Journal http://jcn.sagepub.com/ of Child Neurology
Quality of Life in Children With Cerebral Palsy: Implications for Practice Kim-Michelle Gilson, Elise Davis, Dinah Reddihough, Kerr Graham and Elizabeth Waters J Child Neurol published online 27 May 2014 DOI: 10.1177/0883073814535502 The online version of this article can be found at: http://jcn.sagepub.com/content/early/2014/05/27/0883073814535502
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Original Article
Quality of Life in Children With Cerebral Palsy: Implications for Practice
Journal of Child Neurology 1-7 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0883073814535502 jcn.sagepub.com
Kim-Michelle Gilson, PhD1, Elise Davis, PhD1, Dinah Reddihough, MD, BSc, FRACP, FAFRM2,3,4, Kerr Graham, MD, FRCS(Ed), FRACS5, and Elizabeth Waters, DPhil1
Abstract The ability to assess the quality of life of children with cerebral palsy to inform and evaluate individual care plans, service planning, interventions, and policies is crucial. In this article, the recent evidence on quality of life in children with cerebral palsy is reviewed, with attention to the determinants of quality of life and role of this construct as a practical outcome indicator in clinical trials. Quality of life measurement advances for children with cerebral palsy are discussed with a focus on conditionspecific quality of life measures, particularly, the Cerebral Palsy Quality of Life–Child, which is the first condition-specific quality of life measure for children with cerebral palsy. The article presents an overview for clinicians and researchers intending to use quality of life measures on children with cerebral palsy and provides recommendations for future research that will better inform practice in the field. Keywords Cerebral Palsy Quality of Life–Child, measurement, disability, outcome, determinants Received April 01, 2014. Received revised April 01, 2014. Accepted for publication April 03, 2014.
Cerebral palsy is the most frequent cause of physical disability in children, occurring in approximately 2 to 2.5 per 1000 live births.1 Cerebral palsy refers to a group of disorders with a diverse range of etiological pathways, pathophysiologies, and clinical features that persist through the life span. The most recent definition is as follows: ‘‘A group of developmental disorders of movement and posture, causing activity restriction or disability attributed to disturbances occurring in the foetal or infant brain. The motor impairment may be accompanied by a seizure disorder and by impairment of sensation, cognition, communication and/or behaviour and by secondary musculoskeletal problems.’’2
indication of their well-being across several life domains such as physical health and social and emotional well-being. The aim of this article is to introduce the quality of life construct and provide an overview of what is now known about quality of life in children with cerebral palsy, with a particular focus on measurement, determinants, and relevance for clinical practice.
Quality of Life Quality of life is considered a broad and multidimensional concept that includes subjective evaluations of both the 1
Treatment of the motor disorder alone may involve a range of therapies and interventions such as physiotherapy, the use of orthoses, orthopedic surgery, and medications for spasticity.3 It is not surprising that cerebral palsy can have profound effects on the physical, social, and emotional health and wellbeing of the child, including the parents.4 Children with cerebral palsy not only have to contend with a range of physical problems, such as muscle weakness, stiffness, and clumsiness, they are also 4 times more likely than their peers to experience emotional and behavioral problems.5 Quality of life is a very relevant and important construct in the context of children with cerebral palsy because it can provide a broad subjective
The Jack Brockhoff Child Health and Wellbeing Program, Academic Centre for Health Equity, Melbourne School of Population and Global Health, University of Melbourne, Australia 2 Department of Developmental Medicine, Royal Children’s Hospital, Melbourne, Australia 3 Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia 4 Murdoch Children’s Research Institute, Melbourne, Victoria, Australia 5 Department of Orthopaedic Surgery, Royal Children’s Hospital, Melbourne, Australia Corresponding Author: Kim-Michelle Gilson, PhD, Melbourne School of Population and Global Health, Level 5, 207 Bouverie Street, University of Melbourne, Australia. Email: [email protected]
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positive and negative aspects of life. It embraces the notion of holistic well-being and encompasses elements about general functioning and health, but also extends beyond these to include a person’s appraisal of their life experiences and social and emotional well-being.6 Definitions of quality of life vary across disciplines and are often complex and difficult to operationalize.7 The most cited definition by the World Health Organization is ‘‘an individuals’ perception of their position in life, in the context of culture and value systems in which they live and in relation to their goals, expectations, standards and concerns.’’8 Although there is considerable agreement that quality of life is multidimensional, there is much variation in the content of the domains that comprise quality of life for children and, particularly, for children with disabilities. Early conceptualizations of quality of life were based heavily on functioning and measures that purported to assess quality of life focused on physical health and functional limitations (e.g., what a person can and cannot do) rather than an individual’s broader well-being or personal experience.9 The traditional assumption that functioning equates with overall quality of life has been brought into question by several empirical studies that show a weak or nonsignificant relationship between a child’s functioning and overall quality of life,10 in addition to specific domains, such as social and emotional functioning.6,11,12 These findings are important because they suggest that a child can still experience a good quality of life despite experiencing significant functional limitations.
Quality of Life as an Important Outcome Measure In the context of rehabilitation, quality of life has clinical utility as an important health-related outcome measure that can guide practice.13 It can provide a comprehensive picture of the overall impact of a health condition on a child’s social, emotional, and physical well-being and therefore offers essential information for decision making in clinical practice.14,15 Increasingly, patients’ well-being and their functioning in everyday life are an important part of a clinical examination, and determining patient quality of life can assist health professionals to make decisions about how best to plan care interventions that are appropriate and individualized.16,17 In this sense, quality of life measures can be complementary to objective measures of functioning that are administered by rehabilitation professionals.18 Children and their families may also benefit from the information that quality of life assessments can provide, particularly giving them hope if positive scores are observed on particular domains, or by facilitating discussions over treatment suitability.
