Advances in the management of children with gastrointestinal and hepatic diseases have led to increased survival. Greater attention is now being given to the quality of life (QOL) of children living with chronic illness. From the perspective of the patients and their families, QOL can be as important as improved survival. Quality of life encompasses not only the physical and mental health of a person but also the impact of culture, environment, and economic factors. In the context of health care this translates to an attempt to measure the impact of a disease and its treatment on the lives of patients (ie, health-related QOL [HRQOL]). For the purpose of this review, QOL refers to HRQOL unless stated otherwise. Assessment of QOL should emphasize the patient's subjective impression of his or her life because previous studies have shown that there is little concordance between issues that are important to the lives of children with chronic illness and issues that health professionals think are important to their patients (1). Quality of life measures should encompass issues related to the physical illness and qualities that are independent of it, such as psychologic state, social relationships, and environment. Quality of life measurement may help to make health professionals more aware of issues that are important to the patient, including the emotional and psychological impact of an illness and its treatment. Patients may feel their opinion is being valued and may be more likely to adhere to the advice of health professionals.
Quality of life can be assessed by interviews or by using validated instruments, usually questionnaires. Quality of life instruments can be generic, providing an overall assessment of the child's QOL, or disease specific, in which the instrument deals with issues surrounding a particular illness. Generic instruments can be used in healthy people or patients with any illness. Disease-specific instruments include aspects of QOL relevant to that patient group and may be more sensitive in the detection of important changes.
Steps in the development of a QOL instrument may include definition of the purpose of the measurement of QOL, choice of a population of patients and their settings, generation of items of concern related to the illness (ie, item generation), and identification of the most important and common areas of concern (ie, item reduction).
Items of concern can be identified from patients by semistructured interviews or open-ended questionnaires and may also include the views of involved health professionals. Item reduction may be achieved by ranking the items in order of importance by the patients. These top-ranking items should then be incorporated into the questionnaire.
Validation of the instrument is important to make sure it is measuring what it is intended to measure. Different aspects of validity include face validity (ie, whether the questions are sensible), content validity (ie, whether the questions are relevant and the content is comprehensive), and criterion validity (ie, how it compares to existing measures). Reliability means that a similar score would be achieved if the questionnaire was repeated 4 to 6 weeks later (assuming there has been no change in clinical status and no major life events). Internal reliability, or internal consistency, refers to the domain structure of the instrument and assesses how well individual questions fit into particular domains. Sensitivity means that the instrument is sensitive to changes in clinical status.
The instrument also needs to be suitable for the population being studied: for children it needs to be age appropriate. Other important factors include administration time, comprehensibility, mode of administration, mode of response (eg, visual analogue scale or Likert scale), and mode of analysis (eg, via computer).
Eiser and Morse demonstrated a lack of validated QOL instruments in their review of QOL measures in chronic diseases of childhood (2). Assessment of QOL in children may be more complicated than in adults because they are undergoing constant physical, psychological, and emotional development. In younger children it may be further complicated by the need to involve the parents or caregivers (eg, in the completion of questionnaires).
The purpose of this review is to give an overview of studies that have attempted to assess QOL in children with gastrointestinal and hepatological diseases and to identify and appraise validated disease-specific QOL instruments.
A literature search was undertaken using MEDLINE, EMBASE, CINAHL and PsycINFO. The aim of the search was to identify all publications addressing QOL in children with gastrointestinal and liver diseases until the end of 2005. The search strategy was adapted from that used by Eiser and Morse (2), but instead of chronic diseases, we searched for gastrointestinal and liver diseases. The following search strategy was used to search MEDLINE and was modified for the other databases.
