The first steps in the development of a cancer-specific patient-reported experience measure item bank (PREM-item bank): towards dynamic evaluation of experiences.

OBJECTIVE: Since the implementation of value-based healthcare, there has been a growing emphasis on utilizing patient-reported experience measures (PREMs) to enhance the quality of care. However, the current PREMs are primarily generic and static, whereas healthcare is constantly evolving and encompasses a wide variety of aspects that impact care quality. To continuously improve care requires a dynamic PREM. The aim of this study was to propose an item bank for the establishment of a dynamic and care-specific patient-reported evaluation. METHODS: In co-creation with patients, a mixed methods study was conducted involving: (1) an explorative review of the literature, (2) a focus group analysis with (ex-)patients, (3) qualitative analyses to formulate themes, and (4) a quantitative selection of items by patients and experts through prioritization. RESULTS: Eight existing PREMs were evaluated. After removing duplicates, 141 items were identified. Through qualitative analyses of the focus group in which the patient journey was discussed, eight themes were formulated: "Organization of healthcare," "Competence of healthcare professionals," "Communication," "Information & services," "Patient empowerment," "Continuity & informal care," "Environment," and "Technology." Seven patients and eleven professionals were asked to prioritize what they considered the most important items. From this, an item bank with 76 items was proposed. CONCLUSION: In collaboration with patients and healthcare professionals, we have proposed a PREM-item bank to evaluate the experiences of patients' receiving cancer care in an outpatient clinic. This item bank is the first step to dynamically assess the quality of cancer care provided in an outpatient setting.

Type D Personality as a Risk Factor for Adverse Outcome in Patients With Cardiovascular Disease: An Individual Patient-Data Meta-analysis.

OBJECTIVE: Type D personality, a joint tendency toward negative affectivity and social inhibition, has been linked to adverse events in patients with heart disease, although with inconsistent findings. Here, we apply an individual patient-data meta-analysis to data from 19 prospective cohort studies ( N = 11,151) to investigate the prediction of adverse outcomes by type D personality in patients with acquired cardiovascular disease. METHOD: For each outcome (all-cause mortality, cardiac mortality, myocardial infarction, coronary artery bypass grafting, percutaneous coronary intervention, major adverse cardiac event, any adverse event), we estimated type D's prognostic influence and the moderation by age, sex, and disease type. RESULTS: In patients with cardiovascular disease, evidence for a type D effect in terms of the Bayes factor (BF) was strong for major adverse cardiac event (BF = 42.5; odds ratio [OR] = 1.14) and any adverse event (BF = 129.4; OR = 1.15). Evidence for the null hypothesis was found for all-cause mortality (BF = 45.9; OR = 1.03), cardiac mortality (BF = 23.7; OR = 0.99), and myocardial infarction (BF = 16.9; OR = 1.12), suggesting that type D had no effect on these outcomes. This evidence was similar in the subset of patients with coronary artery disease (CAD), but inconclusive for patients with heart failure (HF). Positive effects were found for negative affectivity on cardiac and all-cause mortality, with the latter being more pronounced in male than female patients. CONCLUSION: Across 19 prospective cohort studies, type D predicts adverse events in patients with CAD, whereas evidence in patients with HF was inconclusive. In both patients with CAD and HF, we found evidence for a null effect of type D on cardiac and all-cause mortality.

The association of hypoglycemia with outcome of critically ill children in relation to nutritional and blood glucose control strategies.

