrity of a cerebral network involved in vision and cognition. People with MS with visual complaints may benefit from neurovisual rehabilitation, in which low-vision rehabilitation and neuropsychological rehabilitation are closely intertwined.

Innovative vision rehabilitation method for epiretinal membrane: Enhancing visual functions through Biofeedback Training.

Purpose: To report a case with epiretinal membrane (ERM) treated with audio-luminous Biofeedback training (BT) which resulted in improvement of monocular and binocular visual functions. Observations: We report a case of ERM with distance Best Corrected Visual Acuity (BCVA) of 20/40 in the right dominant eye, and 20/20 in the left eye. The main symptom was binocular blurry vision, and the patient did not desire to pursue posterior vitrectomy due to its risks. Using a 10-2 microperimetry test with the MAIA Microperimeter, it was possible to identify an inferior paracentral relative scotoma in the right eye with splitting fixation. The right eye was trained with five 20-minute sessions with Biofeedback training towards a trained retinal locus (TRL) with better retinal sensitivity and 1.46° superior and temporal to the current preferred retinal locus (PRL). After training, the patient reported total improvement of symptoms, and BCVA was 20/25 in the right eye and 20/20 in the left eye. Conclusions and importance: Biofeedback training is a valuable treatment with no known adverse effects that can be performed as an alternative to patients with ERM with no surgical indication and debilitating symptoms, or as a post-surgery adjuvant treatment.

Barriers to the Utilization of Low-Vision Rehabilitation Services among Over-50-Year-Old People in East and Southeast Asian Regions: A Scoping Review.

East and Southeast Asia (ESEA) are facing age-related eye health issues. Low-vision rehabilitation (LVR), which is a special rehabilitation service for individuals with vision impairment, is a promising solution for these health issues; however, poor accessibility to LVR services has been reported globally, including ESEA. Therefore, this scoping review aimed to summarize and understand the barriers to accessing LVR services in ESEA. In total, 20 articles were ultimately considered eligible for this scoping review after an electronic database search using MEDLINE (PubMed), Web of Science, Academic Search Ultimate (EBSCO), and Ichushi-Web (Japanese medical literature database), and an independent review by two reviewers. Twenty-one potential barrier factors were identified in the full-text review. Notably, age, education, economic status, "previous experience using eye care service", and "knowledge, information, and awareness" were the possible barrier factors that were examined for their association with LVR utilization, with supportive evidence in many eligible studies. We also identified research gaps relating to geographical and ethnic diversity, the scope of LVR services, and barriers among eligible articles. Therefore, by conducting further studies addressing the research gaps identified in this scoping review, these findings can be used to make LVR services more accessible to people in ESEA.

Role of eccentric viewing training with microperimeter for low vision rehabilitation in multifocal choroiditis: Case report.

A 21-year-old student with healed multifocal choroiditis involving the macula presented with difficulty in seeing distance and reading her college books. Based on microperimetr'y, the location of her preferred retinal locus and the position of new trained retinal locus (TRL) were identified. With multiple sessions of biofeedback training, her mean retinal sensitivity improved from 4.8 to 8.8 dl, distance vision from 3/60 to 6/36, and near vision from N32 to N8 in the right eye. At 6-month follow-up, her TRL position and visual acuity were found to be stable. This case report highlights that eccentric viewing training using microperimeter can significantly improve the efficiency of functional vision in patients with choroiditis.

Addressing Current Issues in Vision Rehabilitation as a Specialty Practice of Occupational Therapy.

The purpose of this paper was to examine the low number of occupational therapy practitioners in the United States that possessed specialty or advanced certification in low vision. The discussion explores possible reasons for this finding, including insufficient educational accreditation standards to prepare occupational therapy students to work with people with visual conditions, lack of clarity on the definition of low vision leading to misalignment with the profession's scope of practice, inconsistent requirements for advanced certification, scarcity of post-professional preparation programs, and other issues. We propose several solutions to prepare occupational therapy practitioners to meet the challenges and needs of people of all ages with visual impairments.

Retinitis Pigmentosa: Current Clinical Management and Emerging Therapies.

Retinitis pigmentosa (RP) comprises a group of inherited retinal dystrophies characterized by the degeneration of rod photoreceptors, followed by the degeneration of cone photoreceptors. As a result of photoreceptor degeneration, affected individuals experience gradual loss of visual function, with primary symptoms of progressive nyctalopia, constricted visual fields and, ultimately, central vision loss. The onset, severity and clinical course of RP shows great variability and unpredictability, with most patients already experiencing some degree of visual disability in childhood. While RP is currently untreatable for the majority of patients, significant efforts have been made in the development of genetic therapies, which offer new hope for treatment for patients affected by inherited retinal dystrophies. In this exciting era of emerging gene therapies, it remains imperative to continue supporting patients with RP using all available options to manage their condition. Patients with RP experience a wide variety of physical, mental and social-emotional difficulties during their lifetime, of which some require timely intervention. This review aims to familiarize readers with clinical management options that are currently available for patients with RP.

