Review about the impact of growing up with a chronic disease showed delays achieving psychosocial milestones.

AIM: This review aimed to provide a comprehensive overview of the psychosocial developmental trajectory of various diseases during childhood and adolescence. METHODS: Studies of Dutch young adults aged 18-35 years, who had grown up with a chronic disease, were included if the Course of Life Questionnaire had been used to assess psychosocial developmental milestones in three domains: social, autonomy and psychosexual. Differences between the disease groups and the general population were presented as Cohen's d and odds ratios. RESULTS: We included 17 studies comprising 1899 young adults, who had grown up with 18 different paediatric diseases. Psychosocial development was delayed in all three questionnaire domains. Remarkable findings with regard to specific milestones were as follows: less participation in sports clubs in the social domain, less likely to have had paid jobs in the autonomy domain and later sexual intimacy in the psychosexual domain. End-stage renal disease, galactosaemia (males), childhood cancer and orthotopic liver transplants were the most affected disease groups. CONCLUSION: Children and adolescents with chronic diseases risked delays in psychosocial development. This should be addressed by healthcare providers, along with the physical aspects of diseases, and they should focus on the optimal psychosocial development of the patient.

Screening for child abuse using a checklist and physical examinations in the emergency department led to the detection of more cases.

AIM: We studied the accuracy of a screening checklist (SPUTOVAMO), complete physical examination (top-to-toe inspection, TTI) and their combination in detecting child abuse in the emergency department (ED). METHODS: Consecutive patients admitted to the ED of the Academic Medical Center in Amsterdam between January 2011 and 1 July 2013 were included. An Expert Panel assigned a consensus diagnosis to positive cases. For all other and missed cases, the Child Abuse Counselling and Reporting Centre diagnosis was used. RESULTS: We included 17 229 admissions of 12 198 patients. In 46%, SPUTOVAMO was performed, in 33% TTI; 421 children (4.3%) tested positive on either or both, with 68 positive consensus diagnoses. In eight children not reported to the Expert Panel, the Child Abuse Counselling and Reporting Center diagnosis was positive. Ten of 3519 (0.3%) children testing negative on both were child abuse cases; 0.88% of the study group had a final child abuse diagnosis. The estimated PPV was 0.46 for SPUTOVAMO, 0.44 for TTI and 0.43 for the combination. CONCLUSION: Combining screening tests significantly increased the number of test positives and led to more child abuse cases detected. Combined screening for child abuse in all children less than 18 years old presenting to an ED is recommended.

Bi-allelic Loss-of-Function Mutations in the NPR-C Receptor Result in Enhanced Growth and Connective Tissue Abnormalities.

The natriuretic peptide signaling pathway has been implicated in many cellular processes, including endochondral ossification and bone growth. More precisely, different mutations in the NPR-B receptor and the CNP ligand have been identified in individuals with either short or tall stature. In this study we show that the NPR-C receptor (encoded by NPR3) is also important for the regulation of linear bone growth. We report four individuals, originating from three different families, with a phenotype characterized by tall stature, long digits, and extra epiphyses in the hands and feet. In addition, aortic dilatation was observed in two of these families. In each affected individual, we identified a bi-allelic loss-of-function mutation in NPR3. The missense mutations (c.442T>C [p.Ser148Pro] and c.1088A>T [p.Asp363Val]) resulted in intracellular retention of the NPR-C receptor and absent localization on the plasma membrane, whereas the nonsense mutation (c.1524delC [p.Tyr508∗]) resulted in nonsense-mediated mRNA decay. Biochemical analysis of plasma from two affected and unrelated individuals revealed a reduced NTproNP/NP ratio for all ligands and also high cGMP levels. These data strongly suggest a reduced clearance of natriuretic peptides by the defective NPR-C receptor and consequently increased activity of the NPR-A/B receptors. In conclusion, this study demonstrates that loss-of-function mutations in NPR3 result in increased NPR-A/B signaling activity and cause a phenotype marked by enhanced bone growth and cardiovascular abnormalities.