Measuring Quality of Life in Children With Cerebral Palsy In recent years, there has been increasing interest in developing valid measures of quality of life. This has been both an attempt
to understand the impact of cerebral palsy on a child’s whole life, and in strong efforts to obtain empirical-based evidence to guide practice in the field particularly with therapeutic interventions for children with cerebral palsy. There are now a number of generic and condition-specific scales available to measure QOL in children with cerebral palsy, and these are available in the form of child self-report or parent proxy versions. Given the subjective nature of quality of life, it has been argued that whenever possible, quality of life should be self-reported by the individual.19 However, given the range of impairment in children with cerebral palsy, this is not always possible and parents may rate (1) their own perceptions of the child’s quality of life or (2) what they believe is the child’s own perception. Generic quality of life scales are designed to be applicable to all population subgroups and are useful when making comparisons between groups and with norms from the general population.20,21 It therefore follows that generic instruments used to measure quality of life in children with cerebral palsy might not have been designed for that purpose and might not include domains or items that specifically tap into their lived experience. For example, the Pediatric Outcomes Data Collections Instrument, Child Health Questionnaire, and Lifestyle Assessment Questionnaire have been used to measure quality of life 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. Another common instrument is the KIDSCREEN,13 which is a health-related quality of life measure not specific to children with cerebral palsy. Condition-specific scales, on the other hand, are designed to be applicable to 1 group and therefore target issues pertinent to the individual’s condition. The domains and items in these instruments are derived from the lived experience of children with cerebral palsy and therefore correspond to what they consider important to their life. This also means that they are suitable for detecting change in the quality of life domains that are important for a specific condition.20,22 Over the last 8 years, 4 new condition-specific instruments have been developed to measure the quality of life of children with cerebral palsy. Measures now include the Cerebral Palsy Quality of Life–Child (CP QOL-Child23); the Caregiver Priorities and Child Health Index of Life with Disabilities24; the Pediatric Quality of life Inventory CP Module27; and the DISABKIDS CP Module.25 A recent systematic review of these condition-specific instruments that exist for school-aged children with cerebral palsy reported that the Cerebral Palsy Quality of Life–Child measure has the strongest psychometric properties and clinical utility followed by Caregiver Priorities and Child Health Index of Life with Disabilities.26 This was determined by reviewing published data on concurrent, construct, and content validity and evidence of internal and test-retest reliability for all measures. The review highlighted that some of these instruments had a heavy emphasis on functioning, such as the Pediatric Quality of life Inventory, where 23 of the 35 items related
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Table 1. Overview of the Condition Specific Quality of Life Instruments for Children With Cerebral Palsy. Cerebral Palsy Quality of life Questionnaire for Children (CP QOL-Child)
Caregiver Priorities and Child Pediatric Quality of Life Inventory (PedsQL) Health Index of Life with CP Module Disabilities (CPCHILD)
Description
A condition-specific outcome measure intended to gauge and assess quality of life changes in children with cerebral palsy
Age range (y) Completion time (min) Proxy (caregiver) report
4-12 15-25 Yes (for children aged 4-12)
Self-report (age) Country of origin Number of items Domains
Yes (9þ) Australia 52 Social well-being and acceptance, functioning, participating and physical health, access to services,a family health,a emotional wellbeing, pain and feelings about disability
To measure health-related To measure healthA measure of health status related quality of quality of life in a popuand well-being for children life and assess lation of children and with cerebral palsy burden of disease adolescents with cerein children and bral palsy adolescents 5-18 2-18 4-16 20-30 5 N/A Yes (for children aged 5-12) Yes (for children aged 2-18) Yes (for children aged 4-16) No Yes (5-18) Yes (4-16) Canada USA Europe 36 35 16 Generic module, a Daily activities, school Personal care; positioning, chronic generic activities, movement and transfers and mobility; module and a balance, pain and hurt, communication and condition-specific fatigue, eating activities comfort, emotions and module and speech and behavior; health; and communication overall quality of life
Instrument
DISABKIDS-CP module
Abbreviation: CP, cerebral palsy. a Only the primary caregiver-proxy version of the CP quality of life contains the 2 domains of access to services and family health.
to functional tasks such as school activities, daily activities, or eating activities. Another important finding was that the Cerebral Palsy Quality of Life–Child was the only measure to base its development on a theoretical construct of quality of life, that is, measuring well-being rather than ill-being and difficulty, and having broad domains. The attributes of the condition-specific instruments, including their domains and psychometric properties, are listed in Tables 1 and 2.
The Cerebral Palsy Quality of Life Questionnaire The Cerebral Palsy Quality of Life–Child was developed by a team of clinicians and researchers in Australia and was the first condition-specific quality of life measure for children with cerebral palsy aged 4 to 12 years.23 In contrast to other measures, the Cerebral Palsy Quality of Life–Child was based on qualitative interviews with children with cerebral palsy and their parents. These interviews focused on what the child needed to be happy and have a good quality of life and provided content for the development of items.30 Until recently, instruments have measured quality of life domains that were decided a priori by professional ‘‘experts’’ (researchers and clinicians) rather than through eliciting the perspective of the audience for which they are designed. There is now an increasing recognition that families and children should be consulted and measures developed based on their perspectives. The Cerebral Palsy Quality of Life–Child assesses several aspects of well-being, rather than functioning, and 2 parallel
forms of the measure exist: a primary caregiver-proxy version that is for parents of children aged 4 to 12 years, and a child self-report version for children aged 9 to 12 years. It is important to have a child self-report version, given that quality of life is, by definition, an individual’s perceptions. Furthermore, parents’ views of their children’s quality of life may be affected by their own mental health and well-being associated with caregiving.31 Since the development of the Cerebral Palsy Quality of Life–Child in 2007, it has been translated into 20 different languages (Greek, Hebrew, Polish, Arabic, Chinese, Flemish, Tamil, Bahasa, Italian, Dutch, Thai, French, Spanish, Korean, Turkish, Farsi, Burmese, Malayalam, Finnish, and Portuguese) and cited nearly 70 times. The international support for the translation and psychometric properties of the Cerebral Palsy Quality of Life–Child demonstrates the widespread need for such a measure and its applicability cross-culturally. Qualitative research in the United Kingdom has also demonstrated that items of Cerebral Palsy Quality of Life–Child correspond with the perspectives provided by children and their parents who discussed the quality of life of their child.32
Guidance on Selecting the Most Appropriate Measure for Assessing Quality of Life Although it is encouraging that there are now several quality of life instruments relevant for children with cerebral palsy, the wider choice means that it is becoming increasingly difficult for clinicians and researchers to select the most appropriate quality of life instrument for their purpose. From our work in
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Table 2. Condition-Specific Quality of Life Instruments for Children With CP: A Comparison of Psychometric Attributes.26
Instrument Reliability tested for CP Internalreliability
Retest reliability (intraclass correlation coefficient statistic) Validity tested for CP Content validity
Concurrent validity
Construct validity
Sensitivity to change examined
Cerebral Palsy Quality of Caregiver Priorities and Child Pediatric Quality of Life Inventory (PedsQL)–CP Health Index of Life with life Questionnaire for Module Children (CP QOL-Child) Disabilities (CPCHILD) Yes23 Yes24 a ¼ 0.74-0.92 (caregivers) a ¼ 0.74-0.93 across all domains a ¼ 0.80-0.90 (child-self report) 0.76-0.89 0.97 for the total questionnaire; 0.88-0.96 across the 6 domains; (95% CI ¼ 0.88-0.99) Yes23 Yes24 Caregiver rated importance Domains determined in of items x ¼ 3.95 on 6consultation with point ordinal scale (SD ¼ children and their 0.63, range 2.67-4.90) parents (28 families) Domains moderately Domains moderately correlated to those of correlated to those of CHQ and PEDI KIDSCREEN and CHQ Higher GMFCS scores Global QOL r ¼ 0.18correlated with higher 0.62; Global Health CPCHILD scores (worse r ¼ 0.21-0.56 outcome). No No
Yes27 Child self-report: 0.77-0.93 N/A
Yes27 N/A
N/A
Can distinguish between typically developing children and children with CP Yes, across gross motor function levels29
DISABKIDS-CP Module Yes28 a ¼ 0.71-0.91 interrater reliability and 0.14-0.84 across scales N/A
Yes28 Domains determined in consultation with children and their parents (9 families) N/A
Can differentiate between levels of disability than CHQ and KINDL No
Abbreviations: CP, Cerebral Palsy; CPCHILD, Caregiver Priorities and Child Health Index of Life with Disabilities; GMFCS, Gross Motor Function Classification System; PEDI, Pediatric Evaluation of Disability System; Inventory; CHQ, Child Health Questionnaire; KINDL, a generic health-related quality of life measure. This table is based on the systematic review provided by Carlon et al.26
developing a condition-specific quality of life measure, and reviewing existing quality of life instruments for children with cerebral palsy, brief criteria have been developed that can assist researchers and clinicians in their choice of a quality of life measure for children with cerebral palsy. These characteristics (shown in Table 3) have been used to compare quality of life scales for children with cerebral palsy33,34 and pediatric populations without a disability.9 With a focus on the health professional within a clinical context, we also suggest a number of core questions that might help guide the clinician through their choice of instrument (Table 4). It is recognized that future research is also needed to provide clearer guidance in relation to these questions.