Visual and cognitive functioning among older adults with low vision before vision rehabilitation: A pilot study.

Introduction: The occurrence of age-related vision changes is inevitable. However, some of these changes can become pathological. Research indicates that vision and hearing loss is correlated with age-related cognitive decline, and with a higher risk of developing dementia due to Alzheimer's disease. Low vision rehabilitation could possibly be a protective factor against cognitive decline, as it provides the clients with compensatory strategies to overcome their visual deficits. Objectives and hypothesis: The aim of this pilot study was to assess correlations between visual and cognitive functions in older adults referred for low vision rehabilitation. We hypothesized that more severe impairment of visual acuity and contrast sensitivity would be correlated with more advanced levels of cognitive impairment. The second objective was to examine which of these correlations would remain significant once established variables that influence cognition are statistically removed (e.g., age, education). Methods: Thirty-eight older adults (age range: 66-97 years old) with a visual impairment (acuity <20/70) were recruited before the onset of their low vision rehabilitation. They underwent vision (reading acuity, reading speed, contrast sensitivity), hearing (audiogram, speech-in-noise perception) and cognitive (global cognition, memory, executive functions) testing, and demographic information was obtained. Results and discussion: Correlations among global cognition and visual aid use, memory and reading speed, memory and contrast sensitivity, memory, and visual aid use, and between executive functions and contrast sensitivity were significant. Correlations between contrast sensitivity and memory, as well as between global cognition and visual aid use remained significant after controlling for age and education. The present study is relevant to clinicians who are assessing the cognitive status of older adults, such as neuropsychologists, because it highlights the importance of considering low vision when administering neuropsychological tests, especially to persons who have not yet received rehabilitation for their visual impairment.

An Evaluation of a Clinic-Based Low Vision Device Lending Library.

As the efficacy of low vision service provision is facilitated by clients' access to and proper use of low vision devices, the objective of this study was to evaluate an outpatient clinic-based low vision device lending library program and the functional and psychosocial impact that device use had upon clients. Twenty individuals borrowed portable video magnifiers during the study period. Line items from the Revised-Self-Report Assessment of Functional Visual Performance and the Reading Behavior Inventory were analyzed before and after device loan at two months. The Psychosocial Impact of Assistive Devices Scale-10 and a semi-structured interview were also completed at two months. Reported improvements in reading performance and satisfaction levels on the Reading Behavior Inventory were significant (p<.001). The Revised-Self-Report Assessment of Functional Visual Performance indicated improved independence in reading medications, bills and labels. Higher scores in happiness, independence, sense of control and adaptability on the Psychosocial Impact of Assistive Devices Scale-10 indicated device retention at two months. Qualitative themes included improved independence, time needed to acclimate to the device, personal appraisal impacting motivation and challenges specific to low vision. This article provides occupational therapists a model to facilitate access, person-device fit and successful use of low vision devices to promote therapy outcomes.

Impact of Lighting Assessment and Optimization on Participation and Quality of Life in Individuals with Vision Loss.

This pilot study was designed to investigate the effects of a holistic lighting intervention on the quality of life for individuals with low vision. Sixty participants (44 women; median age 69 years) with visual impairment received lighting interventions, including a home visit and consultation in a lighting lab. Assisted by low vision consultants, participants evaluated their performance using the Canadian Occupational Performance Measure (COPM) before and after the intervention. Improvements in visual functioning and quality of life were evaluated using the 39-item National Eye Institute Visual Function Questionnaire (NEI VFQ-39), the Groffman Visual Tracing Test, and the Farnsworth Dichotomous Test (D15). Following the lighting intervention, scores improved for all activities in the COPM (p < 0.01), for near activities and vision-specific role difficulties in the VFQ-39 (p < 0.05), and overall in the D15 test (p < 0.05). These results suggest the intervention provided an effective method for improving the participants' quality of life and performance.

Low Vision Referral Patterns in Intermediate Age-Related Macular Degeneration.

PURPOSE: Sparse data exist regarding low vision (LV) services referral patterns. We retrospectively examined our institution's intermediate age-related macular degeneration (iAMD) patients to determine factors influencing referral. METHODS: We compared visual acuity (VA) and Visual Function Questionnaire (VFQ-25) composite and subscale scores for referred and non-referred iAMD patients. VA was collected at time of referral or most recent visit, and VFQ-25 was taken upon enrollment into the registry. RESULTS: Thirty-six (15.5%) of the 232 iAMD patients were referred to LV. Referred patients were more likely to have older age, worse VA in both eyes, and lower VFQ-25 scores. Univariate analysis of VFQ-25 subscales demonstrated worse scores in general vision, near, distance, mental health, role limitations, dependency, and driving. Multivariable analysis revealed lower scores in general health, general vision, and driving. Forty-eight percent of non-referred patients had VA or VFQ-25 composite scores at least as poor as the median for referred patients. Two-thirds of patients who were not referred had no discernable obstacle to referral. CONCLUSIONS: Our institution refers patients with worse objective and functioning vision, but more patients may benefit from referral. Future studies should identify metrics to prompt referral and evaluate this approach.

