Pediatric Global Health in Children with Very Early-Onset Inflammatory Bowel Disease.

OBJECTIVE: Children with very early-onset inflammatory bowel disease (VEO-IBD) represent a distinct group of patients with IBD with unique phenotypic and genetic characteristics; however, they are frequently omitted from psychosocial research. This study used a novel, brief measure of pediatric global health to assess (1) overall health-related quality of life (HRQOL) in children with VEO-IBD, (2) HRQOL compared to healthy children, and (3) whether gastrointestinal symptoms account for the differences in HRQOL between these groups. METHODS: Caregivers of 51 children with VEO-IBD (Mage = 4.26 years, 75% male) and 54 healthy children (Mage = 3.50 years, 54% male) completed the PROMIS Pediatric Global Health Scale (PGH-7) parent-proxy form to assess HRQOL and a questionnaire assessing gastrointestinal symptoms. Descriptive statistics, analysis of variance with covariates (ANCOVA), and meditation analyses with bootstrapping were conducted. RESULTS: Caregivers of children with VEO-IBD rated their HRQOL as relatively positive, although children with greater disease yielded lower ratings on some PGH-7 items (e.g., fun with friends, physical health, sadness). Compared to healthy youth, children with VEO-IBD scored lower on the PGH-7, with significantly lower item-level scores on overall health, physical health, mental health, and quality of life. Gastrointestinal symptoms mediated the association between health status (i.e., VEO-IBD vs. healthy) and HRQOL, αß = -2.84, 95% CI = -5.70, -0.34. CONCLUSIONS: While some children with VEO-IBD are at risk for deficits in HRQOL, many are quite resilient. Psychosocial screening is necessary for providing appropriate referrals to behavioral health services and learning more about psychosocial adjustment in children with VEO-IBD.

Health behaviours reported by adults with congenital heart disease across 15 countries.

BACKGROUND: Health behaviours are essential to maintain optimal health and reduce the risk of cardiovascular complications in adults with congenital heart disease. This study aimed to describe health behaviours in adults with congenital heart disease in 15 countries and to identify patient characteristics associated with optimal health behaviours in the international sample. DESIGN: This was a cross-sectional observational study. METHODS: Adults with congenital heart disease (n = 4028, median age = 32 years, interquartile range 25-42 years) completed self-report measures as part of the Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study (APPROACH-IS). Participants reported on seven health behaviours using the Health Behaviors Scale-Congenital Heart Disease. Demographic and medical characteristics were assessed via medical chart review and self-report. Multivariate path analyses with inverse sampling weights were used to investigate study aims. RESULTS: Health behaviour rates for the full sample were 10% binge drinking, 12% cigarette smoking, 6% recreational drug use, 72% annual dental visit, 69% twice daily tooth brushing, 27% daily dental flossing and 43% sport participation. Pairwise comparisons indicated that rates differed between countries. Rates of substance use behaviours were higher in younger, male participants. Optimal dental health behaviours were more common among older, female participants with higher educational attainment while sports participation was more frequent among participants who were younger, male, married, employed/students, with higher educational attainment, less complex anatomical defects and better functional status. CONCLUSIONS: Health behaviour rates vary by country. Predictors of health behaviours may reflect larger geographic trends. Our findings have implications for the development and implementation of programmes for the assessment and promotion of optimal health behaviours in adults with congenital heart disease.

Prevalence and Effects of Cigarette Smoking, Cannabis Consumption, and Co-use in Adults From 15 Countries With Congenital Heart Disease.

