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Principal Investigator: Kanishka Thiran Jayasundera
Organization: UNIVERSITY OF MICHIGAN AT ANN ARBOR
Fiscal Year: 2024
Award: $695,936
Funding agency: National Eye Institute
Abstract
The distinct phenotypes making up inherited retinal degenerations (IRDs) affect all forms of functional vision,
and therefore, provide an illustrative disease model for low vision and blindness. Usual care for individuals with
IRDs consists of low vision rehabilitation (LVR). Patients experience variable effectiveness with LVR that is
often accompanied by elevations in depression and anxiety, which can complicate the delivery, impact, and
efficacy of LVR. There remains a critical need to develop an individualized treatment approach for patients with
IRDs addressing vision related anxiety. The Principal Investigator has created IRD-specific, psychometrically
validated, patient reported outcome (PRO) measures that capture and quantify specific disabilities of patients
in categories of physiologically distinct forms of visual dysfunction and distress that are seen in variable
phenotypic expressions of IRDs. Our goal in this grant application is to quantify the difficulties, limitations, and
distress experienced by the heterogeneous expressions of IRDs and intervene with LVR and psychotherapy.
Building on our earlier work, we propose to assess the impact of LVR coupled with a well validated cognitive
behavior therapy, called Emotion Regulation Therapy (ERT), to enhance functioning and resolve elevations in
depression and anxiety. The proposed research relates closely with the National Eye Institute’s Vision for the
Future, where standards of care must come to emphasize the individual quality of life—with a particular focus
on the patient perspective and encompassing blindness rehabilitation and brain plasticity, assistive devices
and independent living, while also addressing depression in patients coping with vision loss. We propose to
quantify levels of vision-related difficulties, limitations, and distress detected by both the Michigan Retinal
Degeneration Questionnaire (MRDQ) and the Michigan Vision-related Anxiety Questionnaire (MVAQ) in a
cross-sectional study of 600 patients with varying phenotypic manifestations of IRDs. We will quantify
longitudinal change in vision-related and emotional difficulties, limitations, and impairment by intervening with
LVR and ERT applied by targeting the quantified functional deficits detected by MRDQ and MVAQ in patients
with varying phenotypic manifestations of IRDs, by conducting a three-arm trial in 180 IRD patients comparing
LVR in settings of low and high vision-related anxiety, and ERT given to individuals with high vision-related
anxiety to determine the comparative efficacy signal from IRD validated PROs. We see the current work as a
crucial first step to set the stage for a multicenter trial in provision of LVR and ERT for vision-related distress,
and thereby improving the quality of life of patients with progressive visual impairment due to genetic etiology.
This program of research may also offer a blueprint for tailoring and optimizing care for visually impaired
patients well beyond IRDs.
