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Visual training and emotional state of people with retinitis pigmentosa

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Abstract

The purpose of the study was to improve the visual functioning of people with restriction in contrast sensitivity (CS), such as retinitis pigmentosa (RP), by means of a visual training program. Twenty-six volunteers with RP participated, distributed in two groups: 15 who made up the experimental group (who received the training program) and 11 who participated as a control group (without training). Participants were evaluated before beginning training, on completion, and 3 mo following completion for CS with the Pelli-Robson Contrast Sensitivity (P&R) test, visual functioning with the Visual Function Questionnaire (VFQ), and in emotional state with the Beck Depression Inventory (BDI). The training program is based on software that generates luminous stimuli of varying duration and intensity and registers the stimuli perceived by the subject. The outcomes showed significant differences posttraining in the experimental group in depression (F1,14 = 5.42; p < 0.04), VFQ (Z = -2.27; p < 0.02), and P&R in the right eye (Z = -1.99; p < 0.046) and left eye (Z = -2.30; p < 0.02) but not in binocular (Z = -0.96; p < 0.34). The outcomes showed that the experimental group made significant progress in all variables and these effects remained after 3 mo, which suggests that the program could be a helpful addition to RP rehabilitation and help mitigate the damage.
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JRRD Volume 50, Number 8, 2013
Pages 1157–1168
Visual training and emotional state of people with retinitis pigmentosa
Helena Chacón-López, PhD;1* Francisco J. Pelayo, PhD;2 María D. López-Justicia, PhD;1 Christian A. Morillas,
PhD;2 Raquel Ureña, MSc;2 Antonio Chacón-Medina, PhD;3 Begoña Pino, PhD2
1Department of Developmental and Educational Psychology, Faculty of Educational Sciences, 2Department of Computer
Architecture and Technology, High Technical School of Informatics and Telecommunications, and 3Department of Didactics
and School Organization, Faculty of Educational Sciences, University of Granada, Spain
Abstract—The purpose of the study was to improve the visual
functioning of people with restriction in contrast sensitivity (CS),
such as retinitis pigmentosa (RP), by means of a visual training
program. Twenty-six volunteers with RP participated, distributed
in two groups: 15 who made up the experimental group (who
received the training program) and 11 who participated as a con-
trol group (without training). Participants were evaluated before
beginning training, on completion, and 3 mo following comple-
tion for CS with the Pelli-Robson Contrast Sensitivity (P&R) test,
visual functioning with the Visual Function Questionnaire
(VFQ), and in emotional state with the Beck Depression Inven-
tory (BDI). The training program is based on software that gener-
ates luminous stimuli of varying duration and intensity and
registers the stimuli perceived by the subject. The outcomes
showed significant differences posttraining in the experimental
group in depression (F1,14 = 5.42; p < 0.04), VFQ (Z = 2.27; p <
0.02), and P&R in the right eye (Z = 1.99; p < 0.046) and left
eye (Z = 2.30; p < 0.02) but not in binocular (Z = 0.96; p <
0.34). The outcomes showed that the experimental group made
significant progress in all variables and these effects remained
after 3 mo, which suggests that the program could be a helpful
addition to RP rehabilitation and help mitigate the damage.
Key words: adults, contrast sensitivity, depression, emotional
state, rehabilitation, retinal degenerative diseases, retinitis pig-
mentosa, visual functioning, visual performance, visual training.
INTRODUCTION
The degree of autonomy in personal and social per-
formance is assessed by efficiency in carrying out vari-
ous daily tasks. In conducting these activities, people
with low vision, such as those with retinitis pigmentosa
(RP), can have serious difficulties, which may adversely
affect their social and personal welfare.
RP belongs to a group of degenerative diseases of the
retina characterized by a progressive loss of vision that
can lead to blindness. This disorder specifically implies
night blindness, peripheral restrictions and/or scotomas
(scattered spots in which vision is absent or deficient) in
the visual field (VF) [1–3], frequent reduction of visual
acuity (VA) [1–4], and alterations in contrast sensitivity
(CS) [5–6], showing a significant reduction of CS in a
wide range of spatial frequencies. These symptoms affect
daily visual functioning, lifestyle, and social develop-
ment and influence the emotional state [7–8], as well as
the visual-perceptual state [9]; therefore, paying attention
to these aspects is fundamental in our study.
Abbreviations: BDI = Beck Depression Inventory, CS = con-
trast sensitivity, HMD = head-mounted display, MMSE =
Mini-Mental State Examination, P&R = Pelli-Robson Contrast
Sensitivity, RP = retinitis pigmentosa, SD = standard deviation,
VA = visual acuity, VF = visual field, VFQ = Visual Function
Questionnaire.
*Address all correspondence to Helena Chacón-López, PhD;
Department of Developmental and Educational Psychology,
Faculty of Educational Sciences, University of Granada,
Campus Universitario de La Cartuja, s/n, 18071–Granada,
Spain; +34-958-243-975. Email: helenachacon@ugr.es
http://dx.doi.org/10.1682/JRRD.2012.06.0113
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Until now, there have been no medical solutions or
pharmacological treatments for this pathology or for
other degenerative visual problems.
Contrast Sensitivity
One of the parameters used to assess visual function-
ing, which significantly influences the performance of
daily living activities, is CS [10–12], the ability to dis-
criminate between shades of gray [13]. Assessment of CS
is useful for evaluating the effects of some visual deficits
because a person may have good VA but diminished CS
and therefore may experience some difficulties in certain
real-life situations [6–11]. This is the case of some people
with RP.
Improvements in CS can be obtained in subjects with
normal vision by training them with challenging tasks that
involve vision [14]. This training is of great interest to peo-
ple with RP since the loss of CS is one of the main difficul-
ties faced [15] and significantly affects their ability to carry
out daily living tasks [11].
Visual Functioning and Retinitis Pigmentosa
Various studies have indicated that differences in visual
functioning in daily living activities are significant among
people with visual impairments such as RP; therefore, their
functional performance is considered in their assessment
[11,16–18]. It has even been stressed that the outcomes of
this assessment are as valuable and complete as the data
provided by ophthalmological tests [11–18]. Procedures to
evaluate visual functioning include lists or self-report tools
used in the tasks in order to record easily observable behav-
iors. These behaviors make it possible to study visual func-
tioning in daily living activities by measuring visual and
psychosocial aspects. Other studies have shown that daily
visual functioning can be affected by negative emotional
states such as depression [11–18], although depression is
also affected by daily living activities.
Emotional State and Retinitis Pigmentosa
Numerous authors have noted that adults who develop
visual restriction have a greater risk of depression [18–21].
Furthermore, depression constitutes a major source of
functional disability, and the consequences in adults also
affect visual rehabilitation [22–23]. A previous research
study showed that emotional adjustment worsens over
time [24].
Depression in people with RP is frequent [7,11,18].
The prevalence has been estimated at 25.7 percent (while
in the general population it amounts to 10%). Nemshick
et al. showed that the period of greatest crisis or stress
occurs during or immediately following diagnosis [25],
and López-Justicia et al. recommended evaluating the
depression variable just after the diagnosis of the disease
and again over time [7].
Visual Stimulation and Retinitis Pigmentosa
Some years ago, procedures for assessment and for
perceptual and visual training began to be applied to
adults with visual deficiencies (including RP) in order to
improve their visual functioning and performance in cer-
tain situations of everyday life [16–17]. These studies
concluded that practice and training could improve the
functional use of residual vision, although it was also
observed that people who were more actively involved in
training made better use of their residual vision [16].
Thus, both practice and motivation seem to be decisive
factors in improving the use of residual vision.
Visual stimulation and training are highly relevant in
interventions with people affected by low vision, even
when the level of remaining vision is very low. This type
of training has been proven to be effective in enhancing
their quality of life regardless of the patient’s age [26–
27]. The aim of visual stimulation and visual training is
to train affected people in using their visual functions so
that they achieve both a quantitative and a qualitative
enhancement in social functioning [26–27]. Likewise,
visual stimulation and training allow affected people to
use their remaining VA ability [28]; this aspect should
not be disregarded, because it has been confirmed that a
large number of RP-affected individuals maintain some
VA to the end of their lives, even if it is minimal [29].
