Weight status and health behaviors of adolescents and young adults with sickle cell disease: The emerging risk for obesity.

Historically, youth with sickle cell disease (SCD) were at risk for being underweight, but recent data suggests this population is replicating obesity trends of youth in the United States. The current observational study assessed the weight status and health behaviors of 44 adolescents and young adults with SCD via a self-report survey and chart review. Using height and weight data closest to survey completion date, 27% of participants were either overweight or obese. With respect to obesogenic risk behaviors, 77% ate fast food 1-3 times per week, 25% had no fruits/vegetables with any of their meals, 11% drank no water, and 57% watched 4 or more hours of television per day. Though more research is needed, this preliminary study adds to the SCD literature suggesting an emerging shift toward obesity in this population. As such, adolescents with SCD may benefit from interventions to decrease obesity risk factors as being overweight or obese has the potential to worsen SCD-related symptoms and complications.

A Description of Children Dependent on Long Term Ventilation via Tracheostomy and Their Hospital Resource Use.

PURPOSE: To describe the proportion of children with an index hospitalization in 2014 who had established long-term invasive ventilator dependence (LTVD), and determine regional variation in hospital length of stay, charges, and readmissions. DESIGN AND METHODS: Multicenter, longitudinal, retrospective cohort study using a recently established algorithm to identify children with LTVD from the Pediatric Health Information System database with an index hospitalization at least once during 2014, excluding normal newborn care or chemotherapy, and the subset with established LTVD. Hospitals were grouped by geographic regions. Analysis included descriptive statistics and multi-variable mixed modeling for length of stay, charges, and readmissions. RESULTS: Of the 615,883 unique children discharged from 45 children's hospitals in 2014, 2235 (0.4%) had established LTVD. Of these, 342 (15%) were hospitalized in the Northeast, 677 (30%) Midwest, 733 (32%) South and 481 (22%) West. Most had at least two complex chronic conditions (97%) and used a medical device for at least two body systems (71%). No statistically significant regional variation was found for length of stay, charges, or readmissions after adjustment for child demographics, admission type, disposition, primary diagnosis, ICU stay, and number of chronic conditions. CONCLUSIONS: This study characterized the population of children with LTVD hospitalized in 2014. No regional variation was found for length of stay, charges, or readmissions. PRACTICE IMPLICATIONS: Children with established LTVD make up a small subset of all children admitted to children's hospitals however, they require substantial, costly, multifaceted care as most have additional complex chronic conditions and require multiple medical devices.

Facilitators and Barriers to Implementing Standardized Outcome Measurement for Children With Cleft Lip and Palate.

OBJECTIVE: Identify facilitators and barriers to implementing standardized outcome measurement in cleft care. DESIGN: Cross-sectional, qualitative study. SETTING/PARTICIPANTS: Participants included 24 providers and staff from a large, multidisciplinary cleft team in the southwest United States, 5 caregivers of children with cleft palate (with or without cleft lip) treated by this team, and 3 experts involved in implementing a cleft-specific standardized outcome measurement in the United Kingdom. INTERVENTIONS: Semistructured, qualitative interviews were conducted exploring perceived facilitators and barriers to implementing standardized outcome measurement in cleft care. Interviews were audio-recorded, transcribed, and analyzed for content. The Consolidated Framework for Implementation Research was used to guide the interviews and analysis. The analysis focused on the characteristics of standardized outcome measurement that directly influence its adoption. RESULTS: Participants identified both facilitators and barriers to implementing standardized outcome measurement. Facilitators included the strength and quality of evidence supporting improvements in cleft care delivery following implementation of standardized outcome measurement and the relative advantage of standardized outcome measurement over continuing the status quo. Barriers included the difficulty adapting standardized outcome measurement to meet local context and patient-specific needs and the complexity of implementing standardized outcome measurement. CONCLUSIONS: Providers, staff, and caregivers involved in cleft care perceive multiple benefits from standardized outcome measurement, while also recognizing substantial barriers to its implementation. Results from this study can be used to guide development of an implementation strategy for standardized outcome measurement that builds upon perceived strengths of the intervention and reduces perceived barriers.

