DEVELOPMENTAL MEDICINE & CHILD NEUROLOGY REVIEW Quality of life instruments for children and adolescents with neurodisabilities: how to choose the appropriate instrument | ELISE DAVIS PHD 1 | GABRIEL M RONEN MD MSC 2 | PETER ROSENBAUM MD 2 | MICHAEL LIVINGSTON BASC 3 | SAROJ SAIGAL MD 2 ELIZABETH WATERS MPH DPHIL 1 1 McCaughey Centre, VicHealth Centre for the Promotion of Mental Health and Community Wellbeing, School of Population Health, University of Melbourne, Melbourne Australia. 2 Department of Pediatrics, Faculty of Health Sciences, McMaster University, Hamilton, Ontario, Canada. 3 University of Western Ontario, Canada. Correspondence to Dr Elise Davis, Senior Research Fellow, McCaughey Centre: VicHealth Centre for the Promotion of Mental Health and Community Wellbeing, 207 Bouverie Street, University of Melbourne, Victoria 3010, Australia. E-mail: [email protected] ABBREVIATIONS CHQ HRQoL PedsQL QoL Child Health Questionnaire Health-related quality of life Pediatric Quality of Life Inventory Quality of life AIM There are many misconceptions about what constitutes ‘quality of life’ (QoL). It is often difficult for researchers and clinicians to determine which instruments will be most appropriate to their purpose. The aim of the current paper is to describe QoL instruments for children and adolescents with neurodisabilities against criteria that we think are important when choosing or developing a QoL instrument. METHOD QoL instruments for children and adolescents with neurodisabilities were reviewed and described based on their purpose, conceptual focus, origin of domains and items, opportunity for self report, clarity (lack of ambiguity), potential threat to self-esteem, cognitive or emotional burden, number of items and time to complete, and psychometric properties. RESULTS Several generic and condition-specific instruments were identified for administration to children and adolescents with neurodisabilities – cerebral palsy, epilepsy and spina bifida, and hydrocephalus. Many have parent-proxy and self-report versions and adequate reliability and validity. However, they were often developed with minimal involvement from families, focus on functioning rather than well-being, and have items that may produce emotional upset. INTERPRETATION As well as ensuring that a QoL instrument has sound psychometric properties, researchers and clinicians should understand how an instrument’s theoretical focus will have influenced domains, items, and scoring. The concept of ‘good health’ now embraces subjective well-being and quality of life (QoL), not just absence of disease. This is a more positive approach consistent with the World Health Organization’s definition of health as ‘a state of complete physical, mental, and social well-being and not merely the absence of disease or infirmity’. Traditional biomedical outcome measures, such as survival or improvement of symptoms, do not capture all the ways in which a person may be affected by illness or treatment. The inclusion of more holistic outcomes such as QoL and ª The Authors. Journal compilation ª Mac Keith Press 2009 660 DOI: 10.1111/j.1469-8749.2009.03324.x health-related quality of life (HRQoL) is attracting interest. However, it is often difficult for researchers and clinicians to choose an appropriate instrument. DEFINING QoL QoL is an evolving theoretical construct which is variously defined for children, adolescents, and adults. A generally accepted definition is that QoL is the ‘individual’s perceptions of their position in life in the context of the culture and value systems in which they live, and in relation to their goals, expectations, and concerns’.1 A useful operational definition of QoL is ‘an overall assessment of wellbeing across various domains’.2 For children, QoL includes, but is not limited to, the child’s perception of their social, physical, and emotional well-being, and as such must be sensitive to the changes that occur throughout development3. HRQoL is considered to be a subdomain of the more global construct of QoL which includes health-related domains of life.4,5 MEASURING QoL Research studies which measure QoL often do not define what QoL means or justify the choice and content of the measure(s) used.6 QoL measures should be viewed as indices of relatively unrelated items and domains. Unlike single-trait scales such as anxiety, the measured variables in each QoL instrument define the QoL construct of that particular measure rather than being defined by it.7 There may be no normal range or standard mean.6 Therefore, clinicians may not know how to select the most appropriate instrument, or how to interpret scores and integrate them into clinical practice.8,9 There will always