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The aim of this study was to assess internal consistency and construct validity (known-groups validity) and to provide Dutch norm data for the Dutch Pediatric Quality of Life Inventory Multidimensional Fatigue Scale for Young Adults ages 18–30 years (PedsQL fatigue_YA). A Dutch sample of 649 young adults completed online a sociodemographic questionnaire and the PedsQL fatigue_YA including three subscales: general fatigue, sleep/rest fatigue and cognitive fatigue (0–100: Higher scores indicate less fatigue symptoms). The PedsQL fatigue_YA showed satisfactory to good internal consistency (Cronbach’s alpha = .70–.94), except for one scale (.68). The mean scale scores were 68.23 (SD 19.15) for ‘general fatigue,’ 67.04 (SD 15.54) for ‘sleep/rest fatigue’ and 74.62 (SD 19.02) for ‘cognitive fatigue.’ Men reported significantly higher scores on ‘general fatigue’ and ‘sleep/rest fatigue’ than women. The PedsQL fatigue_YA distinguished between healthy young adults and young adults with chronic health conditions, with higher scores on all scales in healthy young adults than in those with a chronic health condition. The results demonstrate good psychometric properties of the PedsQL fatigue_YA in a sample of Dutch young adults. With the current norms available, it is possible to evaluate fatigue in the Netherlands from childhood to adulthood with the PedsQL Multidimensional Fatigue Scale.

Chronically ill children require several hours of additional care per day compared to healthy children. As parents provide most of this care, they have to incorporate it into their daily schedule, which implies a reduction in time for other activities. The study aimed to assess the effect of having a chronically ill child on parental employment and parental leisure activity time, and to explore the role of demographic, social, and disease-related variables in relation to employment and leisure activities. Outcomes of 576 parents of chronically ill children and 441 parents of healthy school children were analyzed with multivariate regression. Having a chronically ill child was negatively related with family employment, maternal labor force participation, and leisure activity time. Use of child care was positively related to family and maternal employment of the total group of parents. Within parents of chronically ill children, most important finding was the negative relation of dependency of the child on daily care and low parental educational level with family and maternal employment. In conclusion, parents of chronically ill children, mothers in particular, are disadvantaged in society probably due to the challenge of combining child care with work and leisure time.

The objective of this study was to investigate satisfaction with and feasibility of an online cognitive behavioral-based group intervention (OK Onco Online) for adolescent childhood cancer survivors (CCS). The intervention, carried out by pediatric psychologists, aimed to prevent psychosocial problems. High levels of satisfaction were reported by the psychologists (n = 6) and the CCS (n = 11, age 11-17). The dropout rate was very low. Some recommendations for optimizing the intervention were mentioned. In conclusion, the positive evaluations by the CCS and the psychologists indicate that the online chat intervention OK Onco Online is a promising innovative group intervention for adolescent CCS. Further research is needed to confirm feasibility and to establish efficacy

SUMMARY The main objective of the present study was to determine which variables predict the emotional adjustment of parents of children with cancer. Therefore, parents’ emotional adjustment, in terms of depression, anxiety, feelings of loneliness, helplessness, uncertainty and positive feelings, were predicted with three models. (1) With a child model (including age of the child, time since diagnosis, being in remission or having a relapse, and depression of the child); (2) with a control strategies model (including four distinguished control strategies of parents); and (3) with a child and control strategies model (including a combination of the aforementioned variables). The four control strategies of parents of children with cancer included: the reliance on predictive control (having positive expectations); vicarious control (attributing power to the medical setting); illusory control (relying on luck and wishful thinking); and interpretative control (gaining knowledge). A total of 84 mothers and 79 fathers, of 84 children with cancer with different survival perspectives (in remission or with a relapse) participated in the study, and were assessed about the use of control strategies and adjustment. Lack of positive expectations about the course of the illness was most strongly related to negative emotions for mothers and for fathers. For mothers having a child with a relapse, predicted feelings of helplessness and uncertainty, and reported feelings of depression of the child, proved to be related to the feelings of uncertainty of the fathers. The findings demonstrate that the use of secondary control strategies contribute significantly to the emotional adjustment of parents of children with cancer. © 1997 by John Wiley & Sons, Ltd. Psycho-Oncology 6: 115‐128, 1997. No. of Figures: 0. No. of Tables: 6. No. of Refs: 36.

Aim: Kawasaki disease (KD) is an acute paediatric vasculitis. The psychosocial consequences of this sudden illness for parents are unknown. This study aimed to evaluate health related quality of life (HRQOL) and parental perceptions of child vulnerability (PPCV) in parents of children with KD, and to identify variables associated with PPCV. Methods: This cross-sectional study included 288 parents (83% mothers) of KD patients (mean age 8.7 years). HRQOL was assessed using the TNO-AZL Questionnaire for Adult’s HRQOL (TAAQOL) and PPCV using the Child Vulnerability Scale (CVS). Scores of KD parents were compared with reference groups of Dutch parents. Logistic regression analyses were performed to examine associated variables. Results: The HRQOL of KD parents was comparable to the HRQOL of parents of healthy children. However, KD parents showed significantly higher PPCV, regarding both the median CVS total score and the percentage in the clinical range. No differences were found in CVS outcomes between KD parents and parents of a chronically ill child. None of the studied parental, child and disease characteristics were significantly associated with PPCV. Conclusion: Parents perceived their KD child more vulnerable to illness than healthy children, while in reality the majority had fully recovered from KD.

Background: More and more pediatric patients reach adulthood. Some of them are successfully integrating in adult life, but many others are not. Possibly Illness cognitions (IC) - the way people give meaning to their illness/disability - may play a role in individual differences on long-term adjustment. This study explored the association of IC with disease-characteristics and Health Related Quality of Life (HRQoL), anxiety and depression in young adults with a disability benefit due to childhood-onset chronic condition. Methods: In a cross-sectional study, young adults (22-31 years, N = 377) who claimed a disability benefit because of a somatic condition since childhood, completed the Illness Cognition Questionnaire (acceptance-helplessness-benefits), RAND-36 (HRQoL) and HADS (anxiety and depression) online. Besides descriptive statistics, linear regression analyses were conducted to predict (1) illness cognitions by age, gender and disease-characteristics, and (2) HRQoL (Mental and Physical Component Scale), Anxiety and Depression by illness cognitions, controlling for disease-characteristics, age and gender. Results: Respectively 90.2%, 83.8% and 53.3% of the young adults with a disability benefit experienced feelings of acceptance, benefits and helplessness. Several disease-characteristics were associated with IC. More acceptance and less helplessness were associated with better mental (beta = 0.31; beta = -0.32) and physical (beta = 0.16; beta = -0.15) HRQoL and with less anxiety (beta = -0.27; beta = 0.28) and depression (beta = -0.29; beta = 0.31). Conclusions: IC of young adult beneficiaries were associated with their HRQoL and feelings of anxiety and depression. Early recognition of psychological distress and negative IC might be a key to the identification of pediatric patients at risk for long-term dysfunction. Identification of maladaptive illness cognitions enables the development of psychosocial interventions to optimise their well-being and adaptation to society