Determinants of Quality of Life in Children With Cerebral Palsy Understanding the determinants of quality of life gives us some insight into what factors may be important to consider for programs and interventions aiming to have an impact on quality of life. As rehabilitation and other pediatric health care specialists continue to broaden their intervention goals to include quality of life measures, it is essential that the contributing factors, or determinants, of quality of life in children with cerebral palsy become better understood. This can also assist clinicians to identify children who are more likely to have poor quality of life and, therefore, inform their treatment plan.
Studies on children with cerebral palsy have focused mainly on small groups of children selected from clinics with homogenous degrees of impairment severity, being skewed toward less motor impairment rather than across the spectrum.10,35 In line with a greater appreciation of the multidimensionality of quality of life, studies have reported independent predictors for different quality of life domains such as physical and psychosocial well-being. Focusing on domains has also shown significant variability across studies, with evidence to show that some children with cerebral palsy experience similar quality of life domains compared to typically developing children in spite of important functional limitations.6,16,35 One of the few population-based approaches to study the determinants was the European Union funded project (SPARCLE) conducted across 7 countries.35 This examined whether the severity of the child’s motor impairment and associated difficulties in IQ and pain predicted a comprehensive set of quality of life domains using the KIDSCREEN. Severity of motor impairment was associated with poor quality of life in terms of physical well-being and autonomy but not to psychosocial quality of life domains. Lower IQ was associated with the poor social support domain but not the moods and emotions and self-perception domains. In contrast, pain was associated with multiple poor quality of life domains, specifically, physical well-being, psychological well-being, and self-perception. Other studies36 have also found associations between pain and motor impairment severity and to adverse
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Table 3. Characteristics to Evaluate and Select an Appropriate Quality of Life Instrument. Characteristic
Gold standard
Original purpose of the instrument Origin of items
Developed to measure quality of life
Actual focus of the instrument Opportunity for self-report Potential threat to self-esteem Length Psychometric properties Conceptual underpinningsa
Based on the perspectives of children and parents Focuses on well-being
targeting stress management may contribute in helping to improve child quality of life. Clinical trials have focused primarily on medical interventions to treat the child’s impairment and assess change in functioning quality of life domains.38 There is a need to look toward interventions that consider other quality of life domains as being important outcomes for the child. This could include the consideration of caregiver support and the participation of the child as important components to interventions where quality of life is an outcome measure.
Has a self-report version available Positively worded Small number of items Has excellent psychometrics properties Strong conceptual underpinnings
a To have strong conceptual underpinnings, Davis et al9 suggest that the measure (1) must rest on a clear, operationalized definition of quality of life; (2) must be based on a theory of quality of life; (3) must include the important domains of life for children; and (4) must have well-constructed items.
Table 4. Questions for Clinicians Choosing a Quality of Life Instrument. Questions for clinicians What is the reason for measuring quality of life? Who is the respondent? Is a generic or condition-specific quality of life instrument needed? What is level of functioning of the child according to the GMFCS? What domains of quality of life do I need to measure? Does the intended measure have strong psychometric properties? Abbreviation: GMFCS, Gross Motor Function Classification System.
educational and social consequences. In regards to the family, parental stress consistently predicted poor quality of life in all domains, and single-parent households was associated with poor quality of life in the mood and emotions domain. These findings convey the complexity in delineating the predictors of quality of life in children with cerebral palsy, particularly given the wide range of impairment that accompanies cerebral palsy and the multidimensionality of quality of life. A study that assessed quality of life by both child and parent report was conducted by Majnemer et al,6 who found that poor socialization skills, reduced general competence, and behavioral difficulties were significantly associated with poor psychosocial well-being on both the Child Health Questionnaire (CHQ) and the Pediatric Quality of life Inventory in children with cerebral palsy. This highlights important areas for intervention beyond improving the motor impairment, particularly as social skills and behavioral problems can potentially be modified by health professionals to enhance quality of life.6 Higher levels of parental stress were also strongly associated with child’s psychosocial quality of life. Poor parental mental health is associated with poor quality of life on a number of different quality of life measures in children with cerebral palsy.35,37 From a clinical point of view, this suggests that targeted support by health professionals in the form of resources to enhance caregiver coping or programs
Conclusion and Directions for Future Research Research on the quality of life in children with cerebral palsy has made significant progress in the last 10 years with improved knowledge of the determinants and measurement approaches to quality of life and clinical practice. An overall advancement is our improved understanding of the quality of life construct and how this can be investigated within the complexity of cerebral palsy. There is a much stronger foundation to work toward improving the quality of life of children with cerebral palsy, which will enable significant progress over the forthcoming years. A number of important areas for future research are recommended. The first concerns the lack of research on sensitivity to change of quality of life instruments and on how quality of life instruments are used to evaluate the effectiveness of rehabilitation interventions. This is a major area for further work because quality of life instruments are increasingly being relied upon, yet there is still little understanding about if and how quality of life can be improved. This is a particularly complex issue given the many factors that can determine an individual’s quality of life. There is very limited data on if and how measures are being used and what quality of life instruments service providers and health professionals prefer to use. Therefore, further work should assess the role of quality of life instruments in clinical settings, which may then guide measure development or refinement. A wider understanding of the determinants of quality of life in children with cerebral palsy is needed. A multidimensional conceptual framework such as the ICF model1 could be applied to elucidate the determinants that either facilitate or hinder quality of life. This should be done using a population-based approach with greater inclusion of children from across the spectrum of gross motor impairment. Finally, research is needed on how responsive quality of life instruments are according to different Global Motor Function Classification levels, which would guide clinicians in their decision to selecting the most appropriate instrument.
Key Take Home Messages
Quality of life is a multidimensional construct. Measures encompass a number of domains and may be useful in evaluating the effectiveness of interventions and informing treatment care plans.
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Impairment does not correlate with poor quality of life. Impairment may predict poor physical well-being and other functional quality of life domains, but not more psychosocial domains of quality of life. There are now a number of psychometrically sound condition-specific quality of life measures for children with cerebral palsy. When choosing among various quality of life instruments, researchers and clinicians should consider the purpose of the instrument, number of items, the domains, country of origin, reliability, validity, sensitivity to change, age range, and respondents. Further work is needed on population-based samples across the spectrum of gross motor impairment levels to draw firm conclusions about the determinants and modifiers of child quality of life.
Author Contributions K-MG wrote the first draft of the manuscript and all authors contributed equally to the editing and revision of this manuscript.
Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: For underlying research conducted by the authors that is discussed in this special issue paper, funding was received by the National Health Medical Research Grant (NHMRC 284514), Murdoch Children’s Research Institute, and Victorian Health Promotion Foundation.
