The Pediatric Quality of Life Inventory™ in children with heart disease
Introduction
In recent decades we have enjoyed a progressive reduction in mortality in children with heart disease. Surgical advances have allowed increased longevity for many children with complex heart defects, which were previously considered inoperable. Quality of life has emerged as an important outcome in these children. In the past, most outcome studies in patients with congenital heart disease (CHD) have examined health status, including anatomic or hemodynamic sequelae, electrophysiologic sequelae, and exercise capacity following surgical intervention [1], [2], [3]. The Second Natural History Study of Congenital Heart Defects [4] included ‘quality of life’ parameters such as marital status, number of offspring, employment status, educational attainment, and health insurance coverage. These studies have included a personal assessment of health limited to a single question with responses ranging from excellent to poor. Traditional clinical and biomedical measures lack the ability to assess the full impact of heart disease during childhood and adolescence. As recent studies suggest that quality of life cannot be simply inferred from clinical and laboratory findings [5], [6], [7], development of a measure for assessing quality of life in this pediatric population is imperative to optimize health care interventions and outcomes.
Most health-related quality of life measures have evolved from the World Health Organization's [8] definition of health as ‘not only the absence of disease and infirmity, but the presence of physical, mental and social well-being’ [9]. In addition to being multidimensional, pediatric quality of life measures must also consider developmental changes during childhood. In a review of health-related quality of life measures in pediatric populations, Spieth and Harris [10] found that past measurements have utilized a battery of measures focused on either a general or a disease-specific population, utilized a global rating (summary of functioning), or been insensitive to variations in age or developmental differences. The long length of some commonly utilized child-report instruments can be a barrier to data completeness [9]. Other measurements have relied on parent or proxy rating of the child's quality of life [11] despite an observed lack of significant agreement, referred to as ‘cross-informant variance’, in multiple pediatric studies [12], [13], [14], [15]. Finally, controversy exists regarding the relative merits of generic measures that allow standardized comparisons across healthy and patient populations vs. disease-specific measures that may have greater clinical utility in assessing concerns that are particular to a disease or population [10].
In children with heart disease, there has been a paucity of studies evaluating health-related quality of life which evaluate the patient's self perceptions or parent perceptions of the child's psychological and social functioning. Recently, neurodevelopmental status has received attention in outcome studies of children with CHD [16], [17], [18], [19], [20], [21]. Studies suggest that the majority of children have IQ scores within the normal range, but attention problems and learning disabilities are more prevalent in these patients. The latter can negatively impact psychosocial outcomes in these children. Campbell and colleagues [22] examined educational performance in 48 children with congenital heart disease. The children were at risk for learning problems regardless of the severity of the cardiac defect and, as reported by teachers, had more problems with socialization. Casey and colleagues [23] examined the behavioral adjustment at school age of 26 children with surgically palliated complex congenital heart disease. Children were rated by their parents as more withdrawn, having more social problems, and engaging in fewer physical activities. In children as young as 1–3 years, ‘functional disabilities’ have been reported to be highly prevalent, including difficulties in socialization and daily living activities [24]. An important study by DeMaso and associates [25] found that the severity of heart disease had no significant relationship to the child's emotional adjustment. Most of these studies of psychosocial functioning are limited by small sample size or measurement issues, especially lack of self-report.
The Pediatric Quality of Life Inventory (PedsQL™) is a modular instrument for measuring health-related quality of life dimensions in children and adolescents ages 2–18 years [26]. The PedsQL™ 4.0 Generic Core Scales are multidimensional (physical, emotional, social, school/role functioning) child self-report and parent proxy report scales developed as a generic measure to be integrated with the PedsQL™ disease-specific modules. The purpose of the current study was to determine the reliability and validity of the PedsQL™ 4.0 Core scales and the PedsQL™ 3.0 Cardiac Module in a diverse sample of children with heart disease.
Section snippets
Participants and setting
A convenience sample of children aged 5–18 years (n=250) and parents of children aged 2–18 years (n=344) were recruited from the pediatric cardiology clinic at Cincinnati Children's Hospital Medical Center. The average age of the child with heart disease for the entire group was 9.0 years, including 206 boys (60%) and 138 girls (40%). The mean age of child respondents was 11.7 years, with 50 children between 5 and 7 years, 97 children 8–12 years old, and 103 children 13–18 years of age. The
Missing item responses
To assess the feasibility or practicality of administration of the PedsQL™ 4.0 Generic Core Scales and the Cardiac Module scales, the percentage of missing values was calculated. For child self-report and parent proxy-report PedsQL™ 4.0 Core Scales, the percentage of missing item responses was 1 and 2%, respectively. If the toddler school subscale is eliminated from the parent-proxy report analysis, since toddlers frequently do not attend school or day care, the missing values for parents
Discussion
The study findings support the feasibility, reliability and validity of the PedsQL™ 4.0 Generic Core Scales and PedsQL™ 3.0 Cardiac Module scales as a child self-report for children 8–18 years and parent proxy report measure of health-related quality of life in pediatric cardiology. Items on the PedsQL™ had minimal missing responses, suggesting that children and parents are willing and able to provide good-quality data regarding the child's health-related quality of life. The PedsQL™ 4.0
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