BACKGROUND: Withholding parenteral nutrition (PN) until one week after PICU admission facilitated recovery from critical illness and protected against emotional and behavioral problems 4 years later. However, the intervention increased the risk of hypoglycemia, which may have counteracted part of the benefit. Previously, hypoglycemia occurring under tight glucose control in critically ill children receiving early PN did not associate with long-term harm. We investigated whether hypoglycemia in PICU differentially associates with outcome in the context of withholding early PN, and whether any potential association with outcome may depend on the applied glucose control protocol. METHODS: In this secondary analysis of the multicenter PEPaNIC RCT, we studied whether hypoglycemia in PICU associated with mortality (N = 1440) and 4-years neurodevelopmental outcome (N = 674) through univariable comparison and multivariable regression analyses adjusting for potential confounders. In patients with available blood samples (N = 556), multivariable models were additionally adjusted for baseline serum NSE and S100B concentrations as biomarkers of neuronal, respectively, astrocytic damage. To study whether an association of hypoglycemia with outcome may be affected by the nutritional strategy or center-specific glucose control protocol, we further adjusted the models for the interaction between hypoglycemia and the randomized nutritional strategy, respectively, treatment center. In sensitivity analyses, we studied whether any association with outcome was different in patients with iatrogenic or spontaneous/recurrent hypoglycemia. RESULTS: Hypoglycemia univariably associated with higher mortality in PICU, at 90 days and 4 years after randomization, but not when adjusted for risk factors. After 4 years, critically ill children with hypoglycemia scored significantly worse for certain parent/caregiver-reported executive functions (working memory, planning and organization, metacognition) than patients without hypoglycemia, also when adjusted for risk factors including baseline NSE and S100B. Further adjustment for the interaction of hypoglycemia with the randomized intervention or treatment center revealed a potential interaction, whereby tight glucose control and withholding early PN may be protective. Impaired executive functions were most pronounced in patients with spontaneous or recurrent hypoglycemia. CONCLUSION: Critically ill children exposed to hypoglycemia in PICU were at higher risk of impaired executive functions after 4 years, especially in cases of spontaneous/recurrent hypoglycemia.

Early brain magnetic resonance imaging findings and neurodevelopmental outcome in children with congenital heart disease: A systematic review.

AIM: To investigate the association between early brain magnetic resonance imaging (MRI) findings and neurodevelopmental outcome (NDO) in children with congenital heart disease (CHD). METHOD: A search for studies was conducted in Embase, Medline, Web of Science, Cochrane Central, PsycINFO, and Google Scholar. Observational and interventional studies were included, in which patients with CHD underwent surgery before 2 months of age, a brain MRI scan in the first year of life, and neurodevelopmental assessment beyond the age of 1 year. RESULTS: Eighteen studies were included. Thirteen found an association between either quantitative or qualitative brain metrics and NDO: 5 out of 7 studies showed decreased brain volume was significantly associated with worse NDO, as did 7 out of 10 studies on brain injury. Scanning protocols and neurodevelopmental tests varied strongly. INTERPRETATION: Reduced brain volume and brain injury in patients with CHD can be associated with impaired NDO, yet standardized scanning protocols and neurodevelopmental assessment are needed to further unravel trajectories of impaired brain development and its effects on outcome.

Hospital-to-home transitions for children with medical complexity: part 2-a core outcome set.

Appropriate outcome measures as part of high-quality intervention trials are critical to advancing hospital-to-home transitions for Children with Medical Complexity (CMC). Our aim was to conduct a Delphi study and focus groups to identify a Core Outcome Set (COS) that healthcare professionals and parents consider essential outcomes for future intervention research. The development process consisted of two phases: (1) a three-round Delphi study in which different professionals rated outcomes, previously described in a systematic review, for inclusion in the COS and (2) focus groups with parents of CMC to validate the results of the Delphi study. Forty-five professionals participated in the Delphi study. The response rates were 55%, 57%, and 58% in the three rounds, respectively. In addition to the 24 outcomes from the literature, the participants suggested 12 additional outcomes. The Delphi rounds resulted in the following core outcomes: (1) disease management, (2) child's quality of life, and (3) impact on the life of families. Two focus groups with seven parents highlighted another core outcome: (4) self-efficacy of parents.   Conclusion: An evidence-informed COS has been developed based on consensus among healthcare professionals and parents. These core outcomes could facilitate standard reporting in future CMC hospital to home transition research. This study facilitated the next step of COS development: selecting the appropriate measurement instruments for every outcome. What is Known: • Hospital-to-home transition for Children with Medical Complexity is a challenging process. • The use of core outcome sets could improve the quality and consistency of research reporting, ultimately leading to better outcomes for children and families. What is New: • The Core Outcome Set for transitional care for Children with Medical Complexity includes four outcomes: disease management, children's quality of life, impact on the life of families, and self-efficacy of parents.

