Abstract
Objective
To determine the validity and reliability of a novel questionnaire to measure vision related quality of life (VRQOL) in children 8–18 years old for use in juvenile idiopathic arthritis-associated uveitis (JIA-U) –Effects of Youngsters’ Eyesight on Quality of Life (EYE-Q).
Methods
Several steps validated the EYE-Q. We interviewed experts and children on how vision affects a child’s activities. We developed new items and selected relevant items from existing instruments. We administered initial versions of the EYE-Q to normal-sighted children and those with JIA-U. For this study, children with various (or no) ocular conditions were recruited from a clinical population. Visual acuity (VA) and contrast sensitivity were performed, and the EYE-Q and Pediatric Quality of Life Inventory (PedsQL) were administered. The EYE-Q was repeated 10 days later. Patients, parents and physicians rated vision severity.
Results
Of 120 patients, 48% were female, 46.7% had no visual impairment (VI), and 52% had bilateral eye involvement. Mean age was 11.3 years. There were significant differences in the measures based on VA (p<0.001). Children with more severe VA and bilateral eye involvement had worse EYE-Q scores (p<0.001). There were significant associations between the EYE-Q and PedsQL (r = 0.375), repeat EYE-Q (r = 0.864), and clinical measures of ocular disease (r = −0.620).
Conclusions
Our study provides evidence of the validity and reliability of the EYE-Q in the measurement of VRQOL. The EYE-Q may complement clinical measures of VI and overall QOL and become an important tool in the assessment of QOL in JIA-U.
Visual impairment (VI) in children is a significant problem in the U.S. Approximately 6.8% of children <18 years old have a diagnosed vision problem, and 25 per 1,000 children in the U.S. suffer from VI or blindness (1, 2). Juvenile idiopathic arthritis (JIA)-associated uveitis is commonly associated with VI. JIA-associated uveitis (JIA-U) is the most prevalent extra-articular manifestation of JIA in North America and occurs in 10–20% of children with JIA (3, 4). It is a chronic non-granulomatous inflammatory eye disease which accounts for up to 80% of all pediatric anterior uveitis (3). JIA-U is associated with ocular diseases that can result in VI such as cataracts, glaucoma and cystoid macular edema (5–9).
Visual Related Quality of Life
Quality of life (QOL) is known to be an important construct, particularly related to chronic disease. Vision-related quality of life (VRQOL) is related to, but not identical to, visual function. VRQOL represents the degree to which vision impacts an individual’s ability to complete activities of daily living and one’s social, emotional and economic well-being. VRQOL can be assessed by measuring the degree of impairment experienced in activities of daily living that rely on sight, (i.e., impaired daily function secondary to visual difficulties is a proxy for visual function). Thus, significant VI would lead to difficulty in performing tasks that rely on vision.
A child’s activities in the home and school largely depend on vision. Since uveitis sequelae can result in compromise of visual function, studies in children with uveitis should account for visual disability. Unfortunately, there are few validated instruments to assess VRQOL in children 8 to 18 years old. Instead, we rely on the clinical ophthalmological exam (e.g., visual acuity (VA), visual field, and contrast sensitivity (CS)), measures of overall QOL, or adult based instruments (10–12). It is unclear whether these measures accurately represent the child’s perspective of the impact of VI on their daily life. Importantly, studies in adult patients use standardized visual function questionnaires in addition to clinical measures to assess visual disability (National Eye Institute Visual Function Questionnaire - NEI-VFQ) (13, 14).
To date, studies examining QOL and outcomes in JIA focus on physical function assessments (i.e. Childhood Health Assessment Questionnaire, joint counts) either as proxies for or predictors of QOL (15, 16). Hence, current methods to assess QOL in JIA may underestimate the impact of the disease on a child’s experience because they fail to account for disease-specific limitations, particularly those relating to vision. This led to the development of a new instrument to evaluate the performance of activities that rely on vision in the home and school, Effects of Youngsters’ Eyesight on QOL (EYE-Q) (17). Our objective is to provide further evidence of the validity and reliability of our instrument for children 8–18 years old.
PATIENTS AND METHODS
This was a validation study that was approved by the Institutional Review Board of Emory University and conformed to the requirements of the United States Health Insurance Portability and Privacy Act. Consent was obtained from the parent/legal guardian, and assent was obtained from the children prior to administration of the instruments.