References 1. ICF. International Classification of Functioning, Disability and Health. Available at: http://www.who.int/icf. 2. Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl. 2007;109:8-14. 3. Boyd RN, Hays RM. Current evidence for the use of botulinum toxin type A in the management of children with cerebral palsy: a systematic review. Eur J Neurol. 2001;8(suppl 5):1-20. 4. Glenn S, Cunningham C, Poole H, Reeves D, Weindling M. Maternal parenting stress and its correlates in families with a young child with cerebral palsy. Child. 2009;35:71-78. 5. Sigurdardottir S, Indredavik MS, Eiriksdottir A, Einarsdottir K, Gudmundsson HS, Vik T. Behavioural and emotional symptoms of preschool children with cerebral palsy: a population-based study. Dev Med Child Neurol. 2010;52:1056-1061. 6. Majnemer A, Shevell M, Rosenbaum P, Law M, Poulin C. Determinants of life quality in school-age children with cerebral palsy. J Pediatr. 2007;151:470-475, 475.e1-475.e3. 7. Eiser C, Morse R. Can parents rate their child’s health-related quality of life? Results of a systematic review. Qual Life Res. 2001;10:347-357.
8. Group W. Study protocol for the World Health Organization project to develop a Quality of Life assessment instrument (WHOQOL). Qual Life Res. 1993;2:153-159. 9. Davis E, Waters E, Mackinnon A, et al. Paediatric quality of life instruments: a review of the impact of the conceptual framework on outcomes. Dev Med Child Neurol. 2006;48: 311-318. 10. Livingston MH, Rosenbaum PL, Russell DJ, Palisano RJ. Quality of life among adolescents with cerebral palsy: what does the literature tell us? Dev Med Child Neurol. 2007;49:225-231. 11. Wake M, Hesketh K, Cameron F. The Child Health Questionnaire in children with diabetes: cross-sectional survey of parent and adolescent-reported functional health status. Diabetic Med. 2000;17:700-707. 12. Waters EB, Wake MA, Hesketh KD, Ashley DM, Smibert E. Health-related quality of life of children with acute lymphoblastic leukaemia: comparisons and correlations between parent and clinician reports. Int J Cancer J. 2003;103:514-518. 13. Ravens-Sieberer U, Gosch A, Rajmil L, et al. KIDSCREEN-52 quality-of-life measure for children and adolescents. Exp Rev Pharmacoecon Outcomes Res. 2005;5:353-364. 14. Dieruf K, Burtner PA, Provost B, Phillips J, Bernitsky-Beddingfield A, Sullivan KJ. A pilot study of quality of life in children with cerebral palsy after intensive body weight-supported treadmill training. Pediatr Phys Ther. 2009;21:45-52. 15. Seid M, Varni JW, Kurtin PS. Measuring quality of care for vulnerable children: challenges and conceptualization of a pediatric outcome measure of quality. Am J Med Qual. 2000;15:182-188. 16. Dickinson HO, Parkinson KN, Ravens-Sieberer U, et al. Selfreported quality of life of 8-12-year-old children with cerebral palsy: a cross-sectional European study. Lancet. 2007;369:2171-2178. 17. Vinson J, Shank L, Thomas P, Warschausky S. Self-generated domains of quality of life in children with and without cerebral palsy. J Dev Phys Disabil. 2010;22:497-508. 18. Majnemer A, Mazer B. New directions in the outcome evaluation of children with cerebral palsy. Semin Pediatr Neurol. 2004;11: 11-17. 19. Ravens-Sieberer U, Gosch A, Abel T, et al. Quality of life in children and adolescents: a European public health perspective. Soz Pra¨ventivmed. 2001;46:294-302. 20. Bjornson KF, McLaughlin JF. The measurement of health-related quality of life (HRQL) in children with cerebral palsy. Eur J Neurol. 2001;8(suppl 5):183-193. 21. Maher CA, Olds T, Williams MT, Lane AE. Self- reported quality of life in adolescents with cerebral palsy. Phys Occup Ther Pediatr. 2008;28:41-57. 22. Sakzewski L, Ziviani J, Boyd RN. Best responders after intensive upper-limb training for children with unilateral cerebral palsy. Arch Phys Med Rehabil. 2011;92:578-584. 23. Waters E, Davis E, Mackinnon A, et al. Psychometric properties of the quality of life questionnaire for children with CP. Dev Med Child Neurol. 2007;49:49-55. 24. Narayanan UG, Fehlings D, Weir S, Knights S, Kiran S, Campbell K. Initial development and validation of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD). Dev Med Child Neurol. 2006;48:804-812.
Downloaded from jcn.sagepub.com at UMKC University Libraries on June 16, 2014
Gilson et al
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25. Baars RM, Atherton CI, Koopman HM, Bullinger M, Power M. The European DISABKIDS project: development of seven conditionspecific modules to measure health related quality of life in children and adolescents. Health Qual Life Outcomes. 2005;3:70. 26. Carlon S, Shields N, Yong K, Gilmore R, Sakzewski L, Boyd R. A systematic review of the psychometric properties of quality of life measures for school aged children with cerebral palsy. BMC Pediatr. 2010;10:81. 27. Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care. 2001;39:800-812. 28. Ravens-Sieberer U, Schmidt S, Gosch A, Erhart M, Petersen C, Bullinger M. Measuring subjective health in children and adolescents: results of the European KIDSCREEN/DISABKIDS Project. Psychosoc Med. 2007;4:Doc08. 29. Varni JW, Burwinkle TM, Sherman SA, et al. Health-related quality of life of children and adolescents with cerebral palsy: hearing the voices of the children. Dev Med Child Neurol. 2005;47:592-597. 30. Waters E, Maher E, Salmon L, Reddihough D, Boyd R. Development of a condition-specific measure of quality of life for children with cerebral palsy: empirical thematic data reported by parents and children. Child. 2005;31:127-135. 31. White-Koning M, Arnaud C, Dickinson HO, et al. Determinants of child-parent agreement in quality-of-life reports: a European
32.
33.
34.
35.
36.
37.
38.
study of children with cerebral palsy. Pediatrics. 2007;120: e804-e814. Parkinson KN, Rice H, Young B. Incorporating children’s and their parents’ perspectives into condition-specific quality-of-life instruments for children with cerebral palsy: a qualitative study. Value Health. 2011;14:705-711. Davis E, Shelly A, Waters E, et al. Quality of life of adolescents with cerebral palsy: perspectives of adolescents and parents. Dev Med Child Neurol. 2009;51:193-199. Waters E, Davis E, Ronen GM, Rosenbaum P, Livingston M, Saigal S. Quality of life instruments for children and adolescents with neurodisabilities: how to choose the appropriate instrument. Dev Med Child Neurol. 2009;51:660-669. Arnaud C, White-Koning M, Michelsen SI, et al. Parent-reported quality of life of children with cerebral palsy in Europe. Pediatrics. 2008;121:54-64. Houlihan CM, O’Donnell M, Conaway M, Stevenson RD. Bodily pain and health-related quality of life in children with cerebral palsy. Dev Med Child Neurol. 2004;46:305-310. Wiley R, Renk K. Psychological correlates of quality of life in children with cerebral palsy. J Dev Phys Disabil. 2007;19: 427-447. Tsoi WS, Zhang LA, Wang WY, Tsang KL, Lo SK. Improving quality of life of children with cerebral palsy: a systematic review of clinical trials. Child. 2012;38:21-31.