A Study of Environmental Factors in Low Vision Rehabilitation.

Healthcare has the past decades shifted from a narrow medical perspective to a more holistic, biopsychosocial perspective. Disability understood as a contextual condition constituted by the relation of the individual to their social and physical context. The disability model of the International Classification of Functions (ICF) contextualizes activity, participation, body functions and structure by including environmental and personal factors. However, illustrated by the consideration of the environmental factors as a neutral dimension, the dynamic interrelation of the individual parts of the system is rather unchartered. In 2017-2019, a lighting assessment was developed and tested on 60 participants in low vision rehabilitation. An action research project accompanied the pilot study from 2018. Ethnographic participatory observations of the low vision consultants in 15 consultations, semi-structured interviews, and a document analysis of the project material of the pilot project has been analyzed using the theoretical framework of science and technology studies. Mapping the physical environment showed a range of factors from spatial organization to luminaires and light bulbs. Moreover, in relation to specific activities, relevant factors were identified and assessed, and in the intervention adjusted to relevant personal and social factors. Identifying overlapping personal, environmental, and professional spheres illustrates the complexity of practicing rehabilitation in people's everyday lives. Acknowledging and coordinating different versions of lighting enabled low vision consultants to work across these spheres relationally. ICF was embedded in the practice of low vision consultants as a frame of reference, however, implementing this framework occurred through an assemblage of tools from different fields. The focus on lighting as an active element in low vision rehabilitation demonstrated a way to work across the personal and environmental to reduce the gap that caused disability. In everyday life, the physical environment was pivotal in the person-environment relationship and in enabling or disabling the individual. However, the physical environment was also key to the rehabilitation process, facilitating the individual's learning and change processes and reconfiguring their understanding and use of the environment. Consequently, the physical environment was not a neutral background to the other factors but rather enabling the rehabilitation and recovery processes.

Comparison of Reading Test Parameters from the Print and Tablet Application Forms of the Minnesota Low Vision Reading Test.

Objectives: To compare reading parameters measured with the Turkish version of the Minnesota Low Vision Reading Test (MNREAD-TR) printed acuity chart and the tablet application version of the same test for both normally sighted and low-vision individuals. Materials and Methods: A total of 116 individuals (92 normally sighted and 24 low-vision) were included in the study. All participants were tested with both the print version of the MNREAD-TR chart (method 1) and the tablet application version (method 2). Reading acuity (RA), critical print size (CPS), maximum reading speed (MRS), and reading accessibility index (ACC) were compared statistically. Results: No statistically significant difference was found in RA and CPS between the two methods for the normally sighted individuals (p=0.083 and p=0.075, respectively). There was no statistically significant difference in RA and ACC between the two methods for the patients with low vision (p=0.159 and p=0.103, respectively). The mean MRS was 233.1±34.7 words per minute (wpm) with method 1 and 169.3±23.4 wpm with method 2 in the normally sighted group (p<0.001) and 93.2±50.2 wpm with method 1 and 68.2±34.7 wpm with method 2 in the low-vision group (p<0.001). Conclusion: In our study, it was found that the parameters RA and CPS in the normally sighted individuals and RA and ACC in the low vision individuals provided similar results in both forms of the MNREAD. The tablet application method can be preferred to eliminate evaluators' bias of scoring the printed chart. In addition, applications have other advantages such as being faster and more practical and providing automatic analysis of parameters, especially in low-vision rehabilitation.

Fixation Stability and Preferred Retinal Locus in Advanced Age-Related Macular Degeneration

Objectives: To evaluate fixation stability and characteristics of the preferred retinal locus (PRL) in patients with advanced age-related macular degeneration (AMD). Materials and Methods: Sixty-three eyes of 63 patients with AMD who presented to the low vision unit were included in this prospective study. Sociodemographic characteristics, eye examination findings, and reading performance results with the Minnesota Low Vision Reading test were evaluated. Microperimetry was used to evaluate fixation stability and PRL characteristics. Results: There was unstable fixation in 68% of the eyes, relative stable fixation in 27%, and stable fixation in 5%. The mean PRL-foveal distance was 5.15°±3.31° (range 0.75°-14.2°). PRL-foveal distance was greater in cases with unstable fixation than cases with stable fixation (p=0.023). Distance of the PRL from the lesion margin was not associated with absolute scotoma size or fixation stability (p=0.315, p=0.095, respectively). PRLs were most frequently located in the nasal quadrant (31%), followed by the superior quadrant (26%) of the retina. There was no significant relationship between PRL location and fixation stability (p=0.088). Fixation stability was significantly associated with reading speed (p=0.003). Conclusion: In advanced AMD, PRL-foveal distance is an important factor in fixation stability. Knowing the factors that affect fixation stability may be important in determining low vision rehabilitation strategies for these patients because of the strong association between fixation stability and reading speed.