be gaps between what we want to understand and our efforts to quantify it and this is especially true for QoL.10 It is often difficult to decide which domains to include in a QoL measure. Increasingly, it is recognized that children and parents should be consulted about the important domains of life.2 GENERIC AND CONDITION-SPECIFIC QoL INSTRUMENTS Domains of QoL in adult instruments include health, material well-being, safety, community, productivity, emotional well-being, family, and friends.11 These domains cannot be assumed to be generalizable to children and adolescents. A recent review of QoL instruments for children and adolescents reported that the most common domains of QoL refer to emotions, social interactions, medical issues ⁄ treatment, cognition, activities, school, family, independence ⁄ autonomy, pain, behavior, future, leisure, and body image.12 There are two broad types of instrument for assessment of QoL ‘generic’ and ‘condition-specific’. Generic instruments address a comprehensive array of domains of wellbeing, and have the advantage that the data acquired can be compared across demographic or clinical populations. A potential limitation is that they might lack the sensitivity to detect subtle aspects of specific conditions or disorders in a way that provides meaningful information to patients and professionals, although the empirical evidence in this regard is contradictory. Condition-specific instruments assess characteristics of a particular condition. As such, they are generally seen to be more relevant and sensitive to the nuances of that particular condition. The domains included in QoL instruments will differ between generic and condition-specific instruments. For example, based on a qualitative study of children with cerebral palsy (CP) and their parents, the following domains were considered to be important to the QoL of children with CP: social well-being and acceptance, feelings about functioning, participation and physical health, emotional well-being, access to services, pain and impact of disability, and family health.14 While some of these domains overlap with common generic instruments (social well-being, emotional well-being, family health), others are specific to children with CP (e.g. pain and impact of disability, functioning). For children with epilepsy, condition-specific issues include disclosure ⁄ concealment of their epilepsy and the quest for normality.15 CHARACTERISTICS OF QoL INSTRUMENTS Essential characteristics of a QoL instrument are outlined in Table I and discussed below. The aim of this paper is to describe and analyze QoL instruments for children and adolescents with neurodisabilities against these characteristics. Original purpose of instrument An instrument used to measure QoL may not have been designed for that purpose. For example, in studies of children with CP, the Pediatric Outcomes Data Collections Instrument (PODCI),16 Child Health Questionnaire (CHQ),17 and Lifestyle Assessment Questionnaire18 have been used to measure QoL but the PODCI is designed to measure functional status, the CHQ to measure functional health and well-being, and the Lifestyle Assessment Questionnaire to measure impact of disability. In studies of QoL in childhood epilepsy, the Impact of Pediatric Epilepsy on the Family Scale (IPES),19 Hague Restrictions in Childhood Epilepsy Scale (HARCES),20 and Impact of Table I: Characteristics of a quality of life instrument Characteristics Original purpose of instrument Actual focus of instrument Origin of domains and items Opportunity for self-report Clarity of items, using children’s own phrases Threat of negative wording to self-esteem Number of items and time to complete Psychometric properties QoL Instruments for Children and Adolescents with Neurodisabilities Elizabeth Waters et al. 661 Childhood Illness Scale21 have been used but none were designed to measure QoL. Actual focus of instrument An instrument should focus on the personal well-being of an individual, or their feelings and perceptions about life. However, many so-called ‘QoL’ or ‘HRQoL’ instruments focus on functioning (what the person can do) rather than well-being. For example, the Pediatric Quality of Life Inventory–CP module (PedsQL–CP module) assesses whether children have difficulty moving one or both of their legs, difficulty using scissors, and difficulty brushing their teeth,22 and the CHQ measures physical functioning by assessing a child’s limitations in terms of activities such as playing soccer and riding a bike.17 While functional status and impairment may have an impact on QoL they are not synonymous with it;6 and a meta-analysis of adult studies measuring both found them to be distinct