The objective of this study was to evaluate health-related quality of life (HRQoL) and behavioral functioning in pediatric patients with hereditary spherocytosis (HS). A cross-sectional study was conducted in 132 Dutch children and adolescents with HS and aged 8–18 years of whom 48 underwent splenectomy prior to the study. HRQoL was assessed using the KIDSCREEN-27, and behavioral functioning was evaluated using the strength and difficulties questionnaire (SDQ). Scores of pediatric patients with HS were compared to a Dutch norm population. Additionally, the effects of three factors were assessed: fatigue, self-image, and parents’ perceived vulnerability (measured with the checklist individual strength, the self-perception profile for children and adolescents, and the child vulnerability scale). Both unsplenectomised and splenectomised pediatric patients reported lower HRQoL on the domain physical well-being (KIDSCREEN-27) compared to Dutch peers. For behavioral functioning, parents of both groups reported more emotional problems (SDQ) compared to the norm population. Pediatric patients with lower scores on physical well-being experienced more fatigue. The patients’ perceived social acceptance and parents’ perceived vulnerability appeared as determinants of emotional problems. Conclusion: Pediatric patients in the current study generally report few complaints, and the results suggest that these patients overall have a strong ability to cope with HS. Despite these few complaints, fatigue and parents’ perceived vulnerability seem to be important determinants for lower HRQoL and more emotional problems. Therefore, screening on these factors could serve as an addition to the treatment of HS, to help pediatric patients who are at risk for lower HRQoL or more emotional problems.

Raising a child with Down syndrome (DS) has been found to be associated with lowered health related quality of life (HRQoL) in the domains cognitive functioning, social functioning, daily activities and vitality. We aimed to explore which socio-demographics, child functioning and psychosocial variables were related to these HRQoL domains in parents of children with DS. Parents of 98 children with DS completed the TNO-AZL adult quality of life questionnaire (TAAQOL) and a questionnaire assessing socio-demographic, child functioning and psychosocial predictors. Using multiple linear regression analyses for each category of predictors, we selected relevant predictors for the final models. The final multiple linear regression models revealed that cognitive functioning was best predicted by the sleep of the child (β = .29, p < .01) and by the parent having given up a hobby (β = −.29, p < .01), social functioning by the quality of the partner relation (β = .34, p < .001), daily activities by the parent having to care for an ill friend or family member (β = −.31, p < .01), and vitality by the parent having enough personal time (β = .32, p < .01). Overall, psychosocial variables rather than socio-demographics or child functioning showed most consistent and powerful relations to the HRQoL domains of cognitive functioning, social functioning, daily activities and vitality. These psychosocial variables mainly related to social support and time pressure. Systematic screening of parents to detect problems timely, and interventions targeting the supportive network and the demands in time are recommended.

The model of Rothbaum et al. ( 1982), in which primary and secondary control are outlined in strategies of predictive vicarious, illusory and interpretative control, was used to describe parental efforts to cope with childhood cancer. Primary control strategies are efforts directed at modifying objective conditions (in the case of a life-threatening disease e.g. changing treatment schedules, changing other people's behavior or changes in life-style), whereas secondary control strategies refer to attempts directed at modifying oneself (e.g. changing one's hopes, expectations or interpretations). Manifestations of secondary interpretative, illusory, predictive and vicarious control strategies were operationalized and assessed. Therefore, an inventory with 38 items was administered to 163 parents (n = 84 mothers and n = 79 fathers) of 84 children with cancer with different survival perspectives, that is in remission or with a relapse. Differences according to sex, survival perspective, and educational level were investigated in the use of the four control strategies. Mothers appeared to rely more on interpretative and illusory control than fathers do, while parents of children in remission relied more on predictive and vicarious control. Parents with a lower educational level tended to rely more on all control strategies with the exception of interpretative control

BACKGROUND AND OBJECTIVE: Monitoring health-related quality of life (HRQoL) by using electronic patient-reported outcomes (ePROs) has been only minimally evaluated in pediatrics. Children with juvenile idiopathic arthritis (JIA) are at risk for HRQoL problems. The aim of this study was to investigate the effectiveness of ePROs in clinical pediatric rheumatology care. METHODS: All children (aged 0–18 years) with JIA visiting any of the 4 pediatric rheumatology clinics in Amsterdam between February 2009 and February 2010 were eligible for this sequential cohort intervention study. Before an outpatient consultation, children (aged 8–18 years) or parents (of children aged 0–7 years) completed web-based questionnaires. The resulting ePROfile was provided to the pediatric rheumatologist (PR). The study was divided into a control period in which the ePROfile was not discussed during consultation, and an intervention period in which the ePROfile was provided and discussed during consultation. Effectiveness was evaluated in terms of communication about different HRQoL topics, referral to a psychologist, and satisfaction with the consultations. RESULTS: Out of the eligible JIA patients, 176 (65%) participated in the study. Use of the ePROfile increased discussion of psychosocial topics (P < .01), as well as the PR’s satisfaction with provided care during consultation (P < .01). The use of ePROfiles did not affect referrals to a psychologist or parental satisfaction. Parents and PRs evaluated the use of the ePROfile as positive in 80% to 100% of the consultations. CONCLUSIONS: Our web-based application to systemically monitor HRQoL problems in pediatric rheumatology contributed significantly to communication about psychosocial issues in a positive way. We recommend implementation of ePROs in pediatric clinical practice.

This study aimed to assess health-related quality of life (HRQOL)-mean scores and percentages at risk for impaired HRQOL in childhood brain tumor survivors (CBTS) and to explore differences between CBTS treated with surgery only (SO) versus CBTS treated with surgery and adjuvant therapy (SA). HRQOL was evaluated in 34 CBTS (mean age = 14.7 y; mean time since the end of treatment = 6.4 y) with the KIDSCREEN. Being at risk for impaired HRQOL was defined as a T-score ≥ 1 SD below the norm population mean. The total and the SA group, but not the SO group, had significantly lower mean scores than the Dutch norm population in the domains of "physical well-being," "psychological well-being," and "peers and social support." High percentages (35% to 53%) of both the SO and the SA groups appeared to be at risk for impaired HRQOL in the domains of "physical well-being," "moods and emotions," "peers and social support," and "bullying," compared to 16% in the norm population. In conclusion, although HRQOL in some domains appeared similar to the norm population, a considerable number of CBTS-reported impaired HRQOL in several other domains. It is recommended to systematically monitor HRQOL in CBTS regardless of the therapy applied. Copyright © 2013 by Lippincott Williams & Wilkins.

Background Neurotoxicity caused by treatment for a brain tumor is a major cause of neurocognitive decline in survivors. Studies have shown that neurofeedback may enhance neurocognitive functioning. This paper describes the protocol of the PRISMA study, a randomized controlled trial to investigate the efficacy of neurofeedback to improve neurocognitive functioning in children treated for a brain tumor. Methods/Design Efficacy of neurofeedback will be compared to placebo training in a randomized controlled double-blind trial. A total of 70 brain tumor survivors in the age range of 8 to 18 years will be recruited. Inclusion also requires caregiver-reported neurocognitive problems and being off treatment for more than two years. A group of 35 healthy siblings will be included as the control group. On the basis of a qEEG patients will be assigned to one of three treatment protocols. Thereafter patients will be randomized to receive either neurofeedback training (n=35) or placebo training (n=35). Neurocognitive tests, and questionnaires administered to the patient, caregivers, and teacher, will be used to evaluate pre- and post-intervention functioning, as well as at 6-month follow-up. Siblings will be administered the same tests and questionnaires once. Discussion If neurofeedback proves to be effective for pediatric brain tumor survivors, this can be a valuable addition to the scarce interventions available to improve neurocognitive and psychosocial functioning. Trial registration ClinicalTrials.gov NCT00961922.