- “quality of life” OR satisfaction OR happiness OR “functional status” OR “health status” OR “well being”
- “quality of life” mesh/explode all subheadings
- #1 OR #2
- infant* OR child, preschool OR child* OR adoles*
- “infant” mesh/explode all subheadings
- “child, preschool” mesh/explode all subheadings
- “child” mesh/explode all subheadings
- “adolescent” mesh/explode all subheadings
- #4 OR #5 OR #6 OR #7 OR #8
- gastrointestinal disease OR (gastr* reflux) OR esophag* OR oesophag* OR gastr* OR ulcer* OR Hirschsprung OR intussusception OR (inflammatory bowel disease) OR crohn* OR intolerance OR malabsorp* OR celiac OR coeliac OR diarrh* OR constipat* OR (cystic fibrosis) OR pancrea* OR liver OR hepat*
- “digestive system diseases” mesh/explode all subheadings
- #10 OR #11
- #3 AND #9 AND #12
Reference lists of identified studies were searched for additional studies and the Patient-reported Outcome and Quality of Life Instruments Database (3) was searched for additional instruments. The abstracts of the publications identified in the search were then reviewed independently by the authors. Publications were excluded if no attempt was made to measure QOL, they did not relate to children younger than 17 years of age, or they did not relate to gastrointestinal or hepatological disorders. Review articles were also excluded. Full versions of the remaining articles were obtained and appraised using a data extraction form, modified from Eiser and Morse (2).
Details of the excluded and included articles are recorded in the Appendixes at the end of this article. All of the identified disease-specific instruments relating to pediatric gastrointestinal or liver disease are listed in Table 1. Because there are no agreed-upon guidelines for rating the quality of QOL instruments, we used the following criteria, which are considered (2) to be to be essential aspects of pediatric QOL instruments: developed according to established procedures including child participation in item generation, validity and reliability tested in children with the disease in question using established techniques, and able to be completed by child (or parent/caregiver when the child is unable to complete because of age or a learning disability).
A total of 2379 publications were identified from the initial search, from which 2194 were excluded from the title because they were not related to paediatrics or gastrointestinal or hepatic disease. The abstracts of the 185 remaining articles were reviewed and a further 73 were excluded for the same reasons. From the remaining 112 articles, 42 were excluded after review of the full article by the present authors (see Appendix I), leaving 70 included studies (see Appendix II). These were assigned to the following categories: inflammatory bowel disease (IBD), n = 17; cystic fibrosis (CF), n = 20; liver disease, n = 11; surgery, n = 15; and miscellaneous, n = 7. Meta-analysis of results was not possible because data were collected in different ways and with the use of different instruments.
Inflammatory Bowel Disease
In a meta-analytic review of chronic diseases, IBD had the most profound effect on mental health (4). In one study, 56% of children with IBD compared to 18% of control subjects had a psychiatric disorder, and these were almost exclusively emotional disorders (5). A further comparison of children with chronic illnesses reported psychiatric disorders in 60% of children with IBD, 30% with tension headaches, 20% with diabetes, and 15% of healthy controls (6). The first published study on QOL in children with IBD by Rabbett et al (7) reported that many of the affected children had problems with school attendance, sports, holidays, and staying at friends' houses. Children who were receiving steroids had more depressive symptoms. A recent study in the Netherlands (8) showed that younger children (ages 8–12 years) with IBD had comparable QOL and better cognitive function than a reference population, whereas adolescents had a significantly impaired QOL in 4 domains (body complaints, motor functioning, autonomy, and negative emotions).
An instrument to measure QOL in children with IBD (IMPACT) was developed in Canada (9). Interviews were held with 82 children with IBD and these generated a list of ways in which their lives were affected by IBD. These issues were incorporated into an item reduction questionnaire, which was administered to a further 117 children with IBD. Patients indicated how important each item was to them and how often it bothered them. The top-ranking items were then used to develop the IMPACT questionnaire. A subsequent validation study in 147 Canadian children (ages 9.2–18.0 years) confirmed that IMPACT is a valid and reliable measure of QOL in older children and adolescents with ulcerative colitis and Crohn disease (10). Mean total IMPACT score was significantly higher among patients with quiescent IBD versus those with active disease. The original instrument has been simplified and validated in the Netherlands (11) and has also been translated into French. A cross-cultural comparison has shown that the concerns of children with IBD in the United Kingdom are broadly similar to those of the Canadian children (12). Further validation is under way in the United Kingdom and a computerized touch-screen version has been developed (13).