This fact is undoubtedly valuable for planning education
and rehabilitation activities [30].
On the other hand, methods for training the visual sys-
tem in people with VF deficit have been developed using
computer programs to stimulate, through luminous points,
the edge of the region of the VF situated between a visu-
ally intact area and a damaged area [31], resulting in a sig-
nificant increase in visual function. For a long time, it was
believed that these problems could not be treated since it
was thought that vision required a high degree of neuronal
organization produced during the early stages of life. Nev-
ertheless, despite this specific period for organization, a
considerable degree of plasticity has been documented in
the adult visual system damaged by a lesion: a reorganiza-
tion of the neuron receptive field occurs following lesions
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CHACÓN-LÓPÉZ et al. Visual training, emotional state, and RP
in the retina or cortex, with cortical neurons with receptive
fields associated with the damaged area of the retina
acquiring new fields in adjacent areas [32]. Some of the
criticisms of these procedures have pointed out that the
increase in VF produced could be explained by movement
of the eyes toward the affected area in an attempt to com-
pensate for the deficit in VF [33–34].
Purpose of Study
The final purpose of this study was to improve the
CS of people with limited CS, such as those with RP, by
means of a visual training program.
Assuming as a starting hypothesis that it is possible
to improve the CS of people with difficulties in this func-
tion, we have applied a training program based on the
stimulation of VF with different levels of contrast and
using covert direction of attention. It is known that covert
attention, or orientation of the attention toward visual
stimuli that appear in areas other than the fixation point,
improves the response of the visual system [35]. We also
hypothesized that visual training would lead to an
improvement both in functional vision in daily living
activities (specifically CS) and in emotional state.
METHODS
Participants
A meeting was organized for people with RP, mem-
bers of the RP Association of Andalusia (who participated
in research coordinated by two of the authors), to inform
them about the objectives of the study and the activities
involved. A sample of 26 volunteers with RP was then
selected from all those who agreed to participate and who
fulfilled the inclusion criteria: having bilateral VF loss
ranging between 5° and 40° (binocular), having VA rang-
ing between 20/20 (0.0 logMAR unit) and 20/200 (1.0
logMAR unit) in the better eye, and presenting no cogni-
tive impairment (score greater than 24 measured with
Mini-Mental State Examination [MMSE]). These partici-
pants were asked to provide an ophthalmological report,
including the diagnosis and the degree of VA and VF.
Their VA was measured with Snellen acuity charts, and
their monocular and binocular kinetic VF was measured
with a Goldmann Perimeter (V4, III4, I4, II2). Later, they
were randomly assigned (considering VA and VF) into
two groups: 15 (13 women and 2 men) who made up the
experimental group (who received the training program),
and 11 (8 women and 3 men) who participated as a control
group (without training program). There were no signifi-
cant differences between the groups in VA (right eye: χ2 =
2.36, p = 0.50; left eye: χ2 = 6.36, p = 0.10; binocular:
χ2 = 6.36, p = 0.10) or in VF (χ2 = 11.46, p > 0.99). The
participants were evaluated before starting the training
period (pre-), on completion (post-) and 3 mo following
completion (post 3 mo). At each evaluation, participants
completed the following tests: the Pelli-Robson Contrast
Sensitivity (P&R) test, the Visual Function Questionnaire
(VFQ), and the Beck Depression Inventory (BDI). VA
was measured only before beginning training and on com-
pletion. The participants in the control group were
informed that they could undertake the training program
in a second phase of the study.
Table 1 shows the demographic characteristics of the
participants by groups. Four had associated incipient cata-
racts, one had mild central macular edema in one eye,
another had very incipient macular degeneration in one eye,
and three had photopsia (sensation of seeing lights, sparks,
or colors). The presence of ring scotomas or temporal
islands was not included in the reports. Participants had
been diagnosed between 2 and 57 yr before (mean = 14.58,
standard deviation [SD] = 11.16). None of the participants
Characteristic Experimental
Group Control Group
Age (yr)
22–57 17–57
43.00 ± 10.55 36.64 ± 13.06
Sex
13 8
2 3
VA (logMAR unit)
0.00–1.00 0.00–1.00
0.30–1.00 0.30–1.00
VF
5.00–40.00 5.00–40.00
14.80 ± 9.49 17.73 ± 13.11
Associated Visual Pathologies
2 —
2 1
1 —
1 —
Table 1.
Demographic characteristics of participants by group.
Range
Mean ± SD
Female
Male
RE Range
LE Range
Range
Mean ± SD
Incipient Cataracts
Photopsia
Mild Central Macular Edema
Mild Macular Degeneration
LE = left eye, logMAR = logarithm of minimum angle of resolution, RE =
right eye, VA = visual acuity, VF = visual field.
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were receiving any type of treatment for depression at the
time of the study. One participant had mild central macular
edema in one eye.
Materials
Contrast Sensitivity
To evaluate CS, we used the P&R test. The P&R test,
as indicated by Pelli et al. [36], consists of two printed
optotype charts, 97 × 82 cm, with eight lines each show-
ing a different sequence of letters (6 per line). All the let-
ters are the same size and are arranged in groups whose
contrast varies from high to low. Each group has three
letters of the same contrast level, and the contrast is
lower in the group on the right. The test can measure up
to 16 different contrast values in steps of 0.15 log units,
from 0.0 to 2.25.
Visual Functioning
The National Eye Institute VFQ (VFQ-25, version
2000) was used to obtain a measure of the individual
visual functioning in everyday life [37]. This instrument
is composed of 38 items that provide a general measure
of the difficulties associated with vision in daily life in
people with chronic eye diseases, as well as 11 subscales
that evaluate emotional well-being and social function-
ing: general health and general vision, near vision, dis-
tance vision, driving, peripheral vision, color vision,
ocular pain, specific visual limitations (role difficulties),
dependency, social functioning, and mental health. The
questionnaire measures, therefore, visual and psychoso-
cial aspects that belong to visual functioning in everyday
life. The answers to the items range between 1 and
5 points, depending on which best fits the respondent’s
situation. These scores are converted to a 0 to 100 scale,
so higher scores mean better visual functioning. The
questionnaire enabled us to obtain scores in each of the
subscales (although in the present study we have omitted
the results in the driving subscale because only one par-
ticipant could do it) and an overall score.
We chose this scale because it is much used and cited
in recent years and there are studies that underpin its util-
ity in the population with RP [18]. The psychometric
properties of the scale are robust (the reliability ranges
between 0.71 and 0.85 and it has reliability equal to or
greater than 0.70, in all the subscales) [37].
Depression
We used the BDI to evaluate depression [38]. The
BDI is a self-applicable instrument validated for the
Spanish population [39] to quantify symptoms of depres-
sion in normal and clinical populations. The BDI has an
average reliability (alpha coefficient) of 0.86 [40]. The
version used in this study was the abbreviated scale of 13
items, and there is a high correlation (0.96) between both
forms [38]. In this version, the respondent must choose a
sentence from four options, listed in order of severity.
Each item is assessed with different options of answers
from 0 to 3, giving a total possible score of 39 points. The
following scores were taken into account: 0 to 4 =
absence of depression, 5 to 7 = mild depression, 8 to 15 =
moderate depression, and >15 = serious depression [41].
Training Program
The instruments for the training program consisted of
a personal computer, a head-mounted display (HMD),
and software that generates the training patterns and reg-
isters the responses of the user during each session.
Visual stimuli consisted of bright spots of varying inten-
sity, duration, and position generated within the VF of the
HMD, first for each eye in monocular vision and then in
binocular vision.