Improving self-management in adolescents with sickle cell disease.

BACKGROUND: Sickle cell disease (SCD) is associated with significant medical challenges that often worsen in adolescence when caregivers are beginning to transfer responsibility for disease management. Behavioral activation (BA) is an important precedent to improvements in self-management and ultimately health outcomes; however, few interventions targeting BA have been developed for the SCD population. The goal of the present study was to evaluate a technology-enhanced self-management intervention for adolescents and young adults (AYA) with SCD targeting BA domains (ie, disease knowledge, self-efficacy, motivation, and self-management skills). DESIGN/METHODS: Participants were randomized to one of two study arms. SCThrive participants (N = 26) completed six weekly group sessions, an in-person booster session, and used a companion app (iManage) to record symptoms, progress on goals, and connect with other group members. Each SCHealthEd participant (N = 27) received six weekly phone calls on SCD-related and general health education topics. All AYA completed questionnaires assessing BA at baseline and posttreatment. RESULTS: Separate mixed ANOVA analyses to assess for the effects of group (SCThrive/SCHealthEd), time (baseline/posttreatment), and group × time interaction indicated that there was a clinically meaningful improvement (8-point change) in self-efficacy, with a medium effect size, P = .09, η2  = .06, and there was statistically significant improvement in one self-management skill (tracking health), P = .001, d = .71, among SCThrive participants. CONCLUSIONS: The results support the potential for a self-management intervention to improve self-efficacy in AYA with SCD. Health care providers are encouraged to target BA skills to support self-management of AYA with SCD.

Marked Variation Exists Among Surgeons and Hospitals in the Use of Secondary Cleft Lip Surgery.

OBJECTIVE: To identify child-, surgeon-, and hospital-specific factors at the time of primary cleft lip repair that are associated with the use of secondary cleft lip surgery. DESIGN: Retrospective cohort study. SETTING: Forty-nine pediatric hospitals. PARTICIPANTS: Children who underwent cleft lip repair between 1999 and 2015. MAIN OUTCOME MEASURE: Time from primary cleft lip repair to secondary lip surgery. RESULTS: By 5 years after primary lip repair, 24.0% of children had undergone a secondary lip surgery. In multivariable analysis, primary lip repair before 3 months had a 1.22-fold increased hazard of secondary surgery (95% confidence interval [CI]: 1.02-1.46) compared to repair at 7 to 12 months of age, and children with multiple congenital anomalies had a 0.77-fold decreased hazard of secondary surgery (95% CI: 0.68-0.87). After adjusting for cleft type, age at repair, presence of multiple congenital anomalies, and procedure volume, there remained substantial variation in secondary surgery use among surgeons and hospitals (P < .01). For children with unilateral cleft lip repaired at 3 to 6 months of age, the predicted proportion of children undergoing secondary surgery within 5 years of primary repair ranged from 4.9% to 21.8% across surgeons and from 4.5% to 24.7% across hospitals. CONCLUSIONS: There are substantial differences among surgeons and hospitals in the rates of secondary lip surgery. Further work is needed to identify causes for this variation among providers.

Acceptability and Feasibility of a Disease-specific Patient Portal in Adolescents With Sickle Cell Disease.

OBJECTIVE: To examine the acceptability, feasibility, and efficacy of a health care portal. OBSERVATIONS: Adolescents and young adults with sickle cell disease were taught how to use sickle cell disease conditions page in MyChart and completed questionnaires at baseline, postintervention (T2, 6 wk after baseline) and after 3 months (T3). In total, 44 participants (M age=18.82, SD=2.72) viewed an average of 58.07 pages from T1 to T2. The portal was highly accepted (90.32%). Efficacy data indicated that portal use was associated with improved patient-provider communication. CONCLUSIONS: Electronic portals are promising tools for improving medical self-management.