concepts.23 It is not possible to estimate a patient’s distress by accumulating their range of problems. Therefore, QoL measures are needed to provide an evaluation that is separate from the biomedical health state.24 Questionnaires that aggregate impairments with QoL to a single score should be avoided. QoL assessment shows that similar medical conditions may affect individuals in different ways.25 People with significant health problems can be highly satisfied with some aspects of their lives (the so-called ‘disability paradox’).26 Recent studies of children with CP, show that functioning is only weakly related to domains of QoL.27 Origin of domains and items Until recently, instruments have measured domains that were decided a priori by professional ‘experts’ (researchers and clinicians); however, there is increasing recognition that families and children should be consulted.2,28,29 Qualitative research is well suited to this29 and instrument developers should report in detail the methods they use. Instruments are more likely to have content validity if items are derived from a sample of the population in which the instrument is to be used.30 Children identify more items than health professionals or their own parents, and contribute significantly to the wording of items.31 Opportunity for self-report In the adult literature self-report questionnaires are the primary method of assessing QoL whereas childhood literature has suggested that parents may be better able than the child to rate their child’s QoL because of their child’s cognitive immaturity, limited social experience, and continued dependency.32 However this view is changing, especially for children aged 8 years and over. New instruments have child self-report versions. The instruments usually 662 Developmental Medicine & Child Neurology 2009, 51; 660–669 start at about age 8 years32 because test–retest reliability was poor in children with epilepsy aged 6 to 7 years, while those 8 years and older showed acceptable results.15 However, where child ⁄ adolescent self-reports cannot be collected because of age, disability, or cognitive impairment, parent proxy measures are valuable. A systematic review of 14 studies assessing the relationship between parent-proxy and child self-reported QoL demonstrated that the level of agreement depended on the domain.32 Generally there was good agreement (correlations >0.5) between parents and children for domains reflecting physical activity, functioning, and symptoms but poorer agreement (correlations <0.30) for domains which reflected social or emotional domains. Threat of negative wording to self-esteem Items may threaten self-esteem by making assumptions about an illness. For example, the DISABKIDS-CP Module includes items such as ‘Does it bother you that you have to explain to others what you can and can’t do?’ ‘Is it frustrating to be unable to keep up with other children?’ ‘Do people think that you are not as clever as you are?’ Although these items may have sound psychometric properties, there may be ethical implications to including such questions. Number of items and time to complete The number of items and time required to complete a QoL instrument are important considerations, particularly for children. We think that completion of a measure should not take longer than 10 to 20 minutes. Psychometric properties Instruments should demonstrate adequate test–retest reliability, validity, and factor structure. If designed to evaluate interventions, they should also demonstrate evidence of sensitivity to change. Cronbach’s coefficient alpha (a) is a useful measure for internal consistency in scales that tap a single dimension construct but not necessarily in multidimensional measures such as QoL where the items may not correlate closely with each other. Because the a is sensitive to the length of the instrument, a long scale may have a high value even where there is heterogeneity. Therefore, an optimal value of the a to measure the internal consistency of a measure is a necessary but not a sufficient index of reliability; and values over 0.9 are likely to indicate redundancy.33,34 Good test–retest reliability confirms that the scale is stable over time and values of the intraclass coefficient should be >0.6. Construct validity assesses an instrument in the absence of a criterion standard and refers to predictions regarding how the instrument should behave based on hypotheses. The process is usually established over a number of studies, tapping various aspects of the hypothetical construct. Factor analysis is important for developing multiconstruct measures because it may reveal patterns of interrelationships among variables not otherwise apparent, identify