Using an HMD allowed us greater control over the
illumination conditions, as well as helping to avoid any
possible sources of distraction. The software included in
the training program generated the visual stimuli on the
HMD and registered the response of the participant when
he or she perceived it and pressed a key. The visual field
was divided into a regular grid of eight by eight areas or
cells arranged into four quadrants of the screen, with the
stimuli located at the center of the cells. Stimuli were
shown in all the defined positions at three different levels
of intensity, 1/3, 2/3, and 1, with 1 corresponding to the
highest intensity. During each complete training stage
(either monocular or binocular), the 64 defined areas were
stimulated once with each of the three intensity levels in
each position presented randomly (64 stimuli per intensity
level).
During the whole training session, a fixation point
remained in the center of the screen, where the partici-
pants had to direct his or her gaze at all times. Before pre-
senting each new stimulus, the quadrant in which it was
due to appear was pointed at by an arrow in the center of
the visual field behind the fixation point. The arrow
remained in this position for a random variable time
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CHACÓN-LÓPÉZ et al. Visual training, emotional state, and RP
(400–600 ms) before the stimulus appeared. The stimulus
was then displayed for 200 ms. In some randomly
selected cases, the stimulus was not provided, with the
intention of avoiding any tendency toward false posi-
tives. Once the stimulus disappeared, there was a variable
period of time during which the participant had to press a
computer key to register the event as soon as he or she
saw it. The time for registering events was also chosen
randomly within a range between 400 to 600 ms.
The use of variable temporal ranges both in the stage
prior to the display of the stimulus and in the phase in
which, if it is perceived, it is recorded was intended to cre-
ate a different duration for different stimulation cycles;
we hoped that this would avoid participants registering
stimuli that they had not seen because they were simply
following a repetitive periodic response to the stimuli.
With the information generated in each session, a
complete and detailed analysis of the development of
each participant can be carried out.
Procedure
The procedure followed for applying the P&R test
consisted of all the participants in the study reading (in
monocular and binocular vision) the letters located on the
optotype, beginning with the top row and continuing
until two of the three letters in the same group were read
incorrectly.
The participants sat in front of the chart at a distance
of 1 m with the center of the chart at eye level, avoiding
reflections on the surface of the chart. All participants
were assessed at the same location and under identical
conditions, maintaining the illumination constant and
consistent with that established by the authors. The illu-
mination was measured, following the recommendation
of the test instructions, using a Lumix DMC-L1 camera
with a Leica D Vario-Elmarit 14–50mm f/2.8–3.5 lens
(Panasonic; Kadoma, Japan), adjusted to 100 ASA, so
that the illumination of the room corresponded to the
combination of 1/15 s and aperture of f/5.6.
Next, following a break after the CS test, we pro-
ceeded to evaluate VFQ (VFQ-25, version 2000) [37]
and depression. The evaluation was carried out by the
same researcher, administered in the same laboratory
with the same luminance levels. Approximately 2 h were
required to conduct all the tests.
The training phase for each participant was planned
for a period of 3 mo, with daily 15 min sessions in the par-
ticipant’s home and 1 d off per week. Each session was
divided into three phases: two to train each eye separately
and a third for binocular training. The software program
and HMD were installed on the laptops of all participants
so that they could carry out the training at home, without
having to travel. In the beginning of each phase at every
training session, the software repeated the instructions
through a message displayed on the HMD and a recorded
speech. These instructions consisted of keeping their gaze
on the central fixation point and pressing the laptop key-
board every time a stimulus was perceived. At the end of
the session, when the three phases were completed, a mes-
sage was displayed showing a measure related to the per-
formance of the session, computed using the number of
stimuli at each intensity that the user perceived. For each
session, a file was generated containing all the relevant
information for a later analysis: perceived stimuli (their
intensity and location) and a timestamp to control the
training follow-up. We recommended that participants
complete the training every day during the same time
frame, at a time when they were calm and could concen-
trate and when other factors would not interfere.
The participants were instructed to keep their eyes on
the fixation point and to avoid eye movements during the
intervention, although this was not monitored in every
training session. However, at the beginning of each of the
three stages, participants were reminded of this instruc-
tion with a message displayed on the HMD and with a
spoken message that they carry out the training task by
keeping their eyes on the fixation point.
During the training period, the process was moni-
tored by a telephone call every 15 d, registering the most
notable aspects of the participants’ experience. No proce-
dures were applied to affect the emotional state.
RESULTS
Statistical Analyses
Pretraining Measures
Since the violation of the homogeneity of variance
between the experimental group and control group can
lead to biased results in the analyses of unequal sample
sizes, the Kolmogorov-Smirnov test was performed in
the depression variable in the experimental group (p >
0.90) and in the control group (p > 0.66). No significant
differences were found between the two groups (F =
1.99, p = 0.18). It was also performed for differences
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between groups in depression (F = 1.99; p = 0.18) and
VFQ variables (χ2 = 3.08, p > 0.99). The depression
scores and VFQ scores at pretest did not differ between
the experimental group and control group.
Table 2 shows the mean score and SD of both groups
in depression, P&R test, and VFQ before beginning train-
ing, on its completion, and 3 mo following completion.
VA only shows two measures, before beginning training
and after finishing it.
Table 3 shows the mean score and SD of both groups
in VFQ subscales in the three assessments.
Correlation Studies
Spearman correlation analysis between the two
groups showed a negative and significant relationship
between levels of depression and VFQ (ρ = 0.64, p <
0.01) in both the experimental group (ρ = 0.73, p < 0.01)
and in the control group (ρ = 0.84, p < 0.001). No corre-
lation was found between levels of depression and VA in
the right eye (ρ = 0.25, p < 0.26), left eye (ρ = 0.12,
p < 0.59), or binocular (ρ = 0.08, p < 0.97) or between
levels of depression and CS in the right eye (ρ = 0.18,
p < 0.42), left eye (ρ = 0.39, p < 0.09), or binocular (ρ =
0.17, p < 0.47).
Results of Depression Variable
Different linear models of repeated measurements (2 ×
2: two groups × two levels of measurements: pretraining and
posttraining) were carried out for the depression variable.
The main effect of group (F1,24 = 0.34, p < 0.57) and the
two levels of measurements (F1,24 = 0.01, p < 0.91) showed
Measure Pre Post Post 3 mo
EG CG EG CG EG CG
Depression 5.08 ± 4.54 2.56 ± 2.69 3.66 ± 3.89 4.11 ± 5.60 3.25 ± 4.09 4.66 ± 4.82
VA - R E 0.23 ± 0.59 0.21 ± 0.62 0.17 ± 0.55 0.21 ± 0.62 — —
VA - L E 0.19 ± 0.59 0.20 ± 0.64 0.15 ± 0.60 0.20 ± 0.64 — —
VA - B 0.19 ± 0.59 0.20 ± 0.64 0.15 ± 0.60 0.20 ± 0.64 — —
P&R-RE 1.45 ± 0.47 1.53 ± 0.42 1.55 ± 0.46 1.53 ± 0.42 1.58 ± 0.52 1.53 ± 0.42
P&R-LE 1.49 ± 0.33 1.48 ± 0.45 1.57 ± 0.30 1.48 ± 0.45 1.65 ± 0.34 1.48 ± 0.45
P&R-B 1.69 ± 0.26 1.63 ± 0.44 1.73 ± 0.26 1.63 ± 0.44 1.74 ± 0.27 1.63 ± 0.44
VFQ 63.92 ± 15.64 60.64 ± 19.33 67.08 ± 16.01 61.53 ± 18.78 65.60 ± 18.11 61.48 ± 18.99
Subscale Pre Post Post 3 mo
EG CG EG CG EG CG
General Health 68.66 ± 13.39 71.36 ± 22.42 69.83 ± 15.10 72.72 ± 20.44 71.83 ± 16.72 72.00 ± 20.73
General Vision 58.66 ± 15.75 54.54 ± 18.50 57.66 ± 17.71 55.00 ± 20.00 59.33 ± 16.24 54.72 ± 19.93
Ocular Pain 78.33 ± 21.37 76.13 ± 18.07 85.38 ± 16.63 80.07 ± 17.38 84.16 ± 19.17 79.80 ± 17.54
Near Activities 66.10 ± 23.03 59.84 ± 28.52 64.44 ± 23.82 61.66 ± 28.79 63.83 ± 25.05 61.49 ± 28.95
Distance Activities 57.77 ± 19.08 53.37 ± 22.50 55.55 ± 16.49 56.16 ± 22.11 56.10 ± 15.65 56.43 ± 22.22
Social Functioning 63.88 ± 19.83 62.05 ± 21.26 65.55 ± 24.16 62.66 ± 23.59 62.21 ± 23.11 62.57 ± 23.72
Mental Health 67.66 ± 23.05 60.45 ± 29.19 73.00 ± 22.89 65.45 ± 29.10 70.33 ± 23.25 65.45 ± 29.10
Role Difficulties 62.50 ± 19.33 50.00 ± 19.96 58.75 ± 20.16 47.45 ± 14.44 56.25 ± 21.65 47.45 ± 14.44
Dependency 72.50 ± 27.01 63.63 ± 29.02 80.41 ± 23.12 65.45 ± 31.74 71.66 ± 29.77 65.18 ± 31.75
Color Vision 75.00 ± 25.00 77.27 ± 26.11 78.33 ± 20.84 75.00 ± 27.38 80.00 ± 27.05 75.00 ± 27.38
Peripheral Vision 43.33 ± 22.09 36.36 ± 20.50 45.00 ± 16.90 36.36 ± 23.35 43.33 ± 19.97 36.18 ± 23.24
Tab le 2.