1. Phase I - Item generation
We interviewed pediatric rheumatologists, pediatric ophthalmology professionals (e.g. ophthalmologists, optometrists, clinical research technicians), and children with and without VI regarding the ways vision affects a child’s daily activities. We also selected relevant items from existing instruments and adapted them to increase their relevance to a U.S.-based sample of children (18, 19). This led to the development of our initial instrument consisting of 20 items thought to measure VRQOL. Based on expert consensus, we thus established face and content validity demonstrating that the items generated were appropriate and fully measured the complexity of the target construct (20).
2. Phase 2 - Operationalism
Thirteen normal-sighted 8–18 year old children then evaluated the questionnaire and items for comprehensibility, format and relevance. Items were retained if ≥70% of respondents agreed that they performed the task and it was important to be able to do so. We also asked for feedback regarding additional tasks that rely on vision. This led to further refinement of the EYE-Q, and 13 items were retained as a result of this analysis.
3. Phase 3 - Pre-testing
The refined version of the EYE-Q was administered in a cross-sectional study of children with JIA, with and without uveitis (21). Children with uveitis had worse EYE-Q scores compared to children without uveitis, supporting the utility of the EYE-Q as an assessment tool for VRQOL in children with JIA and uveitis. This establishes criterion-and construct-related validity by demonstrating that the EYE-Q correlates with external standards, criteria, or diagnoses and conforms to the theoretical concepts or constructs it was designed to measure (20, 22).
4. Revised version
After discussion with other pediatric rheumatology, ophthalmology and epidemiology experts, additional questions for children 16–18 years old were added. As children mature and engage in new developmental tasks, some of which are vision-related, the ability or inability to perform certain tasks would likely contribute to QOL. Therefore, we included items related to driving from the NEI-VFQ-25 (14). We also modified the phrasing of items for increased comprehensibility, and incorporated questions to evaluate photosensitivity, night vision, use of visual aids, and the patient’s perception of overall visual function. This led to the current version of the EYE-Q used in this study.
Subjects
A convenience sample of 120 children with and without ocular disorders was enrolled from the pediatric ophthalmology and uveitis outpatient clinics at the Emory Eye Center. All eligible children and their parents were approached while waiting for their ophthalmology appointment. Inclusion criteria include: 1) ages 8–18 years, and 2) at least a 3rd grade reading and comprehension level. Exclusion criteria include: 1) recently dilated, 2) inability to complete the questionnaires for any reason, 3) major developmental disorders, or 4) refusal to participate.
1) Demographic/Baseline
Parent and patient-based questionnaires were completed in person. A member of the research team was available to read the questions to subjects whose VI prevented completion of the instruments on their own. The individual administering the questionnaires was masked to the child’s ophthalmologic diagnosis and best corrected visual acuity (VA) at the time of visit. Date of visit, date of birth, medical record number, address, phone number, gender, ethnicity, race, and presence of ophthalmologic disease was collected from the parent/guardian. Medical record review confirmed eye disease and laterality of eye involvement.
2) Assessment of Vision Related QOL
The EYE-Q was administered to all child subjects who completed the age-appropriate modules accordingly. The EYE-Q is a patient-based self-report that consists of 23 items for children 8–15 years old, and 26 items (3 additional items on driving) for children 16–18 years old. Items consider tasks that rely on distance (6 items), near (7), color (1), and night vision (1); functionality (3); and photosensitivity (2). There is a subjective assessment of vision severity where the child rates their eyesight as: 1 (excellent), 2 (good), 3 (fair), 4 (poor), 5 (very poor), or 6 (blind). A 5-point Likert scale response format is used to assess difficulty performing each task with response options including: 1 (Not hard), 2 (A little hard), 3 (Hard), 4 (Very hard), and 5 (Cannot do). Each item also provides the child with the option of endorsing “does not apply”. We have chosen not to use a visual analog scale, as this may be problematic for children with visual disability. The EYE-Q takes less than 10 minutes to complete.
The module for 16–18 year olds contains items related to driving. If the teen is not driving or has never driven, the module is identical to that for 8–15 year olds. If driving was given up because of eyesight, 2 points are deducted (similar to needing a visual aid). If the teen is driving presently, 3 additional items inquire about difficulty driving 1) during the day, 2) at night, and 3) in difficult conditions.
One question asks about the need for visual aids (special lamps, magnifying glass and large print material) and allows the child to specify other aids used. Two points are deducted for the use of each aid, with a maximum of 8 points (4 aids) deducted.