Five-Fold Variation Among Surgeons and Hospitals in the Use of Secondary Palate Surgery.

OBJECTIVE: To identify child-, surgeon- and hospital-specific factors at the time of primary cleft palate repair that are associated with the use of secondary palate surgery. DESIGN: Retrospective cohort study. SETTING: Forty-nine pediatric hospitals. PARTICIPANTS: Children who underwent cleft palate repair between 1998 and 2015. MAIN OUTCOME MEASURE: Time from primary cleft palate repair to secondary palate surgery. RESULTS: By 5 years after the primary palate repair, 27.5% of children had undergone secondary palate surgery. In multivariable analysis, cleft type and age at primary palate repair were both associated with secondary surgery ( P < .01). Children with unilateral cleft lip and palate had a 1.69-fold increased hazard of secondary surgery (95% confidence interval [CI]: 1.54-1.85) compared to children with cleft palate alone. Primary palate repair before 9 months had a 3.99-fold increased hazard of secondary surgery (95% CI: 3.39-4.07) compared to repair at 16 to 24 months of age. After adjusting for cleft type, age at repair, and procedure volume, there remained substantial variation in secondary surgery use among surgeons and hospitals ( P < .01). For children with isolated cleft palate, the predicted proportion of children undergoing secondary surgery within 5 years of primary repair ranged from 8.5% to 46.0% across surgeons and 9.1% to 49.4% across hospitals. CONCLUSIONS: There are substantial differences among surgeons and hospitals in the rates of secondary palate surgery. Further work is needed to identify causes for this variation among providers and develop interventions to reduce the need for secondary surgery.

Systemwide Implementation of Patient-Reported Outcomes in Routine Clinical Care at a Children's Hospital.

BACKGROUND: Despite a growing literature on patient-reported outcomes (PROs), little has been written to guide development of a standardized, systemwide PRO program across multiple clinics and conditions. A PRO implementation program, which was created at Cincinnati Children's Hospital Medical Center, a large children's hospital, can serve as a standardized approach for the use of PROs in a clinical setting. METHODS: Recommended standardized PRO implementation components include identification of a committed clinical leader and team, selection of an instrument that addresses the identified outcome of interest, specifying threshold scores that indicate when an intervention is needed, identification of clinical interventions to be triggered by threshold scores, provision of training for providers and staff involved in the PRO implementation process, and the measurement and monitoring of PRO use. RESULTS: For each instrument, the completion goal is 80%, defined as the number of PRO measures that were actually completed divided by the number that should have been completed. The overall combined completion rate is 75% for the 68 unique instruments currently in use. Case studies of specific clinical team experiences demonstrate the value of using PROs and the implementation components and shows how PROs are used to promote patient-centered care. CONCLUSION: Data on PRO implementation are collected on an ongoing basis to confirm the value of the program, define ongoing improvement, and fuel collaborative research to further refine essential implementation components and successful spread. Next steps include measuring the influence of PRO use on coproduction of patient-centered clinical care and the impact PRO measurement has on patient outcomes.

Development and evaluation of iManage: A self-management app co-designed by adolescents with sickle cell disease.

BACKGROUND: Adolescents and young adults (AYAs) with sickle cell disease (SCD) are a vulnerable population with high risk of morbidity that could be decreased with effective self-management. Previous research suggests that mobile applications (apps) may facilitate AYA engagement in health-promoting behaviors. The objectives of this study were: (i) describe Internet access and use in AYA with SCD; (ii) identify barriers for self-management in this population; (iii) collaborate with AYA to co-design a mobile app that would minimize barriers; and (iv) evaluate the feasibility and acceptability of the app. PROCEDURE: In phase 1, 46 AYAs with SCD 16-24 years of age completed a survey of Internet access and use. During phase 2, 19 AYAs with SCD (average age 20 ± 2.5 years) and eight healthcare providers participated in interviews to identify barriers and co-design sessions to develop the app. In phase 3, five AYAs with SCD completed app feasibility and usability testing. RESULTS: AYAs with SCD had daily Internet access (69%) using their computers (84%) or mobile phones (70%). Participants went online for health information (71%) and preferred Web sites with interactive/social features (83%). Barriers to self-management included failing to believe that their health would suffer, lack of tailored self-management support, lack of a mechanism to visualize self-management progress, and limited opportunities for peer interaction around self-management. The prototype app (iManage) was rated as highly feasible and beneficial. CONCLUSIONS: A mobile app prototype co-designed by AYAs with SCD may be a useful tool for engaging them in self-management strategies designed to improve health.

