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INTRODUCTION

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Measures of physical function/disability and health-related quality of life (HRQOL) have become critical determinants of outcomes for patients with pediatric-onset systemic lupus erythematosus (SLE) (1–3). HRQOL has been defined as a “multi-dimensional concept that includes the physical, psychological and social functioning associated with an illness or its treatment” (4). At least one measure of HRQOL has been included in recent studies aimed to develop a core set of variables to assess flare criteria and response to therapy in pediatric SLE (5–7). The Childhood Health Assessment Questionnaire, which is a measure of physical function/disability, and several HRQOL instruments (Child Health Questionnaire, Pediatric Quality of Life Inventory Generic Core Module, Pediatric Quality of Life Inventory Rheumatology Module, and Simple Measure of Impact of Lupus Erythematosus in Youngsters) have been validated in pediatric SLE, and will be discussed here. This review includes both a description of the measures' content as well as their psychometric properties as it relates to pediatric SLE.

CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Description

Purpose.

The C-HAQ measures functional health status for pediatric patients 6 months to 18 years of age with a chronic rheumatic disease. The original publication in 1994 described validation of the C-HAQ for patients with juvenile idiopathic arthritis (JIA) (8). Revised versions of the C-HAQ have been suggested for JIA (9, 10); this review pertains to the original version of the C-HAQ.

Content.

The C-HAQ assesses disability in 8 domains, including dressing and grooming, arising, eating, walking, hygiene, reach, grip, and activities. The C-HAQ also includes visual analog scale (VAS) scores for pain and overall well-being.

Number of items.

In the disability index, there are 30 items in 8 categories. Each category has 2–5 component items. In addition, questions are asked about assistive devices or personal aids needed to perform the 30 functions. The pain and overall well-being scales are individual questions.

Response options/scale.

For each category in the disability index, the respondent selects the amount of difficulty the child may have with a particular task as a result of their condition. Each item is rated from 0–3 with 0 = “without any difficulty,” 1 = “with some difficulty,” 2 = “with much difficulty,” and 3 = “unable to do.” If the item is not applicable to the subject (i.e., they would not be expected to perform that particular task due to young age), then the parent would select “Not applicable.” The highest score for any component question within a category determines the score for the category. Use of assistive devices or a personal aide automatically increases the score to a 2. If a component score is left blank, the score for that category is determined by the remaining completed questions. If all component questions are left blank, that category is left blank. Pain severity is measured on a VAS with 0 = no pain and 100 = very severe pain, and a score from 0–3 is determined based on the location of the respondent's mark. Well-being (“rate how your child is doing”) is measured on a VAS scale with 0 = very well and 100 = very poor.

Recall period for items.

Questions pertain to the week preceding the assessment.

Practical Application

How to obtain.

A copy of the C-HAQ can be obtained at the following URL: http://aramis.stanford.edu/index.html.

Method of administration.

Interview (in person or telephone) or self-administered by either the child or the parent. Moderate correlations between child and parent reports have been demonstrated in pediatric systemic lupus erythematosus (SLE) (11).

Scoring.

There are specific scoring instructions for the C-HAQ.

Score interpretation.

The C-HAQ score is calculated as the mean of the 8 category scores in the disability index. Scores range from 0–3; higher scores reflect more disability. There are no normative values for the C-HAQ in healthy children. The minimum clinically important differences (MCIDs) for the C-HAQ in patients with JIA ranged between a score improvement of −0.188 and a score worsening of +0.125 (12).

Respondent burden.

The C-HAQ takes <10 minutes to complete.

Administrative burden.

The C-HAQ takes <10 minutes to administer and <5 minutes to score.

Translations/adaptations.

The C-HAQ has been cross-culturally adapted and validated in multiple languages.

Psychometric Information

Method of development.

The C-HAQ was adapted from the adult Stanford Health Assessment Questionnaire (8). There is at least 1 question per domain relevant to children of all ages. The face validity of the instrument was evaluated by a group of 20 health professionals and the parents of 22 healthy children.

Acceptability, reliability, and validity in pediatric SLE.