In a study of children with active Crohn disease treated with exclusive enteral nutrition, 23 of 26 exhibited clinical remission at 8 weeks, with improvement in QOL scores measured via the IMPACT questionnaire (14). The change in QOL score was predictive of achieving a clinical remission but not of histological improvement. A prospective study of children with IBD attending a 1-week camp showed a significant improvement in total IMPACT score and several of the individual domain scores (15).
Adolescents with IBD have been shown to have higher QOL scores when they have a closer social support network. Those with recent-onset IBD relied more on family members than peers for emotional support and depended more on their parents' coping skills than their own. There was a significant agreement between adolescent and parental QOL score and stressful event ratings (16). Parents were also found to be adequate raters of objective components of the child's QOL but not of more subjective components (17). Paediatricians were found to overestimate physical symptoms (1). Adolescents with IBD used more avoidant coping styles than did healthy peers (18). Use of a predictive coping style and less use of a depressive reaction pattern was associated with better QOL in 3 of 6 domains.
A cross-sectional study of Dutch children showed that adolescents with IBD (especially boys) have worse QOL and show more internalizing problem behavior compared with healthy peers (19). An important predictor of QOL was self-esteem. A cross-sectional study of long-term psychosocial outcomes in children with IBD in the United States showed that 20% had behavioral/emotional symptoms, which was similar to healthy control subjects (20). An Italian study of the long-term effects 10 years after surgery in 21 children with ulcerative colitis showed that 15 had normal emotional status and 14 had a normal social life (21).
In a study of parents and siblings of children with IBD, parents were concerned about the effect of IBD on their child's future (eg, job prospects, marriage, independence) and were worried about problems the ill child encountered at school (22). Other concerns included side effects of medication and feelings of guilt. Siblings were concerned about their brother's or sister's illness and its treatment. They were also concerned about bullying and that their parents were withholding information about their brother's or sister's illness. A cross-sectional survey of 46 parents of children with IBD in Australia showed that the most common concerns were related to the side effects of medications and future prospects for the child. Parents were satisfied with aspects of care within the IBD clinic, but many requested additional personnel such as counselors and educators, continuing education, and easy access to up-to-date information (23).
Two studies reported that parents of children and adolescents with CF rated their child's QOL worse than their children did (24,25). The parents also believed their child's QOL was worse in comparison with healthy children or those with other chronic illness (24). Quality of life in children with asthma, diabetes, and CF was worse than that in a community sample. In children with CF, physical health declined during the 2-year study period, but there was no change in scores for physical and family activities (26). A study of family assessment of QOL showed no relation to adolescent assessment of QOL (27). A cross-cultural comparison showed poorer QOL in English versus German adolescents with CF and healthy controls compared in several domains (28). A cross-sectional study in Australia showed lower levels of psychopathology than population norms (29).
Early studies of QOL in children with CF used generic instruments such as Quality of Well Being (QWB) (30,31) and the Child Health Questionnaire (CHQ) (32). A survey in 2001 showed that 92% of CF centers in the United States did not measure QOL for clinical or research use (33). Since then, several disease-specific instruments have been developed. The CFQ is a disease-specific instrument developed and validated in France. The CFQ 14+ is for teenagers and adults, the CFQ Child P is a parent/proxy evaluation for children ages 8 to 13 years (34). They include 3 modules for assessing QOL, symptoms, and health perception. Nine QOL dimensions were identified: physical functioning, energy/well-being, emotions, social limitations, role, embarrassment, body image, eating disturbances, and treatment burden. Items in the instrument were derived from 33 interviews with patients and parents. Item reduction and assessment of internal consistency and convergent and discriminant validity were based on a cross-sectional survey of 393 patients and parents. A second study with 124 patients and 85 parents confirmed reproducibility and responsiveness. All psychometric properties were successfully demonstrated. This instrument has been translated into American English (35,36), German (37), and Dutch (38).
