A national postgraduate nurse practitioner and physician assistant fellowship in cystic fibrosis: An innovative approach to the provider shortage in complex and rare disease.

ABSTRACT: Cystic fibrosis (CF) is a complex life-limiting genetic condition that affects the respiratory, digestive, reproductive system, and sweat glands. Advances in treatment have led to improved survival and quality of life. Today, most persons with CF live to adulthood but require highly specialized care at accredited CF Care Centers. The growing and aging CF population combined with the provider workforce shortage have increased the demand for qualified CF providers. Nurse practitioners (NPs) and physician assistants (PAs) have been providing CF care for decades, but most learned on the job. The Leadership and Education for Advanced Practice Provider (LEAPP) fellowship in CF care aims to address the provider gap, ease transition to practice, and ensure access to specialized care. Unlike other institutional based joint NP/PA fellowships, LEAPP was designed to train providers at various locations across the national CF care center network. The program is innovative in several ways: (1) LEAPP employs a flipped classroom that pairs an online curriculum with case-based virtual discussion with content experts from the CF care network; (2) fellows receive mentored clinical training at their home CF center; (3) LEAPP partnered with a university-based team to ensure best practices and evaluation for adult learners; and (4) LEAPP promotes organizational enculturation through program components of professional mentoring, quality improvement, and leadership. This innovative approach may be suitable for other complex conditions that require highly specialized care, such as sickle cell disease, spina bifida, and solid organ transplant.

Human Milk Feeding and Direct Breastfeeding Improve Outcomes for Infants With Single Ventricle Congenital Heart Disease: Propensity Score-Matched Analysis of the NPC-QIC Registry.

Background Infants with single ventricle congenital heart disease undergo 3 staged surgeries/interventions, with risk for morbidity and mortality. We estimated the effect of human milk (HM) and direct breastfeeding on outcomes including necrotizing enterocolitis, infection-related complications, length of stay, and mortality. Methods and Results We analyzed the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry (2016-2021), examining HM/breastfeeding groups during stage 1 and stage 2 palliations. We calculated propensity scores for feeding exposures, then fitted Poisson and logistic regression models to compare outcomes between propensity-matched cohorts. Participants included 2491 infants (68 sites). Estimates for all outcomes were better in HM/breastfeeding groups. Infants fed exclusive HM before stage 1 palliation (S1P) had lower odds of preoperative necrotizing enterocolitis (odds ratio [OR], 0.37 [95% CI, 0.17-0.84]; P=0.017) and shorter S1P length of stay (rate ratio [RR], 0.87 [95% CI, 0.78-0.98]; P=0.027). During the S1P hospitalization, infants with high HM had lower odds of postoperative necrotizing enterocolitis (OR, 0.28 [95% CI, 0.15-0.50]; P<0.001) and sepsis (OR, 0.29 [95% CI, 0.13-0.65]; P=0.003), and shorter S1P length of stay (RR, 0.75 [95% CI, 0.66-0.86]; P<0.001). At stage 2 palliation, infants with any HM (RR, 0.82 [95% CI, 0.69-0.97]; P=0.018) and any breastfeeding (RR, 0.71 [95% CI, 0.57-0.89]; P=0.003) experienced shorter length of stay. Conclusions Infants with single ventricle congenital heart disease in high-HM and breastfeeding groups experienced multiple significantly better outcomes. Given our findings of improved health, strategies to increase the rates of HM/breastfeeding in these patients should be implemented. Future research should replicate these findings with granular feeding data and in broader congenital heart disease populations, and should examine mechanisms (eg, HM components, microbiome) by which HM/breastfeeding benefits these infants.

Predictors of Human Milk Feeding and Direct Breastfeeding for Infants with Single Ventricle Congenital Heart Disease: Machine Learning Analysis of the National Pediatric Cardiology Quality Improvement Collaborative Registry.