Short-Term Clinical Results of Preferred Retinal Locus Training

Objectives: This study evaluated acoustic biofeedback training using microperimetry in patients with foveal scars and an eligible retinal locus for better fixation. Materials and Methods: A total of 29 eligible patients were enrolled in the study. The acoustic biofeedback training module in the MAIA (Macular Integrity Assessment, CenterVue®, Italy) microperimeter was used for training. To determine the treatment efficacy, the following variables were compared before and after testing: best corrected visual acuity (BCVA); MAIA microperimeter full threshold 4-2 test parameters of average threshold value, fixation parameters P1 and P2, and bivariate contour ellipse area (BCEA) for 63% and 95% of fixation points; contrast sensitivity (CSV 1000E Contrast Sensitivity Test); reading speed using the Minnesota Low-Vision Reading Test (MNREAD reading chart); and quality of life (NEI-VFQ-25). In addition, fixation stability parameters were recorded during each session. Results: The study group consisted of 29 patients with a mean age of 68.72±8.34 years. Median BCVA was initially 0.8 (0.2-1.6) logMAR and was 0.8 (0.1-1.6) logMAR after 8 weeks of preferred retinal locus training (p=0.003). The fixation stability parameter P1 improved from a mean of 21.28±3.08% to 32.69±3.69% (p=0.001) while mean P2 improved from 52.79±4.53% to 68.31±3.89% (p=0.001). Mean BCEA 63% decreased from 16.11±2.27°2 to 13.34±2.26°2 (p=0.127) and mean BCEA 95% decreased from 45.87±6.72°2 to 40.01±6.78°2 (p=0.247) after training. Binocular reading speed was 38.28±6.25 words per minute (wpm) before training and 45.34±7.35 wpm after training (p<0.001). Statistically significant improvement was observed in contrast sensitivity and quality of life questionnaire scores after training. Conclusion: Beginning with the fifth session, biofeedback training for a new trained retinal locus improved average sensitivity, fixation stability, reading speed, contrast sensitivity, and quality of life in patients with macular scarring.

An Overview of Preferred Retinal Locus and Its Application in Biofeedback Training for Low-Vision Rehabilitation.

Central vision loss (CVL) caused by macular damage generally disables common daily tasks, which cannot be reversed by present treatments. Fortunately, it has been found that biofeedback training by inducing or reinforcing preferred retinal locus (PRL) as an eccentric fixation reference contributes to the improvement of visual performance in patients with CVL. However, the clinical application is still under controversy due to poor knowledge of its fundamental and inconsistent practical standards. This article aims to summarize the possible rationale for the development, location, re-location and evaluating indicators of PRL, and the general apparatus, protocol, and outcome of biofeedback PRL training.

Universal Design and Low-Vision Rehabilitation: The Case for a Holistic Lighting Assessment.

Among various approaches to handling friction between (dis)abilities and the built environment, universal design (UD) has emerged as an interdisciplinary field for research and practice. However, while the literature denotes UD as a design concept, practice, and strategy for rehabilitation, its true impact is still largely unknown. To explore the rehabilitative potential of UD and determine how to evaluate its impact, this paper seeks to turn the tables. It investigates a case regarding low-vision rehabilitation, in which a group of consultants developed a holistic lighting assessment (HLA) that embraced the social and the physical contexts of the visually impaired. The lighting assessment was performed using participant observations from 15 consultations, document analysis, and interviews with the low-vision consultants. Based on an actor-network theory (ANT) approach, the analysis reveals the contextual knowledge of participants, environments, and the interaction between them. The combination of quantitative and qualitative methods in HLA enabled a range of different understandings of light: as a quantitative measure, as an individually perceived aspect of the home environment, as something that enables or disables daily activities, and as a social factor of great importance for social practices. While traditional lighting assessments generally resemble the accessibility approach, with its measures of visual acuity translated into recommendations for an overall lux value, the holistic approach more closely resembles the UD methodology. One finding of this paper is that the concepts of rehabilitation and UD are committed to slightly different levels of abstraction. Rehabilitation focuses on specific individuals and specific environments, with patient rehabilitation as the main goal. UD focuses on user groups and design principles, with design and architectural solutions as the main objectives. While the concepts of UD and HLA represent different fields and different levels of abstraction, the two approaches can enhance both respective practices and theoretical frames.