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Quality of Life in Children With Cerebral Palsy: Implications for Practice Kim-Michelle Gilson, Elise Davis, Dinah Reddihough, Kerr Graham and Elizabeth Waters J Child Neurol published online 27 May 2014 DOI: 10.1177/0883073814535502 The online version of this article can be found at: http://jcn.sagepub.com/content/early/2014/05/27/0883073814535502
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Original Article
Quality of Life in Children With Cerebral Palsy: Implications for Practice
Journal of Child Neurology 1-7 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/0883073814535502 jcn.sagepub.com
Kim-Michelle Gilson, PhD1, Elise Davis, PhD1, Dinah Reddihough, MD, BSc, FRACP, FAFRM2,3,4, Kerr Graham, MD, FRCS(Ed), FRACS5, and Elizabeth Waters, DPhil1
Abstract The ability to assess the quality of life of children with cerebral palsy to inform and evaluate individual care plans, service planning, interventions, and policies is crucial. In this article, the recent evidence on quality of life in children with cerebral palsy is reviewed, with attention to the determinants of quality of life and role of this construct as a practical outcome indicator in clinical trials. Quality of life measurement advances for children with cerebral palsy are discussed with a focus on conditionspecific quality of life measures, particularly, the Cerebral Palsy Quality of Life–Child, which is the first condition-specific quality of life measure for children with cerebral palsy. The article presents an overview for clinicians and researchers intending to use quality of life measures on children with cerebral palsy and provides recommendations for future research that will better inform practice in the field. Keywords Cerebral Palsy Quality of Life–Child, measurement, disability, outcome, determinants Received April 01, 2014. Received revised April 01, 2014. Accepted for publication April 03, 2014.
Cerebral palsy is the most frequent cause of physical disability in children, occurring in approximately 2 to 2.5 per 1000 live births.1 Cerebral palsy refers to a group of disorders with a diverse range of etiological pathways, pathophysiologies, and clinical features that persist through the life span. The most recent definition is as follows: ‘‘A group of developmental disorders of movement and posture, causing activity restriction or disability attributed to disturbances occurring in the foetal or infant brain. The motor impairment may be accompanied by a seizure disorder and by impairment of sensation, cognition, communication and/or behaviour and by secondary musculoskeletal problems.’’2
indication of their well-being across several life domains such as physical health and social and emotional well-being. The aim of this article is to introduce the quality of life construct and provide an overview of what is now known about quality of life in children with cerebral palsy, with a particular focus on measurement, determinants, and relevance for clinical practice.
Quality of Life Quality of life is considered a broad and multidimensional concept that includes subjective evaluations of both the 1
Treatment of the motor disorder alone may involve a range of therapies and interventions such as physiotherapy, the use of orthoses, orthopedic surgery, and medications for spasticity.3 It is not surprising that cerebral palsy can have profound effects on the physical, social, and emotional health and wellbeing of the child, including the parents.4 Children with cerebral palsy not only have to contend with a range of physical problems, such as muscle weakness, stiffness, and clumsiness, they are also 4 times more likely than their peers to experience emotional and behavioral problems.5 Quality of life is a very relevant and important construct in the context of children with cerebral palsy because it can provide a broad subjective
The Jack Brockhoff Child Health and Wellbeing Program, Academic Centre for Health Equity, Melbourne School of Population and Global Health, University of Melbourne, Australia 2 Department of Developmental Medicine, Royal Children’s Hospital, Melbourne, Australia 3 Department of Paediatrics, University of Melbourne, Melbourne, Victoria, Australia 4 Murdoch Children’s Research Institute, Melbourne, Victoria, Australia 5 Department of Orthopaedic Surgery, Royal Children’s Hospital, Melbourne, Australia Corresponding Author: Kim-Michelle Gilson, PhD, Melbourne School of Population and Global Health, Level 5, 207 Bouverie Street, University of Melbourne, Australia. Email: [email protected]
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positive and negative aspects of life. It embraces the notion of holistic well-being and encompasses elements about general functioning and health, but also extends beyond these to include a person’s appraisal of their life experiences and social and emotional well-being.6 Definitions of quality of life vary across disciplines and are often complex and difficult to operationalize.7 The most cited definition by the World Health Organization is ‘‘an individuals’ perception of their position in life, in the context of culture and value systems in which they live and in relation to their goals, expectations, standards and concerns.’’8 Although there is considerable agreement that quality of life is multidimensional, there is much variation in the content of the domains that comprise quality of life for children and, particularly, for children with disabilities. Early conceptualizations of quality of life were based heavily on functioning and measures that purported to assess quality of life focused on physical health and functional limitations (e.g., what a person can and cannot do) rather than an individual’s broader well-being or personal experience.9 The traditional assumption that functioning equates with overall quality of life has been brought into question by several empirical studies that show a weak or nonsignificant relationship between a child’s functioning and overall quality of life,10 in addition to specific domains, such as social and emotional functioning.6,11,12 These findings are important because they suggest that a child can still experience a good quality of life despite experiencing significant functional limitations.
Quality of Life as an Important Outcome Measure In the context of rehabilitation, quality of life has clinical utility as an important health-related outcome measure that can guide practice.13 It can provide a comprehensive picture of the overall impact of a health condition on a child’s social, emotional, and physical well-being and therefore offers essential information for decision making in clinical practice.14,15 Increasingly, patients’ well-being and their functioning in everyday life are an important part of a clinical examination, and determining patient quality of life can assist health professionals to make decisions about how best to plan care interventions that are appropriate and individualized.16,17 In this sense, quality of life measures can be complementary to objective measures of functioning that are administered by rehabilitation professionals.18 Children and their families may also benefit from the information that quality of life assessments can provide, particularly giving them hope if positive scores are observed on particular domains, or by facilitating discussions over treatment suitability.