A cross-sectional study of 24 pediatric SLE patients assessed the correlation between C-HAQ scores and disease activity utilizing the SLE Disease Activity Index (SLEDAI) and Physician's Global Assessment (PGA). The mean ± SD child-report C-HAQ score was 0.35 ± 0.35 (median 0.3), and the mean ± SD parent-report score was 0.14 ± 0.2 (median 0) (11). The median SLEDAI score for the cohort was 4; both the SLEDAI and C-HAQ exhibited a floor effect. The C-HAQ correlated moderately with the SLEDAI (ρ = 0.4, P = 0.04) but did not correlate with PGA. The C-HAQ was also validated in a study of 504 patients with active pediatric SLE who were assessed at baseline (prior to major therapeutic intervention) and then at 6-month followup (13). Mean parent-report C-HAQ scores were 0.83 ± 0.94 at baseline and 0.19 ± 0.43 at followup. In this cohort, subjects had a high degree of disease activity as evidenced by a mean ± SD SLEDAI score of 18.12 ± 10.14 at baseline; SLEDAI scores improved to 6.21 ± 6.46 at followup. Several measures of disease activity, including laboratory parameters and HRQOL, were collected at the 2 time points. In evaluating reliability, the C-HAQ demonstrated excellent internal consistency in this cohort (Cronbach's α = 0.96). With regard to construct validity, there was a moderate correlation for the absolute change from baseline to 6 months (Spearman's correlation coefficient 0.4–0.7) between the C-HAQ and the Child Health Questionnaire (CHQ) physical health score, the parent's global assessment of pain and overall well-being, and the Systemic Lupus Activity Measure. There was poor correlation (Spearman's correlation coefficient of <0.4) with PGA, European Consensus Lupus Activity Measure, 24-hour proteinuria, SLEDAI, and CHQ psychosocial health scores.

Ability to detect change.

In this study of 504 patients with active SLE, the standardized response mean for the C-HAQ was moderate at 0.74. The C-HAQ demonstrated a significant ability (P < 0.0001) to discriminate between subjects who were improved and those who were not improved at 6 months based on the Paediatric Rheumatology International Trials Organisation/American College of Rheumatology juvenile SLE definition of improvement.

Critical Appraisal of Overall Value to the Rheumatology Community

Strengths.

The C-HAQ is easy to administer and is the most widely used measure of health status, physical function, and disability for patients with a chronic rheumatic disease. With regard to pediatric SLE, the C-HAQ has excellent responsiveness over time, particularly among patients with more active SLE.

Caveats and cautions.

The C-HAQ focuses on physical function, and may fail to capture other physical symptoms and dimensions of SLE activity that affect health status (e.g., fatigue). In addition, the C-HAQ has been criticized for its focus on “disability” versus “ability.”

Clinical usability.

The C-HAQ has a significant floor effect, particularly among patients with less active SLE, limiting its clinical utility. In addition, the MCID for pediatric SLE has not been established.

Research usability.

The C-HAQ has demonstrated a reasonable ability to discriminate between patients who have and have not achieved clinical improvement, making it a useful tool in a research setting.

CHILD HEALTH QUESTIONNAIRE (CHQ)

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Description

Purpose.

Modeled after the adult Short Form 36, the CHQ is a generic measure of pediatric health-related quality of life (HRQOL) designed to measure the physical, emotional, and social components of health. The original manuscript describing the development of the CHQ was published in 1998 (14).

Content.

The CHQ comprises 14 domains, including physical functioning, bodily pain or discomfort, general health, change in health, limitations in schoolwork and activities with friends, mental health, behavior, self-esteem, family cohesion, limitations in family activities, and emotional or time impact on the parent (15).

Number of items.

The parent form is available in a 50- (PF-50) or 28-item (PF-28) version. The child self-report version (CHQ-CF87) consists of 87 items and is for children ≥10 years of age. From the PF-50, physical health (PhS) and psychosocial health (PsS) summary scores can be derived.

Response options/scale.

Each item is scored on a Likert-type scale with higher scores indicating better or more positive health states.

Recall period for items.

The change in health subscale is “compared to last year” and no recall period is used for the general health and family cohesion subscales, otherwise the subscales refer to the preceding 4-week period.

Practical Application

How to obtain.

A copy of the CHQ can be obtained at the following URL: http://www.healthact.com/survey-chq.php.

Method of administration.

Self-administered by the child or a parent.

Scoring.

The CHQ can be hand scored or a computer scoring program can be used.

Score interpretation.