Assessments carried out by a child abuse and neglect team in an Amsterdam teaching hospital led to interventions in most of the reported cases.

AIM: This study described cases of child abuse and neglect (CAN) that were reported to the multiagency CAN team at the Emma Children's Hospital in Amsterdam and the resulting interventions. METHODS: We carried out a retrospective review of all cases that were reported to the CAN team from 1 January 2010 to 31 December 2012. RESULTS: There were 27 prenatal cases, 92 referrals based on parental characteristics and 523 children. Overall, 1.2% of the children visiting the emergency department of our hospital, attending the outpatients department or being admitted were reported to the team. More than half of the referrals (55.1%) were confirmed as CAN. The most common diagnoses were as follows: witnessing intimate partner violence, physical neglect and emotional abuse. If CAN was confirmed an intervention was offered in 98.3% of cases. If a CAN diagnosis was undetermined or rejected, the figures were still 83.5% and 64.2%, respectively. CONCLUSION: Our results showed that CAN affected more than one in every 100 children visiting our hospital, and the expertise of our hospital-based CAN Team led to an intervention in the majority of the reported cases. The broad scope of problems that were encountered underlined the importance of a multidisciplinary CAN team.

Parents' Opinion About a Routine Head-to-Toe Examination of Children as a Screening Instrument for Child Abuse and Neglect in Children Visiting the Emergency Department.

INTRODUCTION: To improve detection of child abuse and neglect (CAN), many emergency departments use screening methods. Apart from diagnostic accuracy, possible harms of screening methods are important to consider, especially because most children are not abused and do not benefit from screening. We performed a systematic literature review to assess parents' opinions about CAN screening, in which we could only include 7 studies, all reporting that the large majority of participating parents favor screening. Recently, a complete physical examination (called "top-toe" inspection [TTI], a fully undressed inspection of the child) was implemented as a CAN screening method at the emergency department of a teaching hospital in The Netherlands. This study describes parents' opinions about the TTI. METHODS: We used a questionnaire to assess parents' opinions about the TTI of their children when visiting the emergency department. During the study period, 1000 questionnaires were distributed by mail. RESULTS: In total, 372 questionnaires were returned (37%). A TTI was performed for 194 children (52%). The overall attitude of parents whose children underwent a TTI was positive; 77.3% of the respondents found the TTI acceptable, and 1.5% (N = 3) found it unacceptable. Seventy percent of the respondents agreed with the theorem that all children who visit the emergency department should have a TTI performed, and 7.3% (N = 14) disagreed. DISCUSSION: Contrary to what is commonly believed, both in our systematic literature review and in our questionnaire study, the majority of participating parents agree with screening for CAN in general and with the TTI specifically. Sharing the results of this study with ED personnel and policy makers could take away prejudices about perceived disagreement of parents, thereby improving implementation of and adherence to CAN screening.

Results of the implementation of a new screening protocol for child maltreatment at the Emergency Department of the Academic Medical Center in Amsterdam.

OBJECTIVE: This study examines the results of the implementation of a new screening protocol for child maltreatment (CM) at the Emergency Department (ED) of the Academic Medical Center in Amsterdam, The Netherlands. This protocol consists of adding a so called 'top-toe' inspection (TTI), an inspection of the fully undressed child, to the screening checklist for child maltreatment, the SPUTOVAMO. DESIGN: We collected data from all patients 0-18 years old directly after introduction (February 2010) and 9 months later. Outcome measures were: completion of the screening and reasons for non-adherence. Data were collected on age, gender, reason for visiting the ED (defined by International Classification of Disease, ICD), presence of a chronic illness, type of professional performing the TTI and admission during week or weekend days. RESULTS: In February 560 and in November 529 paediatric patients were admitted. In February the complete screening protocol was performed in 42% of all children, in November in 17%. A correlation between completion of the SPUTOVAMO and having a TTI performed was found. Older age and presence of a chronic illness influenced the chance of having both SPUTOVAMO and TTI performed negatively. The completion rate of SPUTOVAMO was influenced by ICD code. Completion of TTI was influenced by type of investigator. The best performing professional was the ED physician followed by the paediatrician followed by the ED nurse. The reasons for not performing a TTI were not documented. Refusal of the TTI by a patient or parent was reported three times. CONCLUSION: Implementation of this new screening protocol for CM was only mildly successful and declined in time. A negative correlation between older child age and having a chronic illness and completion of the screening was found. A practical recommendation resulting from this study could be that, if CM screening protocols prove to be effective in detecting CM, regular training sessions have to be held. Filling out the checklist is something that could be performed by ED nurses. Performing a TTI is perhaps easier for the ED physicians to make part of their daily routine.