BACKGROUND: The prevalence and effects of cigarette smoking and cannabis use in persons with congenital heart disease (CHD) are poorly understood. We (1) described the prevalence of cigarette smoking, cannabis consumption, and co-use in adults with CHD; (2) investigated intercountry differences; (3) tested the relative effects on physical functioning, mental health, and quality of life (QOL); and (4) quantified the differential effect of cigarette smoking, cannabis use, or co-use on those outcomes. METHODS: APPROACH-IS was a cross-sectional study, including 4028 adults with CHD from 15 countries. Patients completed questionnaires to measure physical functioning, mental health, and QOL. Smoking status and cannabis use were assessed by means of the Health Behaviour Scale-Congenital Heart Disease. Linear models with doubly robust estimations were computed after groups were balanced with the use of propensity weighting. RESULTS: Overall, 14% of men and 11% of women smoked cigarettes only; 8% of men and 4% of women consumed cannabis only; and 4% of men and 1% of women used both substances. Large intercountry variations were observed, with Switzerland having the highest prevalence for smoking cigarettes (24% of men, 19% of women) and Canada the highest for cannabis use (19% of men, 4% of women). Cigarette smoking had a small negative effect on patient-reported outcomes, and the effect of cannabis was negligible. The effect of co-use was more prominent, with a moderate negative effect on mental health. CONCLUSIONS: We found significant intercountry variability in cigarette and cannabis use in adults with CHD. Co-use has the most detrimental effects on patient-reported outcomes.

Camp-based psychosocial intervention dosage and changes in independence in young people with spina bifida.

AIM: To examine associations between camp-based intervention dosage and changes in independence-related skills for young people with spina bifida. METHOD: Participants were 110 individuals (mean age [SD] 14y 7mo [6y 1mo], range 6-32y; 66 females, 54 males) who attended a summer camp for individuals with spina bifida between 2 to 6 times (mean 2.40; operationalized as 'dosage'). Parents of young campers (e.g. those <18y) also participated in data collection. Campers and/or parents completed preintervention measures assessing campers' level of medical responsibility, mastery over medical tasks, and social skills. Outcomes included change in preintervention scores from dose 1 to final dose. RESULTS: Hierarchical regression analyses with and without covariates (age, IQ, and lesion level at dose 1) revealed that increased dosage was significantly associated with greater parent-reported improvements in campers' medical responsibility and mastery over medical tasks. Increased dosage was also significantly associated with camper-report of increased medical responsibility, but this relationship was no longer significant when including covariates. Intervention dosage was not associated with changes in campers' social skills. INTERPRETATION: Repeated participation in a camp-based intervention was associated with improvements in condition-related independence. Future work may focus on the development of interventions to promote improvements in social skills for young people with spina bifida. WHAT THIS PAPER ADDS: Participating in an intervention over multiple summers is associated with increases in campers' responsibility for spina bifida-related tasks. Repeated summer camp intervention participation is associated with improved mastery over condition-related tasks for campers with spina bifida. Repeated camp intervention participation is not associated with changes in social skills for campers with spina bifida.

DOSIS DE INTERVENCIÓN PSICOSOCIAL EN CAMPAMENTOS RECREATIVOS Y CAMBIOS EN LA INDEPENDENCIA EN JÓVENES CON ESPINA BÍFIDA: OBJETIVO: Examinar las asociaciones entre la dosis de intervención recibidas en campamentos recreativos y los cambios en las habilidades relacionadas con la independencia de los jóvenes con espina bífida. MÉTODO: Los participantes fueron 110 individuos (edad media [DS] 14 años 7 meses[6y 1 mes], rango 6-32 años; 66 mujeres, 54 varones) que asistieron a un campamento de verano para personas con espina bífida, con una frecuencia entre 2 a 6 veces (media 2,40; considerada como 'dosificación' de la intervención). Los padres de los jóvenes que concurrieron a los campamentos (por ejemplo, aquellos con <18 años) también participaron en la recopilación de datos. Los participantes y / o los padres completaron las escalas previas a la intervención evaluando el nivel de responsabilidad sobre su salud de los participantes, el dominio sobre las tareas sanitarias y las habilidades sociales. Los resultados incluyeron cambios en las puntuaciones preintervención de la primera dosis a la última dosis. RESULTADOS: Los análisis de regresión jerárquica con y sin covariables (edad, coeficiente intelectual y nivel de lesión) revelaron que el aumento de la dosis se asoció significativamente con mayores mejoras informadas por los padres en la responsabilidad sobre su salud de los participantes y el dominio sobre las tareas sanitarias. El aumento de la dosis también se asoció significativamente con el informe de instructores reportando mayor responsabilidad sobre su propia salud, pero esta relación ya no fue significativa al incluir las covariables. La dosis de la intervención no se asoció con cambios en las habilidades sociales de los participantes INTERPRETACIÓN: La participación repetida en una intervención recibida en el campamento se asoció con mejoras en la independencia relacionada con la condición de salud. El trabajo futuro podría centrarse en el desarrollo de intervenciones para promover mejoras en las habilidades sociales de los jóvenes con espina bífida.