Scores for each subscale range from 0–100, with higher scores reflecting better health status. These scores are standardized with a mean ± SD of 50 ± 10. Higher scores indicate higher HRQOL. Normative values for the different versions of the CHQ, and for different populations (e.g., US, Italy, Mexico), are published (16–18). The mean ± SD national norms for the CHQ PhS is 53.00 ± 8.8 and the PsS is 51.20 ± 9.1 for a population sample of US children. Although the minimal clinically important difference for the CHQ PhS has not been established in pediatric systemic lupus erythematosus (SLE), a single study reported a mean ± SD decrease in CHQ PhS scores among patients who had a disease flare (n = 89 episodes) of −2.35 ± 1.14, while episodes not associated with a flare (n = 438) had an increase in CHQ PhS scores of 0.78 ± 0.51 (P = 0.013) (7).

Respondent burden.

The estimated time to completion depends on the length of the survey (CHQ PF-50, 10–15 minutes; PF-28, 5–10 minutes; and the CHQ-CF87, 16–25 minutes).

Adminstrative burden.

Not reported.

Translations/adaptions.

The CHQ has been validated in multiple languages/countries. A full list of the available translations is available at URL: http://www.healthact.com/translation-chq.php.

Psychometric Information

Method of development.

The CHQ was developed as a part of the Child Assessment Project, an effort that was initiated in 1990 to develop methods for “measuring the physical and psychosocial health status and well-being of children and adolescents” (14).

Acceptablity, reliability, and validity in pediatric SLE.

In a cross-sectional and multinational study, Ruperto and colleagues assessed the HRQOL of 297 pediatric SLE patients utilizing the CHQ (15). For this cohort, the mean ± SD summary PhS score was 40.2 ± 15 and the mean ± SD summary PsS score was 44.8 ± 10.7. These scores were similar to previously reported CHQ scores for patients with juvenile idiopathic arthritis, but significantly below the mean scores for the healthy control populations used in the study. In this cohort, the SLE Disease Activity Index (SLEDAI) score was significantly correlated with the CHQ PhS score (r = −0.29, P < 0.0001) and the CHQ PsS score (r = −0.25, P < 0.0001). The Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) correlated with the CHQ PhS score (r = −0.23, P = 0.0001) but not the CHQ PsS score. In a study examining the relationship between HRQOL and disease course in pediatric SLE, 98 patients were followed every 3 months for up to 18 months (549 total visits) (19). The mean ± SD summary CHQ PhS score was 41.8 ± 12 and the mean ± SD summary CHQ PsS score was 49.2 ± 6.6, which was significantly below the mean scores for the normative population of healthy children. At baseline, the mean ± SD SLEDAI score was 4.8 ± 4.4 and mean ± SD SDI score was 0.42 ± 0.1. To assess the relationship between HRQOL and disease activity, subjects were grouped into 1 of 3 groups of disease activity based on British Isles Lupus Assessment Group (BILAG) score. Subjects with a BILAG score ≤1 had “inactive” or “minimally active” disease, subjects with a BILAG score >1 but ≤5 were classified as “somewhat active” disease, and subjects with a BILAG score >5 had “very active” disease. Higher disease activity (SLEDAI or BILAG score) was associated with lower CHQ PhS and CHQ PsS scores; however, there was no significant difference in CHQ PhS scores between the groups with “somewhat” and “very active disease,” and the CHQ PsS failed to differentiate between the groups of patients with different levels of disease activity. Patients with minimal or absent disease damage (SDI ≤1) had significantly higher CHQ PhS, but not CHQ PsS scores.

Ability to detect change.

In the 3-month interval between study visits, as compared to physician-rated worsening or improvement of disease, the scores of the CHQ PhS changed significantly (P < 0.0005), but the CHQ PsS did not. The standardized response mean (SRM) was <0.4 for both measures. The CHQ PhS SRM improved to 0.57 when correlated to patient-/parent-related worsening of health. Brunner et al included the CHQ PhS as the primary measure of HRQOL in a study designed to validate criteria for the evaluation of response to therapy in pediatric SLE (6). This study included 98 children who were evaluated every 3 months for up to 7 visits (623 total visits). The CHQ PhS was one of the 5 SLE core response variables obtained at each visit. The mean ± SD score at baseline was 42.4 ± 12.14. There was no significant change in the CHQ PhS among the patients who were classified as “improved” during the study time period. In a related study designed to develop flare criteria for pediatric SLE, a combination of physician-rated disease activity, a validated disease activity index (e.g., SLEDAI, BILAG), and change in CHQ PhS (not weighted) were found to be adequate to identify SLE flares in the cohort (area under the curve: 0.81, sensitivity 64%, specificity 86%) (7).