independent domains and redundant domains, reduce the number of items and retain only those items that correlate primarily with a single domain. RESULTS Generic QoL instruments for children and adolescents with neurodisabilities Several generic instruments were identified including the CHQ, PedsQL, and KIDSCREEN. Each is described briefly against the features laid out in Figure 1. Child Health Questionnaire The CHQ is a proxy-completed generic instrument designed to measure functional health status, well-being, and health outcomes of children aged 0 to 18 years.35 Its domains are behavior, bodily pain, general health, mental health, parent impact-emotional, physical functioning, parent impact-time, role-emotional ⁄ behavioral, role-physical, and self-esteem; there are also physical and psychosocial summary scores.35 The items were developed by experts and adapted from a variety of existing instruments. The CHQ only has a self-report version for adolescents and it is long. Although the CHQ is reliable and valid for children with CP, we have not found studies which examined its sensitivity to change. A recent study compared the psychometric properties of the CHQ with the CP QoL Questionnaire for METHOD A systematic review was conducted to identify pediatric condition-specific and generic QoL and HRQoL instruments for children and adolescents with neurodisabilities. It employed a search strategy of the published literature on the Medline and PsychLit electronic databases to identify QoL instruments published between 1990 and 2007, using the terms ‘children, adolescent, QoL (encompassing HRQoL), neurodisabilities, CP, spina bifida, and epilepsy’. A total of 474 abstracts were reviewed to identify QoL ⁄ HRQoL instruments, and further information was then obtained on each instrument. The instruments were reviewed and mapped to the characteristics set out in Table I. Health or functioning Low involvement of families Original purpose of instrument CHQ KIDSCREEN PedsQL Origin of items PedsQL CHQ KIDSCREEN Quality of life High involvement of families Actual focus of the instrument Functioning No opportunity for self-report Negative wording Large number of items Poor or not demonstrated PedsQL CHQ KIDSCREEN Opportunity for self-report CHQ (13+) KIDSCREEN PedsQL (5+) Potential threat to self-esteem PedsQL KIDSCREEN CHQ Length CHQ (self-report 87 items) KIDSCREEN (10,27,52) PedsQL CHQ parent Psychometric properties KIDSCREEN PedsQL CHQ Well-being Self-report version available Positive wording Small number of items Excellent and demonstrated adequately Figure 1: Features of generic quality of life instruments for children and adolescents to with neurodisabilities. CHQ, Child Health Questionnaire; PedsQL, Pediatric Quality of Life Inventory. QoL Instruments for Children and Adolescents with Neurodisabilities Elizabeth Waters et al. 663 Children (CP QoL-Child) and KIDSCREEN-10 for children with CP; it suggests that the CHQ is not the most appropriate instrument available to measure the QoL of children with CP.36 The internal consistency of the CHQ ranged from 0.18 to 0.96 across different domains. Correlations between CHQ and KIDSCREEN were good (r=0.25–0.53) but correlations between CP QoL-Child and CHQ ranged from 0.01 to 0.5 with similar domains correlating moderately.36 Finally, the CP QoL-Child and KIDSCREEN-10 outperformed the CHQ in terms of floor and ceiling effects with floor effects observed for all domains of the CHQ except general health (5–25%), and ceiling effects for all domains of the CHQ (2.9–60.8%).36 Pediatric Quality of Life Inventory The PedsQL is a measure of HRQoL for children and adolescents aged 2 to 18 years. It assesses physical, emotional, social, and school functioning. The four factors correlated highly from 0.75 to 0.88 suggesting that they might tap into related issues of emotional functioning.37 The PedsQL is brief and has a child self-report version; however the items focus on functioning and what the child can do, as well as on how much difficulty a child has doing something. If a child does have a lot of difficulty, the items might threaten self-esteem. The PedsQL Generic Core Scale has adequate psychometric properties with internal consistency ranging from 0.70 to 0.89 for proxy-report and 0.54 to 0.86 for self-report.22 Correlations between the Generic Core Scale and CP module (see below) ranged from weak (0.1–0.2) to moderate (0.40–0.43) to significant (0.5–0.8).22 KIDSCREEN KIDSCREEN is a self- and proxy-completed generic instrument, created with children’s input, that is designed to measure the QoL of healthy and chronically ill children and adolescents aged 8 to 18 years. Its domains include physical well-being, psychological