Physicians' Perceptions of Shared Decision Making in Chronic Disease and Its Barriers and Facilitators.

This study assessed pediatric physicians' use of shared decision making (SDM) in 2 chronic conditions. Most physicians indicated that parent and adolescent trust and emotional readiness facilitated SDM, physicians' preferred approach to decision making. At the same time, they perceived few barriers, other than insurance limitations, to using SDM.

High Levels of Decisional Conflict and Decision Regret When Making Decisions About Biologics.

OBJECTIVES: The aim of the study was to understand the association between parents' perceptions of the decision process and the decision outcomes in decisions about the use of biologics in pediatric chronic conditions. METHODS: We mailed surveys to parents of children with inflammatory bowel disease or juvenile idiopathic arthritis who had started treatment with biologics in the prior 2 years and were treated at either of 2 children's hospitals. The survey included measures of the decision process, including decision control and physician engagement, and decision outcomes, including conflict and regret. We used means and frequencies to assess the response distributions. General linear models were used to test the associations between decision process and decision outcomes. RESULTS: We had 201 respondents (response rate 54.9%). Approximately 47.0% reported using shared decision making. Each physician engagement behavior was experienced by the majority of parents, with the highest percentage reporting that their child's physician used language they understood and listened to them. Approximately 48.5% of parents had decisional conflict scores of 25 or greater, indicating high levels of conflict. Approximately 28.2% had no regret, 31.8% had mild regret, and the remaining 40.0% had moderate to severe regret. Shared decision making was not associated with improved decisional conflict, but physician engagement behaviors were associated with both decisional conflict and regret. CONCLUSIONS: Improving decision outcomes will require more than just focusing on who parents perceive as controlling the final decision. Developing interventions that facilitate specific physician engagement behaviors may decrease parents' distress around decision making and improve decision outcomes.

Making decisions about chronic disease treatment: a comparison of parents and their adolescent children.

OBJECTIVE: To compare factors considered by parents to those considered by adolescents making decisions about chronic disease treatments. METHODS: We conducted individual interviews with 15 parent-adolescent dyads in which the adolescent had either juvenile idiopathic arthritis or Crohn's disease. Questions focused on treatment decisions, with an emphasis on the factors that influenced each individual's preferences related to biologic therapies. A multidisciplinary team developed a coding structure. All interviews were coded by two people with disagreements resolved through discussion. We used content analysis and coding matrices to examine decision factors within and between parent-adolescent dyads. RESULTS: Parents and adolescents both participated in decisions about treatment with biologic therapies but considered decision factors differently. In only half of cases did parents and adolescents agree on the factor that most influenced their decision. Although their decision factors often fell into similar categories (e.g. treatment risks, quality of life), in many cases the specifics varied between adolescents and their parents. Adolescents were more likely to focus on immediate treatment effects and quality of life while parents took a longer term view of the decision. Agreement within dyads was most consistent when a special circumstance influenced the treatment decision. CONCLUSIONS: Differences regarding influential decision factors exist within parent-adolescent dyads. Continued research is needed to determine the extent to which such differences are due to individual preferences or to variations in the information available to each person. Future decision support interventions will need to address parents' and adolescents' potentially disparate views and information needs.

Shared Decision Making in the Care of Children with Developmental and Behavioral Disorders.

