A Modified Protocol for the Assessment of Visual Function in Patients with Retinitis Pigmentosa

Lodha, Nidhi; Westall, Carol A.; Brent, Michael H.; Abdolell, Mohamed; Hèon, Elise

doi:10.1007/978-1-4615-0067-4_7

Cited by 15 publications

(3 citation statements)

References 7 publications

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“…There are currently nine RP-specific patient-reported outcome (PRO) instruments. 31,[34][35][36][37][38][39][40][41] The motive behind our endeavor to develop a comprehensive RP-specific PRO is that none of the existing PRO instrument has undergone comprehensive validation in this disease group and their content coverage is limited to measuring only a few QoL domains (predominantly mobility). [42][43][44] Lack of an appropriate RP-specific PRO measure restricts the understanding of the full impact of RP on QoL.…”

Section: Discussionmentioning

confidence: 99%

Seeing through their eyes: lived experiences of people with retinitis pigmentosa

Senthil

Khadka

Pesudovs

2017

Eye

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PurposeRetinitis pigmentosa (RP) is the most common retinal degeneration causing blindness. Although their clinical problems are amenable for the clinical diagnosis, their day-to-day problems for having to live with the disease are mostly unexplored. This study aims to explore and understand the issues and impact of people with RP on quality of life (QoL).MethodsA qualitative research methodology to facilitate the understanding of the experiences of people with RP was carried out. Data were collected through audio-recorded semi-structured interviews. Thematic analysis occurred through the process of line-by-line coding, aggregation, and theme development using the NVivo-10 software.ResultsTwenty-three interviews were conducted (mean age=56 years; females, 14). We identified five major QoL themes: (1) struggle to perform important day-to-day tasks; (2) concerns about disease progression, disease outcome and personal safety; (3) facing a lot of emotional and psychological challenges; (4) experiencing a myriad of visual symptoms; and (5) adopting different strategies to cope and manage stressful circumstances. Difficulty in performing important day-to-day tasks was the most prominent QoL issue among these people. Their major concerns were going blind and uncertainties about their future. They face a lot of emotional and psychological challenges to adapt to the physiological stress associated with the progressive vision loss. However, they adopt several coping strategies to manage the stressful circumstances.ConclusionsPeople with RP experience a myriad of QoL issues. Despite all the hardship, they remain optimistic and learn to accept their eye condition and move on in life.

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Section: Discussionmentioning

confidence: 99%

Seeing through their eyes: lived experiences of people with retinitis pigmentosa

Senthil

Khadka

Pesudovs

2017

Eye

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“…Prem Senthil and colleagues (2017a) conducted a systematic review and quality evaluation of PROMs for retinal diseases that includes publications up to 2014. The authors identified one ophthalmic, non-disease-specific PROM, the NEI-VFQ, and 11 IRD-specific PROMs (nine for RP, one for Stargardt disease, and one for congenital stationary night blindness), including the following: MDQ (Geruschat et al 1998), the IMQ (Turano et al 1999), FEQ (Kennedy et al 1977, PVFQ (Lodha et al 2003), EDTQ (Lowe and Drasado 1992), V-ADL (Somani et al 2006), ADVQ (Szlyk et al 1998), DTPQ (Szlyk et al 2001), NVQ-39 (Bijveld et al 2013), VDQ (Sumi et al 2000), and SMDVQ (Miedziak et al 2000). These diseasespecific instruments cover only one or two domains across mobility, health concerns, and activity limitations, while the NEI-VFQ (Mangione et al 1998a) covers activity limitations, emotional well-being, social participation, and ocular comfort (Table 1).…”

Section: Ird Proms Developed Prior To the 2009 Fda Guidancementioning

confidence: 99%

Beyond the NEI-VFQ: Recent Experience in the Development and Utilization of Patient-Reported Outcomes for Inherited Retinal Diseases

Durham

Banhazi

Patalano

et al. 2022

Cold Spring Harb Perspect Med

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“…117 Particularly for RCD, Szlyk et al have developed questionnaires that showed strong correlation with BCVA, CS and VF. 118,119 The Vision Function Scale-plus (19 items) survey, initially developed for cataract, has also provided promising results in RCD. 120 Recently, the Michigan Retinal Degeneration Questionnaire was also validated as a PRO for patients with IRD, employing 59 items in 7 domains.…”

Section: Patient-reported Outcome Measuresmentioning

confidence: 99%

Functional evaluation in inherited retinal disease

et al. 2021

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Functional assessments are a fundamental part of the clinical evaluation of patients with inherited retinal diseases (IRDs). Their importance and impact have become increasingly notable, given the significant breadth and number of clinical trials and studies investigating multiple avenues of intervention across a wide range of IRDs, including gene, pharmacological and cellular therapies. Moreover, the fact that many clinical trials are reporting improvements in vision, rather than the previously anticipated structural stability/slowing of degeneration, makes functional evaluation of primary relevance. In this review, we will describe a range of methods employed to characterise retinal function and functional vision, beginning with tests variably included in the clinic, such as visual acuity, electrophysiological assessment and colour discrimination, and then discussing assessments often reserved for clinical trials/research studies such as photoaversion testing, full-field static perimetry and microperimetry, and vision-guided mobility testing; addressing perimetry in greatest detail, given it is commonly a primary outcome metric. We will focus on how these tests can help diagnose and monitor particular genotypes, also noting their limitations/challenges and exploring analytical methodologies for better exploiting functional measurements, as well as how they facilitate patient inclusion and stratification in clinical trials and serve as outcome measures.

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A Modified Protocol for the Assessment of Visual Function in Patients with Retinitis Pigmentosa

Cited by 15 publications

References 7 publications

Seeing through their eyes: lived experiences of people with retinitis pigmentosa

Seeing through their eyes: lived experiences of people with retinitis pigmentosa

Beyond the NEI-VFQ: Recent Experience in the Development and Utilization of Patient-Reported Outcomes for Inherited Retinal Diseases

Functional evaluation in inherited retinal disease

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