DOSAGEM DE INTERVENÇÃO PSICOSSOCIAL BASEADA EM ACAMPAMENTO E MUDANÇAS NA INDEPENDÊNCIA DE JOVENS COM ESPINHA BÍFIDA: OBJETIVO: Examinar associações entre dosagem de intervenção baseada em acampamento e mudanças nas habilidades relacionadas à independência para jovens com espinha bífida. MÉTODO: Participaram 110 indivíduos (idade média [desvio padrão] 14 anos e 7 meses [6 anos e 1 mês], 66 mulheres e 54 homens) que participaram do acampamento de verão para indivíduos com espinha bífida entre 2 e 6 vezes (média de 2,40; operacionalizado como `dosagem`). Os pais de jovens campistas (por exemplo, os <18 anos) também participaram da coleta de dados. Campistas e/ou pais completaram medidas de pré-intervenção avaliando o nível de responsabilidade médica dos campistas, domínio sobre tarefas médicas e habilidades sociais. Os resultados incluíram a mudança nos escores pré-intervenção da dose 1 para a dose final. RESULTADOS: Análises de regressão hierárquica com e sem covariáveis (idade, QI e nível de lesão na dosagem 1) revelaram que o aumento da dosagem foi significativamente associado a maiores melhorias relatadas pelos pais na responsabilidade médica dos campistas e no domínio de tarefas médicas. O aumento da dosagem também foi significativamente associado ao relato por parte do campista de maior responsabilidade médica, mas essa relação não era mais significativa quando se incluíam covariáveis. A dosagem de intervenção não foi associada a mudanças nas habilidades sociais dos campistas. INTERPRETAÇÃO: A participação repetida em uma intervenção baseada em acampamento foi associada a melhorias na independência relacionada à condição. Trabalho futuro pode se concentrar no desenvolvimento de intervenções para promover melhorias nas habilidades sociais para jovens com espinha bífida.

Perceived Health Mediates Effects of Physical Activity on Quality of Life in Patients With a Fontan Circulation.

Patients with a Fontan circulation are at risk of a sedentary lifestyle. Given the direct relationship between physical activity and health, promotion of physical activity has the potential to improve outcomes, including quality of life (QOL). This study aimed to describe self-reported physical activity levels in adult Fontan patients and examine associations between physical activity, perceived health status and QOL. The sample consisted of 177 Fontan patients (Mage = 27.5 ± 7.6 years, 52% male) who reported their physical activity, perceived health status, and QOL as part of the cross-sectional Assessment of Patterns of Patient-Reported Outcomes in Adults with Congenital Heart disease - International Study. Descriptive statistics and univariate analyses of variance with planned contrasts were computed to describe physical activity characteristics. Mediation analyses tested whether perceived health status variables mediated the association between physical activity and QOL. Forty-six percent of patients were sedentary while only 40% met international physical activity guidelines. Higher physical activity was associated with younger age, lower NYHA class, higher perceived general health, and greater QOL. Patients who commuted by walking and engaged in sports reported better perceived health and QOL. Mediation analyses revealed that perceived general health but not NYHA functional class mediated the association between physical activity and QOL (αß = 0.22, 95% confidence interval = 0.04 to 0.49). In conclusion, Fontan patients likely benefit from regular physical activity, having both higher perceived general health and functional capacity; greater perceived health status may contribute to enhanced QOL. In conclusion, these data support the pivotal role of regular physical activity for Fontan patients.

Allocation of Treatment Responsibility in Adolescents With Epilepsy: Associations With Cognitive Skills and Medication Adherence.