An open-ended question asks for feedback regarding any other tasks that rely on vision.
The instrument is then scored as the sum of the items (minus the aids if used) divided by the number of items answered (20 or 23 items depending on the module). Items on vision assessment and additional VRQOL tasks were not included in the scoring. Items were rescaled, hence scores range from 0 to 4 with higher scores indicating better VRQOL. Scores would not be computed if the children did not complete, missed, or marked “does not apply” for more than 50% of the items for any reason.
Distance VA was measured using a Snellen chart and contrast sensitivity (CS) using the Pelli-Robson CS test. Best corrected VA was converted to Logmar for statistical purposes. This provides data to determine construct validity under the assumption that those with worse VA will have poorer VRQOL.
Children were grouped by VI according to best corrected VA: 1) Normal vision: VA 20/20 to <20/40; 2) Mild VI: 20/40 to 20/60; 3) Moderate VI: 20/70 to <20/200; and 4) Severe VI: 20/200 or worse. They were also grouped according to laterality: 1) Bilateral normal vision: VA 20/20 to <20/40 in both eyes (OU); 2) Bilateral Mild VI: 20/40 to 20/60 OU; 3) Bilateral Moderate VI: 20/70 to <20/200 OU; 4) Bilateral Severe VI: 20/200 or worse OU; 5) Unilateral Mild VI: 20/40 to 20/60 in worse eye, <20/40 in better eye; 6) Unilateral Moderate VI: 20/70 to <20/200 in worse eye, <20/40 in better eye; 7) Unilateral Severe VI: >20/200 in worse eye, <20/40 in better eye. Only 116 children were grouped by laterality since 4 were unclassifiable – 1) 20/50 and 20/125, 2) 20/50 and 20/200, 3) 20/80 and no light perception, and 4) 20/80 and 20/200.
To test re-test reliability or consistency of the measure, the EYE-Q was completed twice: first in the clinic and then at home 10 days after the initial assessment. Subjects were called as a reminder to complete the questionnaire and return it in a self-addressed stamped envelope provided by the investigators.
Parents and physicians completed assessments of vision severity and were asked to score how much they thought the child’s life was being affected by their vision from 1–10. One = vision does not affect how he/she functions in daily activities, and 10 = vision affects how he/she functions in daily activities.
3) Quality of Life Assessment
The Pediatric Quality of Life Inventory Version 4.0 (PedsQL) is a validated measure of health-related QOL in children and adolescents from 2 to 18 years of age (23). Four core scales are measured on a 5-point-scale: 1) physical functioning (8 items), 2) emotional functioning (5), 3) social functioning (5), and 4) school functioning (5). Scores range from 0–100 with higher scores indicating better QOL. Aside from the overall QOL score (PedsQL Total), there are Physical Health Summary (PedsQL Physical) and Psychosocial Health Summary (PedsQL Psychosocial) scores. For this study, all scores were calculated, and child-report forms were administered.
Statistics
Data was analyzed using SPSS version 8. Descriptive analyses of baseline demographic and clinical variables based on VI and laterality were performed. Continuous variables that met the assumption of normality were compared using the independent t-test or ANOVA. P-values <0.05 were considered significant. Associations between measures of VRQOL, overall QOL, and the ophthalmologic exam were determined using Pearson’s correlation coefficient. Correlations <0.5 were weak, <0.7 to > 0.5 were moderate, and >0.7 were strong. To determine, test-retest reliability, we examined correlations between the EYE-Q scores at first visit and ten days after. Cronbach’s alpha was performed for internal consistency of the items.
RESULTS
There were 120 children seen in the pediatric ophthalmology clinics who agreed to participate, and 60.8% returned the 2nd EYE-Q after 10 days.
Demographics and Disease Characteristics
Mean age was 11.32 years (SD=2.7), 11.7% were 16 to 18 years, 47.5% were females, 6.7% were Hispanic, and 60.8% were Caucasian (Table 1).
Table 1.