Neurocognitive, Psychosocial, and Quality of Life Outcomes After Multisystem Inflammatory Syndrome in Children Admitted to the PICU.

OBJECTIVES: To investigate neurocognitive, psychosocial, and quality of life (QoL) outcomes in children with Multisystem Inflammatory Syndrome in Children (MIS-C) seen 3-6 months after PICU admission. DESIGN: National prospective cohort study March 2020 to November 2021. SETTING: Seven PICUs in the Netherlands. PATIENTS: Children with MIS-C (0-17 yr) admitted to a PICU. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Children and/or parents were seen median (interquartile range [IQR] 4 mo [3-5 mo]) after PICU admission. Testing included assessment of neurocognitive, psychosocial, and QoL outcomes with reference to Dutch pre-COVID-19 general population norms. Effect sizes (Hedges' g ) were used to indicate the strengths and clinical relevance of differences: 0.2 small, 0.5 medium, and 0.8 and above large. Of 69 children with MIS-C, 49 (median age 11.6 yr [IQR 9.3-15.6 yr]) attended follow-up. General intelligence and verbal memory scores were normal compared with population norms. Twenty-nine of the 49 followed-up (59%) underwent extensive testing with worse function in domains such as visual memory, g = 1.0 (95% CI, 0.6-1.4), sustained attention, g = 2.0 (95% CI 1.4-2.4), and planning, g = 0.5 (95% CI, 0.1-0.9). The children also had more emotional and behavioral problems, g = 0.4 (95% CI 0.1-0.7), and had lower QoL scores in domains such as physical functioning g = 1.3 (95% CI 0.9-1.6), school functioning g = 1.1 (95% CI 0.7-1.4), and increased fatigue g = 0.5 (95% CI 0.1-0.9) compared with population norms. Elevated risk for posttraumatic stress disorder (PTSD) was seen in 10 of 30 children (33%) with MIS-C. Last, in the 32 parents, no elevated risk for PTSD was found. CONCLUSIONS: Children with MIS-C requiring PICU admission had normal overall intelligence 4 months after PICU discharge. Nevertheless, these children reported more emotional and behavioral problems, more PTSD, and worse QoL compared with general population norms. In a subset undergoing more extensive testing, we also identified irregularities in neurocognitive functions. Whether these impairments are caused by the viral or inflammatory response, the PICU admission, or COVID-19 restrictions remains to be investigated.

The eXpectations of Parents regarding Anesthesiology Study (XPAS) from a parental perspective: a two-phase observational cross-sectional cohort study.

BACKGROUND: Uncertainty concerning anesthetic procedures and risks in children requiring anesthesia may cause concerns in parents and caregivers. AIMS: To explore parental expectations and experiences regarding their child's anesthesia using questionnaires designed with parental input. METHODS: This observational cross-sectional cohort study included parents (including caregivers) of children undergoing anesthesia in a tertiary pediatric referral university hospital. The study consisted of two phases. In Phase 1, we developed three questionnaires with parental involvement through a focus group discussion and individual interviews. The questionnaires focused on parental satisfaction, knowledge, concerns, and need for preparation regarding their child's anesthesia. In Phase 2, independent samples of parents completed the questionnaires at three time points: before the preanesthesia assessment (T1), 2 days after the preanesthesia assessment (T2), and 4 days after the anesthetic procedure (T3). RESULTS: In Phase 1, 22 parents were involved in the development of the questionnaires. The three questionnaires contained 43 questions in total, of which 10 had been proposed by parents. In Phase 2, 78% (474 out of 934) parents participated at T1, 36% (610 out of 1705), at T2 and 34% (546 out of 1622) at T3. Parental satisfaction scores were rated on a visual analogue scale for the preanesthesia assessment with a median of 87/100, and with a median of 90/100 for the anesthetic procedure (0: not satisfied and 100: satisfied). Parental concerns were rated with a median of 50/100 (0: no concerns and 100: extremely concerned). Parental answers from the questionnaire at T2 revealed significant knowledge deficits, with only 73% reporting that the anesthesiologist was a physician. Parents preferred to receive more information about the procedure, especially regarding the intended effects and side effects of anesthesia. CONCLUSIONS: Overall, parental satisfaction scores regarding the pediatric anesthesiology procedure were high, with a minority expressing concerns. Parents indicated a preference for their child's anesthesiologist to visit them both before and after the anesthetic procedure. Parental expectations regarding anesthesia did not completely correspond with the information provided; more information from the clinician about the intended effects and side effects of anesthesia was desired.