well-being, social support and peers, and financial resources. The inter-subscale correlation ranges from 0.1 to 0.62 in keeping with an index of relatively independent domains. As indicated in Figure 1, KIDSCREEN is the only generic instrument that has high involvement of children and families in its development, assesses well-being, has a child self-report version, no apparent threat to self-esteem, a limited number of items, and good psychometric properties. In European normative samples, KIDSCREEN-10 has sound psychometric properties with good internal consistency (0.82) and test–retest reliability (r=0.73),38 confirmed by a recent Australian study which also reported good internal consistency (0.86) and reliability with correlations between 664 Developmental Medicine & Child Neurology 2009, 51; 660–669 KIDSCREEN-10 and CP QoL-Child ranging from 0.30 to 0.54.36 CONDITION-SPECIFIC QOL INSTRUMENTS FOR CHILDREN AND ADOLESCENTS WITH NEURODISABILITIES Cerebral palsy (see Fig. 2) The following four condition-specific QoL ⁄ HRQoL instruments were identified: the CP QoL-Child, The Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD), the PedsQL-CP module, and DISABKIDS. CP QoL Questionnaire for Children The CP QoL-Child is designed to measure the QoL of children with CP aged 4 to 12 years. Its seven domains are social well-being and acceptance, functioning, participation and physical health, emotional well-being, access to services (parent-proxy only), pain and feelings about disability, and family health (parent-proxy only).39 The CP QoL-Child items focus on assessment of well-being and present no threat to self-esteem. The parent-proxy version is psychometrically sound while early results of the child self-report version suggest it has good psychometric properties. For parent-proxy, the two week test–retest reliability ranged from r=0.76 to 0.89 and internal consistency ranged from 0.74 to 0.92. The questionnaire was moderately correlated with the CHQ and KIDSCREEN, supporting the validity of the CP QoL-Child parent-proxy version.39 Child Health Index of Life with Disabilities The CPCHILD40 is a Canadian condition-specific health functioning instrument for children and adolescents with severe CP. It focuses on measuring caregivers’ perspectives of the activity limitations, health status, well-being, and ease of care. It consists of 36 items in six sections: personal care; positioning, transfers and mobility; communication and social interaction; comfort, emotions and behavior; health; and overall QoL. Items are rated on degree of difficulty (‘no problem’ to ‘impossible’) and level of assistance (‘independent’ to ‘total assistance’). Early analysis of the CPCHILD suggests that it has sound psychometric properties with 2 week test-retest ranged from r=0.88 to 0.96.40 Pediatric Quality of Life Inventory–CP module The PedsQL-CP module,22 is a condition-specific HRQoL questionnaire for children and adolescents with CP. The 35-item CP module yields the following seven scales: daily activities, school activities, movement and balance, pain and hurt, fatigue, eating activities, and speech and communication. The questionnaire is designed for children aged 2 to 18 years. According to Varni et al.22 the Health or functioning Low involvement of families Original purpose of instrument CP QoL-Child PedsQL-CP DISABKIDS-CP CPCHILD Origin of items CPCHILD PedsQL-CP DISABKIDS-CP CP QoL-Child Quality of life High involvement of families Actual focus of instrument Functioning No opportunity for self-report Negative wording Large number of items Poor or not demonstrated PedsQL-CP CPCHILD DISABKIDS-CP CP QoL-Child Opportunity for self-report CPCHILD CP QoL-Child PedsQL-CP DISABKIDS-CP Potential threat to self-esteem PedsQL-CP DISABKIDS-CP CPCHILD DISABKIDS-CP CPCHILD CP QoL-Child CP QoL-Child Length Psychometric properties CPCHILD CP QoL-Child DISABKIDS-CP PedsQL-CP PedsQL-CP Well-being Self-report version available Positive wording Small number of items Excellent and demonstrated adequately Figure 2: Condition-specific quality of life instruments for children and adolescents with neurodisabilities. CPCHILD, The Caregiver Priorities and Child Health Index of Life with Disabilities; CP Qol-Child, CP QoL Questionnaire for Children; PedsQl, Pediatric Quality of Life Inventory. Module has adequate psychometric properties with internal consistency ranging from 0.88 to 0.96 for proxy-report and 0.63 to 0.93 for self report; (fatigue 0.63, speech and communication 0.95, and daily activities 0.96 ⁄ 0.93). Correlations between the CP module and Generic Core Scale were varied ranging from weak (0.14–0.23), moderate (0.40–0.43), to significant (0.52–0.84). DISABKIDS – CP