Aim This meta-analysis provides a systematic review of studies into intellectual and attentional functioning of paediatric brain tumour survivors (PBTS) as assessed by two widely used measures: the Wechsler Intelligence Scale for Children (3rd edition; WISC-III) and the Conners’ Continuous Performance Test (CPT). Method Studies were located that reported on performance of PBTS (age range 6–16y). Meta-analytic effect sizes were calculated for Full-scale IQ, Performance IQ, and Verbal IQ as measured by the WISC-III, and mean hit reaction time, errors of omission, and errors of commission as measured by the CPT. Exploratory analyses investigated the possible impacts of treatment mode, tumour location, age at diagnosis, and time since diagnosis on intelligence. Results Twenty-nine studies were included: 22 reported on the WISC-III in 710 PBTS and seven on CPT results in 372 PBTS. PBTS performed below average (ps

The long-term survival of children with a central nervous system (CNS) tumour is improving. However, they experience late effects, including altered habits and patterns of sleep. We evaluated the presence and type of sleep disorders and daytime sleepiness in these children, and its associations with clinical characteristics and daily performance (fatigue and psychosocial functioning). In a cross-sectional study at the outpatient clinic of the Emma Children's Hospital AMC (February-June 2010), sleep, fatigue and psychosocial functioning were analysed in 31 CNS tumour patients (mean age: 11.8years; 20 boys) and compared with 78 patients treated for a non-CNS malignancy (mean age: 9.7years; 41 boys) and norm data. Questionnaires applied were the Sleep Disorder Scale for Children, the Epworth Sleepiness Scale, the Pediatric Quality of Life Inventory, and the Strengths and Difficulties Questionnaire. Sleeping habits and endocrine deficiencies were assessed with a self-developed questionnaire. Increased somnolence was found in CNS tumour patients compared with those with a non-CNS malignancy (8.8±2.8 versus 7.5±2.7; P0.05). Both patient groups reported more problems (P0.01) than the norm with initiating and maintaining sleep. No specific risk factors were identified for a sleep disorder in CNS tumour patients, but their excessive somnolence was correlated with lower fatigue related quality of life (QoL) (r=-0.78, P0.001) and worse psychosocial functioning (r=0.63, P0.001). In conclusion, children treated for a CNS tumour have increased somnolence, significantly increasing fatigue and worsening daily functioning. Further investigation should focus on possibilities to improve sleep quality and diminish fatigue.

Objectives. It is generally recognized that for all children the fulfilling of age-specific psychosocial developmental tasks in childhood is of great importance to adjustment in adult life, including participation in society. For young adults with JIA this is more difficult. We assume that the achievement of psychosocial milestones while growing up (psychosocial developmental trajectory) is also related to labour participation. A proportion of all young adults with JIA have to apply for disability benefits. This study assessed the health-related quality of life (HRQOL) and the psychosocial developmental trajectory of young female beneficiaries with JIA compared with peers from the Dutch general population. Methods. Young females with disability benefits because of JIA completed the RAND-36 (HRQOL) and the Course of Life Questionnaire (psychosocial developmental trajectory). Differences between respondents and the peer group were tested using analysis of variance and logistic regression analysis by group and age. Results. The beneficiaries reported worse HRQOL than the peer group and achieved fewer milestones, or achieved the milestones at a later age than the peer group in the autonomy, social and psychosexual domain. Conclusions. Young females with JIA who have to apply for disability benefits are at risk for impaired HRQOL and a delay in their psychosocial developmental trajectory. Parents, physicians and other health-care providers should pay systematic attention to the development of social and independent functioning of children with JIA in order to optimize their adaptation to society at the time of transition to adulthood.

Objective The authors evaluated health-related quality of life (HRQOL) and behavioral functioning in patients with a history of Kawasaki disease (KD). Study design A cross-sectional study was conducted at a tertiary referral center for KD follow-up in 280 patients (mean age 8.6 years, 60.0% male). Patients were eligible when they were aged 0-18 years and had a history of KD. HRQOL was assessed using the TNO-AZL Preschool Children Quality of Life questionnaire for children 0-5 years old and the Pediatric Inventory of Quality of Life Core Scales 4.0 for those 6-18 years old. Behavioral functioning was evaluated using the Strength and Difficulties Questionnaire (8-16 years proxy report and 11-16 years self-report). KD results were compared with Dutch norm data, and patients with and without coronary artery aneurysms were compared. Results HRQOL was significantly worse for male patients aged 0-5 years on 4 of the 12 TNO-AZL Preschool Children Quality of Life questionnaire scales and for female patients on the motor functioning scale. At an older age, the HRQOL of patients was comparable with the norm population. Coronary artery status did not influence HRQOL. Parents reported more behavioral problems on the hyperactivity and emotional subscale in patients compared with the norm population. Conclusions Although at an older age the HRQOL of patients with KD is comparable with the Dutch norm, HRQOL seems to be particularly impaired at younger age. Parents reported more hyperactivity and emotional problems in patients with KD. (J Pediatr 2012;161:314-9)

The use of Patient Reported Outcomes (PROs) in daily clinical practice receives increasing attention. Former studies suggest that discussing PROs improves communication between physicians and patients and facilitates early recognition of HRQOL problems. Especially in paediatrics, there is a need to address HRQOL in daily clinical practice in the context of a child’s development; repeated measurement of HRQOL in different developmental stages can be valuable. With the use of PROs, HRQOL problems can be detected early and tailored intervention can be provided to the child, before the HRQOL problems increase. However research of PROs in paediatrics is still scarce. Children with Juvenile Idiopathic Arthritis (JIA) are at risk for HRQOL problems. Therefore, it is important to monitor their HRQOL during doctor’s visits. The aim of this study is to investigate the effectiveness of a web-based PRO about HRQOL (KLIK PROfile) in paediatric rheumatology practice.

Background Mucopolysaccharidosis type III (MPS III, Sanfilippo syndrome) is a lysosomal storage disorder caused by a deficiency of one of the enzymes involved in the degradation of heparan sulfate. MPS III is characterized by progressive mental deterioration resulting in severe dementia. A number of potentially disease-modifying therapies are studied. As preservation of cognitive function is the ultimate goal of treatment, assessment of cognitive development will be essential in order to evaluate treatment efficacy. However, no large scale studies on cognitive levels in MPS III patients, using formal psychometric tests, have been reported. Methods We aimed to assess cognitive development in all 73 living patients with MPS III in the Netherlands. Results Cognitive development could be assessed in 69 patients. In 39 of them developmental level was estimated > 3 months and formal psychometric testing was attempted. A remarkable variation in the intellectual disability was detected. Conclusions Despite special challenges encountered, testing failed in only three patients. The observed broad variation in intellectual disability, should be taken into account when designing therapeutic trials.