Impact of critical illness and withholding of early parenteral nutrition in the pediatric intensive care unit on long-term physical performance of children: a 4-year follow-up of the PEPaNIC randomized controlled trial.

BACKGROUND: Many critically ill children face long-term developmental impairments. The PEPaNIC trial attributed part of the problems at the level of neurocognitive and emotional/behavioral development to early use of parenteral nutrition (early-PN) in the PICU, as compared with withholding it for 1 week (late-PN). Insight in long-term daily life physical functional capacity after critical illness is limited. Also, whether timing of initiating PN affects long-term physical function of these children remained unknown. METHODS: This preplanned follow-up study of the multicenter PEPaNIC randomized controlled trial subjected 521 former critically ill children (253 early-PN, 268 late-PN) to quantitative physical function tests 4 years after PICU admission in Leuven or Rotterdam, in comparison with 346 age- and sex-matched healthy children. Tests included handgrip strength measurement, timed up-and-go test, 6-min walk test, and evaluation of everyday overall physical activity with an accelerometer. We compared these functional measures for the former critically ill and healthy children and for former critically ill children randomized to late-PN versus early-PN, with multivariable linear or logistic regression analyses adjusting for risk factors. RESULTS: As compared with healthy children, former critically ill children showed less handgrip strength (p < 0.0001), completed the timed up-and-go test more slowly (p < 0.0001), walked a shorter distance in 6 min (p < 0.0001) during which they experienced a larger drop in peripheral oxygen saturation (p ≤ 0.026), showed a lower energy expenditure (p ≤ 0.024), performed more light and less moderate physical activity (p ≤ 0.047), and walked fewer steps per day (p = 0.0074). Late-PN as compared with early-PN did not significantly affect these outcomes. CONCLUSIONS: Four years after PICU admission, former critically ill children showed worse physical performance as compared with healthy children, without impact of timing of supplemental PN in the PICU. This study provides further support for de-implementing the early use of PN in the PICU. Trial registration ClinicalTrials.gov, NCT01536275 ; registered on February 22, 2012.

Neurocognitive functioning and health-related quality of life of children after pediatric intensive care admission: a systematic review.

OBJECTIVE: This study systematically reviewed recent findings on neurocognitive functioning and health-related quality of life (HRQoL) of children after pediatric intensive care unit admission (PICU). DATA SOURCES: Electronic databases searched included Embase, Medline Ovid, Web of Science, Cochrane CENTRAL, and Google Scholar. The search was limited to studies published in the last five years (2015-2019). STUDY SELECTION: Original studies assessing neurocognitive functioning or HRQoL in children who were previously admitted to the PICU were included in this systematic review. DATA EXTRACTION: Of the 3649 identified studies, 299 met the inclusion criteria based on title abstract screening. After full-text screening, 75 articles were included in the qualitative data reviewing: 38 on neurocognitive functioning, 33 on HRQoL, and 4 on both outcomes. DATA SYNTHESIS: Studies examining neurocognitive functioning found overall worse scores for general intellectual functioning, attention, processing speed, memory, and executive functioning. Studies investigating HRQoL found overall worse scores for both physical and psychosocial HRQoL. On the short term (≤ 12 months), most studies reported HRQoL impairments, whereas in some long-term studies HRQoL normalized. The effectiveness of the few intervention studies during and after PICU admission on long-term outcomes varied. CONCLUSIONS: PICU survivors have lower scores for neurocognitive functioning and HRQoL than children from the general population. A structured follow-up program after a PICU admission is needed to identify those children and parents who are at risk. However, more research is needed into testing interventions in randomized controlled trials aiming on preventing or improving impairments in critically ill children during and after PICU admission.