Demographic and disease characteristics (N=120)
| Frequency (%) | |
|---|---|
| Demographics | |
| Age, mean (SD), years | 11.3 (2.7) |
| 8–15 years old | 106 (88.3) |
| 16–18 years old | 14 (11.7) |
| Race, Hispanic (N = 112) | 8 (6.7) |
| Ethnicity (N=115) | |
| White | 73 (60.8) |
| Black | 38 (31.7) |
| Male | 63 (52.5) |
| Disease Characteristics | |
| Ocular disease or condition | 107 (89.2) |
| Duration of eye disease among those with ocular disease, mean (SD), years | 5 (0.6) |
| Patients requiring use of ≥1 visual aids | 25 (20.8) |
| Laterality, visual acuity | |
| Bilateral normal, 20/30 or better | 56 (46.7) |
| Bilateral mild, 20/40 to 20/60 | 6 (5) |
| Bilateral moderate, 20/70 to <20/200 | 8 (6.7) |
| Bilateral severe, ≥20/200 | 7 (5.8) |
| Unilateral mild, 20/40 to 20/60 in worse eye, ≤20/30 in better eye | 19 (15.8) |
| Unilateral moderate, 20/70 to <20/200 in worse eye, ≤20/30 in better eye | 8 (6.7) |
| Unilateral severe, >20/200 in worse eye, ≤20/30 in better eye | 12 (10) |
| Other | 4 (3.3) |
Although 89.2% of children had one or more ocular diseases or conditions, 46.7% had normal bilateral VA and 53.3% had varying degrees of VI (Table 1). Only 4 subjects had strictly refractive disorders, 9 had disorders affecting the uvea (i.e. uveitis, aniridia), 4 - cornea (i.e. cornea transplant, abrasions), 19 – lens (i.e. cataracts, aphakia), 10 – retina (i.e. retinopathy of prematurity, oculocutaneous albinism, retinoblastoma), and 93 had other diseases (i.e. optic nerve atrophy, esotropia, strabismus, etc.). Visual aids were required by 20.8% (large print material-18%, magnifying glass-14.2%, special lamps-2.5%, and others-6.7%). Other aids included canes, binoculars, Braille materials, special computer applications, and reading rulers. 30.1% had other non-ocular diseases (e.g. JIA, lymphoma, sickle cell).
Mean EYE-Q score was 3.45 (range 0.8 to 4.0), and mean total PedsQL score was 78.99 (range 14 to 100). The mean Logmar VA of the better eye was 0.44 which is equivalent to a VA of 20/50-20/60, and a mean CS of 1.72.
Validity and Reliability of the EYE-Q
The EYE-Q appears to measure one major construct. Cronbach’s α on the first EYE-Q was 0.903 and on the second EYE-Q was 0.925. When considering the age-specific modules, α = 0.870 for the 8 to 15 years old module, and α = 0.962 for the 16 to 18 years old module. This provides evidence for strong internal consistency.
When we removed 4 items with high inter-item correlations in the EYE-Q, our Cronbach’s α = 0.879 which provides evidence that a 16-item module for this population retained strong internal consistency.
The EYE-Q also demonstrates strong evidence for reliability. There was a strong correlation between the scores on the first and second administration of the EYE-Q (r = 0.864).
To evaluate construct- and criterion-related validity, variables potentially associated with VRQOL were assessed: the clinical ocular exam (VA and CS) and the PedsQL (Table 2). The EYE-Q was weakly associated with the PedsQL (r = 0.375, r = 0.344, and r = 0.341), and moderately associated with VA of the better eye (r = −0.620) and CS (r = 0.565). The PedsQL was not related to VA (r = −0.049) or CS (r = 0.065).
Table 2.
Correlations between measures of validity and reliability
| EYE-Q1 Score (range of scores 0–4)+ | R | P value |
|---|---|---|
| EYE-Q repeat (range of scores 0–4)+ | 0.864 | <0.001* |
| PedsQLTotal2 (0–100)+ | 0.375 | <0.001* |
| PedsQL Physical3 (0–100)+ | 0.341 | <0.001* |
| PedsQLPsychosocial4 (0–100)+ | 0.344 | <0.001* |
| Logmar Far VA5, better eye (−0.30–3.00)++ | −0.620 | <0.001* |
| Contrast Sensitivity, both+++ | 0.565 | <0.001* |
| PedsQL2 Total Score (0–100)+ | ||
| Logmar VA, better eye (−0.30–3.00)++ | −0.049 | 0.593 |
| Contrast sensitivity, both | 0.065 | 0.571 |
Effects of Youngsters’ Eyesight on QOL;
Pediatric Quality of Life Inventory;
PedsQL Physical Health Summary Score;
PedsQL Psychosocial Health Summary Score;
Best corrected visual acuity
Higher scores indicate better quality of life,
Higher values indicate worse vision and visual acuity,
Lower numbers indicate better vision
<0.001 using Pearson’s Coefficient
Visual Impairment and Quality of Life
Mean scores of the EYE-Q, PedsQL, and vision assessments in children with and without any VI are shown in Table 3. There were significant differences in the EYE-Q scores, PedsQL Total scores, and patient and physician vision assessments in children with normal vision (bilateral VA 20/20 to <20/40) compared to those with VI (any eye with a VA 20/40 or worse). There was no significant difference in parent vision assessments in both groups.