Patients with classic galactosemia suffer from several long term effects of their disease. Research in a group of mainly female patients has shown that these patients may also have a developmental delay with regard to their social aptitude. To study if male galactosemia patients achieve psychosocial developmental milestones more slowly than male peers from the general Dutch population, we assessed their development with the Course of Life Questionnaire (CoLQ). A total of 18 male galactosemia patients participated in this study (response rate 69%): 11 Dutch patients and seven American patients. We found severe delays in the social and psychosexual scales of this questionnaire, but not on the autonomy axis. These results are comparable to an earlier study with a limited number of male patients. The observed delays could be secondary to less developed social skills, cognitive dysfunction, or disrupted language development. We strongly recommend screening of galactosemia patients for developmental delays, to ensure early intervention through social skills training.

Background Health Related Quality of Life (HRQOL) questionnaires are increasingly used in clinical practice. These Patient Reported Outcomes (PROs) are provided to the paediatrician to facilitate communication with patients during a consultation. The aim of the current article is to describe the development and introduction of a new web-based application for the use of PROs in daily paediatric clinical practice. Methods Currently, the use of PROs in daily clinical practice is very time consuming and often has logistical problems. The use of a web-based programme can overcome these problems and contributes to an improved use of PROs in clinical practice. We therefore developed an easily accessible website (KLIK) for outpatient treatment and a training programme for paediatricians to maximize the effectiveness and the practical use of PROs (KLIK PROfile). Results The KLIK study was launched in August 2008 to evaluate the use of the KLIK PROfile in daily clinical practice. The KLIK study evaluates whether feedback from HRQOL data could influence patient satisfaction with the consultation, the advice given, the type of referrals and topics discussed. In this multicentre study, a control group (without the use of the KLIK PROfile) is compared to an intervention group (with the use of the KLIK PROfile). A sequential cohort design is chosen to avoid contamination between the study groups. Conclusions Based on the positive experiences with the use of the KLIK PROfile acquired during the study we conclude that the KLIK PROfile may contribute to systematically monitor and discuss HRQOL issues during consultations. The next steps will be a comprehensive evaluation of the KLIK study data and the implementation of the KLIK PROfile in daily clinical practice in different patient groups.

Item does not contain fulltext PURPOSE: Perceiving favourable changes from one's illness may go hand in hand with experiencing harmful psychosocial effects. Each of these constructs should be considered when examining children's levels of psychological adjustment following stressful life events. A paediatric instrument that accounts for both positive and negative impact of stressful events has not been investigated in The Netherlands before. The aim of the study was to investigate psychometric properties of the Dutch version of the Benefit and Burden Scale for Children (BBSC), a 20-item questionnaire that intends to measure potential benefit and burden of illness in children. METHODS: Dutch paediatric survivors of childhood cancer aged 8-18 (N = 77) completed the BBSC and other psychological questionnaires: Pediatric Quality of Life Inventory (health-related quality of life), State-Trait Anxiety Inventory for Children (anxiety), Children's Revised Impact of Event Scale (posttraumatic stress) and Strengths and Difficulties Questionnaire (behavioural functioning). Reliability and validity were evaluated. RESULTS: Internal consistency (Cronbach's alpha, benefit 0.84, burden 0.72), test-retest reliability (benefit r = 0.74, burden r = 0.78) and homogeneity (mean inter-item correlation, benefit r = 0.34, burden r = 0.22) were satisfactory. Burden was associated with HRQoL (-), anxiety (+), posttraumatic stress symptoms (+) and behavioural problems. Benefit did not correlate with the psychological outcomes. CONCLUSIONS: The Dutch version of the BBSC shows promising psychometric properties. Perceived benefit and disease-related burden are distinct constructs; both should be considered when examining children's psychological adjustment to potentially traumatic experiences. The BBSC may be useful as monitoring and screening instrument.

Although neurocognitive deficits in children with sickle cell disease (SCD) have been well documented, the etiology of these deficits has not been completely clarified. The aim of this study was to investigate the association of laboratory markers of disease severity and radiological parameters with neurocognitive functioning in children with SCD.Participants were 37 children with SCD ((HbSS or HbS-β(0)-thalassemia) aged 6-18 years. All participants underwent extensive neurocognitive assessment. Further data (TCD values, laboratory test results, and MRI data) were obtained from medical charts. Associations were analyzed by hierarchical regression analysis.Hemoglobin was associated with a decrease in verbal short-term memory. There was no association between TCD velocities and neurocognitive functioning, when controlled for age. Children with silent infarcts did not differ from children with normal MRI in neurocognitive functioning. Children with right-left asymmetries in cerebral blood flow as measured by continuous arterial spin labelling (CASL) MRI had better sustained attention than children without asymmetries.Neurocognitive deficits are associated with the severity of anemia, indicating reduced oxygen delivery to the brain as an etiological mechanism. This implies that children with SCD and normal MRIs may still suffer from neurocognitive impairments, possibly affecting their academic development and full participation in society.

With the aim of assessing parental stress after SCT, 73 parents of children and adolescents who underwent SCT 5 or 10 years ago responded to questionnaires on general distress (General Health Questionnaire (GHQ)), disease-related stress (Pediatric Inventory for Parents-short form (PIP-SF)) and perceptions of child vulnerability (Child Vulnerability Scale (CVS)). General distress scores were comparable with the reference groups, but 40% of the mothers at 5 years after SCT reported increased stress levels as compared with 26% in the community-based reference group. Disease-related stress was comparable with the reference group of parents of children who were just off cancer treatment, 5 years after SCT. At 10 years after SCT, scores were lower than the reference group. Perceived child vulnerability did diminish over time, but remained high in parents of SCT survivors, compared with parents of healthy children: 96% of the parents at 5 years after SCT and 76% of the parents at 10 years after SCT scored above the cutoff point. Perceived vulnerability was found to be a predictor for parental disease-related stress. To conclude, although most parents of SCT survivors are resilient, the majority of parents perceive their child to be much more vulnerable as compared with parents of healthy children. This perception is associated with disease-related stress and may induce overprotective parenting. Bone Marrow Transplantation (2010) 45, 1102-1108; doi:10.1038/bmt.2009.309; published online 2 November 2009

Sickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES-matched control group, in order to gain more insight into the specific deficits of these patients.Forty-one children with homozygous SCD (HbSS or HbS-β0-thalassemia) and 38 controls were assessed on a comprehensive set of well-defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo-spatial working memory, and verbal working memory) as well as visuo-motor functioning.SCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full-scale IQ below 75. Furthermore, children with SCD showed deficits in visuo-motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo-spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory.Children with SCD are at increased risk of lower intelligence, visuo-motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD.