OBJECTIVE: To identify factors that support or limit human milk (HM) feeding and direct breastfeeding (BF) for infants with single ventricle congenital heart disease at neonatal stage 1 palliation (S1P) discharge and at stage 2 palliation (S2P) (∼4-6 months old). STUDY DESIGN: Analysis of the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry (2016-2021; 67 sites). Primary outcomes were any HM, exclusive HM, and any direct BF at S1P discharge and at S2P. The main analysis involved multiple phases of elastic net logistic regression on imputed data to identify important predictors. RESULTS: For 1944 infants, the strongest predictor domain areas included preoperative feeding, demographics/social determinants of health, feeding route, clinical course, and site. Significant findings included: preoperative BF was associated with any HM at S1P discharge (OR = 2.02, 95% CI = 1.74-3.44) and any BF at S2P (OR = 2.29, 95% CI = 1.38-3.80); private/self-insurance was associated with any HM at S1P discharge (OR = 1.91, 95% CI = 1.58-2.47); and Black/African-American infants had lower odds of any HM at S1P discharge (OR = 0.54, 95% CI = 0.38-0.65) and at S2P (0.57, 0.30-0.86). Adjusted odds of HM/BF practices varied among NPC-QIC sites. CONCLUSIONS: Preoperative feeding practices predict later HM and BF for infants with single ventricle congenital heart disease; therefore, family-centered interventions focused on HM/BF during the S1P preoperative time are needed. These interventions should include evidence-based strategies to address implicit bias and seek to minimize disparities related to social determinants of health. Future research is needed to identify supportive practices common to high-performing NPC-QIC sites.

Using data-driven analytics and ecological systems theory to identify risk and protective factors for school absenteeism among secondary students.

Chronic absenteeism is an administrative term defining extreme failure for students to be present at school, which can have devastating long-term impacts on students. Although numerous prior studies have investigated associated variables and interventions, there are few studies that utilize both theory-driven and data-informed approaches to investigate absenteeism. The current study applied data-driven machine learning techniques, grounded in "The Kids and Teens at School" (KiTeS) theoretical framework, to student-level data (N = 121,005) to identify risk and protective variables that are highly associated with school absences. A total of 18 risk and protective variables were identified; all 18 variables were characteristics of the microsystem or mesosystem, emphasizing school absences' proximity to variables within inner ecological systems rather than the exosystem or macrosystem. Implications for future studies and health infrastructure are discussed.

Beyond coping: The role of supportive relationships and meaning making in youth well-being.

INTRODUCTION: The purpose of this study was to identify coping strategies, resources, and strengths that predict well-being in a community-based sample of youth with varying levels of adversity. DESIGN: Grounded in the resilience portfolio model, we used a mixed methods approach with data from a cross-sectional sample of 231 youth ages 8-17. MATERIALS AND METHODS: Data were collected using a survey, participant-generated timeline activity, and brief interview. Measures included assessments of coping and appraisal, resilience resources and assets, and subjective well-being and depression. RESULTS: Active and passive coping strategies predicted subjective well-being and depression. Controlling for demographics and coping, meaning making strengths and supportive relationships were significant predictors of subjective well-being and lower depression, and decreased the impact of adversity on these outcomes. DISCUSSION: The results of this study provide support for the resilience portfolio model in a community-based sample of youth, with relationships as predicted for subjective well-being and symptoms of depression. For both outcomes, family relationships held the strongest associations with positive well-being and lower symptoms of depression. Supportive relationships with peers, meaning making strengths, interpersonal strengths, less passive coping, and fewer adverse life events were also associated with better outcomes. CONCLUSIONS: These findings underscore the need to assess youth resources and strengths and to design interventions that target these protective factors for all youth, regardless of exposure to adversity. CLINICAL RELEVANCE: A theory-informed understanding of resources and strengths that predict youth well-being is essential to inform strengths-based interventions for pediatric research and practice. The resilience portfolio model is a useful framework for understanding predictors of youth well-being.

Human milk feeding and direct breastfeeding improve outcomes for infants with single ventricle congenital heart disease: Propensity score matched analysis of the NPC-QIC registry.