Module DISABKIDS is designed to measure HRQoL and includes a generic module, a chronic generic module, and a condition-specific module. The generic module comes from the KIDSCREEN and is suitable for all children. The chronic generic module is suitable for use with children and adolescents who suffer from any chronic health-related condition. A condition-specific module has been developed for the chronic conditions studied in the DISABKIDS Project, which include CP.41 Childhood epilepsy (see Fig. 3) The quality of life measure for children with epilepsy42 This parent-proxy reported instrument was developed for children with refractory epilepsy, aged 4 to 18 years with average intellectual abilities, and is based on the responses of 68 parents. The items are derived from a survey questionnaire, review of the literature, and related measures. There are 16 domains with 1 to 16 items per domain and a total of 79 items. The high internal consistency in six subscales suggests redundancy of items. Correlation between subscales ranges from 0.07 to 0.84. Items focus on psychological-emotional health and social issues. The sample size precluded the use of factor analysis and the psychometric properties lack test–retest analysis. The acceptable internal consistency of the measure is limited to the characteristics of the sample tested, and may be only marginal for other populations. The quality of life inventory for adolescents with epilepsy43 This is a self-report instrument for adolescents with epilepsy. The measure has 48 items distributed in eight subscales based on reviews of literature, existing measures, focus groups of adolescents with epilepsy, and professional opinion. A conceptual framework is not present. Factor analysis of the 197 completed questionnaires was poorly reported. The majority of the items and subscales seem to portray emotional functioning. Internal consistency points QoL Instruments for Children and Adolescents with Neurodisabilities Elizabeth Waters et al. 665 Health or functioning Low involvement of families Original purpose of instrument QOLCE QVCE-50 CHEQOL-25 QOLIE-AD-48 Origin of items QOLCE QVCE-50 QOLIE-AD-48 Quality of life CHEQOL-25 High involvement of families CHEQOL-25 Well-being CHEQOL-25 QOLIE-AD-48 Self-report version available Actual focus of instrument Functioning No opportunity for self-report Negative wording QOLCE QVCE-50 QOLIEAD48 QOLIE-AD-48 Opportunity for self-report QOLCE QVCE-50 Potential threat to self-esteem QOLIE-AD-48 QOLCE QVCE-50 CHEQOL-25 Positive wording CHEQOL-25 Small number of items Length Large number of items QOLCE Poor or not demonstrated QOLCE QVCE-50 QOLIE-AD-48 Psychometric properties QVCE-50 QOLIE-AD-48 CHEQOL-25 Excellent and demonstrated adequately Figure 3: Condition-specific quality of life instruments for children with epilepsy. QOLCE, Quality of Life for Children with Epilepsy; QUCE, Brazillian quality of life questionnaire for children with epilepsy; CHEQCH-25, Health related quality of life for childhood epilepsy; QOLIE-AD-LO8, quality of life inventory for adults. to redundancy of items in two subscales. Regression analysis for the ‘overall HRQoL’ identified the age of the adolescent, seizure severity, neurotoxicity of antiepileptic drugs, and socio-economic status as the main risk factors43 HRQoL questionnaire for Brazilian children with epilepsy44 This is a caregiver-reported instrument in Portuguese. The items were pooled from other measures, the literature, and the authors’ personal experience. Although the wording was chosen to prevent negative feelings many items read negatively in translation. The 50 items and four domains cover physical health, psychological health, social and family relationships, and cognitive educational issues, suggesting a mix of objective functional and emotional health with subjective perceptions. Impact of pediatric epilepsy on the family19 This 11-item parent response measure intends to evaluate the impact of epilepsy on the family’s and child’s life. The items were generated from the authors’ clinical experience and review of the literature. A theoretical concept is absent, 666 Developmental Medicine & Child Neurology 2009, 51; 660–669 with no a priori explanation why these 11 items were most important. Factor analysis identified three factors, namely participation, interpersonal relationships, and family dynamics. The high values of internal consistency suggest that the items tap into a single factor.34 HRQoL for childhood epilepsy The work of Ronen et al.15,28,29 on the measurement of HRQoL in children and youth with epilepsy captures the experiential aspects of childhood epilepsy from the youths’ and parents’ perspective. These related measures