OBJECTIVE: Behavior problems are common in children with functional constipation. This study assessed the prevalence of overall, internalizing, and externalizing behavior problems in children with functional constipation and explored which clinical characteristics of constipation are associated with these behavior problems. METHODS: Children who had functional constipation, were aged 4 to 18 years, and were referred to the gastrointestinal outpatient clinic at the Emma Children's Hospital were eligible for enrollment. This study made use of baseline data of 133 children who participated in a randomized, controlled trial that evaluated the clinical effectiveness of behavioral therapy compared with conventional treatment. Prevalence of behavior problems was assessed by the Child Behavior Checklist. Univariate and multivariate logistic regression models were used to test the association between clinical characteristics and behavior problems. RESULTS: The prevalence rate of overall, internalizing, and externalizing behavior problems was considerable: respectively 36.8%, 36.1%, and 27.1% compared with 9% in the Dutch norm population. A long duration of treatment was found to have the strongest association with overall and externalizing behavior problems in children with constipation. Children with constipation and nighttime urinary incontinence have an increased risk for having overall behavior problems. Fecal incontinence and the production of large stools seemed to be exclusively related to externalizing behavior problems. CONCLUSIONS: Behavior problems are common in children who have constipation and are referred to gastrointestinal outpatient clinics, suggesting that a behavioral screening should be incorporated into the diagnostic workup of children with constipation.

Background Childhood cancer and its treatment have considerable impact on a child's physical and mental wellbeing. Especially long-term administration of chemotherapy and/or radiotherapy impairs physical fitness both during and after therapy, when children often present with muscle weakness and/or low cardiorespiratory fitness. Physical exercise can improve these two elements of physical fitness, but the positive effects of physical exercise might be further increased when a child's wellbeing is simultaneously enhanced by psychosocial training. Feeling better may increase the willingness and motivation to engage in sports activities. Therefore, this multi-centre study evaluates the short and long-term changes in physical fitness of a child with a childhood malignancy, using a combined physical exercise and psychosocial intervention program, implemented during or shortly after treatment. Also examined is whether positive effects on physical fitness reduce inactivity-related adverse health problems, improve quality of life, and are cost-effective. Methods This multi-centre randomized controlled trial compares a combined physical and psychosocial intervention program for children with cancer, with care as usual (controls). Children with cancer (aged 8-18 years) treated with chemotherapy and/or radiotherapy, and who are no longer than 1 year post-treatment, are eligible for participation. A total of 100 children are being recruited from the paediatric oncology/haematology departments of three Dutch university medical centres. Patients are stratified according to pubertal stage (girls: age ≤10 or >10 years; boys: ≤11 or >11 years), type of malignancy (haematological or solid tumour), and moment of inclusion into the study (during or after treatment), and are randomly assigned to the intervention or control group. Discussion Childhood cancer patients undergoing long-term cancer therapy may benefit from a combined physical exercise and psychosocial intervention program since it may maintain or enhance their physical fitness and increase their quality of life. However, the feasibility, patient need, and effectiveness of such a program should be established before the program can be implemented as part of standard care. Trial registration number NTR1531 (The Netherlands National Trial Register)

Background Low health-related quality of life (HRQoL) of children with sickle cell disease (SCD) may be associated with consequences of the disease, or with the low socio-economic status (SES) of this patient population. The aim of this study was to investigate the HRQoL of children with SCD, controlling for SES by comparing them to healthy siblings (matched for age and gender), and to a Dutch norm population. Methods The HRQoL of 40 children with homozygous SCD and 36 healthy siblings was evaluated by the KIDSCREEN-52. This self-report questionnaire assesses ten domains of HRQoL. Differences between children with SCD and healthy siblings were analyzed using linear mixed models. One-sample t-tests were used to analyze differences with the Dutch norm population. Furthermore, the proportion of children with SCD with impaired HRQoL was evaluated. Results In general, the HRQoL of children with SCD appeared comparable to the HRQoL of healthy siblings, while children with SCD had worse HRQoL than the Dutch norm population on five domains (Physical Well-being, Moods & Emotions, Autonomy, Parent Relation, and Financial Resources). Healthy siblings had worse HRQoL than the Dutch norm population on three domains (Moods & Emotions, Parent Relation, and Financial Resources). More than one in three children with SCD and healthy siblings had impaired HRQoL on several domains. Conclusion These findings imply that reduced HRQoL in children with SCD is mainly related to the low SES of this patient population, with the exception of disease specific effects on the physical and autonomy domain. We conclude that children with SCD are especially vulnerable compared to other patient populations, and have special health care needs.

Objective: To evaluate self-reported health-related quality of life, anxiety, depression, and cognitive function in pediatric septic shock survivors. Design: A retrospective cohort study. Setting: A 14-bed tertiary pediatric intensive care unit. Patients: Children aged >= 8 yrs at the time of the follow-up who were admitted between 1995 and 2004 for septic shock. Inotropic and or vasoconstrictive agents were administered to these patients for >= 24 hrs. Intervention: Health-related quality of life was assessed with the KIDSCREEN-52, anxiety with the State Trait Anxiety Inventory for Children, depression with the Children's Depression Inventory, and cognitive function with the cognitive scale of the TNO-AZL Children's Quality of Life Questionnaire Child Form. Measurements and Main Results: Fifty of 82 eligible pediatric septic shock survivors were evaluated. The median age of the children at pediatric intensive care unit admission was 4.2 yrs (range, 0.0-17.0 yrs); the median age at follow-up was 10.7 yrs (range, 8.0-20.4 yrs). Health-related quality of life and anxiety scores were comparable to the age-related Dutch norm population. Depression scores were significantly better than the norm population, whereas cognitive function was significantly lower than the norm population. We found that 44% of the children had cognitive scores

To identify and appraise all published quality of life (QOL) measures developed for use with children with cancer and childhood cancer survivors. MEDLINE, CINAHL, EMBASE, PsycINFO, CancerLit, and Sociological Abstracts were searched from the inception of each database to 15 June 2009. Included articles were ones that described the development and/or psychometric evaluation of a QOL measure developed for use with children with cancer or childhood cancer survivors. Articles were appraised for adherence to internationally recommended guidelines for item generation, item reduction, and psychometric evaluation. Thirteen QOL questionnaires were identified by our search. Eleven measures are applicable to measuring QOL in children with any type of cancer, and two are specific to children with brain cancer. Four measures can be used to measure QOL in children undergoing cancer treatment, six can be used with children on or off treatment, and three are specific to childhood cancer survivors. While all measures underwent some degree of formal development and validation, item generation often did not involve children with cancer or their parents, and a number of measures did not describe or utilize recommended methods for item reduction and psychometric evaluation. Most of the measures identified in this review were designed to measure QOL concerns of children with any type of cancer and at any time, during treatment or survivorship. Our findings can help researchers and clinicians identify scientifically sound measures.

Background. This study was designed to evaluate generic and disease-specific health-related quality of life (HRQoL), cognitive functioning and behaviour problems of children with Langerhans Cell Histiocytosis (LCH). Furthermore, we investigated which medical determinants and social demographic factors were predictive for HRQoL, cognitive functioning and behavioural problems. Procedure. In this cross-sectional case-control study 24 children ranging from 7 to 17 years of age were administered a HRQoL questionnaire, cognitive tests and behaviour ratings. In addition, a disease-specific HRQoL measure was developed and tested. Results were compared to a reference group consisting of healthy peers and to proxy-ratings by parents and teachers. Results. Children with LCH reported a lower physical HRQoL than the reference group (P � 0.05). Children older than 12 reported lower HRQoL scores. Scores on the disease-specific HRQoL questionnaire were lower than on the generic measure used. Performances on cognitive tests varied widely, short term visual memory was most affected. Twenty-five percent of the children follow special education. According to parents and teachers, children with LCH had more internalising behaviour problems (i.e., anxiety and depression), compared to the instrument norms. Children with Diabetes Insipidus, other CNS involvement and children who have had chemotherapy had more cognitive and behaviour problems than the other children with LCH. Conclusions. HRQoL is affected in children with LCH, especially in older children. Children with LCH show more internalising problem behaviour than their peers. Teachers are important additional informants about behaviour problems. Pediatr Blood Cancer 2009; 52:116‐122. 2008 Wiley-Liss, Inc.