Measuring Quality of Life in Children With Cerebral Palsy In recent years, there has been increasing interest in developing valid measures of quality of life. This has been both an attempt
to understand the impact of cerebral palsy on a child’s whole life, and in strong efforts to obtain empirical-based evidence to guide practice in the field particularly with therapeutic interventions for children with cerebral palsy. There are now a number of generic and condition-specific scales available to measure QOL in children with cerebral palsy, and these are available in the form of child self-report or parent proxy versions. Given the subjective nature of quality of life, it has been argued that whenever possible, quality of life should be self-reported by the individual.19 However, given the range of impairment in children with cerebral palsy, this is not always possible and parents may rate (1) their own perceptions of the child’s quality of life or (2) what they believe is the child’s own perception. Generic quality of life scales are designed to be applicable to all population subgroups and are useful when making comparisons between groups and with norms from the general population.20,21 It therefore follows that generic instruments used to measure quality of life in children with cerebral palsy might not have been designed for that purpose and might not include domains or items that specifically tap into their lived experience. For example, the Pediatric Outcomes Data Collections Instrument, Child Health Questionnaire, and Lifestyle Assessment Questionnaire have been used to measure quality of life 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. Another common instrument is the KIDSCREEN,13 which is a health-related quality of life measure not specific to children with cerebral palsy. Condition-specific scales, on the other hand, are designed to be applicable to 1 group and therefore target issues pertinent to the individual’s condition. The domains and items in these instruments are derived from the lived experience of children with cerebral palsy and therefore correspond to what they consider important to their life. This also means that they are suitable for detecting change in the quality of life domains that are important for a specific condition.20,22 Over the last 8 years, 4 new condition-specific instruments have been developed to measure the quality of life of children with cerebral palsy. Measures now include the Cerebral Palsy Quality of Life–Child (CP QOL-Child23); the Caregiver Priorities and Child Health Index of Life with Disabilities24; the Pediatric Quality of life Inventory CP Module27; and the DISABKIDS CP Module.25 A recent systematic review of these condition-specific instruments that exist for school-aged children with cerebral palsy reported that the Cerebral Palsy Quality of Life–Child measure has the strongest psychometric properties and clinical utility followed by Caregiver Priorities and Child Health Index of Life with Disabilities.26 This was determined by reviewing published data on concurrent, construct, and content validity and evidence of internal and test-retest reliability for all measures. The review highlighted that some of these instruments had a heavy emphasis on functioning, such as the Pediatric Quality of life Inventory, where 23 of the 35 items related
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Table 1. Overview of the Condition Specific Quality of Life Instruments for Children With Cerebral Palsy. Cerebral Palsy Quality of life Questionnaire for Children (CP QOL-Child)
Caregiver Priorities and Child Pediatric Quality of Life Inventory (PedsQL) Health Index of Life with CP Module Disabilities (CPCHILD)
Description
A condition-specific outcome measure intended to gauge and assess quality of life changes in children with cerebral palsy
Age range (y) Completion time (min) Proxy (caregiver) report
4-12 15-25 Yes (for children aged 4-12)
Self-report (age) Country of origin Number of items Domains
Yes (9þ) Australia 52 Social well-being and acceptance, functioning, participating and physical health, access to services,a family health,a emotional wellbeing, pain and feelings about disability
To measure health-related To measure healthA measure of health status related quality of quality of life in a popuand well-being for children life and assess lation of children and with cerebral palsy burden of disease adolescents with cerein children and bral palsy adolescents 5-18 2-18 4-16 20-30 5 N/A Yes (for children aged 5-12) Yes (for children aged 2-18) Yes (for children aged 4-16) No Yes (5-18) Yes (4-16) Canada USA Europe 36 35 16 Generic module, a Daily activities, school Personal care; positioning, chronic generic activities, movement and transfers and mobility; module and a balance, pain and hurt, communication and condition-specific fatigue, eating activities comfort, emotions and module and speech and behavior; health; and communication overall quality of life
Instrument
DISABKIDS-CP module
Abbreviation: CP, cerebral palsy. a Only the primary caregiver-proxy version of the CP quality of life contains the 2 domains of access to services and family health.
to functional tasks such as school activities, daily activities, or eating activities. Another important finding was that the Cerebral Palsy Quality of Life–Child was the only measure to base its development on a theoretical construct of quality of life, that is, measuring well-being rather than ill-being and difficulty, and having broad domains. The attributes of the condition-specific instruments, including their domains and psychometric properties, are listed in Tables 1 and 2.
The Cerebral Palsy Quality of Life Questionnaire The Cerebral Palsy Quality of Life–Child was developed by a team of clinicians and researchers in Australia and was the first condition-specific quality of life measure for children with cerebral palsy aged 4 to 12 years.23 In contrast to other measures, the Cerebral Palsy Quality of Life–Child was based on qualitative interviews with children with cerebral palsy and their parents. These interviews focused on what the child needed to be happy and have a good quality of life and provided content for the development of items.30 Until recently, instruments have measured quality of life domains that were decided a priori by professional ‘‘experts’’ (researchers and clinicians) rather than through eliciting the perspective of the audience for which they are designed. There is now an increasing recognition that families and children should be consulted and measures developed based on their perspectives. The Cerebral Palsy Quality of Life–Child assesses several aspects of well-being, rather than functioning, and 2 parallel
forms of the measure exist: a primary caregiver-proxy version that is for parents of children aged 4 to 12 years, and a child self-report version for children aged 9 to 12 years. It is important to have a child self-report version, given that quality of life is, by definition, an individual’s perceptions. Furthermore, parents’ views of their children’s quality of life may be affected by their own mental health and well-being associated with caregiving.31 Since the development of the Cerebral Palsy Quality of Life–Child in 2007, it has been translated into 20 different languages (Greek, Hebrew, Polish, Arabic, Chinese, Flemish, Tamil, Bahasa, Italian, Dutch, Thai, French, Spanish, Korean, Turkish, Farsi, Burmese, Malayalam, Finnish, and Portuguese) and cited nearly 70 times. The international support for the translation and psychometric properties of the Cerebral Palsy Quality of Life–Child demonstrates the widespread need for such a measure and its applicability cross-culturally. Qualitative research in the United Kingdom has also demonstrated that items of Cerebral Palsy Quality of Life–Child correspond with the perspectives provided by children and their parents who discussed the quality of life of their child.32
Guidance on Selecting the Most Appropriate Measure for Assessing Quality of Life Although it is encouraging that there are now several quality of life instruments relevant for children with cerebral palsy, the wider choice means that it is becoming increasingly difficult for clinicians and researchers to select the most appropriate quality of life instrument for their purpose. From our work in
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Table 2. Condition-Specific Quality of Life Instruments for Children With CP: A Comparison of Psychometric Attributes.26
Instrument Reliability tested for CP Internalreliability
Retest reliability (intraclass correlation coefficient statistic) Validity tested for CP Content validity
Concurrent validity
Construct validity
Sensitivity to change examined
Cerebral Palsy Quality of Caregiver Priorities and Child Pediatric Quality of Life Inventory (PedsQL)–CP Health Index of Life with life Questionnaire for Module Children (CP QOL-Child) Disabilities (CPCHILD) Yes23 Yes24 a ¼ 0.74-0.92 (caregivers) a ¼ 0.74-0.93 across all domains a ¼ 0.80-0.90 (child-self report) 0.76-0.89 0.97 for the total questionnaire; 0.88-0.96 across the 6 domains; (95% CI ¼ 0.88-0.99) Yes23 Yes24 Caregiver rated importance Domains determined in of items x ¼ 3.95 on 6consultation with point ordinal scale (SD ¼ children and their 0.63, range 2.67-4.90) parents (28 families) Domains moderately Domains moderately correlated to those of correlated to those of CHQ and PEDI KIDSCREEN and CHQ Higher GMFCS scores Global QOL r ¼ 0.18correlated with higher 0.62; Global Health CPCHILD scores (worse r ¼ 0.21-0.56 outcome). No No
Yes27 Child self-report: 0.77-0.93 N/A
Yes27 N/A
N/A
Can distinguish between typically developing children and children with CP Yes, across gross motor function levels29
DISABKIDS-CP Module Yes28 a ¼ 0.71-0.91 interrater reliability and 0.14-0.84 across scales N/A
Yes28 Domains determined in consultation with children and their parents (9 families) N/A
Can differentiate between levels of disability than CHQ and KINDL No
Abbreviations: CP, Cerebral Palsy; CPCHILD, Caregiver Priorities and Child Health Index of Life with Disabilities; GMFCS, Gross Motor Function Classification System; PEDI, Pediatric Evaluation of Disability System; Inventory; CHQ, Child Health Questionnaire; KINDL, a generic health-related quality of life measure. This table is based on the systematic review provided by Carlon et al.26
developing a condition-specific quality of life measure, and reviewing existing quality of life instruments for children with cerebral palsy, brief criteria have been developed that can assist researchers and clinicians in their choice of a quality of life measure for children with cerebral palsy. These characteristics (shown in Table 3) have been used to compare quality of life scales for children with cerebral palsy33,34 and pediatric populations without a disability.9 With a focus on the health professional within a clinical context, we also suggest a number of core questions that might help guide the clinician through their choice of instrument (Table 4). It is recognized that future research is also needed to provide clearer guidance in relation to these questions.