Physical, Emotional/Behavioral, and Neurocognitive Developmental Outcomes From 2 to 4 Years After PICU Admission: A Secondary Analysis of the Early Versus Late Parenteral Nutrition Randomized Controlled Trial Cohort.

OBJECTIVES: PICU patients face long-term developmental impairments, partially attributable to early parenteral nutrition (PN) versus late-PN. We investigated how this legacy and harm by early-PN evolve over time. DESIGN: Preplanned secondary analysis of the multicenter PEPaNIC-RCT (ClinicalTrials.gov, NCT01536275) that enrolled 1,440 critically ill children from 2012 to 2015 and its 2- (2014-2018) and 4-year (2016-2019) cross-sectional follow-up studies. SETTING: PICUs of Leuven (Belgium), Rotterdam (The Netherlands), and Edmonton (Canada). PATIENTS: Patients and demographically matched healthy control children that underwent longitudinal assessment for physical/emotional/behavioral/neurocognitive functions at both follow-up time points. INTERVENTIONS: In the PEPaNIC-RCT, patients were randomly allocated to early-PN versus late-PN. MEASUREMENTS AND MAIN RESULTS: This within-individual longitudinal study investigated changes in physical/emotional/behavioral/neurocognitive functions from 2 to 4 years after PICU admission for 614 patients (297 early-PN and 317 late-PN, tested at mean ± sd age 5.4 ± 4.2 and 7.3 ± 4.3 yr) and for 357 demographically matched healthy children tested at age 5.6 ± 4.3 and 7.5 ± 4.3 years. We determined within-group time-courses, interaction between time and group, and independent impact of critical illness and early-PN on these time-courses. Most deficits in patients versus healthy children remained prominent over the 2 years ( p ≤ 0.01). Deficits further aggravated for height, body mass index, the executive function metacognition, intelligence, motor coordination (alternating/synchronous tapping), and memory learning-index, whereas verbal memory deficits became smaller (working/immediate/delayed memory) ( p ≤ 0.05). Adjustment for risk factors confirmed most findings and revealed that patients "grew-into-deficit" for additional executive functions (flexibility/emotional control/total executive functioning) and "grew-out-of-deficit" for additional memory functions (recognition/pictures) ( p ≤ 0.05). Time-courses were largely unaffected by early-PN versus late-PN, except for weight loss and limited catch-up for visual-motor integration and alertness in early-PN patients ( p ≤ 0.05). CONCLUSIONS: From 2- to 4-year post-PICU admission, developmental impairments remained prominent. Within that time-window, impaired growth in height, executive functioning and intelligence aggravated, and impaired memory and harm by early-PN only partially recovered. Impact on development into adulthood requires further investigation.

Perceived stress, family impact, and changes in physical and social daily life activities of children with chronic somatic conditions during the COVID-19 pandemic.

BACKGROUND: The COVID-19 pandemic has inevitably affected children and their families. This study examines the impact of the COVID-19 measures in children with chronic somatic conditions (CSC) and their parents and compares them with a Dutch general population sample. METHODS: We included a sample of children with CSC (0-18 years, n = 326) and compared them with children (8-18 years, n = 1,287) from the Dutch general population. Perceived stress, coping, social interaction with friends and family, physical activity, eating behavior, family support, parenting perception, and financial situation were assessed once with the self-reported and parent-reported COVID-19 child check questionnaire, between November 2020 and May 2021. Comparisons between the two samples were made by using t-tests and chi square tests. RESULTS: The proportion of children who reported being less physically active and having less social interaction with friends since the COVID-19 pandemic was higher in children with CSC than in children from the general population. Children with CSC and their parents experienced less stress than children and parents from the general population. Moreover, parents of children with CSC aged 0-7 years and parents of children aged 8-18 years from the general population experienced less support and more financial deterioration than parents of children with CSC aged 8-18 years. In the parents from the general population only, this deteriorated financial situation was associated with more stress, worse family interaction and parenting perception, and less received support. CONCLUSIONS: The impact of COVID-19 on children with CSC and their parents differed from those in the general population. Addressing the collateral damage of COVID-19 measures in children and their families may give direction to policy and potentially prevent lifelong impact.