The CFQOL is a disease-specific instrument developed in the United Kingdom to measure QOL in adolescents and adults with CF (39). Areas of concern to adults and adolescents with CF were identified by unstructured interviews, self-administered questionnaires, consultation with multidisciplinary staff, literature review, and examination of other QOL instruments. Subsequent validation, test-retest reliability, and sensitivity testing were undertaken. Nine domains were identified: physical functioning, social functioning, treatment issues, chest symptoms, body image, interpersonal relationships, career concerns, relationships at work, and concerns for the future. Discrimination of disease severity and sensitivity to changes in health were robust.
The European DISABKIDS project developed and validated a chronic generic module and condition-specific modules for children and adolescents with 7 different chronic illnesses, including CF (40,41). Development of the condition-specific modules included focus groups to construct the pilot version and factor analysis to determine domain structures and for item reduction. The final version was tested in 360 respondents in a pilot study and subsequently in a field test of 1152 respondents in 7 European countries. Internal consistency was confirmed by good to excellent Cronbach α scores.
A number of studies have examined correlation between QOL and respiratory function. Two studies in the United States used generic instruments to assess QOL. The first study showed that QOL decreased as respiratory symptoms increased, the number of concurrent medical conditions increased and the number of medications increased (33). The second study in the United States showed that QWB was responsive to the treatment of an acute exacerbation and validity was shown by correlation between QWB score and respiratory function (42). In validation studies the CFQ and the CFQOL have shown correlation between disease severity and QOL (35,39).
Quality of life is not just related to disease severity; QOL in children and adults was low in those with poor coping skills even when the Schwachman score (CF disease severity score) was good (43). This study also showed that subjective health perceptions of patients explained their variance of QOL. Ways of coping were most significantly correlated with QOL.
A study evaluating a CF neonatal screening project in the United States did not show any difference in QOL scores between the screened and control groups. Thirty-six patients ages 10 to 15.5 years who were enrolled in the screened or control groups of the Wisconsin CF neonatal screening project completed the CHQ (44). This was a part of a larger randomised prospective study on the benefits and risks of the CF neonatal screening test.
The Caregiver QOL CF (CQOLCF) scale (45) was developed in the United States and has been used to show that the QOL of the caregiver decreased as the child's disease severity increased (46).
A study into the effect of α-interferon therapy in 94 children ages 3 to 14 years with chronic viral hepatitis showed that QOL (measured with the Sickness Impact Profile) was good before treatment, deteriorated during treatment, and returned to baseline within 3 months of stopping treatment (47). A pilot study of metformin in 10 children ages 10–17 years with biopsy-proven nonalcoholic steatohepatitis showed impaired baseline QOL (using Pediatric QOL Inventory) and significant improvement following treatment (48).
The first attempt to study QOL in 25 Australian children ages 0.58 to 14.2 years after liver transplantation using the Vineland Adaptive Behaviour Scales showed adequate mean composite and domain scores (49). An instrument to measure QOL in children after liver transplantation was developed by Zamberlan based on a review of the literature, clinical experience, and consultation with experts (50). The instrument has not been formally validated. Zamberlan interviewed a cohort of 20 children (ages 5–12 years) 3 to 6 years after liver transplantation. There were delays in socialization, difficulty in establishing peer relationships, and feelings of loneliness and vulnerability. The child's family was the greatest source of social support and family functioning returned to normal. Children were distressed by their physical appearance as a result of illness and medication. Five were worried about needing another transplant. Children were satisfied with their life and positive about the future but were more anxious and forgetful, slower in school work, and had difficulty with peer relationships. Six had behavior difficulties at home. Kita et al (51) asked 72 Japanese children how their QOL had been since liver transplantation. Most thought their QOL had greatly improved and there was no difference between brain-death and living related–donor groups. Several studies have shown that QOL in children after liver transplantation is worse than that of a reference population (52–55). Buchuvulas et al (56) reported QOL similar to children with other chronic illness. A study by Cole et al in 2004 (57) showed significant increases in all subscale scores except for global mental health 1 year after liver transplantation. The assessment of QOL was completed by parents in most of the studies; in the study by Schultz et al (53), children and their parents completed the assessment and their assessment of the child's QOL diverged strongly.