Mean ± standard deviation scores in depression, visual acuity (VA), Pelli-Robson Contrast Sensitivity (P&R) test, and Visual Function
Questionnaire (VFQ) of two groups.
B = binocular, CG = control group, EG = experimental group, LE = left eye, RE = right eye.
Tab le 3.
Mean Visual Function Questionnaire subscale scores (mean ± standard deviation) in two groups.
CG = control group, EG = experimental group.
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no significant differences, but there was a significant inter-
action between the two variables (F1,24 = 6.12, p < 0.02). A
new linear model of the effects of the two levels of measure-
ments on the levels of groups showed significant differences
in the experimental group (F1,14 = 5.42, p < 0.04) but not in
the control group (F1,10 = 1.89, p < 0.21). This confirms an
improvement in the participants of the experimental group
in the depression variable.
In order to know if the improvement was maintained,
a new analysis posttraining and post 3 mo (using the t-test)
was carried out. Since a decrease in depression can be
expected as a result of training, we used a one-sided test,
that is, halving the p-values found in the two-side t-test.
In the experimental group, no significant differences
were found between posttraining and post 3 mo (t = 0.62,
p = 0.27), but significant differences were found between
pretraining and post 3 mo scores (t = 2.30, p = 0.02). No
significant differences were found in the control group
between posttraining and post 3 mo (t = 1.64, p = 0.07),
but significant differences were found between pretrain-
ing and post 3 mo scores (t = 2.22, p = 0.03), confirming
an increase in the depression variable, contrary to what
was observed in the experimental group.
Results in Visual Function Questionnaire Variable
The Wilcoxon signed-rank test was used to compare
the scores of the two groups in VFQ. The results of this
test in pretraining and posttraining showed significant dif-
ferences in the experimental group (Z = 2.27, p < 0.02),
but not in the control group (Z = 1.12, p < 0.26). No
significant differences were found in posttraining and post
3 mo measurements in the experimental group (Z = 1.36,
p < 0.17), nor in the control group (Z = 0.68, p < 0.50).
Results in Pelli-Robson Contrast Sensory Test Variable
In the P&R test, Wilcoxon signed-rank test showed sig-
nificant differences in pretraining and posttraining scores in
the experimental group in the right eye (Z = 1.99, p <
0.046) and the left eye (Z = 2.30, p < 0.02), but not in bin-
ocular (Z = 0.96, p < 0.34). No significant differences
were found in the control group in the right eye, left eye,
and binocular (Z = 1.00, p < 0.32). In the posttraining and
post 3 mo scores, no significant differences were found in
the experimental group in the right eye score (Z = 0.21, p <
0.83), left eye (Z = 1.49, p < 0.14) and binocular (Z = ,
p > 0.99), although significant differences were found
between pretraining and post 3 mo scores in the right
eye (Z = 2.68, p < 0.01) and in the left eye (Z = 2.30,
p < 0.02), but not in binocular (Z = 0.72, p < 0.47). In
the control group, no significant differences were found
between posttraining and post 3 mo scores, or between
pretraining and post 3 mo scores.
Results in Visual Acuity Variable
In the VA evaluation posttraining, Wilcoxon signed-
rank test showed no significant differences in the experi-
mental group score in the right eye (Z = 0.16, p < 0.11),
left eye (Z = 0.92, p < 0.36), and binocular (Z = 0.18,
p < 0.85). No significant differences were found in the
control group in the right eye, left eye, or binocular,
maintaining the initial mean scores.
Results in Training Program
To assess the gain score for each trained contrast level
(1/3, 2/3, and 1), the average number of stimuli perceived in
the last seven training sessions as compared to the first
seven sessions was calculated over a maximum of 64 shown
stimuli, taking into account the stimuli perceived with each
eye individually and in binocular. Tabl e 4 shows these num-
bers and the average gain score at each contrast level, which
has been calculated individually for each participant with
the following expression: (ba)/(64 – a), where b is the
average number of stimuli perceived in the last seven ses-
sions, and a the average of the first seven sessions.
The average gain score achieved by the group was
26 percent for low-contrast stimuli, 20 percent for medium-
contrast, and 6 percent for high-contrast. These results
enabled us to confirm that the participants undergoing train-
ing improved in the three contrast levels, but especially in
the low-contrast stimuli, as can be seen from the percentage
values and also from the increase in the average number of
perceived stimuli. The average number of completed train-
ing sessions was 58.1 (74.5%).
Contrast
Level First Week Last Week Gain Score
1/3 23.87 31.76 0.26
2/3 33.37 37.41 0.20
136.85 39.32 0.06
Table 4.
Number of perceived stimuli (average of both eyes and binocular) at
beginning and end of training program, and gain score.
1164
JRRD, Volume 50, Number 8, 2013
DISCUSSION
The final aim of the present study was to improve the
CS of people with restriction in contrast, such as those
with RP, through the training of CS. The results obtained
confirm an improvement in the participants of the experi-
mental group in CS, depression, and visual functioning,
associated with the training. No significant improvement
was found in VA.
Contrast Sensitivity and Visual Acuity
Given the data obtained on CS, we can confirm posi-
tive progression for people who carried out the training
program. That improvement was maintained 3 mo after
the conclusion of the training. There was also an improve-
ment in the three contrast levels, especially in the low-
contrast stimuli. We believe that these results are very
interesting because, as has been widely argued, improve-
ments in CS may facilitate the performance of visual pro-
cessing at different stages of the visual system [13]. It is
important to highlight this because the loss of CS is one of
the main difficulties faced by affected individuals and
seriously affects their daily life [11]. A look at the scores
obtained in the P&R test reveals an improvement in each
eye separately and in binocular vision after training
(Table 2). However, in the case of binocular vision the
improvement is not significant. Perhaps a larger sample or
completing a higher number of training sessions would
have allowed us to detect improvement in binocular
vision. Note that there are no changes between the two
evaluations in the control group, with scores remaining at
the initial levels.
These findings are partially consistent with findings
of Fahle and Poggio [42], who reported improvements in
VA and CS after visual training, proving that perceptual
training, previously considered not applicable to the
treatment of adults, is effective for the treatment of, for
instance, amblyopia and presbyopia [13,43–44], although
in this study we have found no significant differences in
VA. It should be noted that a possible limitation of the
study lies in the wide range of VA and VF. Although a
smaller range would have been desirable, the groups
were, at least, homogeneous as shown by the statistical
tests carried out. Also, in accordance with Hahm et al.