Terms: <3-arm randomized trial><3-arm trial><Address><Affect><Age><Alcohol Chemical Class><Alcohols><Anxiety><Applications Grants><Assistive Technology><Behavior><Biological><Biological Response Modifier Therapy><Biological Therapy><Blindness><Brain><Brain Nervous System><Caring><Categories><Cell Communication and Signaling><Cell Signaling><Chronic><Clinical><Clinical Research><Clinical Study><Cognition Therapy><Cognitive Psychotherapy><Cognitive Therapy><Cognitive treatment><Cone><Consumption><Coupled><Cross Sectional Analysis><Cross-Sectional Analyses><Cross-Sectional Studies><Cross-Sectional Survey><DNA Therapy><Diminished Vision><Disease><Disease Frequency Surveys><Disease Progression><Disorder><Distress><Drugs><Dysfunction><Effectiveness><Emotional><Emotional well being><Encephalon><Feels well><Functional disorder><Future><Gene Transfer Clinical><Genetic Intervention><Genetic Predisposition><Genetic Predisposition to Disease><Genetic Susceptibility><Genetic propensity><Goals><Grant Proposals><Health><Hereditary><History><Impairment><Independent Living><Individual><Inherited><Inherited Predisposition><Inherited Susceptibility><Intracellular Communication and Signaling><Legal Blindness><Life><Link><Low Vision><Measurable><Measures><Medical Care Costs><Medical Rehabilitation><Medication><Mental Depression><Methodology><Michigan><Multi-center trial><Multicenter Trials><National Eye Institute><Normal mental condition><Normal mental state><Normal psyche><Outcome Measure><Partial Sight><Patient Outcomes Assessments><Patient Reported Measures><Patient Reported Outcomes><Patients><Peripheral><Personalized medical approach><Persons><Pharmaceutical Preparations><Phenotype><Physiologic><Physiological><Physiopathology><Pigmentary Retinopathy><Principal Investigator><Provider><Psychological Well Being><Psychometrics><Psychotherapy><QOL><Quality of life><Questionnaires><Randomized><Recording of previous events><Reduced Vision><Rehabilitation><Rehabilitation therapy><Research><Retina><Retinal Degeneration><Retinal Diseases><Retinal Disorder><Retinitis Pigmentosa><Rod><Self-Help Devices><Sense of well-being><Sight><Signal Transduction><Signal Transduction Systems><Signaling><Subgroup><Subnormal Vision><Tapetoretinal Degeneration><Testing><Therapeutic><Unhealthy Diet><Vision><Visual Acuity><Visual Fields><Visual disability><Visual impairment><Visually disabled><Well in self><Work><ages><assisted device><assistive device><biologic><biological signal transduction><biological therapeutic><biological treatment><biologically based therapeutics><biotherapeutics><biotherapy><birth complications><care as usual><cognitive behavior intervention><cognitive behavior modification><cognitive behavior therapy><cognitive behavioral intervention><cognitive behavioral modification><cognitive behavioral therapy><cognitive behavioral treatment><comparable efficacy><comparative><comparative efficacy><compare efficacy><coping><customized therapy><customized treatment><degenerative retina diseases><delivery complications><depression><diet and exercise><disability><disease model><disorder model><drug/agent><efficacious therapy><efficacious treatment><emotion regulation><emotional distress><emotional regulation><emotional wellbeing><emotional wellness><experience><eye field><feeling distress><feeling upset><functional gain><functional improvement><gene repair therapy><gene therapy><gene-based therapy><genetic etiology><genetic mechanism of disease><genetic therapy><genetic vulnerability><genetically predisposed><genomic therapy><histories><improve function><improved><improved functional outcomes><individual patient><individualized approach><individualized medicine><individualized patient treatment><individualized therapeutic strategy><individualized therapy><individualized treatment><inherited retinal degeneration><intervention cost><legally blind><measurable outcome><medical costs><medical expenses><meeting><meetings><mental well-being><mental wellbeing><mental wellness><new drug treatments><new drugs><new pharmacological therapeutic><new therapeutics><new therapy><next generation therapeutics><novel drug treatments><novel drugs><novel pharmaco-therapeutic><novel pharmacological therapeutic><novel therapeutics><novel therapy><open label><open label study><outcome measurement><pathophysiology><patient specific therapies><patient specific treatment><personalized approach><poor diet><precision approach><programs><psychologic><psychological><psychological wellbeing><psychological wellness><psychosocial><randomisation><randomization><randomly assigned><rehab therapy><rehabilitative><rehabilitative therapy><retina degeneration><retina disease><retina disorder><retinal degenerative><retinal degenerative diseases><retinopathy><rod and cone dystrophy><rod-cone dystrophy><self wellness><sense of wellbeing><sex><shared decision making><stem><success><tailored approach><tailored medical treatment><tailored therapy><tailored treatment><three-arm randomized trial><three-arm trial><treatment as usual><unique treatment><usual care><vision disability><vision impairment><vision loss><vision rehabilitation><visual dysfunction><visual function><visual loss><visually impaired>