Psychosocial well-being in young adults with chronic illness since childhood: the role of illness cognitions.

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 (ß = 0.31; ß = -0.32) and physical (ß = 0.16; ß = -0.15) HRQoL and with less anxiety (ß = -0.27; ß = 0.28) and depression (ß = -0.29; ß = 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.

Consequences of caring for a child with a chronic disease: Employment and leisure time of parents.

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.

Health-related quality of life, anxiety and depression in young adults with disability benefits due to childhood-onset somatic conditions.

BACKGROUND: As the treatment of chronic or life-threatening diseased children has dramatically over recent decades, more and more paediatric patients reach adulthood. Some of these patients are successfully integrating into adult life; leaving home, developing psychosocially, and defining a role for themselves in the community through employment. However, despite careful guidance and support, many others do not succeed. A growing number of adolescents and young adults who have had a somatic disease or disability since childhood apply for disability benefits. The purpose of this study was to assess the health-related quality of life (HRQoL), anxiety and depression of young adults receiving disability benefits because of somatic conditions compared to reference groups from the general Dutch population and to explore factors related to their HRQoL, anxiety and depression. METHODS: Young adults (N = 377, 22-31 yrs, 64.3% female) claiming disability benefits completed the RAND-36 and an online version of the HADS. Differences between respondents and both reference groups were tested using analysis of variance and logistic regression analysis by group and age (and gender). Regression analyses were conducted to predict HRQoL (Mental and Physical Component Scale; RAND-36) and Anxiety and Depression (HADS) by demographic and disease-related variables. RESULTS: The respondents reported worse HRQoL than the reference group (-1.76 Physical Component Scale; -0.48 Mental Component Scale), and a higher percentage were at risk for an anxiety (29.7%) and depressive (17.0%) disorder. Better HRQoL and lower levels of anxiety and depression were associated with a positive course of the illness and the use of medical devices. CONCLUSIONS: This study has found worse HRQoL and feelings of anxiety and depression experienced by young adults claiming disability benefits. Healthcare providers, including paediatric healthcare providers, should pay systematic attention to the emotional functioning of patients growing up with a somatic condition in order to optimise their emotional well-being and adaptation to society during their transition to adulthood. Future research should focus on emotional functioning in more detail in order to identify those patients that are most likely to develop difficulties in emotional functioning and who would benefit from specific psychosocial support aimed at workforce participation.

Gastrointestinal manifestations of cow's milk protein allergy and gastrointestinal motility.

UNLABELLED: Cow's milk protein allergy (CMPA) may cause gastrointestinal motility disorders. Symptoms of both conditions overlap and diagnostic tests do not reliably differentiate between both. A decrease of symptoms with an extensive hydrolysate and relapse during challenge is not a proof of allergy, because hydrolysates enhance gastric emptying, a pathophysiologic mechanism of gastro-oesophageal reflux (GER). Thickened formula reduces regurgitation, and failure to do so suggests CMPA. A thickened extensive hydrolysate may induce more rapid improvement, but does not always differentiate between CMPA and GER. Different hypotheses are discussed: is the overlap between CMPA and functional disorders coincidence, or do both entities present with identical symptoms, or does the fact that symptoms are identical indicates that there is only one entity involved? Studies on the prevention of CMPA focused on 'at-risk families', and resulted in a decrease of CMPA and atopic dermatitis, but did not provide data on the incidence of GER. CONCLUSION: As long as there are no objective diagnostic tools to separate GER from CMPA, the physician has two options: first treat the most likely diagnosis, and switch if after 2-4 weeks there is no improvement, or treat both conditions with one intervention, what will not result in a diagnosis.