The first study assessing QOL in children undergoing gastrointestinal surgery used an unvalidated questionnaire in families of children after repair of high imperforate anus (58). In the majority of young children QOL was not related to achieving faecal continence, but in older children, faecal continence became a determinant of QOL. In a subsequent study a proxy version (parent-completed) of the EuroQOL in children treated for imperforate anus was found to be feasible and valid (59).
A Hirschsprung disease/anorectal malformation QOL instrument has also been developed (60). The hypothesised domain structure was confirmed with good internal reliability, there was good discriminant ability in adults but not in children, and there were problems with the diet domains in children. A study of children after surgery for anorectal malformation in China reported problems with peer relationships, school absence, restricted diets, and behavioral problems (61). The QOL was lower in patients than controls and lower in children with poor faecal continence. Similar results were found in a Chinese study of QOL after the Swenson procedure for Hirschsprung disease (62). A Dutch study showed that QOL was severely affected in young children with anorectal malformation, QOL was less severely affected in older children, and in adults there was little difference compared with the general population (63). There were similar findings for patients with congenital diaphragmatic hernia (63).
Quality of life after ileoanal pull-through for ulcerative colitis and familial adenomatous polyposis showed no difference from population norms (64). A US study in children undergoing ileal pouch/anal anastomosis reported lower general health scores than US norms, but scores for most psychosocial factors were normal (65).
A retrospective survey (using an unvalidated questionnaire) of caregiver satisfaction after surgery for gastrooesophageal reflux in neurologically impaired children suggested that the QOL of the child and the caregiver were improved after surgery (66). A subsequent study of QOL in the caregivers' of neurologically impaired children showed significant improvement in those undergoing oesophagogastric disassociation compared with fundoplication (67).
Another retrospective survey (using an unvalidated questionnaire) in children with severe disabilities with feeding problems suggested that the QOL of the child and the caregiver improved after the child had a gastrostomy (with fundoplication if there was severe reflux) (68). A recent study of maternal caregivers of gastrostomy-dependent children showed they required twice as much care time and that their caregivers were no more depressed or less satisfied with their lives than caregivers of children without a gastrostomy (69). Sullivan et al (70) measured the QOL of caregivers of children with cerebral palsy before gastrostomy tube insertion and 6 and 12 months later. Caregivers reported significant reduction in feeding times, improved ease of drug administration, and reduced concern about their child's nutritional status. Mean scores in several domains of the Short Form-36 questionnaire were improved at 6 months, and by 12 months, they were comparable to normal reference standards.
In an uncontrolled study of children with oesophageal atresia, parental reports showed higher QOL in those who had gastrostomy, cervical oesophagostomy, and no attempt at anastomosis compared with those who had previous attempts at reconstruction (71). There was no difference in the children's assessment of QOL in the 2 groups.
A disease-specific instrument to measure QOL in children with constipation or functional nonretentive faecal soiling has been developed in the Netherlands using accepted guidelines (72). It consists of 37 items in 4 domains. Reliability was acceptable to good, but validity compared with the Dutch Tacqol generic instrument (TNO-AZL) was only moderate. In a study in the United States, children with chronic constipation had a lower QOL than population norms and those with IBD and gastrooesophageal reflux disease (GERD) (73). Duration of symptoms was correlated with reduced QOL. An Australian study of children with constipation treated with an antegrade continence enema showed improved QOL (74). A Dutch study of children with celiac disease showed that their QOL was similar to that of the reference population (75).
Two studies have looked at QOL in children with GERD. Thomson et al (76) showed that children with severe GERD receiving an endoluminal gastroplication had improved QOL scores (using an adult measure) 6 weeks after surgery, which was sustained for 12 months. Another study using a disease-specific paediatric asthma instrument demonstrated that acid suppression did not improve QOL in children with concomitant asthma and GERD (77).