Critical Appraisal of Overall Value to the Rheumatology Community

Strengths.

The CHQ is a comprehensive measure for assessing HRQOL. The PhS and PsS summary scores are useful tools for simplifying the multiple domains measured by the CHQ. In pediatric SLE, the CHQ PhS correlates well with cross-sectional measures of both disease activity and damage, while the CHQ PsS only correlates with disease activity.

Caveats and cautions.

The CHQ PsS does not discriminate well between patients with different levels of disease activity, and is not responsive to worsening or improvement of disease over time. The CHQ PhS appears to be a more accurate measure for discriminating between patients who have had an increase in disease activity (i.e., disease flare) versus those who have a decrease in disease activity.

Clinical usability.

Given its relatively low respondent burden and the response of the CHQ PhS to change in disease activity over time, the CHQ may be a useful tool for measuring HRQOL in a clinical setting.

Research usability.

The CHQ PhS summary score appears to be a useful tool in identifying increased disease activity (i.e., flares) among study subjects with pediatric SLE, so it may be more useful as a research tool in observational studies versus therapeutic trials, where response to therapy is the primary outcome.

PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Description

Purpose.

The PedsQL-GC is a generic pediatric measure of health-related quality of life (HRQOL).

Content.

The questionnaire encompasses 4 health domains: physical functioning, emotional functioning, social functioning, and school functioning.

Number of items.

The questionnaire contains 23 items. Physical and psychosocial health summary scores can be calculated.

Response options/scale.

Items are scored using a 5-point Likert scale (0 = never a problem, 1= almost never a problem, 2 = sometimes a problem, 3 = often a problem, and 4 = almost always a problem).

Recall period for items.

Questions refer to the preceding 4 weeks.

Examples of use.

Disease-specific modules are available for rheumatology, asthma, diabetes mellitus, cancer, and cardiac conditions.

Practical Application

How to obtain.

A copy of the PedsQL-GC can be obtained at the following web site: http://www.pedsql.org/contact.html.

Method of administration.

Includes parallel child/ adolescent self-report (ages 5–18) or parent proxy report (ages 2–18).

Scoring.

There are specific scoring instructions.

Score interpretation.

From the sum of the raw scores from the 23 items, a summary score ranging from 0–100 can be calculated, with higher scores indicating higher HRQOL. Mean ± SD normative values for a population of school-age US children were 80.64 ± 13.34 for the child self-report total score, and 76.92 ± 16.81 for the parent proxy-report total score (20). The minimum clinically important difference for the child self-report score in a diverse pediatric population was a change of 4.4, and for the parent proxy report was a change of 4.5 (21).

Respondent burden.

The survey takes <4 minutes to complete.

Administrative burden.

The time to administer the survey is <10 minutes. The PedsQL-GC is described as “easy to score,” but no specific time period is recorded.

Translation/adaptations.

The PedsQL-GC has been translated into multiple languages, available at URL: http://www.pedsql.org/translations.html.

Psychometric Information

Method of development.

The current PedsQL-GC 4.0 represents the fourth version of the PedsQL-GC tool. It has been field tested with children and adolescents in multiple settings.

Acceptability, reliability, and validity in pediatric systemic lupus erythematosus (SLE).

In a cross-sectional study of 24 patients with pediatric SLE, the mean ± SD summary score for the PedsQL-GC parent proxy report was 69 ± 18 and the mean ± SD child self-report score was 67 ± 20 (11). No significant correlation was found between the PedsQL-GC and measures of disease activity, including the SLE Disease Activity Index (SLEDAI) or Physician's Global Assessment (PGA). Mild correlation was found between the PedsQL-GC and the the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). The corresponding child-parent pair (n = 19) responses were significant for the PedsQL-GC (ρ = 0.7, P = 0.001); the intraclass correlation was 0.7 (confidence interval 0.4–0.8). In a cross-sectional study of 98 pediatric SLE patients designed to assess HRQOL and its relationship to disease activity, the mean ± SD summary score for the PedsQL-GC parent proxy report was 74.6 ± 16.7 and the mean ± SD child self-report score was 78.1 ± 15; both scores were significantly lower than the mean for the normative population (US population sample of healthy children) (19). Higher disease activity, as measured by the British Isles Lupus Assessment Group (BILAG), was associated with lower PedsQL-GC scores (P < 0.05). With regard to disease damage, patients with minimal or no disease damage (SDI score ≤1) had higher PedsQL-GC scores than patients with more than minimal disease damage (SDI score >1; P < 0.05).