Aim To study the prevalence of posttraumatic stress disorder (PTSD), anxiety and depression in parents three months after pediatric intensive care treatment of their child and examine if peritraumatic dissocation and coping styles are related to these mental health problems. Methods This is a prospective cohort study and included parents of children unexpectedly admitted to the Pediatric Intensive Care Unit (PICU) from January 2006 to March 2007. At three months follow-up parents completed PTSD (n = 115), anxiety and depression (n = 128) questionnaires. Immediately after discharge, parents completed peritraumatic dissocation and coping questionnaires. Linear regression models with generalized estimating equations examined risk factors for mental health problems. Results Over 10% of the parents were likely to meet criteria for PTSD and almost one quarter for subclinical PTSD. Respectively 15% to 23% of the parents reported clinically significant levels of depression and anxiety. Peritraumatic dissocation was most strongly associated with PTSD, anxiety as well as depression. Avoidance coping was primarily associated with PTSD. Conclusion A significant number of parents have mental health problems three months after unexpected PICU treatment of their child. Improving detection and raise awareness of mental health problems is important to minimize the negative effect of these problems on parents' well-being.

Although the need for insight in factors influencing the quality of life of patients with an inborn error of metabolism is recognized, psychological adjustment of adults with metabolic diseases has not been properly studied. Adult patients with PKU were demonstrated not to differ from healthy controls in terms of their course of life (CoL) and health-related quality of life (HRQoL). However, adults with galactosaemia had a lower HRQoL with significant lower scores on the domains of cognitive and social function. This study investigated the CoL and the social demographical outcomes in these young adults with galactosaemia, and compared them with the general population and with PKU patients. A total of 15 (88%) adult patients with classical galactosaemia participated in this study. Classical galactosaemia patients had a delayed social and psychosexual development compared to their peers from the general population and to PKU patients. Also, they were significantly less frequently married or living together and significantly less frequently employed than the general population. Our study shows a stark contrast between patients with galactosaemia and patients with PKU, although both are diagnosed in the neonatal period and need life-long dietary restrictions. The observed difference is likely due to the long-term somatic complications frequently seen in galactosaemia and thus not due to the burden of a chronic disease necessitating life-long dietary restrictions. We conclude that it is essential that parents and clinicians encourage children with galactosaemia to participate in peer-related activities in order to stimulate social performance, which may result in a more normal CoL.

Background Parents of chronically ill children are at risk for a lower Health Related Quality of Life (HRQoL). Insight in the dynamics of factors influencing parental HRQoL is necessary for development of interventions. Aim of the present study was to explore the influence of demographic and disease related factors on parental HRQoL, mediated by employment, income, leisure time, holiday and emotional support in a comprehensive model. Methods In a cross-sectional design, 543 parents of chronically ill children completed questionnaires. A conceptual model of parental HRQoL was developed. Structural equation modeling was performed to explore the relations in the conceptual model, and to test if the model fitted the data. Results The model fitted the data closely (CHISQ(14) = 11.37, p = 0.66; RMSEA = 0.0, 90%CI [0.00;0.034]. The effect of socio-demographic and medical data on HRQoL was mediated by days on holiday (MCS: β = .21) and emotional support (PCS: β = .14; MCS: β = .28). Also, female gender (β = -.10), age (β = .10), being chronically ill as a parent (β = -.34), and care dependency of the child (β = -.14; β = -.15) were directly related to parental HRQoL. Conclusion The final model was slightly different from the conceptual model. Main factors explaining parental HRQoL seemed to be emotional support, care dependency, days on holiday and being chronically ill as a parent. Holiday and emotional support mediated the effect of demographic and disease-related factors on HRQoL. Hours of employment, leisure time and household income did not mediate between background characteristics and HRQoL, contrasting the hypotheses.

Objectives With a disease-specific questionnaire, this study aimed to evaluate health-related quality of life (HRQoL) in children with constipation in association with clinical characteristics. Study design Children with constipation-associated fecal incontinence (n = 114), 8 to 18% years, filled out the Defecation Disorder List at a Dutch tertian, hospital. Correlations and linear regression analysis between clinical characteristics and scores on emotional and social functioning were calculated. Specific concerns of children were described by individual item scores of these domains. Results Higher frequency of fecal incontinence episodes was associated with lower emotional and social functioning. Linear regression analysis showed a significant association between social functioning and fecal incontinence, but the variance of the model was low (adjusted R-2 = 0.08). Between 70% to 80% of children were concerned about experiencing fecal incontinence unnoticeably and the attendant social consequences. Children did not report having fewer friends and participated well in social events. Conclusion Lower HRQoL regarding disease-specific emotional and social functioning was reported in children with frequent episodes of constipation-associated fecal incontinence. However. other nonspecified factors may also influence HRQoL of these children. Most children reported relatively more emotional concerns than social consequences. (J Pediatr 2009;154:749-53)

Parents of children with congenitally malformed hearts can suffer from stress as a result of the medical condition of their child. In this cross-sectional study, we aimed to describe levels of parental stress, and perceived vulnerability, in parents of children who underwent major cardiac surgery, by using both generic and disease-related measures for assessment. We included parents of children who underwent open-heart surgery over the period 2002 through 2007 in the Center for congenital Anomalies Heart Amsterdam/Leiden, abbreviated to provide the acronym CAHAL. In total, we assessed 114 mothers and 82 fathers of 131 children, using the Pediatric Inventory for Parents, short form, General Health Questionnaire, Parental Stress Index-Short Form, State-Trait Anxiety Index and the Child Vulnerability Scale. Compared to the reference groups of the instruments used, parents of children with congenitally malformed hearts did not report higher generic nor disease-related stress scores, and parenting levels of stress were also comparable to reference groups. State anxiety levels, however, were higher in mothers of children with congenitally malformed hearts. Both fathers and mothers reported significantly higher rates of perceived vulnerability than did parents of healthy children. Risk factors for increased anxiety and perceived vulnerability were found in the number of surgical procedures, the time past since the last procedure, and ethnicity. Severity of the lesion did not influence parental levels of stress, but parents of children with hypoplastic left heart syndrome did report higher levels of stress than other parents. Psychosocial screening of parents of children with congenitally malformed hearts is important in order to provide appropriate counselling to those parents most in need.