Health-related quality of life of children and their parents 2 years after critical illness: pre-planned follow-up of the PEPaNIC international, randomized, controlled trial.

BACKGROUND: Pediatric intensive care unit (PICU) survivors are at risk for prolonged morbidities interfering with daily life. The current study examined parent-reported health-related quality of life (HRQoL) in former critically ill children and parents themselves and aimed to determine whether withholding parenteral nutrition (PN) in the first week of critical illness affected children's and parents' HRQoL 2 years later. METHODS: Children who participated in the pediatric early versus late parenteral nutrition in critical illness (PEPaNIC) trial and who were testable 2 years later (n = 1158) were included. Their HRQoL outcomes were compared with 405 matched healthy controls. At PICU admission, children had been randomly assigned to early-PN or late-PN. In the early-PN group, PN was initiated within 24 h after PICU admission. In the late-PN group, PN was withheld for up to 1 week in the PICU. Parents completed the Infant Toddler Quality of Life Questionnaire (ITQOL; age 2-3 years) or the Child Health Questionnaire-Parent Form 50 (CHQ-PF50; age 4-18 years). Besides, they completed the Health Utility Index (HUI) and the Short Form Health Survey (SF-12) regarding their child's and their own HRQoL, respectively. RESULTS: For the total age group of 786 post-PICU survivors, parents reported lower scores for almost all HRQoL scales compared to healthy children. Age-specifically, younger critically ill children (2.5 to 3 years old) scored worse for growth and development and older children (4-18 years old) scored worse for role functioning and mental health. Parents' own mental and physical HRQoL was comparable to that of healthy control parents. No HRQoL differences were found between children in the late-PN and those in the early-PN group. CONCLUSIONS: Parent-reported HRQoL of children 2 years after critical illness was impaired compared with healthy controls. In relation to their child's HRQoL, parents reported impairments in emotions, personal time, and family activities; however, their own HRQoL was not impaired. Withholding PN in the first week during critical illness had no impact on longer-term HRQoL of the child. TRIAL REGISTRATION: Clinical trials, NCT01536275. Registered 22 February 2012.

Health-related quality of life of children and their parents 6 months after children's critical illness.

PURPOSE: This study aimed to examine health-related quality of life (HRQoL) of children and their parents, 6 months after the child's admission to the Pediatric Intensive Care Unit (PICU). Associations between parents' reports regarding HRQoL of their child and of themselves were investigated, as well as associations between children's baseline variables and their parent-reported HRQoL outcomes. METHODS: This is a secondary analysis of cross-sectional data collected in a group of children who participated in the PEPaNIC trial. Six months after discharge from the PICU, parents of critically ill children completed the Infant-Toddler Quality of Life Questionnaire (ITQOL, for age 0-3 years) or the Child Health Questionnaire-Parent Form 50 (CHQ-PF50, for age 4-18 years), which are parallel questionnaires. Parents completed the Short Form Health Survey (SF-12) regarding their own HRQoL. Results were compared with normative data. RESULTS: At 6 months' follow-up, 86 children of the 1343 (6%) had died which resulted in 1257 eligible children. Parents of 576 surviving children (46%) completed the questionnaires. Children of responding parents had less often an acute reason for admission and differed in diagnosis compared with children of non-responders. PICU children scored lower on most ITQOL (n = 390) scales and CHQ-PF50 (n = 186) scales compared with normative data. Parents reported (n = 570) higher scores on the physical (p < 0.001) and lower scores on the mental SF-12 scale (p < 0.001) compared with normative data. Parents̕ mental HRQoL correlated with HRQoL they reported for their child (Pearson Correlations range 0.25-0.57, p < 0.001-0.002). Shorter length of stay, lower risk of mortality, younger age, and cardiac diagnosis were associated with higher parent-reported HRQoL outcomes for the child. CONCLUSIONS: Six months after PICU discharge, critically ill children have lower HRQoL compared with normative data. The mental component of HRQoL is impaired in parents and is associated with lower overall parent-reported HRQoL of their child.

