An Exercise Program in Youngsters with Complex Congenital Heart Disease: does it improve Health Related Quality of Life and Psychosocial Functioning? A randomized controlled trial

__Abstract__ A 12-week standardized exercise program improved self-reported cognitive functioning, and parent-reported social functioning in children and adolescents with complex congenital heart disease. Furthermore, it resulted in a decrease in passive leisure time spending. In contrast, it had no effect on emotional and behavioural problems, on sports enjoyment, or on active leisure time spending. Parental mental health moderated changes in social functioning: adolescents in the exercise group whose parents’ overall mental health was worse, reported a pre-post decrease in social functioning, compared with controls

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 &amp; services,” “Patient empowerment,” “Continuity &amp; 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.</p

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

Netherton syndrome; neuropsychological and psychosocial functioning of child and adult patients and their parents

Background: Netherton syndrome is a rare severe skin disease. Clinical experience showed considerable psychosocial burdens among Netherton syndrome patients/families. Their (neuro)psychological functioning has never been investigated. Objective: To investigate neuropsychological/psychosocial functioning of Netherton syndrome patients and parents. Methods: A total of 12 Netherton syndrome patients and/or parents completed neuropsychological tests, semi-structured-interviews, and psychological-questionnaires. Results: Intelligence results showed disharmonic profiles, with below-average scores on processing speed. Neuropsychological problems and unfavorable outcomes on health-related quality of life, illness-appearance-related problems, and negative social consequences among patients/parents were found. Psychopathological (emotional) problems were reported; stigmatization, bullying was common among Netherton syndrome patients. Conclusion: Compared with normative data, Netherton syndrome patients showed neuropsychological and psychosocial problems. Standard follow-up is necessary to identify problems at early stage

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.</p

Health-related quality of life of children and their parents 2 years after critical illness

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, childre

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

Predictors of subjective health status 10 years post-PCI

Background: Subjective health status is an increasingly important parameter to assess the effect of percutaneous coronary intervention (PCI) in clinical practice.Aim of this study was to determine medical and psychosocial predictors of poor subjective health status over a 10 years' post-PCI period. Methods: We included a series of consecutive PCI patients (n = 573) as part of the RESEARCH registry, a Dutch single-center retrospective cohort study. Results: These patients completed the 36-item Short-Form Health Survey (SF-36) at baseline and 10 years post-PCI. We found 6 predictors of poor subjective health status 10 years post-PCI: SF-36 at baseline, age, previous PCI, obesity, acute myocardial infarction as indication for PCI, and diabetes mellitus (arranged from most to least numbers of sub domains). Conclusions: SF-36 scores at baseline, age, and previous PCI were significant predictors of subjective health status 10 years post-PCI. Specifically, the SF-36 score at baseline was an important predictor. Thus assessment of subjective health status at baseline is useful as an indicator to predict long-term subjective health status. Subjective health status becomes better by optimal medical treatment, cardiac rehabilitation and psychosocial support. This is the first study determining predictors of subjective health status 10 years post-PCI

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 &lt; 0.0001), completed the timed up-and-go test more slowly (p &lt; 0.0001), walked a shorter distance in 6 min (p &lt; 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.</p