Objectives: To describe allocation of treatment responsibility (ATR) in adolescents with epilepsy, investigate associations between cognitive skills and ATR, and examine whether ATR for antiepileptic drugs (AEDs) predicted electronically monitored adherence. Method: Sixty adolescents with epilepsy and their caregivers completed the Allocation of Treatment Responsibility Scale and a battery of self-report measures. Medical chart review data and electronically monitored AED adherence were collected for 1 year. Descriptive data assessed ATR for caregivers and adolescents; multivariate hierarchical regressions tested associations between variables. Results: ATR for labs and clinic appointments was greatest for caregivers, while ATR for AEDs was more likely to be shared between caregiver and adolescent. Poorer attention was associated with greater caregiver responsibility for AEDs. Greater caregiver responsibility for AEDs was associated with higher electronically monitored adherence over 12 months. Conclusions: In adolescents with epilepsy, caregivers are responsible for most treatment tasks, although responsibility for taking medication was shared with the adolescent. Greater caregiver responsibility for medication results in better long-term AED adherence. ATR is an important construct that warrants further attention in research and clinical practice, especially in the context of transition and health outcomes in pediatric epilepsy.

Examination of the uses, needs, and preferences for health technology use in adolescents with asthma.

Objective: To examine the health technology uses and preferences of adolescents with asthma using a qualitative descriptive individual interview approach. Methods: Twenty adolescents were recruited from regularly scheduled asthma clinic appointments from February to July 2016. Patients were interviewed about their technology use and ways in which health technology could improve their asthma management using an open-ended semi-structured interview format. Interviews were audio recorded, transcribed, and coded into themes. Results: Social media (e.g. Snapchat, Instagram) and general communication (e.g. messaging) were the most common uses of technology while medical reminders (e.g. appointment, refill, medication) were the most common use of health technology. Adolescents identified ways in which health technology could improve their asthma management including (1) tracking symptoms and medication, (2) medical reminders, and (3) asthma and self-management knowledge specifically related to medications and individual action plans. Other themes that emerged included a desire to customize health technology to fit with individual schedules and medical routines and use of health technology data with medical providers. Conclusions: Adolescents and parents experience a number of challenges related to managing asthma, and health technology interventions should focus on ways to improve adherence and self-management. Future research considerations and potential interventions including ways to integrate adolescent preferences with evidence-based interventions are discussed.

A pilot investigation of a mobile phone application and progressive reminder system to improve adherence to daily prevention treatment in adolescents and young adults with migraine.

BACKGROUND: Effective management of migraine requires adherence to treatment recommendations; however, adolescents with migraine take their daily medications only 75% of the time. Low-cost adherence-focused interventions using technology may improve adherence, but have not been investigated. METHODS: Thirty-five adolescents and young adults (13-21 years) with migraine participated in an AB-design pilot study to assess the use of a mobile phone adherence-promotion application ("app") and progressive reminder system. Adherence was calculated using electronic monitoring during the baseline period and medication adherence intervention. RESULTS: Relative to baseline, adherence significantly improved during the first month of the intervention. Specifically, improvements existed for older participants with lower baseline adherence. Self-reported app-based adherence rates were significantly lower than electronically monitored adherence rates. Participants rated the intervention as acceptable and easy to use. CONCLUSIONS: "Apps" have the potential to improve medication adherence and are a promising intervention for adolescents and young adults with low adherence. Involving parents in the intervention is also helpful. Providers should assess barriers to adherence and use of technology-based interventions, encourage parents to incorporate behavioral incentives, and provide referrals for more intensive interventions to improve long-term outcomes. Further, tracking adherence in an app may result in an underestimation of adherence. Future full-scale studies should be conducted to examine adherence promotion app interventions.

A multinational observational investigation of illness perceptions and quality of life among patients with a Fontan circulation.

OBJECTIVE: First, to compare QOL and illness perceptions between patients with a Fontan circulation and patients with anatomically simple defects (ie, atrial septal defects [ASD] or ventricular septal defects [VSD]). Second, to explore illness perceptions as a mediator of the association between congenital heart disease (CHD) diagnosis and QOL. DESIGN: Cross-sectional observational study. SETTING: Twenty-four cardiology centers from 15 countries across five continents. PATIENTS: Four hundred thirty-five adult patients with congenital heart disease (177 Fontan and 258 ASD/VSD) ages 18-83 years. OUTCOME MEASURES: QOL and illness perceptions were assessed by the Satisfaction With Life Scale and the Brief Illness Perceptions Questionnaire, respectively. RESULTS: Patients with a Fontan circulation reported lower QOL (Wald Z = -3.59, p = <.001) and more negative perceptions of their CHD (Wald Z = -7.66, p < .001) compared with patients with ASD/VSD. After controlling for demographics, anxiety, depressive symptoms, and New York Heart Association functional class, path analyses revealed a significant mediation model, αß = 0.15, p = .002, 95% CI = 0.06-0.25, such that CHD diagnosis was indirectly related to QOL through illness perceptions. CONCLUSIONS: The Fontan sample's more negative perceptions of CHD were likely a reflection of life with a more complex defect. Illness perceptions appear to account for unique differences in QOL between groups of varying CHD complexity. Psychosocial screening and interventions may be important treatment components for patients with CHD, particularly those with Fontan circulations.