have a conceptual basis that de facto provides a more specific definition of HRQoL in childhood epilepsy than any previous definitions.45 The conceptual model was generated by interviewing children and their parents separately, using focus groups.29 Their perception of QoL while living with epilepsy was derived from a textual analysis of their narratives.28 The findings informed the preliminary HRQoL instrument. The measure was administered to 381 children with epilepsy and their parents across Canada and the data were submitted to factor analysis. This generated selected and put into question form scaled with a 5-point Likert scale. The following ten domains were identified: social, emotional, intellectual, financial, medical, independence, environmental, physical functioning, recreation, and vocational, a mixture of well-being and functioning domains. The parents of the 5- to 12-year olds were particularly concerned about the emotional needs of the child and family. The 13- to 20-year-old adolescents were particularly concerned about opportunities for independence and the general public’s perceptions of them. Parents and children were most concerned about the development of social and self-care skills.49 the conceptual domains compromising HRQoL and became the subscales used in its measurement.15 This model comes closer than many others to what youth mean by HRQoL, as the domains were not generated by professionals.46,47 The measure has the following five domains: (1) interpersonal ⁄ social consequences, (2) worries and concerns mostly in daily life experiences, (3) intrapersonal ⁄ emotional issues, (4) secrecy and concealment of epilepsy, and (5) quest for normality. The correlation between the subscales ranged from 0.26 to 0.52. Factor analysis of the Chinese translation of this measure in Hong Kong identified the same factor structure, providing additional cross-cultural validity to the conceptual model and the instrument.48 The measure’s responsiveness has yet to be tested in longitudinal studies. Hydrocephalus Outcome Questionnaire50 (HOQ) and Parental Concerns Questionnaire51 The HOQ instrument for children with hydrocephalus is intended to measures the objective functional status of the child. Factor analysis was not performed and the psychometric properties suggest redundancy of items. The related Parental Concerns Questionnaire originated from focus groups with the parents of children with hydrocephalus during the HOQ development. The nine items with the highest severity and importance scores were included. The Spina-bifida and hydrocephalus (see Fig. 4) Parkin et al.49 generated items by peer interviews with health professionals and in-depth interviews with children with spina bifida and their parents. Approximately 600 items were ranked by the children and parents according to their importance, and the highest ranked 50 items were Health or functioning Original purpose of instrument HOQ HRQL-SB Quality of life HRQL-SB High involvement of families Origin of items Low involvement of families HOQ Actual focus of the instrument Functioning HOQ No opportunity for self-report HOQ Negative wording Opportunity for self-report HRQL-SB Potential threat to self-esteem HOQ HRQL-SB Length Large number of items Poor or not demonstrated Well-being HRQL-SB HOQ HRQL-SB Psychometric properties HOQ HRQL-SB Self-report version available Positive wording Small number of items Excellent and demonstrated adequately Figure 4: Condition specific quality of life instruments for children with spina bifida and hydrocephalus. HOQ, Hydrocephalus Outcome Questionnaire; HRQL-SB, health related quality of life for children with spina bifida. QoL Instruments for Children and Adolescents with Neurodisabilities Elizabeth Waters et al. 667 internal consistency measured 0.89, test–retest reliability 0.86, and interrater reliability 0.67 between mothers and fathers. Although this scale taps into subjective feelings of parents it is not intended to measure QoL of children with hydrocephalus. DISCUSSION The measurement of QoL of children and adolescents with neurodisabilities has advanced significantly over the last decade. There are now several generic and condition-specific QoL instruments available. This paper shows these instruments are often developed with little involvement of families, focus on functioning rather than well-being, and have items that are negatively worded. Researchers and clinicians should be aware of how an instrument was developed and its theoretical focus as these will influence the domains, items, and resulting scores. They should also ensure that an instrument has sound psychometric properties. 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