Ability to detect change.

With assessment of change in disease status over time by either physician-rated worsening/improvement or parent-/patient-rated change in health, there was no significant change in PedsQL-GC parent proxy-report scores and patient self-report scores. The standardized response mean was <0.4, indicating a moderate response.

Critical Appraisal of Overall Value to the Rheumatology Community

Strengths.

The PedsQL-GC is a relatively brief and easily administered generic tool for measuring HRQOL.

Caveats and cautions.

The PedsQL-GC scores correlate with BILAG and SDI scores, but not with SLEDAI scores or PGA, and it may limit the ability to assess change in disease status over time.

Clinical usability.

The PedsQL-GC may be useful in the clinical setting because of the low respondent burden; however, its usefulness in measuring HRQOL over time is unclear.

Research usability.

Given its limited ability to assess change in disease activity over time, the PedsQL-GC summary score may have more limited utility in the research setting.

PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Description

Purpose.

The PedsQL-RM is used in combination with the Pediatric Quality of Life Inventory Generic Core Module (PedsQL-GC), and is a pediatric rheumatology–specific measure of health-related quality of life (HRQOL).

Content.

The PedsQL-RM is a brief parallel patient- and parent-report questionnaire designed for children ages 2–18 that encompasses 5 domains: pain and hurt, daily activities, treatment, worry, and communication. Parent report of the toddler age group (2–4 years) does not include a worry and communication domain.

Number of items.

The PedsQL-RM 3.0 is a 22-item questionnaire.

Response options/scale.

Items are scored using a 5-point Likert scale (never, almost never, sometimes, often, and always).

Recall period for items.

Questions refer to the preceding 4 weeks.

Practical Application

How to obtain.

A copy of the PedsQL-RM can be obtained at the following web site: http://www.pedsql.org/contact.html.

Method of administration.

Child self-report (ages 5–18) or parent proxy-report (ages 2–18) questionnaire.

Scoring.

Items are scored on a 5-point Likert scale (never, almost never, sometimes, often, and always).

Score interpretation.

From the sum of the raw scores from the 22 items, a summary score ranging from 0–100 can be calculated, with higher scores indicating higher HRQOL. Normative values are available for patients with juvenile idiopathic arthritis (JIA) (22).

Respondent burden.

Time to complete is <4 minutes.

Administrative burden.

Time to administer is <10 minutes. Time to score is not reported.

Translations/adaptations.

The PedsQL-RM has been translated into multiple languages, available at URL: http://www.pedsql.org/translations.html.

Psychometric Information

Method of development.

PedsQL-RM was designed to measure pediatric rheumatology–specific HRQOL. The original study designed to demonstrate the reliability, validity, and responsiveness of the PedsQL-RM included 231 children and 244 parents recruited from a pediatric rheumatology clinic (23).

Acceptability, realiability, and validity in pediatric systemic lupus erythematosus (SLE).