Topical Review: Adherence Interventions for Youth on Gluten-Free Diets.

Objective: To summarize gluten-free diet (GFD) nonadherence risk factors, nonadherence rates, and current intervention research within an integrative framework and to develop a research agenda for the development and implementation of evidence-based GFD adherence interventions. Methods: Topical review of literature published since 2008 investigating GFD adherence in pediatric samples. Results: Reviews of pediatric studies indicate GFD nonadherence rates ranging from 19 to 56%. There are few evidence-based, published pediatric GFD adherence interventions. Novel assessments of GFD adherence are promising but require further study. Nonmodifiable and modifiable factors within individual, family, community, and health systems domains must be considered when developing future interventions. Clinical implications are discussed. Conclusions: Avenues for future research include development and refinement of adherence assessment tools and development of evidence-based GFD adherence interventions. Novel technologies (e.g., GFD mobile applications) require empirical study but present exciting opportunities for adherence intervention.

Social Skills in Youth With Spina Bifida: A Longitudinal Multimethod Investigation Comparing Biopsychosocial Predictors.

Objective: To examine the relative contributions of neuropsychological (attention and executive function), family (cohesion and conflict), and health (body mass index, lesion level, gross motor function) domains on social skills over time in youth with spina bifida (SB). Methods: In all, 140 youth with SB (T1 mean age = 11.43 years) and their families participated in the study at baseline with an additional visit 2 years later. Study variables were assessed with multiple methods (questionnaire, medical chart review, observation, neuropsychological tests) and reporters (parents, teachers). Multivariate hierarchical linear regressions determined the predictive power of the three domains for T2 social skills. Results: Neuropsychological variables accounted for significant variance in mother- and father-reported T2 social skills. Neither family nor health variables contributed significantly to later social skills when other domains were included in the model. Conclusions: Neuropsychological factors are particularly important for social skill development in youth with SB. Findings can inform screening and intervention practices.

Milestone achievement in emerging adulthood in spina bifida: a longitudinal investigation of parental expectations.

AIM: To assess changes over time in parents' expectations of adult milestone achievement (college attendance, full-time job attainment, independent living, marriage, parenthood) for young people with spina bifida, to examine how expectancies relate to actual milestone achievement, and to compare milestone achievement in emerging adults with spina bifida with that of peers with typical development. METHOD: Sixty-eight families of children with spina bifida (mean age 8y 4mo, 37 males, 31 females) and 68 families of children with typical development (mean age 8y 6mo, 37 males, 31 females) participated at Time 1. At all subsequent timepoints, parents of young people with spina bifida were asked to rate their expectations of emerging adulthood milestone achievement. At Time 7, when participants were 22 to 23 years old, milestone achievement was assessed. RESULTS: Parents of young people with spina bifida lowered their expectations over time for most milestones; parents of children with higher cognitive ability reported decreases of lower magnitude. Parent expectancies were optimistic and unrelated to actual milestone achievement. Emerging adults with spina bifida were less likely than individuals with typical development to achieve all milestones. INTERPRETATION: Optimistic parental expectations may be adaptive for children with spina bifida and their families, although it is important for families to set realistic goals. Healthcare providers serve a key role in helping families of young people with spina bifida prepare for emerging adulthood.

Observed differences in social behaviors exhibited in peer interactions between youth with spina bifida and their peers: neuropsychological correlates.