[18] and Szlyk et al. [11], the assessment of visual func-
tioning was as valuable and complete as the data pro-
vided by ophthalmologic tests and the results of VFQ
pretreatment showed no significant differences between
the two groups.
Emotional State and Visual Functioning
The results of Spearman correlation analysis high-
light that there is a negative and significant correlation
between the level of depression and visual functioning,
which is in line with the data found in the studies by
Hahm et al. [18] and Szlyk et al. [11]. For this reason, it
seems reasonable to conclude that an improvement in
visual functioning favors the emotional state and quality
of life. The results obtained in our study showed an
improvement in both variables of those who participated
in the training program compared with the control group.
It should be noted that the initial level of depression was
in the limit range of mild depression and we observed a
decrease in the scores obtained after the training and post
3 mo. This result has to be borne in mind when compared
with those of the control group, which showed a slight
increase (also in post 3 mo evaluation) in an even lower
range of mild depression.
In spite of the great variability and heterogeneity
between the participants in both groups (as demonstrated
by the high SDs) and the slightly higher initial score in
VFQ score in the experimental group, an improvement
was noted in the experimental group. Certainly, the data
from our study do not allow us to confirm emphatically
that the improvement was due to participation in the pro-
gram; however, they seem to confirm that participating in
the program benefitted both visual functioning and emo-
tional well-being. This improvement cannot be ascribed to
the implementation of any psychological procedure to
reduce levels of depression, because no such procedure or
treatment was applied. Instead, the improvement could be
related to the mutual influence of emotional state and
visual functioning, also observed in other studies. For
example, Hahm et al. [18] and Szlyk et al. [11] have
pointed out the negative influence of the emotional state
on visual functioning, which reduces people’s vision-
related quality of life, while Grant et al. [22] also stressed
that the psychological state may influence vision rehabili-
tation programs.
Possibly, the opportunity to participate in the experi-
ment and to make improvements in their training sessions
(once the training session was completed the participants
received an overall evaluation of the use of the session cal-
culated by the number of stimuli perceived at each inten-
sity) had an effect on the improvement of their emotional
1165
CHACÓN-LÓPÉZ et al. Visual training, emotional state, and RP
state. In this sense, it must be stressed that all participants
were volunteers with a moderate level of involvement,
especially those in the experimental group (as evidenced by
the regular and normal development of training for 74.5%
training sessions). This may explain the favorable changes
found, corroborating the results obtained in previous stud-
ies that demonstrate that practice and motivation seem to
determine improvement [16]. Possibly, as stated by Herse
[45], the simplest intervention may prove highly effective
in enhancing quality of life and personal well-being.
Limitations
Although the results obtained seem encouraging, we
are aware that they should be considered with caution
because of the small sample size. Additional studies with
larger samples will be needed to confirm our findings.
Another limitation is the voluntary nature of participation
in the study, which may explain, at least in part, the mod-
erate degree of motivation.
Although the participants were instructed to keep
their eyes on the fixation point and to avoid eye move-
ments during the intervention, it would be desirable to
control this factor. This task would be easier to carry out
with the help of an eye-tracker if the training were carried
out with a conventional monitor instead of the HMD we
used. Nevertheless, we consider that the use of the HMD
not only allows training to be less affected by the condi-
tions of ambient illumination, but also decreases the
effect of possible distractions that divert the attention,
and therefore less effort is required to maintain the fixa-
tion point during the training session.
CONCLUSIONS
The results obtained seem encouraging since they
highlight an improvement in CS, visual functioning, and
emotional state of people with a degenerative retinal dis-
ease for which there is currently no treatment and the
development and prognosis are not favorable. Although
we did not find studies that analyze training to improve
CS in people with RP that would have enabled us to com-
pare our results, we believe that initiatives such as this
can contribute to better functional and emotional well-
being of this population.
For this reason, we think that the training program
applied is a helpful addition to RP rehabilitation and that
the findings of our study have important implications in
planning interventions with people with RP. We should
not forget the repercussions that visual impairments such
as RP have on the emotional state and daily activities of
those who are affected [7,11,18]. Hence, training to
improve visual functioning may favor their personal,
social, and professional integration.
ACKNOWLEDGMENTS
Author Contributions:
Study concept and design: H. Chacón-López, F. J. Pelayo, M. D.
López-Justicia, C. A. Morillas, R. Ureña, A. Chacón-Medina, B. Pino.
Acquisition of data: H. Chacón-López, F. J. Pelayo, M. D. López-
Justicia, C. A. Morillas, R. Ureña, A. Chacón-Medina, B. Pino.
Analysis and interpretation of data: H. Chacón-López, F. J. Pelayo,
M. D. López-Justicia, C. A. Morillas, R. Ureña, A. Chacón-Medina,
B. Pino.
Statistical analysis: H. Chacón-López, F. J. Pelayo, M. D. López-
Justicia, C. A. Morillas, R. Ureña, A. Chacón-Medina, B. Pino.
Critical revision of manuscript for important intellectual content:
H. Chacón-López, F. J. Pelayo, M. D. López-Justicia, C. A. Morillas,
R. Ureña, A. Chacón-Medina, B. Pino.
Financial Disclosures: The authors have declared that no competing
interests exist.
Funding/Support: This material was based on work partially supported
by the Cátedra Bidons Egara, the Spanish MICINN research project
RECVIS (Ref. TIN2008–06893-C03–02), the project GENIL-PYR-
2010–19 funded by CEI BioTIC GENIL CEB09–0010, and the Junta de
Andalucía project P06-TIC02007.
Additional Contributions: The authors wish to express their grati-
tude to Dr. Joaquin López (from Bidons Egara, S.L.) and Dr. Eduardo
Fernández (from the Miguel-Hernández University) for their support
and interest in this research; to Dr. José R. Jiménez from the Depart-
ment of Optics (University of Granada); and to Bridgit McQue for
translating parts of the original manuscript into English.
Institutional Review: This research study was approved by the Insti-
tutional Review Board of the University of Granada (Spain) in 2009.
Informed consent was obtained from all participants.
Participant Follow-Up: The authors plan to inform participants of
the publication of this study.
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Submitted for publication June 15, 2012. Accepted in
revised form February 11, 2013.
This article and any supplementary material should be
cited as follows:
Chacón-López H, Pelayo FJ, López-Justicia MD, Moril-
las CA, Ureña R, Chacón-Medina A, Pino B. Visual
training and emotional state of people with retinitis pig-
mentosa. J Rehabil Res Dev. 2013;50(8):1157–68.
http://dx.doi.org/10.1682/JRRD.2012.06.0113
ResearcherID/ORCID: Helena Chacón-López, PhD: L-
3677-2013; Francisco J. Pelayo, PhD: E-2428-2012
... Such kinds of trainings are essential to maintain and enhance the vision of such children (Chacón-López et al., 2013). Besides, the response time of children with low vision can be shortened with the help of these trainings (Tsai et al., 2013). ...
... Besides, the response time of children with low vision can be shortened with the help of these trainings (Tsai et al., 2013). There are a variety of trainings including showing objects on the researcher's hands, providing visual therapy or offering computer-based visual objects whose sizes and durations on the screen and position are adjustable (Chacón-López et al., 2013;Coetzee & Pienaar, 2013;Werth & Seelos, 2005). Consequently, all of the trainings serve the same purpose to improve scanning, focusing, and tracking skills. ...