Health-related quality of life and psychosocial developmental trajectory in young female beneficiaries with JIA.

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.

Growing into disability benefits? Psychosocial course of life of young adults with a chronic somatic disease or disability.

AIM: A growing number of young adults with somatic diseases/disabilities since childhood apply for disability benefits. The achievement of psychosocial milestones while growing up (course of life) is assumed to be related to job participation. This study assessed the course of life of young adult beneficiaries with somatic limitations compared with peers from the general Dutch population. METHODS: Young adult beneficiaries (22-31 years, N = 415) completed the Course of Life Questionnaire assessing the achievement of milestones on autonomy, psychosexual and social development and risk behaviour. Differences between respondents and peers were tested using analysis of variance and logistic regression analysis by group, age and gender. Associations were expressed as odds ratios (OR) with confidence intervals. RESULTS: The beneficiaries achieved fewer milestones or achieved the milestones at a later age than peers. The differences were substantial: most effect sizes were moderate to large and most of the ORs lower than 0.5. CONCLUSION: Young adult beneficiaries with somatic limitations since childhood are at risk of a delayed course of life. Healthcare providers should pay systematic attention to the psychosocial developmental trajectory of their patients in order to optimize their development to adulthood and, consequently, create conditions for an optimal labour market position.

The process of end-of-life decision-making in pediatrics: a national survey in the Netherlands.

OBJECTIVE: The goal of this study was to investigate how Dutch pediatric specialists reach end-of-life decisions, how they involve parents, and how they address conflicts. METHODS: We conducted a national cross-sectional survey among pediatric intensivists, oncologists, neurologists, neurosurgeons, and metabolic pediatricians practicing in the 8 Dutch university hospitals. We collected information on respondents' overall opinions and their clinical practice. RESULTS: Of the 185 eligible pediatric specialists, 74% returned the questionnaire. All responding physicians generally discuss an end-of-life decision with colleagues before discussing it with parents. In half of the reported cases, respondents informed parents about the intended decision and asked their permission. In one-quarter of the cases, respondents informed parents without asking for their permission. In the remaining one-quarter of the cases, respondents advised parents and consequently allowed them to have the decisive voice. The chosen approach is highly influenced by type of decision and type and duration of treatment. Conflicts within medical teams arose as a result of uncertainties about prognosis and treatment options. Most conflicts with parents arose because parents had a more positive view of the prognosis or had religious objections to treatment discontinuation. All conflicts were eventually resolved by a combination of strategies. In 66% of all cases, pain and symptom management were intensified before the child's death. CONCLUSIONS: Within Dutch pediatrics, end-of-life decisions are team decisions. Pediatric specialists differ considerably in how they involve parents in end-of-life decision-making, ranging from benevolent paternalism to parental autonomy. Main conflict-solving strategies are taking more time and extending discussions.

Monitoring health-related quality of life in paediatric practice: development of an innovative web-based application.

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.

Analysis of the question-answer service of the Emma Children's Hospital information centre.