Background Glucocorticoids are important in the treatment of childhood acute lymphoblastic leukaemia (ALL). However, cyclic administration of high dose glucocorticoids may cause rapid and substantial changes in quality of life (QoL). The maintenance phase of the Dutch ALL-9 protocol consisted of alternating two weeks on and five weeks off dexamethasone (6 mg/m2/day). The present study was performed to assess the effect of dexamethasone on QoL during treatment for ALL according to this protocol. Methods In a multicentre prospective cohort study, QoL was assessed halfway (T1) and at the end of the two-year treatment (T2). A generic (Child Health Questionnaire) and disease specific (PedsQL™ cancer version) QoL questionnaire were used to assess QoL in two periods: on and off dexamethasone, respectively. Results 41 children (56% males) were evaluated, mean age at diagnosis was 5.6 years. The CHQ physical and psychosocial summary scores were significantly lower than population norms. At T1 and T2, overall QoL showed no significant change. However, regarding specific domains (pain, cognitive functioning, emotion/behaviour and physical functioning) QoL decreased over time. QoL was significantly more impaired during periods on dexamethasone. Conclusion Dexamethasone was associated with decreased QoL. At the end of treatment, reported QoL during dexamethasone deteriorated even more on certain scales (pain, cognitive functioning, emotion/behaviour and physical functioning). Knowledge of the specific aspects of QoL is essential to improve counselling and coping in paediatric oncology. Adverse effects of specific drugs on QoL should be taken into account when designing treatment protocols.

Purpose: To determine whether childhood medulloblastoma and acute lymphoblastic leukemia (ALL) survivors have decreasedwhite matter fractional anisotropy (WMFA) and whether WMFA is related to the speed of processing and motor speed. Methods and Materials: For this study, 17 patients (6 medulloblastoma, 5 ALL treated with high-dose methotrexate(MTX)(4 � 5g/m 2 )and6withlow-doseMTX (3 � 2g/m 2 ))and17age-matchedcontrolsparticipated.Ona3.0T magnetic resonance imaging (MRI) scanner, diffusion tensor imaging (DTI) was performed, and WMFAvalues were calculated, including specific regions of interest (ROIs), and correlated with the speed of processing and motor speed. Results: Mean WMFA in the patient group, mean age 14 years (range 8.9 ‐ 16.9), was decreased compared with the control group (p= 0.01), as well as WMFA in the right inferior fronto-occipital fasciliculus (IFO) (p=0.03)andinthe genu of the corpus callosum (gCC) (p = 0.01). Based on neurocognitive results, significant positive correlations were present between processing speed and WMFA in the splenium (sCC) (r =0 .53,p = 0.03) and the body of the corpus callosum (bCC) (r =0 .52,p = 0.03), whereas the right IFO WMFAwas related to motor speed (r =0 .49,p < 0.05). Conclusions: Whitemattertracts,usinga3.0-TMRIscanner,showimpairmentinchildhoodcancersurvivors,medulloblastoma survivors, and also those treated with high doses of MTX. In particular, white matter tracts in the sCC,bCC andright IFOarepositivelycorrelatedwithspeedofprocessingand motorspeed. 2009ElsevierInc. DTI, Children, Cancer, Neuropsychology, MTX.

Background Improved survival in children with critical illnesses has led to new disease patterns. As a consequence evaluation of the well being of survivors of Pediatric Intensive Care Units (PICU) has become important. Outcome assessment should therefore consist of evaluation of morbidity, functional health and Health Related Quality of Life (HRQoL). Awareness of HRQoL consequences and physical sequelae could lead to changes in support during the acute phase and thereafter. The aim of this study was to evaluate HRQoL in PICU survivors. Methods Prospective follow-up study three and nine months after discharge from a 14-bed tertiary PICU. Eighty-one of 142 eligible, previously healthy children were included from December 2002 through October 2005. HRQoL was assessed with the TNO-AZL Preschool Children Quality of Life Questionnaire (TAPQOL-PF) for children aged 1 to 6 years of age, the TNO-AZL Children's Quality of Life Questionnaire Parent Form (TACQOL-PF) for children aged 6 to 12 years of age, and the TNO-AZL Children's Quality of Life Questionnaire Child Form (TACQOL-CF) for children aged 8 to 15 years of age. The studied patients were compared with age appropriate normative data using non-parametric tests and effect sizes. Results Thirty-one and 27 children, and 55 and 50 parents completed questionnaires respectively three and nine months after discharge. In 1–6 year old children parents reported more lung problems (3 and 9 months), worse liveliness (9 months) and better appetite and problem behaviour (3 months); in 6–12 year old children parents reported worse motor functioning (3 months); and 12–15 year old adolescents reported worse motor functioning (3 months). Large effect sizes indicating clinical significant differences in HRQoL with healthy control subjects were found on more domains. Conclusion In this small group of PICU survivors differences in HRQoL with the normative population exist three and nine months after discharge. Calculated effect sizes were smaller nine months after discharge. These changes suggest that HRQoL improves over time. More research is necessary but we believe that HRQoL assessment should be incorporated in follow-up programs of PICU survivors.

[CONTEXT]: The number of parents who care for a chronically ill child is increasing. Because of advances in medical care, parental caring tasks are changing. A detailed description of parental health-related quality of life will add to the understanding of the impact of caring for a chronically ill child. This will contribute to pediatric family care. Objective. Our goal was to determine the health-related quality of life of parents of chronically ill children compared with parents of healthy schoolchildren. [DESIGN, SETTING, AND PARTICIPANTS]: A survey of 533 parents of children with chronic conditions (10 diagnosis groups, children aged 1-19 years, diagnosed > 1 year ago, living at home) and 443 parents of schoolchildren was conducted between January 2006 and September 2007. Parents were approached through Emma Children's Hospital (which has a tertiary referral and a regional function) and through parent associations. The comparison group included parents of healthy schoolchildren. Health-related quality of life was assessed with the TNO-AZL Questionnaire for Adult's Health Related Quality of Life. [MAIN OUTCOME MEASURE]: Health-related quality of life measures gross and fine motor function, cognitive functioning, sleep, pain, social functioning, daily activities, sexuality, vitality, positive and depressive emotions, and aggressiveness. The health-related quality of life of the study group was compared with that of the comparison group, and effect sizes were estimated. The percentages of parents at risk for a low health-related quality of life were compared with the 25th percentile scores of the comparison group. Results. Parents of chronically ill children had a significantly lower health-related quality of life. Subgroup analysis showed lower health-related quality of life on sleep, social functioning, daily activities, vitality, positive emotions, and depressive emotions in disease-specific groups. On average, 45% of the parents were at risk for health-related quality-of-life impairment. [CONCLUSIONS]: Parents of chronically ill children report a seriously lower health-related quality of life, which should receive attention and supportive care if necessary. A family-centered approach in pediatrics is recommended. Copyright © 2008 by the American Academy of Pediatrics.