Thesis
This study explores the design principles of eye movement-based computer game applications as training material for children with low vision to enhance their vision skills. It aims to provide children with interactive materials to improve their vision. For the study, design-based research was employed in four phases, namely analysis, development, evaluation and testing, and documentation and reflection. In the analysis phase, a focus group meeting and interviews were conducted with experts from the field of low vision. Besides, interviews were conducted with special education teachers and an ophthalmologist to understand the current eye training applications for children with low vision. In the design phase, interviews were conducted with expert academicians from the field of low vision and instructional technology to gather expert opinion and to prepare storyboards for the eye training games. After that, two prototypes were developed by including formative evaluation supported with interviews with special education teachers, and interviews and focus group meeting with expert academicians. After these steps, the final product of the game-based eye training program for children with low vision was finalized, and design principles for developing eye gaze games were gathered. In the evaluation and testing phase, the eye training program was tested with children with low vision, and the data were collected from their parents, ophthalmologists, and expert academicians as summative evaluation. In the documentation and reflection phase, the design principles were finalized for developing eye training programs for children with low vision. This study revealed that eye-tracking technology can be used by people with low vision. The findings revealed design principles for designing and developing a game-based eye training program for children with low vision. By following these design principles, there is a need for developing more games with a variety of visual objects to help children with low vision to enhance their visual acuity and visual field through scanning and focusing on objects and controlling of eye movements as scanning and tracking by considering color discrimination, contrast sensitivity, and light sensitivity.
... In the absence of XLRP specific studies, studies summarized in Table 2 describe the humanistic burden of RP and include a qualitative assessment based on a semistructured interview of 23 people with RP in Australia; 25 an online survey of 166 people with RP who have vision impairment, recruited through a patient advocacy group; 26 findings from a focus group of eight people with RP who are legally blind in the US; 27 a retrospective self-study of a person living with RP in Ireland; 28 and a clinical assessment of 26 people with RP in Spain. 29 Qualitative studies indicate that people living with RP experience difficulties with everyday tasks, psychosocial burden, and barriers to work and career. 25,30 A retrospective study of patient registry and health insurance data in Korea from 2011 to 2014 identified an increased mortality risk among people with RP compared with the general population. ...
... One study observed that, compared with the wider RP population, people with XLRP had lower perceived vision-related ability (as assessed with a modified version of the Vision Function Scale). 38 The correlation between reducing visual function and increasing levels of depression in RP supports the hypothesis that humanistic burden can reasonably be expected to be greater for XLRP than for other forms of RP. 29 An increase in patient burden with increasing severity of the condition has been observed in RP more generally: a study that stratified patients with RP by visual field and visual acuity found that HRQL and emotional state worsened as patients approached legal blindness (residual visual field diameter below 20° and a visual acuity less than 0.3 [0.5 log minimum angle of resolution]). 39 Similarly, patient reports highlight the greater impact on activities of daily living associated with the progression of RP as XLRP progress, as illustrated in Figure 1. ...
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X-linked retinitis pigmentosa (XLRP) is a severe form of retinitis pigmentosa (RP), a rare, inherited retinal degenerative disorder, that causes blindness. The aim of this literature review was to identify what is currently known about the burden of XLRP. Literature databases were searched for articles describing the clinical, humanistic, or economic burden of XLRP or RP in the US, Japan, France, Germany, Italy, Spain, and the UK, published in English between 2014 and 2019; gray literature and cited references were reviewed. Literature describing XLRP is limited as this is an ultra-rare condition; findings relating to burden of RP have been reported with interpretation of how burden differs for XLRP. In XLRP, night blindness usually presents in the first decade of life, followed by loss of peripheral and then central vision; legal blindness is reported at a median of 45 years in affected males (vs median 70 years for RP). There is limited evidence of humanistic or economic burden specific to XLRP; one study identified greater vision-related activity limitations in patients with XLRP compared with the wider RP population. Qualitative studies describe increased humanistic burden for people living with RP; difficulty undertaking everyday tasks (driving, hobbies, reading), psychosocial burden and barriers to work and career. People described the emotional impact of dealing with progression of RP, ongoing social and physical challenges, and the impact of RP on relationships. The economic burden of RP is associated with lost productivity, greater healthcare costs and increasing requirement for formal and informal care. In summary, XLRP remains an untreatable condition that can impact people from childhood. The humanistic burden of RP has been shown to increase as the disease progresses; hence, in XLRP the earlier onset and earlier progression to blindness during prime working years may mean a comparatively greater lifetime burden of disease.
... RP-related visual impairments alone can negatively affect performance in daily activities, which can be further compounded by the presence of anxiety or depression. 41 These effects on daily activity can also result in a larger dependence on others for daily tasks, which may consequently further aggravate symptoms of depression. 23,35,[41][42][43][44][45] The cyclic nature and implications of anxiety and depression on patients with RP highlight the importance of wholeperson care in patients with RP. ...
... 41 These effects on daily activity can also result in a larger dependence on others for daily tasks, which may consequently further aggravate symptoms of depression. 23,35,[41][42][43][44][45] The cyclic nature and implications of anxiety and depression on patients with RP highlight the importance of wholeperson care in patients with RP. Ophthalmologists and primary care physicians can collaborate and raise awareness about common mental health problems among patients with RP. ...
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Purpose Retinitis pigmentosa (RP) is a chronic progressive disease with no curative treatments. Understanding the variables involved with improving patients’ quality of life is important in managing this population. The literature investigating the relationship of anxiety and depression with RP relies on the analysis of smaller subset populations of patients with RP, and no study has quantified the effect size of the potential association. This study aims to elucidate and quantify the association between RP, anxiety, and depression. Methods A retrospective case-control study was conducted of 6 093 833 medical records within the University of North Carolina Hospital and outpatient clinic system from July 1, 2004, to August 30, 2019. Patients with a diagnosis of RP, anxiety, and depression were identified within the Carolina Data Warehouse for Health by International Classification of Diseases, Ninth and Tenth Revision codes. Results From the base population of 6 093 833 patients' medical records, 690 patients were diagnosed with RP, 253 065 with anxiety, and 232 541 with depression. Patients with RP have an odds ratio, adjusted for sex and age, of 4.915 (95% CI, 4.035-5.987) for having comorbid anxiety, 5.609 (95% CI, 4.622-6.807) for comorbid depression, and 4.130 (95% CI, 3.187-5.353) for comorbid anxiety and depression. Conclusions Patients with RP have a higher prevalence of anxiety and depression, with increased odds of approximately 5 to 6 times for also carrying a diagnosis of anxiety or depression and about 4 times for carrying diagnoses of anxiety and depression compared with the general population.
... PHQ-9 depression scale is a useful multipurpose questionnaire used for screening, diagnosis, and assessment of depression and other mental disorders, with well-established validity and reliability [20,[24][25][26]. It evaluates patients' emotional status over the past two weeks, by scoring all the 9 criteria of the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV) [27]. The responses are rated from "0" (not at all) to "3" (nearly every day). ...
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Purpose To evaluate the levels of anxiety and depression in patients with symptomatic vitreous floaters and to determine the possible correlations of psychological implications with the symptoms duration and possible improvement, the degree of posterior vitreous detachment, and the discomfort severity. Methods Ninety patients complaining for floaters and fifty-seven age- and gender-matched healthy-control subjects were recruited. Every participant underwent a complete ophthalmological examination, including funduscopy and optical coherence tomography scans, while clinical and demographic data were also gathered. The Patient Health Questionnaire-9 (PHQ-9), the Zung Depression Inventory-Self-Rating Depression Scale (Zung SDS), and the Hospital Anxiety and Depression Scale (HADS) were completed by everyone. Results Between the studied groups, no significant differences were detected regarding the clinical and demographic data (p > 0.05). The patients with floaters had significantly higher scores of PHQ-9, Zung SDS, HADS Anxiety, and HADS Depression (p < 0.001). After adjustment for several confounders, PHQ-9 (p = 0.041), Zung SDS (p = 0.003), and HADS Anxiety (p = 0.036) values remained significantly impaired. Among the patients, PHQ-9 and Zung SDS scores were significantly elevated in the patients with floaters duration less than 4 weeks (p < 0.05). Finally, anxiety and depression were significantly correlated with the symptoms duration and intensity, with the floater-associated discomfort, and with the stage of posterior vitreous detachment. Conclusion Vitreous floaters have a negative impact on patients’ psychological status, by the terms of enhanced depressive and anxiety levels. To the best of our knowledge, our study is the first in the literature to elaborate the aforementioned association, by assessing three different questionnaires simultaneously.