In a cross-sectional study of 24 patients with pediatric SLE, PedsQL-RM summary scores were not reported but the mean ± SD child self-report and parent proxy-report means for each of the subscales were as follows: daily activities, 92 ± 13 and 95 ± 9; treatment, 84 ± 13 and 76 ± 19; pain and hurt, 66 ± 22 and 68 ± 24; communication, 63 ± 30 and 65 ± 40; and worry, 56 ± 34 and 52 ± 28, respectively (11). The only significant correlation with disease activity was between the parent-report worry subscale and the SLE Disease Activity Index (SLEDAI; ρ = 0.53, P = 0.02). No correlation was found with disease damage. Correlations between the corresponding child-parent pair (n = 19) responses were significant for the worry (ρ = 0.5, P = 0.05) and pain and hurt domains (ρ = 0.55, P = 0.02). The intraclass correlation was 0.5 (confidence interval 0.04–0.7). In a cross-sectional study of 98 pediatric SLE patients designed to assess HRQOL and its relationship to disease activity, the mean ± SD summary score for the PedsQL-RM parent proxy report was 79.4 ± 14.3 and the mean ± SD child self-report score was 80.8 ± 14.1; although both scores were lower than the mean for the normative population (children with JIA), the differences were not statistically significant (19). To assess the relationship between HRQOL and disease activity, subjects were grouped into 1 of 3 groups of disease activity based on British Isles Lupus Assessment Group (BILAG) scores. Higher disease activity was associated with lower PedsQL-RM scores, although the difference in scores between the somewhat active and very active disease groups for the PedsQL-RM child self-report did not reach significance. With regard to disease damage, patients with minimal or no disease damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index [SDI] score ≤1) had higher PedsQL-RM scores than patients with more than minimal disease damage (SDI score >1).

Ability to detect change.

There was a significant change with the PedsQL-RM parent proxy-report scores and child self-report scores regardless of the method used to assess change in health status (physician-rated worsening/improvement or parent-/patient-related change in health). The standardized response mean was <0.4, indicating a moderate response.

Critical Appraisal of Overall Value to the Rheumatology Community

Strengths.

The PedsQL-RM appears to have both concurrent and construct validity, and is more responsive than the PedsQL-GC to clinically important changes in pediatric SLE.

Caveats and cautions.

The PedsQL-RM summary scores correlate with BILAG and SDI scores, but not with SLEDAI scores or physician's global assessment. The PedsQL-RM does not distinguish between the highest levels of disease activity on the BILAG.

Clinical usability.

The PedsQL-RM is quick and easy to complete, making it a potentially useful measure in the clinical setting.

Research usability.

The Peds QL-RM has favorable psychometric properties in pediatric SLE and may be a useful tool to assess response to therapy in observational and clinical trials.

SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Description

Purpose.

The SMILEY is the only disease-specific measure of health-related quality of life (HRQOL) in pediatric systemic lupus erythematosus (SLE).

Content.

The survey captures 4 domains: effect on self (5 items), limitations (8 items), social (4 items), and burden of SLE (7 items).

Number of items.

The SMILEY is a 26-item survey. The first 2 survey items are summary questions that are not included in the final score. Item 1 relates to current HRQOL status and item 2 relates to current SLE status.

Response options/scale.

Responses are in the form of a pictorial 5-step scale with different facial expressions.

Recall period for items.

Responses apply to the previous month.

Practical Application

How to obtain.

Contact the developer: L. Nandini Moorthy, MD, MS, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, New Brunswick, New Jersey.

Method of administration.

The SMILEY is a self-administered questionnaire for children with SLE <19 years of age, and is completed by both the parent and the child.

Scoring.

There are specific scoring instructions. If more than 12 questions are not answered, SMILEY cannot be scored.

Score interpretation.

All items, including the first 2 summary questions, score from 1–5. The total score is transformed to a 1–100 scale. Higher scores reflect higher quality of life. Because this is a disease-specific tool, there are no normative values for the SMILEY.

Respondent burden.

SMILEY is at the fifth-grade reading level and takes <10 minutes to complete.

Administrative burden.

SMILEY takes 10 minutes to administer and ≤10 minutes to score.

Translations/adaptations.

The SMILEY has been translated into multiple languages (24).

Psychometric Information

Method of development.

The 5-step scale used in SMILEY was modified with permission from the Wong-Baker FACES Pain Rating Scale. Children with SLE and their parents were involved in the different states of development of SMILEY (25).

Acceptability, reliability, and validity in pediatric SLE.