The information centre of the Emma Children's Hospital AMC (EKZ AMC) is a specialised information centre where paediatric patients and persons involved with the patient can ask questions about all aspects of disease and its social implications. The aim of the study was to evaluate the question-answer service of this information centre in order to determine the role of a specialised information centre in an academic children's hospital, identify the appropriate resources for the service and potential positive effects. For this purpose, a case management system was developed in MS ACCESS. The characteristics of the requester and the question, the time it took to answer questions, the information sources used and the extent to which we were able to answer the questions were registered. The costs of the service were determined. We analysed all questions that were asked in the year 2007. Fourteen hundred thirty-four questions were asked. Most questions were asked by parents (23.3%), healthcare workers (other than nurses; 16.5%) and nurses (15.3%). The scope of the most frequently asked questions include disease (20.2%) and treatment (13.0%). Information on paper was the main information source used. Most questions could be solved within 15 min. Twelve percent to 28% of total working hours are used for the question-answer service. Total costs including staff salary are rather large. In conclusions, taking over the task of providing additional medical information and by providing readily available, good quality information that healthcare professionals can use to inform their patients will lead to less time investment of these more expensive staff members. A specialised information service can anticipate on the information need of parents and persons involved with the paediatric patient. It improves information by providing with relatively simple resources that has the potential to improve patient and parent satisfaction, coping and medical results. A specialised information centre is therefore a valuable and affordable asset to an academic children's hospital.

Probiotics and prebiotics in atopic dermatitis: review of the theoretical background and clinical evidence.

The prevalence of atopic dermatitis (AD) has risen over the past decades, especially in western societies. According to the revised hygiene hypothesis this increase is caused by a changed intestinal colonization pattern during infancy, which has an impact on the immune system. Manipulating the intestinal microflora with pro-, pre- or synbiotics is an innovative way to prevent or treat AD. This review provides an overview of the theoretical basis for using probiotics and prebiotics in AD and presents the current evidence from randomized controlled trials (RCTs) regarding prevention and treatment of AD and food allergy in children with pro-, pre- and synbiotics. Seven RCTs on prevention and 12 RCTs on treatment were found by searching the Pubmed, Embase and Cochrane databases. Results of these trials are conflicting. In conclusion, at this moment there is not enough evidence to support the use of pro-, pre- or synbiotics for prevention or treatment of AD in children in clinical practice.

An explorative study on quality of life and psychological and cognitive function in pediatric survivors of septic shock.

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 >or=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 >or=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 <25% of the norm population. Young age at the time of pediatric intensive care unit admission was predictive of cognitive problems, and cognitive problems were associated with lower emotional function. CONCLUSIONS: In this group of septic shock survivors, health-related quality of life, anxiety, and depression are equal to or slightly better than the age-related Dutch norm population. Cognitive function is decreased, especially in children admitted at younger ages. Follow-up studies with adequate neuropsychological testing are warranted to evaluate the association between septic shock, cognitive function, and risk factors for cognitive problems.

A predictive model of Health Related Quality of life of parents of chronically ill children: the importance of care-dependency of their child and their support system.

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: beta = .21) and emotional support (PCS: beta = .14; MCS: beta = .28). Also, female gender (beta = -.10), age (beta = .10), being chronically ill as a parent (beta = -.34), and care dependency of the child (beta = -.14; beta = -.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.

Predicting health-related quality of life of parents of children with inherited metabolic diseases.

AIM: The aim of this study was to examine medical, socio-demographic and psychosocial determinants of health-related quality of life (HRQoL) of parents of children with metabolic diseases. METHODS: A survey among parents of children with metabolic diseases (children aged 1-19 years, diagnosed >1 year before the start of the study, living at home). Parents were approached through the Emma Children's Hospital, and through a national parent and patient association. HRQoL was assessed using the TNO-AZL Questionnaire for Adult's Health Related Quality of Life (TAAQOL), describing 12 domains of HRQoL. Predictor variables were taken from a self-report questionnaire. Univariate and multivariate logistic regression analyses were performed to predict which parents were at risk for HRQoL impairment. RESULTS: Mainly psychosocial determinants were predictive for parental HRQoL. Emotional support was protective for parental HRQOL while loss of friendship was a risk factor for HRQoL impairment. Medical and socio-demographic variables did not consistently predict parental HRQoL. CONCLUSION: Psychosocial determinants appeared more important in predicting parental HRQoL than medical and socio-demographic variables. Interventions should be focused on supporting parents combining the care for their children with a social life. Further research on this subject is necessary. In the meantime, involved medical specialists should pay structural attention to parental functioning.