... [38][39][40] Furthermore, daily practice and motivation seem to be decisive factors in improving the use of residual vision in subjects with impaired vision. 18,[41][42][43] Thus the 3D-MCSTP comprises four in-office visits (every three weeks) and two customized home-based daily training sessions (30 to 40 minutes in the morning and afternoon) that should be performed a minimum of five days/week or a maximum of seven days/week, over 12 weeks. In other words, the 3D-MCSTP facilitated a minimum of 120 and a maximum of 168 home-based training sessions during the time interval between in-office visits (Fig. 1A). ...
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Purpose: To examine whether a noncomputerized multitasking compensatory saccadic training program (MCSTP) for patients with hemianopia, based on a reading regimen and eight exercises that recreate everyday visuomotor activities using three-dimensional (3D) real-world objects, improves the visual ability/function, quality of life (QL), and functional independence (FI). Methods: The 3D-MCSTP included four in-office visits and two customized home-based daily training sessions over 12 weeks. A quasiexperimental, pretest/posttest study design was carried out with an intervention group (IG) (n = 20) and a no-training group (NTG) (n = 20) matched for age, hemianopia type, and brain injury duration. Results: The groups were comparable for the main baseline variables and all participants (n = 40) completed the study. The IG mainly showed significant improvements in visual-processing speed (57.34% ± 19.28%; P < 0.0001) and visual attention/retention ability (26.67% ± 19.21%; P < 0.0001), which also were significantly greater (P < 0.05) than in the NTG. Moreover, the IG showed large effect sizes (Cohen's d) in 75% of the total QL and FI dimensions analyzed; in contrast to the NTG that showed negligible mean effect sizes in 96% of these dimensions. Conclusions: The customized 3D-MCSTP was associated with a satisfactory response in the IG for improving complex visual processing, QL, and FI. Translational relevance: Neurovisual rehabilitation of patients with hemianopia seems more efficient when programs combine in-office visits and customized home-based training sessions based on real objects and simulating real-life conditions, than no treatment or previously reported computer-screen approaches, probably because of better stimulation of patients´ motivation and visual-processing speed brain mechanisms.
... The literature has already demonstrated that psychosocial health can affect patient performance on visual function testing such as visual field testing [29][30][31] . Depression and poor mental/emotional health have repeatedly been correlated with worse visual function, and in fact may be predictive of poorer physical health in an IRD population 4,5,[32][33][34][35][36] . Furthermore, self-rated health has been more strongly associated with quality of life than traditional clinical tests 2,37 , suggesting that a patient's perception of their quality of vision may be more impactful on quality of life. ...
... Visual function questionnaires (VFQs) have been used to evaluate the subjective impact of RP, starting in at least the early 1990s [2,22]. Some have compared VFQ responses to clinical measures [14,17,[23][24][25][26][27][28][29][30][31][32][33] or to functional ability [27,31,[34][35][36][37][38][39][40][41]. However, some VFQs used with RP have not been formally tested for validity. ...
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Abstract Purpose There have been few systematic reports of vision-related activity limitations of people with retinitis pigmentosa (RP). We report a merging of data from the National Eye Institute Visual Function Questionnaire (NEI-VFQ) obtained in five previous studies. We asked whether the Vision Function Scale (VFS; Pesudovs et al., 2010) which was developed for cataract patients would apply in this new population (condition). Methods Five hundred ninety-four individuals completed a total of 1753 questionnaires, with 209 participants providing responses over at least 4 years. Rasch analysis showed that the 15-item VFS was poorly targeted. A new instrument created by adding four driving-related items to the VFS had better targeting. As an indirect validation, VFS-plus person scores were compared to visual field area measured using a Goldmann perimeter, to the summed score for the combined 30–2 and 30/60–1 Humphrey Field Analyzer programs (HFA), to 30-Hz full-field cone electroretinogram (ERG) amplitude, and to ETDRS visual acuity. Changes in VFS-plus person scores with age and between four common heredity groups were also examined. Results The Rasch model of responses to the 19 VFS-plus items had person and item separation of 2.66 and 24.43 respectively. The VFS-plus person scores were related to each vision measure (p
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Objectives Although inherited retinal disorders (IRDs) related to the gene encoding the retinal pigment epithelium 65kD protein (RPE65) significantly impact the vision-related quality of life (VRQoL), their emotional and social aspects remain poorly investigated in Italy. Narrative Medicine (NM) reveals the more intimate aspects of the illness experience, providing insights into clinical practice. Design and setting This NM project was conducted in Italy between July and December 2020 and involved five eye clinics specialised in IRDs. Illness plots and parallel charts, together with a sociodemographic survey, were collected through the project’s website; remote in-depth interviews were also conducted. Narratives and interviews were analysed through NVivo software and interpretive coding. Participants 3 paediatric and 5 adult patients and eight caregivers participated in the project; 11 retinologists globally wrote 27 parallel charts; 5 professionals from hospital-based multidisciplinary teams and one patient association member were interviewed. Results Findings confirmed that RPE65-related IRDs impact VRQoL in terms of activities and mobility limitations. The emotional aspects emerged as crucial in the clinical encounter and as informative on IRD management challenges and real-life experiences, while psychological support was addressed as critical from clinical diagnosis throughout the care pathway for both patients and caregivers; the need for an IRDs ‘culture’ emerged to acknowledge these conditions, and therefore, promoting diversity within society. Conclusions The project was the first effort to investigate the impact of RPE65-related IRDs on the illness experience through NM, concomitantly addressing the perspectives of paediatric and adult patients, caregivers and healthcare professionals and provided preliminary insights for the knowledge of RPE65-related IRDs and the clinical practice.
Article
Purpose Construct and validate a patient-reported outcome measure for screening and monitoring vision-related anxiety in patients with inherited retinal degenerations. Design Item-response theory and graded response modeling to quantitatively validate questionnaire items generated from qualitative interviews and patient feedback. Methods Patients at the Kellogg Eye Center (University of Michigan) with a clinical diagnosis of an inherited retinal degeneration (n=128) participated in an interviewer-administered questionnaire. The questionnaire consisted of 166 items, 26 of which pertained to concepts of “worry” and “anxiety”. The subset of vision-related anxiety questions was analyzed by a graded response model using Cai’s Metropolis-Hastings Robbins-Monro algorithm R (version 3.6.3) mirt package. Item reduction was performed on the basis of item fit, item information, and item discriminability. To assess test-retest variability, 25 participants completed the questionnaire a second time 4-16 days later. Results The final questionnaire consisted of 14-items divided into two unidimensional domains: rod-function and cone-function anxiety. The questionnaire exhibited convergent validity with the Patient Health Questionnaire (PHQ-4) for symptoms of depression and anxiety. This vision-related anxiety questionnaire has high marginal reliability (0.81, 0.83) and exhibits minimal test-retest variability (ρ=0.81 (0.64, 0.91); 0.83 (0.68, 0.92)). Conclusions The Michigan Vision-related Anxiety Questionnaire (MVAQ) is a psychometrically validated 14-item patient-reported outcome measure to be used as a psychosocial screening and monitoring tool for patients with inherited retinal degenerations. It can be utilized in therapeutic clinical trials for measuring the benefit of an investigational therapy on a patient’s vision related anxiety.
Article
Background: Type 1 Usher syndrome (USH1) is a rare disease and major cause of genetic deaf-blindness. Deafness is present from birth while retinitis pigmentosa (RP) which typically presents during childhood is progressive leading to blindness. The aim of this research was to develop a disease model describing USH1 symptoms and their impact on patients’ lives. Materials and Methods: Qualitative interviews were conducted with patients (pediatric and adult) and parents of children and adolescents with USH1. Interviewed subjects were enrolled through ophthalmologists from specialized eye centers in the USA and in France. Trained interviewers used semi-structured techniques to elicit concepts relevant to patients and their parents. Thematic analysis of interview transcripts led to the identification of concepts which were organized to generate a disease model. Results: A total of 18 patients (7 in the US; 11 in France)– 9 adults, 4 adolescents, and 5 children– and 9 mothers were interviewed. The most cited ocular symptoms were difficulty seeing at night and loss of peripheral vision. Interviewees reported limitations on Physical (e.g. difficulty moving), Mental (e.g. fear about falling), Social (e.g. difficulty discussing disease with others) and Role (e.g. difficulties at school/work) functioning. These impacts were, when possible, mitigated by coping strategies and support (e.g. using electronic devices, having a positive/proactive attitude). Conclusions: This research provides an overview of symptoms experienced by patients with USH1 and highlights the dramatic impact these have on patients’ lives, allowing the identification of concepts of importance when evaluating therapeutic treatments in development for RP.