OBJECTIVE: Shared decision making (SDM) is most needed when there are multiple treatment options and no "right" choice. As with quality and experience of care, frequency of SDM may vary by health condition. The objectives of this study were (1) to compare parent report of SDM between a physical and a behavioral health condition and; (2) to compare parent report of SDM between two different behavioral health conditions. METHODS: Data on children age 3-17 years with asthma, attention deficit/hyperactivity disorder (ADHD), and/or autism spectrum disorder (ASD) were drawn from the 2009/10 National Survey of Children with Special Health Care Needs. Weighted logistic regression was used to compare a parent-reported, composite measure of SDM. Analyses controlled for sociodemographic factors that may influence experience of SDM. RESULTS: Compared to parents of children with asthma, parents of children with ADHD were significantly less likely to report experiencing consistent SDM (AOR 0.73). Compared to parents of children with ADHD, those of children with ASD had significantly lower odds of experiencing consistent SDM (AOR 0.59). Those with both ADHD and ASD had the same odds as those with ASD alone of experiencing consistent SDM. CONCLUSION: Use of SDM is particularly limited in developmental and behavioral conditions, such as ADHD and ASD. These data suggest that challenges to implementing SDM may include disease type, complexity, and use of specialty care. Research to identify specific barriers and facilitators of SDM is needed to inform interventions that will promote SDM in developmental and behavioral conditions.

The Burden of Care for Children With Unilateral Cleft Lip: A Systematic Review of Revision Surgery.

OBJECTIVE: To identify the average rate of revision surgery following cleft lip repair. DESIGN: PubMed, CINAHL, and SCOPUS were searched from database inception through March 2013 using the search terms cleft lip and surgery. Two investigators independently screened all abstracts and determined eligibility from review of full manuscripts using prespecified inclusion and exclusion criteria. Strengths and limitation of the studies were assessed, followed by qualitative synthesis. The I(2) test of homogeneity was performed to determine if meta-analysis was appropriate. RESULTS: The search identified 3034 articles. Of those, 45 met the inclusion criteria. Studies were primarily case series and retrospective cohort studies, with only one randomized controlled trial. One-third of studies (n = 15) did not describe how the study sample was selected. Follow-up duration was not reported in one-fourth of studies (n = 11). Nasolabial aesthetics were reported in 44% of studies (n = 20). The incidence of revision surgery ranged from 0% to 100%. Meta-analysis was precluded because of study heterogeneity (I(2) = 97%). CONCLUSIONS: The average incidence of cleft lip revision surgery cannot be estimated from the published literature, due to significant heterogeneity among existing reports and limited study quality. To provide valid information about the burden of care for unilateral cleft lip, a population-based or multicenter longitudinal cohort study is necessary; this study should measure the number of surgical procedures and the patient's aesthetic outcomes.

Real life clinic visits do not match the ideals of shared decision making.

OBJECTIVE: To use observation to understand how decisions about higher-risk treatments, such as biologics, are made in pediatric chronic conditions. METHODS: Gastroenterology and rheumatology providers who prescribe biologics were recruited. Families were recruited when they had an outpatient appointment in which treatment with biologics was likely to be discussed. Consent/assent was obtained to video the visit. Audio of the visits in which a discussion of biologics took place were transcribed and analyzed. Our coding structure was based on prior research, shared decision making (SDM) concepts, and the initial recorded visits. Coded data were analyzed using content analysis and comparison with an existing model of SDM. RESULTS: We recorded 21 visits that included discussions of biologics. In most visits, providers initiated the decision-making discussion. Detailed information was typically given about the provider's preferred option with less information about other options. There was minimal elicitation of preferences, treatment goals, or prior knowledge. Few parents or patients spontaneously stated their preferences or concerns. An implicit or explicit treatment recommendation was given in nearly all visits, although rarely requested. In approximately one-third of the visits, the treatment decision was never made explicit, yet steps were taken to implement the provider's preferred treatment. CONCLUSIONS: We observed limited use of SDM, despite previous research indicating that parents wish to collaborate in decision making. To better achieve SDM in chronic conditions, providers and families need to strive for bidirectional sharing of information and an explicit family role in decision making.