Contains fulltext : 69061.pdf (Publisher’s version ) (Closed access) BACKGROUND: The aim of the study was to access Health Related Quality of Life (HRQoL) in preschool cancer survivors during the first 3 years of continuous remission after the end of successful treatment, and to identify predictors of HRQoL. PROCEDURE: Parent-reported HRQoL was assessed in 53 preschool children treated successfully for cancer, using the TAPQOL and compared with norm data. Longitudinal mixed models analyses were performed to investigate to what extent demographic and medical variables and parental psychological distress were predictive of HRQoL over time. RESULTS: Two months after the end of successful cancer treatment, survivors showed significantly (P < 0.01) more problem behavior and anxiety, and scored significantly worse (P < 0.01) on sleeping, motor functioning, positive mood and liveliness than the norm. One year after the end of treatment survivors still showed significantly (P < 0.01) more anxiety and worse motor functioning. The level of HRQoL in survivors had normalized 2 and 3 years after the end of treatment. Longer duration of treatment, bad prognosis and greater parental psychological distress were associated with worse scores on the Physical Component Score of the TAPQOL. Medical variables and parental psychological distress were not associated with the Mental Component Score. CONCLUSIONS: Survivors adjusted well to the cancer experience and HRQoL improved with time. Despite overall resilience in survivors over time, physical as well as psychosocial monitoring in follow-up is recommended. Standard aftercare should preferably include psychosocial screening, education, and counseling directed at both survivors and parents.

Background The goals were to determine the presence of posttraumatic stress disorder (PTSD) in children after paediatric intensive care treatment, to identify risk factors for PTSD, and to compare this data with data from a major fire disaster in the Netherlands. Methods Children completed the Dutch Children's Responses to Trauma Inventory at three and nine months after discharge from the paediatric intensive care unit (PICU). Comparison data were available from 355 children survivors who completed the same questionnaire 10 months after a major fire disaster. Results Thirty-six children aged eight to 17 years completed questionnaires at three month follow-up, nine month follow-up, or both. More than one third (34.5%) of the children had subclinical PTSD, while 13.8% were likely to meet criteria for PTSD. Maternal PTSD was the strongest predictor for child PTSD. There were no significant differences in (subclinical) PTSD symptoms either over time or compared to symptoms of survivors from the fire disaster. Conclusion This study shows that a considerable number of children have persistent PTSD after PICU treatment. Prevention of PTSD is important to minimize the profound adverse effects that PTSD can have on children's well-being and future development.

Aim: To study the prevalence of posttraumatic stress in parents after an acute admission to a paediatric intensive care unit (PICU) and to determine risk factors for the development of posttraumatic stress. Methods: Parents completed posttraumatic stress questionnaires three months after their child's discharge. This questionnaire measures both symptoms of posttraumatic stress disorder (PTSD) and enables determination of the full psychiatric diagnosis of PTSD. Medical and demographic data concerning their child were gathered from physical evaluations three months after discharge. Of 250 eligible families, 144 (57.6%) participated in this study. The questionnaires were completed by 140 mothers and 107 fathers. Results: More than three-quarters of the parents experienced persistent symptoms of PTSD. In 21 mothers (15.0%) and 10 fathers (9.3%), the full psychiatric diagnosis of PTSD was determined. In six families, both parents had PTSD. Furthermore, a significant positive correlation was found between symptoms of PTSD of the mothers and the fathers. No obvious medical risk factors could be distinguished. Conclusion: The unexpected admission of a child to a PICU is a stressful event associated with parental posttraumatic stress. Treatment should not end after discharge. Follow-up care is warranted and research should be focused on prevention of these symptoms. © 2007 The Author(s).

In this paper studies conducted in the Netherlands on quality of life and social status of children and young adults who have grown up with a chronic disease are summarized. Only for 21 diseases studies could be found. Growing up with a chronic disease has negative consequences in terms of social functioning, schooling and intellectual functioning. Problems in quality of life domains are reported for children with congenital heart disease, inflammatory bowel disease, spina bifida and for children with end-stage renal disease. Little is known about predictive factors such as medical determinants, personality or family factors which are related to the later functioning. Research with a psychological-theoretical background in this area should be promoted, also to contribute to the development of necessary interventions for these children and young adults.

Summary Background Haemangiomas are the most common tumours of infancy, they are often present on the face and can lead to disfigurement. The aim of our study was to assess the health-related quality of life (HRQoL) of children aged 1–15 years with a haemangioma and their parents in comparison with healthy children, and to assess the impact on their life. Furthermore, we investigated if visibility and a complicated course of the haemangioma had influence on these outcomes. Methods Age-specific validated HRQoL and haemangioma-specific questionnaires were sent to all children seen at the AMC in Amsterdam at the plastic surgery or dermatology departments. Results Two hundred and one parents of children with a haemangioma returned the questionnaire (85%). The majority of parents and patients with a haemangioma are not negatively affected by it. Concerning HRQoL, parents of children aged 8 to 11 years reported their children to have more negative emotions. Children aged 12 to 15 reported a better HRQoL for physical symptoms, and positive emotions. No differences were found in the HRQoL questionnaires between visibility and complicated course. Significant differences occur in the specific haemangioma questionnaire. High scores on feelings of disbelief and panic during the growing phase are given by parents and patients agree with the statement on whether their life would be different without a haemangioma, especially when the haemangioma was visible and/or had a complicated course. Conclusions This is the first study to describe the HRQoL of children with haemangiomas. The psychosocial consequences of a haemangioma are feared by physicians and parents. The most important finding of this study is that the majority of children of all ages and their parents feel children with haemangiomas can live a good life. There is reason, however, to believe that having a haemangioma, especially one with a complicated course or a visible location, could result in psychosocial problems later in life, mostly related to physical appearance.

Context: Changes in the prevalence of chronic health conditions in childhood have considerable societal consequences for health care planning and for employment. To obtain valid and reliable estimates of the prevalence of chronic health conditions, a clear definition is needed. Objective: To present an overview of all definitions and operationalizations that have been applied to measure the prevalence of chronic health conditions in childhood. Data Sources: PubMed and the Web of Science were searched for articles published up to December 2006. Also, references were searched by hand for related articles. Study Selection: Non-English- and non-Dutch-language articles were excluded. Of 7252 articles found, 64 articles that stated a conceptual definition and/or operationalization of chronic health conditions in children (aged 0-18 years) were included. Data Extraction: Data on the (1) definition; (2) operationalization in terms of source of information, method of information retrieval, and study population; and (3) resulting prevalence rate were extracted by 2 independent reviewers. Data Synthesis: A large range of definitions were in use, of which 4 were cited by many authors. Various operationalizations of the concepts that were measured were identified. Chronic health conditions in childhood prevalence estimates ranged from 0.22% to 44%, depending on these operationalizations. Conclusions: The wide variability in reported prevalence rates of chronic health conditions in childhood can be explained by considerable diversity in the concepts and operationalizations used. International consensus about the conceptual definition of chronic health conditions in childhood is needed.

Background Muscular dystrophies are chronic diseases manifesting with progressive muscle weakness leading to decreasing activities and participation. To understand the impact on daily life, it is important to determine patients' quality of life. Objective To investigate Health Related Quality of Life (HRQoL) of children and adults with muscular dystrophy (MD), and to study the influence of type and severity of MD on HRQoL in adult patients. Methods Age-related HRQoL questionnaires were administered to 40 children (8–17 years), and 67 adult patients with muscular dystrophies. Results Significant differences in HRQoL were found in children and adults with MD compared to healthy controls. Patients with Becker muscular dystrophy reported a better HRQoL on the several scales compared to patients with other MDs. Severity was associated with worse fine motor functioning and social functioning in adult patients. Conclusion This is one of the first studies describing HRQoL of patients with MD using validated instruments in different age groups. The results indicate that having MD negatively influences the HRQoL on several domains.