Table 3.
Differences in scores of measures based on vision impairment
| Normal vision§ | Vision Impairment§§ | p-value | |
|---|---|---|---|
| N=56 | N=64 | ||
| EYE-Q1 (0–4)+ | 3.71 (0.30) | 3.22 (0.74) | <0.001* |
| PedsQL2 Total (0–100)+ | 82.4 (12.3) | 76.0 (16.5) | 0.044* |
| Patient global assessment (1–6)++ | 1.7 (0.7) | 2.3 (1.2) | 0.003* |
| Parent global assessment (1–10)++ | 3.9 (3.0) | 5.6 (2.9) | 0.803 |
| Physician global assessment (1–10)++ | 2.1 (1.5) | 4.0 (2.5) | <0.001* |
Normal vision – both eyes with a visual acuity (VA) of <20/40
Vision impairment – any eye with a VA of 20/40 or worse
Effects of Youngsters’ Eyesight on QOL;
Pediatric Quality of Life Inventory Total Score
Higher scores indicate better quality of life,
Higher values indicate worse vision severity
<0.05 using t-test
As expected, the mean EYE-Q score was significantly worse (p<0.001) in children who used visual aids than in those who did not. The PedsQL Total score (p=0.140) was not significantly associated with the use of aids, although a significant difference in the PedsQL Physical Health Summary score (p=0.042) was noted. This association with the Physical score may be secondary to the need for aids for daily functioning such as a cane or large print material.
Mean scores of the EYE-Q and PedsQL categorized by degree of VI are shown in Table 4. As VI worsened, the children had significantly worse VRQOL scores (p<0.001). Likewise, worse EYE-Q scores were seen in children with worsening eye involvement based on laterality (p<0.001). Similar findings were noted on the second administration of the EYE-Q. There was no significant difference in the PedsQL based on laterality or VI.
Table 4.
Association between quality of life measures (mean (SD)) and vision impairment based on visual acuity and laterality
| VA1 in Better Eye* | N (%) | EYE-Q2 (0–4) | PedsQL3 Total (0–100) |
|---|---|---|---|
| Normal 20/20 to <20/40 | 94 (78.3) | 3.65 (0.37) | 80.0 (14.4) |
| Mild vision impairment (VI) 20/40 to 20/60 | 11 (9.2) | 2.99 (0.62) | 72.3 (18.5) |
| Moderate VI 20/70 to <20/200 | 8 (6.7) | 2.83 (0.48) | 78.5 (18.9) |
| Severe VI 20/200 or worse | 7 (5.8) | 2.16 (1.14) | 76.5 (12.0) |
| All subjects | 120 | 3.45 (0.63) | 79.0 (15) |
| Laterality of Vision Impairment** | |||
| Bilateral Normal 20/20 to 20/40 | 56 (46.7) | 3.71 (0.30) | 82.4 (12.3) |
| Bilateral Mild VI 20/40 to 20/60 | 6 (5.0) | 2.96 (0.68) | 75.5 (17.7) |
| Bilateral Moderate VI 20/70 to <20/200 | 8 (6.7) | 2.69 (0.45) | 73.2 (22.4) |
| Bilateral Severe VI 20/200 or worse | 7 (5.8) | 2.16 (1.14) | 76.5 (12.0) |
| Unilateral Mild VI 20/40 to 20/60 in worse eye, <20/40 in better eye | 19 (15.8) | 3.52 (0.56) | 76.7 (20.2) |
| Unilateral Moderate VI 20/70- to <20/200 in worse eye, <20/40 in better eye | 8 (6.7) | 3.72 (0.33) | 71.0 (11.7) |
| Unilateral Severe VI >20/200 in worse eye, <20/40 in better eye | 12 (10.0) | 3.58 (0.33) | 80.9 (11.9) |
Best corrected visual acuity;
Effects of youngsters’ eyesight on quality of life;
Pediatric quality of life inventory
Correlation between VA in better eye and 1) EYE-Q - p<0.001, 2) PedsQL - p = 0.425 using ANOVA
Correlation between laterality of VI and 1) EYE-Q - p<0.001, 2) PedsQL - p = 0.286 using ANOVA
DISCUSSION
VI can have a negative impact on a child’s daily life in relation to school, home, and social activities. VI may also lead to poor QOL in children with JIA-U since adults with uveitis have been noted to suffer from poor QOL (10, 13, 24, 25). Although 65% of children with JIA-U are asymptomatic in early disease, up to 45% have ocular complications at their initial ophthalmology examination (7). A preliminary study reports the use of the EYE-Q in children with JIA-U as a valid measure of VRQOL and demonstrates that VI is an important component of QOL in children with uveitis (17). Data from our study suggests that our EYE-Q has construct- and criterion-related validity and test-retest reliability.