The CHIP-Family study to improve the psychosocial wellbeing of young children with congenital heart disease and their families: design of a randomized controlled trial.

BACKGROUND: Children with congenital heart disease (CHD) are at increased risk for behavioral, emotional, and cognitive problems. They often have reduced exercise capacity and participate less in sports, which is associated with a lower quality of life. Starting school may present more challenges for children with CHD and their families than for families with healthy children. Moreover, parents of children with CHD are at risk for psychosocial problems. Therefore, a family-centered psychosocial intervention for children with CHD when starting school is needed. Until now, the 'Congenital Heart Disease Intervention Program (CHIP) - School' is the only evidence-based intervention in this field. However, CHIP-School targeted parents only and resulted in non-significant, though positive, effects as to child psychosocial wellbeing. Hence, we expanded CHIP by adding a specific child module and including siblings, creating the CHIP-Family intervention. The CHIP-Family study aims to (1) test the effects of CHIP-Family on parental mental health and psychosocial wellbeing of CHD-children and to (2) identify baseline psychosocial and medical predictors for the effectiveness of CHIP-Family. METHODS: We will conduct a single-blinded randomized controlled trial comparing the effects of CHIP-Family with care as usual (no psychosocial intervention). Children with CHD (4-7 years old) who are starting or attending kindergarten or primary school (first or second year) at the time of first assessment and their families are eligible. CHIP-Family consists of a separate one-day workshop for parents and children. The child workshop consists of psychological exercises based on the evidence-based cognitive behavioral therapy Fun FRIENDS protocol and sports exercises. The parent workshop focuses on problem prevention therapy, psychoeducation, general parenting skills, skills specific to parenting a child with CHD, and medical issues. Approximately 4 weeks after the workshop, parents receive an individual follow-up session. The baseline (T1) and follow-up assessment (T2 = 6 months after T1) consist of online questionnaires filled out by the child, parents, and teacher (T2 only). Primary outcome measures are the CBCL for children and the SCL-90-R for parents. DISCUSSION: This trial aims to test the effects of an early family-centered psychosocial intervention to meet the compelling need of young children with CHD and their families to prevent (further) problems. If CHIP-Family proves to be effective, it should be structurally implemented in standard care. TRIAL REGISTRATION: Dutch Trial Registry; NTR6063 on 23 August, 2016.

Psychosocial needs of children undergoing an invasive procedure for a CHD and their parents.

OBJECTIVE: The aim of this study was to investigate the psychosocial needs of both parents of children with CHD (aged 0-18 years) and patients themselves (aged 8-18 years) in the week before cardiac surgery or a catheter intervention. Patients Eligible participants included all consecutive patients (0-18 years) scheduled to undergo cardiac surgery or a catheter intervention in our hospital between March, 2012 and July, 2013. Psychosocial needs were assessed using a disease-specific questionnaire designed for this study, consisting of a 83-item parent version and a 59-item child version (for children ⩾8 years), each covering five domains: physical/medical, emotional, social, educational/occupational, and health behaviour; two items assessed from whom and in what format psychosocial care was preferred. Quality of life was also assessed. Interventions If parents/patients reported a need for psychosocial care, referral to adequate mental health-care professionals was arranged. RESULTS: More than 40% of participating parents and >50% of participating children reported a need for psychosocial care on each of the five domains. Needs for psychosocial care for parents themselves were highest for those with children aged 0-12 years. Parents and patients report clear preferences when asked from whom and in what format they would like to receive psychosocial care. Quality of life was relatively high for both parents and patients. Psychosocial care interventions in our hospital increased significantly after the implementation of this study. CONCLUSIONS: Results show that psychosocial care is rated as (very) important by both parents and children during an extremely stressful period of their life.

Management and outcomes of obstructive sleep apnea in children with Robin sequence, a cross-sectional study.