Determinants of Quality of Life in Children With Cerebral Palsy Understanding the determinants of quality of life gives us some insight into what factors may be important to consider for programs and interventions aiming to have an impact on quality of life. As rehabilitation and other pediatric health care specialists continue to broaden their intervention goals to include quality of life measures, it is essential that the contributing factors, or determinants, of quality of life in children with cerebral palsy become better understood. This can also assist clinicians to identify children who are more likely to have poor quality of life and, therefore, inform their treatment plan.
Studies on children with cerebral palsy have focused mainly on small groups of children selected from clinics with homogenous degrees of impairment severity, being skewed toward less motor impairment rather than across the spectrum.10,35 In line with a greater appreciation of the multidimensionality of quality of life, studies have reported independent predictors for different quality of life domains such as physical and psychosocial well-being. Focusing on domains has also shown significant variability across studies, with evidence to show that some children with cerebral palsy experience similar quality of life domains compared to typically developing children in spite of important functional limitations.6,16,35 One of the few population-based approaches to study the determinants was the European Union funded project (SPARCLE) conducted across 7 countries.35 This examined whether the severity of the child’s motor impairment and associated difficulties in IQ and pain predicted a comprehensive set of quality of life domains using the KIDSCREEN. Severity of motor impairment was associated with poor quality of life in terms of physical well-being and autonomy but not to psychosocial quality of life domains. Lower IQ was associated with the poor social support domain but not the moods and emotions and self-perception domains. In contrast, pain was associated with multiple poor quality of life domains, specifically, physical well-being, psychological well-being, and self-perception. Other studies36 have also found associations between pain and motor impairment severity and to adverse
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Table 3. Characteristics to Evaluate and Select an Appropriate Quality of Life Instrument. Characteristic
Gold standard
Original purpose of the instrument Origin of items
Developed to measure quality of life
Actual focus of the instrument Opportunity for self-report Potential threat to self-esteem Length Psychometric properties Conceptual underpinningsa
Based on the perspectives of children and parents Focuses on well-being
targeting stress management may contribute in helping to improve child quality of life. Clinical trials have focused primarily on medical interventions to treat the child’s impairment and assess change in functioning quality of life domains.38 There is a need to look toward interventions that consider other quality of life domains as being important outcomes for the child. This could include the consideration of caregiver support and the participation of the child as important components to interventions where quality of life is an outcome measure.
Has a self-report version available Positively worded Small number of items Has excellent psychometrics properties Strong conceptual underpinnings
a To have strong conceptual underpinnings, Davis et al9 suggest that the measure (1) must rest on a clear, operationalized definition of quality of life; (2) must be based on a theory of quality of life; (3) must include the important domains of life for children; and (4) must have well-constructed items.
Table 4. Questions for Clinicians Choosing a Quality of Life Instrument. Questions for clinicians What is the reason for measuring quality of life? Who is the respondent? Is a generic or condition-specific quality of life instrument needed? What is level of functioning of the child according to the GMFCS? What domains of quality of life do I need to measure? Does the intended measure have strong psychometric properties? Abbreviation: GMFCS, Gross Motor Function Classification System.
educational and social consequences. In regards to the family, parental stress consistently predicted poor quality of life in all domains, and single-parent households was associated with poor quality of life in the mood and emotions domain. These findings convey the complexity in delineating the predictors of quality of life in children with cerebral palsy, particularly given the wide range of impairment that accompanies cerebral palsy and the multidimensionality of quality of life. A study that assessed quality of life by both child and parent report was conducted by Majnemer et al,6 who found that poor socialization skills, reduced general competence, and behavioral difficulties were significantly associated with poor psychosocial well-being on both the Child Health Questionnaire (CHQ) and the Pediatric Quality of life Inventory in children with cerebral palsy. This highlights important areas for intervention beyond improving the motor impairment, particularly as social skills and behavioral problems can potentially be modified by health professionals to enhance quality of life.6 Higher levels of parental stress were also strongly associated with child’s psychosocial quality of life. Poor parental mental health is associated with poor quality of life on a number of different quality of life measures in children with cerebral palsy.35,37 From a clinical point of view, this suggests that targeted support by health professionals in the form of resources to enhance caregiver coping or programs
Conclusion and Directions for Future Research Research on the quality of life in children with cerebral palsy has made significant progress in the last 10 years with improved knowledge of the determinants and measurement approaches to quality of life and clinical practice. An overall advancement is our improved understanding of the quality of life construct and how this can be investigated within the complexity of cerebral palsy. There is a much stronger foundation to work toward improving the quality of life of children with cerebral palsy, which will enable significant progress over the forthcoming years. A number of important areas for future research are recommended. The first concerns the lack of research on sensitivity to change of quality of life instruments and on how quality of life instruments are used to evaluate the effectiveness of rehabilitation interventions. This is a major area for further work because quality of life instruments are increasingly being relied upon, yet there is still little understanding about if and how quality of life can be improved. This is a particularly complex issue given the many factors that can determine an individual’s quality of life. There is very limited data on if and how measures are being used and what quality of life instruments service providers and health professionals prefer to use. Therefore, further work should assess the role of quality of life instruments in clinical settings, which may then guide measure development or refinement. A wider understanding of the determinants of quality of life in children with cerebral palsy is needed. A multidimensional conceptual framework such as the ICF model1 could be applied to elucidate the determinants that either facilitate or hinder quality of life. This should be done using a population-based approach with greater inclusion of children from across the spectrum of gross motor impairment. Finally, research is needed on how responsive quality of life instruments are according to different Global Motor Function Classification levels, which would guide clinicians in their decision to selecting the most appropriate instrument.
Key Take Home Messages
Quality of life is a multidimensional construct. Measures encompass a number of domains and may be useful in evaluating the effectiveness of interventions and informing treatment care plans.
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Impairment does not correlate with poor quality of life. Impairment may predict poor physical well-being and other functional quality of life domains, but not more psychosocial domains of quality of life. There are now a number of psychometrically sound condition-specific quality of life measures for children with cerebral palsy. When choosing among various quality of life instruments, researchers and clinicians should consider the purpose of the instrument, number of items, the domains, country of origin, reliability, validity, sensitivity to change, age range, and respondents. Further work is needed on population-based samples across the spectrum of gross motor impairment levels to draw firm conclusions about the determinants and modifiers of child quality of life.
Author Contributions K-MG wrote the first draft of the manuscript and all authors contributed equally to the editing and revision of this manuscript.
Declaration of Conflicting Interests The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: For underlying research conducted by the authors that is discussed in this special issue paper, funding was received by the National Health Medical Research Grant (NHMRC 284514), Murdoch Children’s Research Institute, and Victorian Health Promotion Foundation.