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El propósito de este trabajo era conocer el desempeño perceptivo visual de adolescentes y jóve-nes con una enfermedad degenerativa visual (Retinosis Pigmentaria-RP) (frente a un grupo sin difi-cultades visuales) y averiguar la relación con su funcionamiento visual, el cual determina la calidad de vida asociada a la visión; otro cometido era averiguar si la agudeza visual cercana (AVC) y el campo visual (CV) tienen alguna relación con su desempeño perceptivo-visual y con el funciona-miento visual. Los resultados mostraban que el grupo de afectados tenía dificultades en todos los ámbitos de la percepción visual evaluados, excepto en discriminación visual. También se constata-ba que la AVC y el CV parecen jugar un rol importante en su percepción visual y en la funcionalidad visual, pero las variables que mejor predicen su funcionamiento son la discriminación figura-fondo y el CV. Los resultados obtenidos constituyen un primer acercamiento a la problemática señalada que permite entender mejor las características viso-perceptivas de esta población y su influencia en la vida diaria, además de identificar posibles objetivos de la intervención psico-educativa, dadas sus limitaciones en un buen número de actividades educativas, sociales y laborales. Palabras clave: retinosis pigmentaria, agudeza visual cercana, campo visual, percepción visual y funcionalidad visual.
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Retinitis pigmentosa (RP) causes restriction of the visual field, progressive vision loss, and night blindness. This article presents an overview of the current management options for O&M and describes the scientific and mobility research on RP that is relevant to the work of O&M instructors.
Article
Active and passive perceptual training methods were tested with 30 macular degeneration patients to improve their residual vision. Four different measures assessed improvement: a reading test, the Frostig Figure Ground Test (FFG), the Bender-Bestalt Test, and the Farnsworth-Munsell 100-Hue Test (FM-100). Participants were grouped according to two levels of impairment (moderate or severe) and three training conditions (active, passive, and control) in a 2 x 3 multivariate design. It was expected that the training sessions would improve visual task performance, and that active participants would show more improvement than other groups. A multivariate ANOVA found an overall main effect for the learning factor, and univariate tests showed that the active group improved significantly more than the passive and control groups on the FFG test. The main conclusion was that perceptual training may contribute to successful visual adjustment and that the effect of training is not limited to a particular level of visual impairment.
Article
El objetivo del presente estudio era determinar la posible presencia de depresión y/o ansiedad en adultos con retinosis pigmentaria (RP), así como en sus familiares, frente a un grupo control. Se pretendía además averiguar si la agudeza visual (AV) tiene alguna repercusión en estas variables. Se utilizó el Cuestionario de Ansiedad STAI y el Inventario de Depresión de Beck. Los resultados mostraban que la ansiedad era mayor en personas con RP y sus familiares, como estado y como rasgo; también se ponía de manifiesto la presencia de depresión leve en los afectados, mientras que no se hallaba en el resto de los grupos. No se encontró relación entre estas variables y la AV en el grupo con RP. Se recomienda evaluar la ansiedad y la depresión tras el diagnóstico de la enfermedad y transcurrido un tiempo, con el fin de proporcionar apoyo psicológico a los afectados y a los familiares.
Article
Retinitis pigmentosa (RP) refers to a group of inherited retinal degenerative disorders in which night and peripheral vision are gradually lost. A national sample of young adults with RP reveals that a significant number feel that RP adversely affects their education, employment, mobility and socialization. Participants fell that special counseling is needed to accept and adjust to their condition, and that sharing with peers would be beneficial.
Article
• We compared visual acuity with visual field radius in 235 patients with typical retinitis pigmentosa and no evidence of other visual acuity-limiting problems (such as cataract or foveal cystoid edema). Results show a strong relationship between visual acuity loss and proximity to the fovea of the visual field border (shortest distance from the foveal center to the border of the V-4-e isopter) for these patients. Ninety-six percent of patients with central visual field radii greater than 30° have visual acuities of 20/40 or better; 32° of patients with central visual field radii smaller than 10° have visual acuity of 20/40 or better.
Article
Objective To develop and test the psychometric properties of a 25-list-item version of the National Eye Institute Visual Function Questionnaire (NEI VFQ-25).Design Prospective observational cohort study of persons with 1 of 5 chronic eye diseases or low vision who were scheduled for nonurgent visits in ophthalmology practices and a reference sample of persons without eye disease.Setting Eleven university-based ophthalmology practices and the NEI Clinical Center.Patients Eligible participants had to have 1 of the following eye conditions: age-related cataracts, age-related macular degeneration, diabetic retinopathy, primary open-angle glaucoma, cytomegalovirus retinitis, or low vision from any cause. Seven of the 12 sites also enrolled persons in a reference sample. Reference sample participants had no evidence of underlying eye disease but were scheduled for either screening eye examinations or correction of refractive error. All eligible persons had to be 21 years or older, English speaking, and cognitively able to give informed consent and participate in a health status interview.Measurements and Main Results To provide the data needed to create the NEI VFQ-25, all subjects completed an interview that included the 51-list-item NEI VFQ. Estimates of internal consistency indicate that the subscales of the NEI VFQ-25 are reliable. The validity of the NEI VFQ-25 is supported by high correlations between the short- and long-form versions of the measure, observed between-group differences in scores for persons with different eye diseases of varying severity, and the moderate-to-high correlations between the NEI VFQ-25 subscales that have the most to do with central vision and measured visual acuity.Conclusions The reliability and validity of the NEI VFQ-25 are comparable to those of the 51-list-item NEI VFQ field test version of the survey. This shorter version will be more feasible in settings such as clinical trials where interview length is a critical consideration. In addition, preliminary analyses indicate that the psychometric properties of the NEI VFQ-25 are robust for the eye conditions studied; this suggests that the measure will provide reproducible and valid data when used across multiple conditions of varying severity.
Article
purpose. The purpose of this study was to evaluate the properties of foveal temporal integration in patients with retinitis pigmentosa (RP) within the framework of contrast processing by the magnocellular (MC) and parvocellular (PC) pathways. methods. Temporal integration functions were measured in eight patients with RP whose visual acuities ranged from 20/25 to 20/63. Contrast thresholds were obtained at durations ranging from 15 to 480 ms, using steady-pedestal and pulsed-pedestal paradigms to bias performance toward the MC and PC pathways, respectively. The patients’ results were compared with those of 10 age-similar control observers with normal vision. For both paradigms, contrast thresholds as a function of duration were fit with a two-limbed function to derive the critical duration for temporal integration (t c) and the asymptotic threshold at long durations (ΔL ∞ ). results. The log t cs of the patients with RP were significantly longer than those of the control subjects for the steady-pedestal paradigm (presumed MC-pathway mediation; t = 3.67, P < 0.001), but not for the pulsed-pedestal paradigm (presumed PC-pathway mediation; t = 0.76, P = 0.45). Further, the patients with RP showed a significant correlation between log t c and log ΔL ∞ for the steady-pedestal paradigm (r = 0.72, P < 0.05) but not for the pulsed-pedestal paradigm (r = −0.37, P = 0.36). conclusions. The patients with RP in this study showed greater deficits in contrast sensitivity and a more prolonged critical duration under test conditions that favor the MC rather than the PC pathway. A likely explanation is a high-frequency response attenuation at the level of the cone photoreceptors that has a differential effect on contrast-processing tasks that emphasize different postreceptoral mechanisms.