OBJECTIVE: To identify differences in social behaviors in observed peer interactions between children with spina bifida (SB) and peers, and to examine neuropsychological correlates of these differences. METHOD: A total of 100 youth (aged 8-15 years) with SB and peers participated in video-recorded interaction tasks, which were coded for interaction style, affect, and collaboration. Children with SB also completed a neuropsychological test battery. RESULTS: Children with SB demonstrated less adaptive social behaviors in peer interactions, particularly within the interaction style domain. Observational items found to be different between children with SB and their peers were best predicted by social language and attention abilities. CONCLUSIONS: Children with SB exhibit a less adaptive interaction style and lower levels of social dominance but are comparable with typically developing peers on other social behaviors. The observed group differences may have a neuropsychological basis.

Development and validation of the Peer Interaction Macro-Coding System Scales (PIMS): a new tool for observational measurement of social competence in youth with spina bifida.

Many children with chronic health conditions encounter enduring difficulties in their peer interactions and friendships. This study aimed to create and validate scales derived from an observational coding system (i.e., Peer Interaction Macro-Coding System, or PIMS) in a sample of children with spina bifida and their peers. Participants were 106 target child-peer dyads who completed a battery of questionnaires and interviews and were videotaped performing 4 interaction tasks, which were then coded across multiple domains of social functioning. Five scales (i.e., Control, Prosocial Skills, Positive Affect, Conflict, and Dyadic Cohesion) were rationally derived based on a review of the literature and a panel of experts. Internal consistency and interrater reliability at the scale level were good to excellent for all 5 scales. Interscale correlations were in the low-to-moderate range for 4 of the scales, although the Dyadic Cohesion Scale was highly correlated with two other scales and was subsequently dropped. Convergent validity and discriminant validity were established by examining the 4 remaining scales in association with comparable questionnaire and interview data. The 4 PIMS scales appear to be reliable and valid measures of social competence and may enhance future multimethod research efforts aimed at learning more about peer interactions and overall social competence.

A camp-based psychosocialiIntervention to promote independence and social function in individuals with spina bifida: moderators of treatment effectiveness.

PURPOSE: To replicate and extend O'Mahar and colleagues' (O'Mahar, K., Holmbeck, G. N., Jandasek, B., & Zuckerman, J. [2010]. A camp-based intervention targeting independence among individuals with spina bifida. Journal of Pediatric Psychology, 35, 848-856) findings in a new and larger sample of youth and young adults with spina bifida who participated in a modified camp-based intervention targeting independence and social skills. Moderators of intervention effectiveness and clinical significance were examined. METHOD: In all, 119 campers aged 7-41 years participated in an intervention that included goal setting and interactive workshops. Campers and parents completed measures of campers' goal attainment, independence, and social functioning at preintervention and postintervention; counselors reported on campers' goal attainment daily throughout the intervention. RESULTS: Parents and campers reported improvements in campers' goal attainment, management of health-related self-care, and independence. Although benefits were found for most campers, cognitive functioning and family income moderated some outcomes. Campers who improved most on their social goals perceived the intervention to be more effective. CONCLUSIONS: Further support is provided for the effectiveness of a camp-based intervention targeting independence and social skills for individuals with spina bifida. More attention should be directed toward those with cognitive difficulties and low-income backgrounds.

Individual adjustment, parental functioning, and perceived social support in Hispanic and non-Hispanic white mothers and fathers of children with spina bifida.

OBJECTIVE: To compare Hispanic and non-Hispanic White mothers and fathers of children with spina bifida on measures of individual adjustment, parental functioning, and perceived social support. METHOD: Mothers (29 Hispanic, 79 non-Hispanic white) and fathers (26 Hispanic, 68 non-Hispanic white) completed questionnaires regarding psychological distress, parental functioning, and perceived social support. RESULTS: Mothers and fathers reported similar individual adjustment across groups. Hispanic mothers reported lower levels of parenting satisfaction, competence as a parent, and social support, as well as higher perceptions of child vulnerability. Hispanic fathers reported lower levels of parenting satisfaction and higher perceptions of child vulnerability. Effect sizes were reduced when socioeconomic status was included as a covariate. CONCLUSIONS: Hispanic parents, particularly mothers, are at risk for lower feelings of satisfaction and competence as parents. More research is needed to understand cultural factors related to these differences.