"On the scene": Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Cincinnati Children's Hospital Medical Center is transforming the way it cares for its patients by building a sophisticated model that focuses on accountable care across the continuum. As nurses from different parts of the organization, we act as change agents to develop an integrated structure built around the patient's needs, from prevention to self-management. We demonstrate how organizational structure, fluid staffing, professional practice, and healthy behaviors operationally catalyze the continuum of care, and how we utilize self-management, community-based programs, and care integration to change the outcome for our patients and families. While care coordination is taking on many forms in medical centers around the world, Cincinnati Children's is proud and passionate about sharing its best practices along the way.

Individualized plans of care to improve outcomes among children and adults with chronic illness: a systematic review.

BACKGROUND: Adults and children with chronic illness often require services from multiple providers. Individualized plans of care (IPCs) are sometimes developed to improve care coordination. However, their association with improved outcomes is unknown. METHODS: We searched literature published between January 2001 and October 2011, using Medline, CINAHL, EMBASE, PsychINFO, and bibliographic review. Eligible studies involved an IPC with input from the patient and/or family of individuals with chronic illness, evaluated outcomes, and were conducted in the United States. We assessed evidence quality using Oxford Centre for Evidence-Based Medicine criteria. RESULTS: 15 studies met inclusion criteria. Studies were heterogeneous regarding populations and outcomes examined and were generally low quality. Most described IPC use within a multifaceted care coordination intervention. The strongest evidence links IPC use and symptom improvement in depressed adults; the weakest evidence exists for outcomes in children. Vague descriptions of the IPCs' limited analysis. CONCLUSIONS: Current evidence supporting an association between IPC use and improved outcomes, particularly among children, is sparse. Well-designed evaluations of clearly described IPCs are needed to examine who should be involved in their development, what they should include, and how often they should be updated to improve outcomes of care for this vulnerable population.

Usability Testing of Situation Awareness Clinical Decision Support in the Intensive Care Unit.

OBJECTIVE: Our objective was to evaluate the usability of an automated clinical decision support (CDS) tool previously implemented in the pediatric intensive care unit (PICU) to promote shared situation awareness among the medical team to prevent serious safety events within children's hospitals. METHODS: We conducted a mixed-methods usability evaluation of a CDS tool in a PICU at a large, urban, quaternary, free-standing children's hospital in the Midwest. Quantitative assessment was done using the system usability scale (SUS), while qualitative assessment involved think-aloud usability testing. The SUS was scored according to survey guidelines. For think-aloud testing, task times were calculated, and means and standard deviations were determined, stratified by role. Qualitative feedback from participants and moderator observations were summarized. RESULTS: Fifty-one PICU staff members, including physicians, advanced practice providers, nurses, and respiratory therapists, completed the SUS, while ten participants underwent think-aloud usability testing. The overall median usability score was 87.5 (interquartile range: 80-95), with over 96% rating the tool's usability as "good" or "excellent." Task completion times ranged from 2 to 92 seconds, with the quickest completion for reviewing high-risk criteria and the slowest for adding to high-risk criteria. Observations and participant responses from think-aloud testing highlighted positive aspects of learnability and clear display of complex information that is easily accessed, as well as opportunities for improvement in tool integration into clinical workflows. CONCLUSION: The PICU Warning Tool demonstrates good usability in the critical care setting. This study demonstrates the value of postimplementation usability testing in identifying opportunities for continued improvement of CDS tools.

Parents' perceptions of a patient portal for managing their child's chronic illness.

Through interviews, we sought to describe parents' perceptions of a patient portal for the management of their child's chronic illness. Parents perceive patient portals as beneficial, providing easier communication with care providers, convenience, a sense of control, reduced anxiety, and reassurance. Future research should aim to quantitate these benefits.