SMILEY was validated in a cohort of 86 pediatric SLE patients (26). In this cohort, the mean ± SD child-report score was 65 ± 13 (range 37–93) and the mean ± SD parent-report score was 62 ± 16 (range 28–98). The median SLE Disease Activity Index (SLEDAI) was 4 (range 0–23) and the median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) was 1 (range 0–10). The interrater reliability for total score via intraclass correlation was 0.7–0.9 for both the parent and child total scores. Cronbach's alpha for internal consistency was 0.9 for both parent and child total scores. For the SMILEY child and SMILEY parent, correlation with other HRQOL measures were as follows: Childhood Health Assessment Questionnaire, r = 0.6 and r = 0.5; global quality of life (QOL), r = 0.5 and r = 0.6; and Pediatric Quality of Life Inventory Generic Core Module (PedsQL-GC), r = 0.6 and r = 0.6, respectively. For the Pediatric Quality of Life Inventory Rheumatology Module, the correlation for the SMILEY child report and SMILEY parent report by domain were: pain and hurt, r = 0.5 and r = 0.5; daily activities, r = 0.4 and r = 0.4; treatment, r = 0.5 and r = 0.6; worry, r = 0.6 and r = 0.5; and communication, r = 0.5 and r = 0.5, respectively. All P values were ≤0.001. Significant Spearman's correlations (r ≥ 0.4) were seen between child and parent total SMILEY scores and items 1–3 and 5–6 of the impact scale of the PedsQL-GC family information form, the self-concept scale, self-perceived global QOL, and self-perceived global SLE status. The child SMILEY limitation domain had mild correlation (r = 0.3) with physician's global assessment (PGA), SLEDAI (r = 0.2), and SDI (r = 0.2). There was no significant correlation with the total child and parent SMILEY scores and SLEDAI, PGA, SDI, or disease duration. SMILEY total and domain scores were higher in subjects with lower SLEDAI scores, lower PGA scores, lower SDI scores, and in those who had never used immunomodulatory therapy, including cyclophosphamide.

Ability to detect change.

In a longitudinal study, 68 pediatric SLE patients were assessed at baseline and 52 patients (76%) were assessed at followup (27). There were no significant difference in SLEDAI or SDI scores between the 2 time points. With regard to the child-report SMILEY, changes in the total scores correlated with changes in patient/parent assessment of global HRQOL (r = 0.3, P = 0.02), patient/parent assessment of SLE status (r = 0.4, P = 0.002), SLEDAI (r = −0.3, P = 0.01), and SDI (r = −0.4, P = 0.005). Changes in SLEDAI and SDI corresponded most strongly with changes in the “burden of SLE” domain. Changes in the parent-report SMILEY scores correlated with changes in the patient/parent assessment of global HRQOL (r = 0.3, P = 0.02), patient/parent assessment of SLE status (r= 0.4, P = 0.002), and SDI (r = −0.3, P = 0.05). Changes with SDI correlated with the limitation domain. Changes in parent-report SMILEY total and domain scores did not correlate with changes in PGA and SLEDAI scores.

Critical Appraisal of Overall Value to the Rheumatology Community

Strengths.

SMILEY is the only HRQOL measurement tool designed specifically for patients with pediatric SLE. The use of the FACES scale may make it more accessible for use with younger patients.

Caveats and cautions.

The total parent and child SMILEY scores exhibit mild/moderate correlation with markers of disease activity and damage, potentially limiting its ability to predict a change in disease activity. Additional studies are needed to evaluate the performance of the SMILEY over time.

Clinical usability.

The SMILEY is reasonably short and straightforward, making it feasible for use in clinical practice.

Research usability.

The SMILEY has very good psychometric properties; the burden of SLE and limitation domains may be particularly useful in measuring response to therapy in clinical trials.

DISCUSSION

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES

Measures of physical function and health-related quality of life (HRQOL) should be included in the assessment of short- and long-term outcomes of systemic lupus erythematosus (SLE). Although several measures exist, they vary in their correlation with and their ability to predict change in disease activity over time. The Childhood Health Assessment Questionnaire is the primary measure used to assess physical function and disability in pediatric SLE, but its utility is limited due to its floor effect and its emphasis on SLE symptoms related to arthritis. Multiple measures have been used to assess HRQOL in pediatric SLE; it has been proposed that the Child Health Questionnaire physical health summary score be included in criteria for measuring global SLE flare. SMILEY, which is the only disease-specific measure of HRQOL, appears to be an effective tool for measuring multidimensional HRQOL in pediatric SLE.

Table  . Summary Table for Measures of Health-Related Quality of Life in Pediatric Systemic Lupus Erythematosus*
ScalePurpose/contentMethod of administrationRespondent burdenAdministrative burdenScore interpretationReliability evidenceValidity evidenceAbility to detect changeStrengthsCautions
  • *

    PhS = physical health; SLAM = Systemic Lupus Activity Measure; SRM = standardized response mean; SLE = systemic lupus erythematosus; HRQOL = health-related quality of life; PsS = psychosocial health; BILAG = British Isles Lupus Assessment Group index; SDI = Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index; SLEDAI = Systemic Lupus Erythematosus Disease Activity Index; PGA = physician's global assessment; ICC = intraclass correlation coefficient.