Children with chronic intestinal pseudoobstruction have been shown to have greater difficulty in attending school and participating in social activities than healthy children (78). They have more pain, depression, and anxiety than healthy children or children with juvenile rheumatoid arthritis. Their parents also required more time to care for their children and had poorer emotional status. A study of QOL in parents of children receiving home parenteral nutrition showed evidence of psychiatric morbidity with deterioration in social life, family life, sex life, and work (79).
Chronic diseases can have a major impact on the QOL of affected children and their families. In their review of QOL measures in chronic diseases of childhood, Eiser and Morse emphasised the need for the implementation of QOL measures in paediatric research, the adoption of child-centered approaches to measurement, and the clarification of the relationship between child and proxy ratings (2). In this systematic review we have attempted to identify all disease-specific instruments (Table 1) and published studies on QOL in children with gastrointestinal and liver disease.
Early attempts to describe QOL focused on functional problems (58). Subsequent studies used unvalidated questionnaires or generic instruments. Many of the studies identified have used parents as proxies to assess their child's QOL. Although this may be the only practical way to assess QOL in young children or those with learning difficulties, there are inherent difficulties with the use of proxy assessments because they do not always correlate closely with patients' own assessments of their QOL (2). Disease-specific instruments (Table 1) have now been developed for Hirschsprung disease/anorectal malformation (60), liver transplantation (50), CF (34,39), IBD (10), constipation, and functional nonretentive faecal soiling (72). Some of these were developed in the early days of paediatric QOL research, have not been developed from the child's perspective, and have not been formally validated. Using the 3 criteria listed in the methods (item generation from children, validity and reliability formally tested in children, questionnaire completed by child), only the IMPACT questionnaire for IBD, the CFQ, the CFQOL, the DISABKIDS, and the Dutch Defecation Disorder List fulfill all of these criteria and can be recommended for use in the relevant patient groups. It is important to remember that if these instruments are used outside their country of origin, they may need to be translated according to the internationally agreed-upon criteria set by Guillemin et al (80) and may require further validation in the country in which they are to be used.
A number of studies were identified that addressed the QOL of the parents or caregivers of children with chronic illnesses. Quality of life was particularly affected in the caregivers of children receiving home parenteral nutrition (79) and severely disabled children with feeding problems (68,70). Although gastrostomy feeding may be associated with improved QOL in the child and caregiver, the decision to subject their child to a gastrostomy may be difficult for caregivers. In addition, oral feeding may be enjoyable and important for communication and rapport.
Although much remains to be done in terms of assessing QOL in children with chronic gastrointestinal and hepatological disorders, some progress has been made. A number of disease-specific instruments have been developed or are in the process of being developed. Many of the studies identified have used generic instruments and/or proxy assessments, but more emphasis is now being given to the child's and family's view. Quality of life measurement may help to make health professionals more aware of issues that are important to the patient, including the emotional and psychological impact of an illness and its treatment. Patients may feel their opinion is being valued and may be more likely to adhere to the advice of health professionals.
Although it is important to give emphasis to the development of robust disease-specific instruments to measure QOL, it is also important to look at ways to improve QOL. When assessing new treatments, QOL should be considered as a primary outcome. Recent studies have started to look at the relationship between coping strategies and QOL. Perhaps teaching children better coping skills will lead to an improvement in their QOL. In addition, we need to consider interventions specifically designed to improve the QOL in children in whom it is severely affected. This may take the form of counselling, psychological therapy, support groups, play therapy, education, or social events including adventure activities and camps.
The authors thank the Evidence-Based Child Health Unit, University of Liverpool, for their help in developing the search strategy. We also thank the MAPI Research Institute for searching the Patient-Reported Outcome and Quality of Life Instruments Database for relevant QOL instruments.
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APPENDIX I: EXCLUDED STUDIES/PUBLICATIONS
APPENDIX II: INCLUDED STUDIES
Inflammatory Bowel Disease
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