VI is expected to worsen an individual’s QOL. Using the adult based Low Vision Quality of Life Questionnaire, children with VI had significantly lower QOL scores than children with normal eyes (12). The EYE-Q was able to detect differences in children with VI as measured by VA. Of even greater importance, VRQOL appeared to worsen as VI became more severe since EYE-Q scores decreased accordingly (lower scores, worse outcome). This provides evidence for construct validity since the EYE-Q identifies differences in normal and impaired eyes, and differences in the severity of VI.
Bilateral eye disease results in poorer VRQOL which is supported by data from our study. Lower EYE-Q scores were significantly associated with bilateral eye disease and distinguished the severity of bilateral involvement. Although there were significant differences in the EYE-Q scores (p<0.001), children with unilateral eye involvement did not appear to have worsening EYE-Q scores compared to children with bilateral involvement. Intuitively, one would expect that having one normal eye should adequately compensate for a visually impaired eye and allow a child to participate in normal activities without difficulty. This was demonstrated in our study. Despite severe impairment in the worse eye (20/200 or worse), as long as the better eye was normal, VRQOL and overall QOL scores did not appear to differ in the unilateral groups. This provides further support for construct validity of the EYE-Q.
A study by Wong, et. al. showed that healthy adolescents with VI had statistically significant but not clinically impaired health related QOL as measured by the PedsQL, and that refractive errors did not have an impact on overall QOL (11). In our study, the EYE-Q had significant mild correlations with the PedsQL and moderate correlations with VA and CS. The PedsQL was not significantly associated with VA. This indicates that although the PedsQL is a valid and reliable measure of overall QOL (23) it is a relatively poor measure of QOL affected by vision, hence, a measure that is specific to vision is important. Furthermore, the moderate, but not strong, correlations between the EYE-Q and VA and CS denotes that the EYE-Q may measure other components of VI that the clinical measures overlook. These findings emphasize the importance of tools specific to the measurement of VRQOL. Therefore, the EYE-Q may be an important screening tool for eye disease and also an important measure of VRQOL. However, another consideration is that the moderate, and not strong, correlation between the EYE-Q and the clinical measures was secondary to the insensitivity of the tool’s ability to comprehensively measure all aspects of VRQOL and the need for additional items with increased sensitivity to VRQOL and visual ability.
Overall, the EYE-Q demonstrated excellent internal consistency (Cronbach’s α= 0.9). A reduction in items led to a 16 item questionnaire with strong internal consistency (Cronbach’s α= 0.879) which may be appropriate for in a general ophthalmology population. However, further validation studies in a population of children with uveitis would be necessary to determine whether there is item redundancy and a need for item reduction for this group.
Outcomes and QOL studies in JIA focus on physical disability secondary to arthritis and largely overlook impairment from eye disease (15). Visual outcome has been measured by VA and presence of protein flare or cells during the ophthalmologic exam. However, it is important to increase our understanding of the role that VRQOL plays in children with JIA-U in order to better assess patient centered outcomes and the global effects of VI (psychological, physical, mental, social function). Incorporating measures of vision-specific QOL may improve measurement of outcomes, complement ophthalmologic exams and global health measures, and augment the assessment of the impact and utility of treatment (25–27).