OBJECTIVE: The objective of this cross-sectional study is to assess the prevalence, course, and management of obstructive sleep apnea (OSA) in children with Robin sequence (RS) aged 1-18 years. MATERIALS AND METHODS: A cross-sectional study was conducted in 63 children aged 1 to18 years with RS. Patient data were collected on baseline characteristics and management. OSA was evaluated by polysomnography. RESULTS: Sixty-three children with RS were included (median age 8.0 years) and divided into two groups based on the initial treatment: prone positioning or respiratory support. Respiratory support was more often indicated in children with a non-isolated RS (p < 0.05). At cross section, in the prone positioning group (n = 32), one child was diagnosed with OSA. In the respiratory support group (n = 31), 13 children (42 %) had respiratory problems of whom 10 needed respiratory support. CONCLUSIONS: Between the age of 1 and 18 years, almost one out of four children with RS still has respiratory problems. Children with RS, who can be treated with prone positioning only as an infant, are not likely to develop obstructive airway problems at a later age. In contrast, children who need respiratory support early after birth are at risk of continuing or re-developing OSA after the age of 1 year. CLINICAL RELEVANCE: This study shows that those who need respiratory support at an early age need careful monitoring until adulthood.

Association between nutritional status and subjective health status in chronically ill children attending special schools.

PURPOSE: In hospitalized children with a chronic disease, malnutrition was associated with a lower subjective health status. In outpatient children with a chronic disease attending special schools, this association has never been studied. The aim of this study was to assess the association between nutritional status and subjective health status in chronically ill children attending special schools. METHODS: Overall, 642 children, median age 9.8 years (IQR 7.7-11.5), 60 % male, 72 % Caucasian, were included in this prospective study in nine special schools for chronically ill children in the Netherlands. Overall malnutrition was assessed as: acute malnutrition (<-2 SDS for weight for height (WFH)) and chronic malnutrition (<-2 SDS for height for age). The malnutrition risk was assessed with the nutritional risk-screening tool STRONGkids. Subjective health status was assessed with EQ-5D. RESULTS: Overall, 16 % of the children had overall malnutrition: 3 % acute and 13 % chronic malnutrition. Nurses reported 'some/severe problems' on the health status dimensions mobility (15 %), self-care (17 %), usual activities (19 %), pain/discomfort (22 %), and anxiety/depression (22 %) in chronically ill children. Their mean visual analogue scale score (VAS) was 73.0 (SD 11.1). Malnutrition, medication usage, and younger age explained 38 % of the variance of the VAS score. CONCLUSIONS: The presence of overall malnutrition in chronically ill children attending special schools was associated with lower subjective health status, especially in younger children and in those with chronic medication usage. Therefore, it is important to develop and use profile-screening tools to identify these children.

Does functional health status predict health-related quality of life in children after Fontan operation?

PURPOSE: It is important to identify those children with a Fontan circulation who are at risk for impaired health-related quality of life. We aimed to determine the predictive value of functional health status - medical history and present medical status - on both physical and psychosocial domains of health-related quality of life, as reported by patients themselves and their parents. METHODS: We carried out a prospective cross-sectional multi-centre study in Fontan patients aged between 8 and 15, who had undergone staged completion of total cavopulmonary connection according to a current technique before the age of 7 years. Functional health status was assessed as medical history - that is, age at Fontan, type of Fontan, ventricular dominance, and number of cardiac surgical procedures - and present medical status - assessed with magnetic resonance imaging, exercise testing, and rhythm assessment. Health-related quality of life was assessed with The TNO/AZL Child Questionnaire Child Form and Parent Form. RESULTS: In multivariate prediction models, several medical history variables, such as more operations post-Fontan completion, lower age at Fontan completion, and dominant right ventricle, and present medical status variables, such as smaller end-diastolic volume, a higher score for ventilatory efficiency, and the presence of sinus node dysfunction, predicted worse outcomes on several parent-reported and self-reported physical as well as psychosocial health-related quality of life domains. CONCLUSIONS: Medical history and worse present medical status not only predicted worse physical parent-reported and self-reported health-related quality of life but also worse psychosocial health-related quality of life and subjective cognitive functioning. These findings will help in identifying patients who are at risk for developing impaired health-related quality of life.