Childhood Health Assessment Questionnaire (C-HAQ)Measure of disability/health statusChild/parent self- or interviewer-administered<10 minutes to complete<10 minutes to administer; <5 minutes to scoreRange 0–3; higher scores indicate higher disabilityExcellent internal consistency (Cronbach's α = 0.96)Moderate correlation with the CHQ PhS, parent's global assessment of pain and overall well-being, and SLAMModerate SRM of 0.74Easy to administer; widely used; excellent responsiveness over timeLimited to assessing physical disability; significant floor effect with less active SLE
Child Health Questionnaire (CHQ)Generic measure of pediatric HRQOLSelf-administered by child or parent50-item CHQ Parent Form takes 10–15 minutes to completeNot reportedScores for each subscale range from 0–100; higher scores indicate better health status; mean ± SD score is 50 ± 10Not specifically tested in pediatric SLECHQ PhS (physical domain) score correlates well with measures of disease activity and damage; CHQ PsS (psychosocial domain) score only correlates with disease activityThe CHQ PhS changes with disease activity while CHQ PsS does not; SRM for both is <0.4Comprehensive measure for assessing HRQOL; summary scores are useful; CHQ PhS subscale may be useful in predicting increased disease activityCHQ PsS does not discriminate between levels of disease activity or damage
Pediatric Quality of Life Inventory Generic Core Module (PedsQL-GC)Generic measure of pediatric HRQOLSelf-administered by child or parent<4 minutes to complete<10 minutes to administer; reportedly easy to scoreSummary score ranging from 0–100; higher scores indicate higher HRQOLNot specifically tested in pediatric SLECorrelates with BILAG and SDI, but not with SLEDAI or PGANo significant change in the PedsQL-GC with change in disease activity over time; SRM <0.4Brief and easily administeredInconsistent correlation with measures of disease activity; limited ability to assess disease activity over time
Pediatric Quality of Life Inventory Rheumatology Module (PedsQL-RM)Pediatric rheumatology– specific measure of HRQOLSelf-administered by child or parent<4 minutes to complete<10 minutes to administerSummary score ranging from 0–100; higher scores indicate higher HRQOLNot specifically tested in pediatric SLECorrelates with BILAG and SDI, but not with SLEDAI or PGASignificant change with change in health status; SRM <0.4Brief and easily administered; correlates with changes in health status over timeMay not distinguish well between higher levels of disease activity
Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY)Pediatric SLE–specific measure of HRQOLSelf-administered by child or parent<10 minutes to complete; fifth-grade reading level10 minutes to administer; <10 minutes to scoreTotal score is transformed to a 1–100 scale; higher scores reflect higher HRQOLHigh interrater reliability (ICC 0.7–0.9); high internal consistency (Cronbach's α = 0.9 for both child and parent scores)Moderate correlation of the total child and parent score with C-HAQ, global quality of life, PedsQL-GC, and domains of the PedsQL-RM; child SMILEY limitation domain had mild correlation with PGA, SLEDAI, and SDIChanges in the child report SMILEY and particularly the burden of SLE domain correlated best with measures of disease activity and damage over timeOnly disease-specific measure for pediatric SLE; excellent evidence of reliability and validityTotal child and parent scores may not adequately predict change in disease activity over time

REFERENCES

  1. Top of page
  2. INTRODUCTION
  3. CHILDHOOD HEALTH ASSESSMENT QUESTIONNAIRE (C-HAQ)
  4. CHILD HEALTH QUESTIONNAIRE (CHQ)
  5. PEDIATRIC QUALITY OF LIFE INVENTORY GENERIC CORE MODULE (PedsQL-GC)
  6. PEDIATRIC QUALITY OF LIFE INVENTORY RHEUMATOLOGY MODULE (PEDSQL-RM)
  7. SIMPLE MEASURE OF IMPACT OF LUPUS ERYTHEMATOSUS IN YOUNGSTERS (SMILEY)
  8. DISCUSSION
  9. AUTHOR CONTRIBUTIONS
  10. REFERENCES
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