References 1. ICF. International Classification of Functioning, Disability and Health. Available at: http://www.who.int/icf. 2. Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl. 2007;109:8-14. 3. Boyd RN, Hays RM. Current evidence for the use of botulinum toxin type A in the management of children with cerebral palsy: a systematic review. Eur J Neurol. 2001;8(suppl 5):1-20. 4. Glenn S, Cunningham C, Poole H, Reeves D, Weindling M. Maternal parenting stress and its correlates in families with a young child with cerebral palsy. Child. 2009;35:71-78. 5. Sigurdardottir S, Indredavik MS, Eiriksdottir A, Einarsdottir K, Gudmundsson HS, Vik T. Behavioural and emotional symptoms of preschool children with cerebral palsy: a population-based study. Dev Med Child Neurol. 2010;52:1056-1061. 6. Majnemer A, Shevell M, Rosenbaum P, Law M, Poulin C. Determinants of life quality in school-age children with cerebral palsy. J Pediatr. 2007;151:470-475, 475.e1-475.e3. 7. Eiser C, Morse R. Can parents rate their child’s health-related quality of life? Results of a systematic review. Qual Life Res. 2001;10:347-357.
8. Group W. Study protocol for the World Health Organization project to develop a Quality of Life assessment instrument (WHOQOL). Qual Life Res. 1993;2:153-159. 9. Davis E, Waters E, Mackinnon A, et al. Paediatric quality of life instruments: a review of the impact of the conceptual framework on outcomes. Dev Med Child Neurol. 2006;48: 311-318. 10. Livingston MH, Rosenbaum PL, Russell DJ, Palisano RJ. Quality of life among adolescents with cerebral palsy: what does the literature tell us? Dev Med Child Neurol. 2007;49:225-231. 11. Wake M, Hesketh K, Cameron F. The Child Health Questionnaire in children with diabetes: cross-sectional survey of parent and adolescent-reported functional health status. Diabetic Med. 2000;17:700-707. 12. Waters EB, Wake MA, Hesketh KD, Ashley DM, Smibert E. Health-related quality of life of children with acute lymphoblastic leukaemia: comparisons and correlations between parent and clinician reports. Int J Cancer J. 2003;103:514-518. 13. Ravens-Sieberer U, Gosch A, Rajmil L, et al. KIDSCREEN-52 quality-of-life measure for children and adolescents. Exp Rev Pharmacoecon Outcomes Res. 2005;5:353-364. 14. Dieruf K, Burtner PA, Provost B, Phillips J, Bernitsky-Beddingfield A, Sullivan KJ. A pilot study of quality of life in children with cerebral palsy after intensive body weight-supported treadmill training. Pediatr Phys Ther. 2009;21:45-52. 15. Seid M, Varni JW, Kurtin PS. Measuring quality of care for vulnerable children: challenges and conceptualization of a pediatric outcome measure of quality. Am J Med Qual. 2000;15:182-188. 16. Dickinson HO, Parkinson KN, Ravens-Sieberer U, et al. Selfreported quality of life of 8-12-year-old children with cerebral palsy: a cross-sectional European study. Lancet. 2007;369:2171-2178. 17. Vinson J, Shank L, Thomas P, Warschausky S. Self-generated domains of quality of life in children with and without cerebral palsy. J Dev Phys Disabil. 2010;22:497-508. 18. Majnemer A, Mazer B. New directions in the outcome evaluation of children with cerebral palsy. Semin Pediatr Neurol. 2004;11: 11-17. 19. Ravens-Sieberer U, Gosch A, Abel T, et al. Quality of life in children and adolescents: a European public health perspective. Soz Pra¨ventivmed. 2001;46:294-302. 20. Bjornson KF, McLaughlin JF. The measurement of health-related quality of life (HRQL) in children with cerebral palsy. Eur J Neurol. 2001;8(suppl 5):183-193. 21. Maher CA, Olds T, Williams MT, Lane AE. Self- reported quality of life in adolescents with cerebral palsy. Phys Occup Ther Pediatr. 2008;28:41-57. 22. Sakzewski L, Ziviani J, Boyd RN. Best responders after intensive upper-limb training for children with unilateral cerebral palsy. Arch Phys Med Rehabil. 2011;92:578-584. 23. Waters E, Davis E, Mackinnon A, et al. Psychometric properties of the quality of life questionnaire for children with CP. Dev Med Child Neurol. 2007;49:49-55. 24. Narayanan UG, Fehlings D, Weir S, Knights S, Kiran S, Campbell K. Initial development and validation of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD). Dev Med Child Neurol. 2006;48:804-812.
Downloaded from jcn.sagepub.com at UMKC University Libraries on June 16, 2014
Gilson et al
7
25. Baars RM, Atherton CI, Koopman HM, Bullinger M, Power M. The European DISABKIDS project: development of seven conditionspecific modules to measure health related quality of life in children and adolescents. Health Qual Life Outcomes. 2005;3:70. 26. Carlon S, Shields N, Yong K, Gilmore R, Sakzewski L, Boyd R. A systematic review of the psychometric properties of quality of life measures for school aged children with cerebral palsy. BMC Pediatr. 2010;10:81. 27. Varni JW, Seid M, Kurtin PS. PedsQL 4.0: reliability and validity of the Pediatric Quality of Life Inventory version 4.0 generic core scales in healthy and patient populations. Med Care. 2001;39:800-812. 28. Ravens-Sieberer U, Schmidt S, Gosch A, Erhart M, Petersen C, Bullinger M. Measuring subjective health in children and adolescents: results of the European KIDSCREEN/DISABKIDS Project. Psychosoc Med. 2007;4:Doc08. 29. Varni JW, Burwinkle TM, Sherman SA, et al. Health-related quality of life of children and adolescents with cerebral palsy: hearing the voices of the children. Dev Med Child Neurol. 2005;47:592-597. 30. Waters E, Maher E, Salmon L, Reddihough D, Boyd R. Development of a condition-specific measure of quality of life for children with cerebral palsy: empirical thematic data reported by parents and children. Child. 2005;31:127-135. 31. White-Koning M, Arnaud C, Dickinson HO, et al. Determinants of child-parent agreement in quality-of-life reports: a European
32.
33.
34.
35.
36.
37.
38.
study of children with cerebral palsy. Pediatrics. 2007;120: e804-e814. Parkinson KN, Rice H, Young B. Incorporating children’s and their parents’ perspectives into condition-specific quality-of-life instruments for children with cerebral palsy: a qualitative study. Value Health. 2011;14:705-711. Davis E, Shelly A, Waters E, et al. Quality of life of adolescents with cerebral palsy: perspectives of adolescents and parents. Dev Med Child Neurol. 2009;51:193-199. Waters E, Davis E, Ronen GM, Rosenbaum P, Livingston M, Saigal S. Quality of life instruments for children and adolescents with neurodisabilities: how to choose the appropriate instrument. Dev Med Child Neurol. 2009;51:660-669. Arnaud C, White-Koning M, Michelsen SI, et al. Parent-reported quality of life of children with cerebral palsy in Europe. Pediatrics. 2008;121:54-64. Houlihan CM, O’Donnell M, Conaway M, Stevenson RD. Bodily pain and health-related quality of life in children with cerebral palsy. Dev Med Child Neurol. 2004;46:305-310. Wiley R, Renk K. Psychological correlates of quality of life in children with cerebral palsy. J Dev Phys Disabil. 2007;19: 427-447. Tsoi WS, Zhang LA, Wang WY, Tsang KL, Lo SK. Improving quality of life of children with cerebral palsy: a systematic review of clinical trials. Child. 2012;38:21-31.
Downloaded from jcn.sagepub.com at UMKC University Libraries on June 16, 2014