There is a striking paucity of validated measurement tools for VRQOL in children; hence, it is important to develop instruments that correlate clinical measures of vision and functional ability, which allow us to determine the impact of visual dysfunction, and that help direct treatment and services. The adult based NEI-VFQ is a well validated measure of vision related QOL but may be inappropriate for younger children since many items are specific to adult activities (i.e. hobbies such as using hand tools or fixing things around the house, reading medicine bottles or legal forms, figuring out the accuracy of bills, or shaving and makeup use). Likewise, there are five published questionnaires that assess visual function in children. The Children’s Visual Function Questionnaire (CVFQ) is a parent-administered measure for young children ≤7 years old that focuses on: 1) general health, 2) general vision, 3) competence - the ability to perform ordinary, “everyday” tasks, 4) personality- effect on the child’s social behavior and personality, 5) family impact – effect on the parents or the rest of the family, in terms of stress and worry and 6) treatment – effect of treatment on child and family (19). The LV-Prasad Questionnaire (LVPQ) assesses the self-reported functional abilities of visually impaired children who are 8 – 18 years old in India (18). Nineteen items assess difficulty in performing tasks in relation to distance, near, and color vision, and visual field, and an item on the child’s global self-assessment of vision. The Cardiff Visual Ability Questionnaire for Children (CVAQC) measures the visual disability of children 5–18 years old, and the Impact of Vision Impairment for Children (IVI_C) measures the impact of VI on QOL in 8–18 year olds (28, 29). The Vision-related Quality of Life of Young Children and Young People (VQoL_CYP) is limited for use in those who are 10–15 years of age (30). These are promising instruments that are undergoing further validation but are not specific to JIA-U. Some of the items may not apply to children in the US because of differences in the US, UK, and Australian educational structures and content. Therefore, our child-specific comprehensive EYE-Q may offer significant improvements over existing measures and also over tools for adults (14, 18, 19, 28, 29, 31).
Several limitations to this study are present. The first is that a relatively small number of children were assessed, and although 50% had normal vision, the remaining 50% had varied VI. A second limitation is the use of the Snellen chart to measure distance vision instead of the ETDRS (Early-Treatment Diabetic Retinopathy Study) acuity which is the gold standard in ophthalmologic clinical trials (32). However, our data is analyzed using a range of BCVA which would allow for small differences in measurement, and the ETDRS acuity is not used routinely in pediatric ophthalmology clinics. Third, there was a small sample of children 16–18 years old (11.7%). However, this is one of many steps needed to develop an instrument specific to children with JIA-U. Prior to focusing this instrument on this population, it is important to determine whether this tool is relevant for children with and without ocular disease, and with diverse VI.
Our future studies aim to administer this instrument to a larger number of children and validate its’ use specifically for those with uveitis. We aim to create a parent proxy module to compare to the self report and to have available for children who are unable to complete their own instruments for whatever reasons and who are younger than 8 years old. Furthermore, we plan to assess a more comprehensive role for the EYE-Q in looking at the longitudinal course of JIA-U.
The continued improvement of our outcome measure, the EYE-Q, will help us better determine the effects of uveitis, complications, and treatment. This is especially important in children with JIA-U since measures of visual function have depended primarily on the clinical exam. Development of a child-friendly, reliable and valid instrument that assesses visual functioning within the context of QOL would enhance our understanding of the impact of complex, multi-system childhood rheumatic diseases on a child’s QOL and provide an opportunity for us to explore the impact of vision QOL on other complex diseases that impair vision.
Significance and Innovation.
Studies that examine quality of life (QOL) and outcomes in JIA focus on physical function assessments. This may underestimate QOL as its measurement focuses on musculoskeletal function secondary to arthritis, and not the impact of uveitis and the contribution of visual disability to QOL.
There is a paucity of instruments that measure visual disability and vision related QOL (VRQOL) in children. There are none that are specific for children with uveitis which would make the assessment of QOL related to visual disability difficult in this population. The EYE-Q could serve as a validated instrument that measures VRQOL in children with uveitis.
In JIA-U, clinical measures are used to evaluate outcomes, hence the need for a more comprehensive evaluation which takes into account the child’s perspective. The EYE-Q could complement ophthalmologic exams and global health measures and augment the assessment of the impact and utility of treatment.
Acknowledgments
Grant Support: Arthritis Foundation - New York Chapter WCMC CTSC, UL1RR024996 Research to Prevent Blindness Emory Egleston Children’s Research Center Knights Templar Eye Foundation, Inc
Footnotes
Conflict of Interest: Dr. Sunil K Srivastava is on the advisory board for Allergan Inc, Bausch and Lomb and Alimera. Dr. Melanie J Harrison is an employee of Celgene Corporation. However, all work presented in this manuscript was conducted outside her current employment and was not supported by Celgene Corporation.
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