Background: Infants with single ventricle (SV) congenital heart disease (CHD) undergo three staged surgeries/interventions, with risk for morbidity and mortality. We estimated the effect of human milk (HM) and direct breastfeeding (BF) on outcomes including necrotizing enterocolitis (NEC), infection-related complications, length of stay (LOS), and mortality. Methods: We analyzed the National Pediatric Cardiology Quality Improvement Collaborative registry (2016-2021), examining HM/BF groups during stage 1 (S1P) and stage 2 (S2P) palliations. We calculated propensity scores for feeding exposures, then fitted Poisson and logistic regression models to compare outcomes between propensity-matched cohorts. Results: Participants included 2491 infants (68 sites). Estimates for all outcomes were better in HM/BF groups. Infants fed exclusive HM before S1P had lower odds of preoperative NEC (OR=0.37, 95% CI=0.17-0.84, p=0.017) and shorter S1P LOS (RR=0.87, 0.78-0.98, p=0.027). During the S1P hospitalization, infants with high HM had lower odds of postoperative NEC (OR=0.28, 0.15-0.50, p<0.001) and sepsis (0.29, 0.13-0.65, p=0.003), and shorter S1P LOS (RR=0.75, 0.66-0.86, p<0.001). At S2P, infants with any HM (0.82, 0.69-0.97, p=0.018) and any BF (0.71, 0.57-0.89, p=0.003) experienced shorter LOS. Conclusions: Infants with SV CHD in high HM and BF groups experienced multiple significantly better outcomes. Given our findings of improved health, strategies to increase the rates of HM/BF in these patients should be implemented. Future research should replicate these findings with granular feeding data and in broader CHD populations, and should examine mechanisms (eg, HM components; microbiome) by which HM/BF benefits these infants. Clinical Perspective: What is new?: This is the first large, multisite study examining the impact of human milk and breastfeeding on outcomes for infants with single ventricle congenital heart disease.All outcome estimates were better in high human milk and breastfeeding groups, with significantly lower odds of necrotizing enterocolitis, sepsis, and infection-related complications; and significantly shorter length of stay at both the neonatal stage 1 palliation and the subsequent stage 2 palliation.All estimates of all-cause mortality were substantially lower in human milk and breastfeeding groups, with clinically important estimates of 75%-100% lower odds of mortality in direct breastfeeding groups.What are the clinical implications?: There is a critical need for improved, condition-specific lactation support to address the low prevalence of human milk and breastfeeding for infants with single ventricle congenital heart disease.Increasing the dose and duration of human milk and direct breastfeeding has strong potential to substantially improve the health outcomes of these vulnerable infants.

Patterns of Breastfeeding and Human Milk Feeding in Infants with Single-Ventricle Congenital Heart Disease: A Population Study of the National Pediatric Cardiology Quality Improvement Collaborative Registry.

Introduction: Infants with single-ventricle (SV) congenital heart disease (CHD) undergo staged surgical and/or catheter-based palliation and commonly experience feeding challenges and poor growth. Little is known about human milk (HM) feeding or direct breastfeeding (BF) in this population. Aim: To determine (1) HM and BF prevalence for infants with SV CHD, and (2) whether BF at neonatal stage 1 palliation (S1P) discharge is associated with any HM at stage 2 palliation (S2P; ∼4-6 months old). Materials and Methods: Analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry (2016-2021) using (1) descriptive statistics for prevalence, and (2) logistic regression adjusted for multiple variables (e.g., prematurity, insurance, length of stay) to examine early BF/later HM feeding. Results: Participants included 2,491 infants from 68 sites. HM prevalence ranged from 49.3% any/41.5% exclusive before S1P to 37.1% any/7.0% exclusive at S2P. Direct BF ranged from 16.1% any/7.9% exclusive before S1P to 9.2% any/3.2% exclusive at S2P discharge. Prevalence varied among sites; for example, 0-100% any HM before S1P. Infants BF at S1P discharge had greater odds of any HM (odds ratio = 4.11, 95% confidence interval [CI] = 2.79-6.07, p < 0.001) and exclusive HM (1.85, 95% CI 1.03-3.30, p = 0.039) at S2P. Conclusions: The prevalence of HM and BF for infants with SV CHD was low and declined over time. Direct BF at S1P discharge was associated with increased odds of any HM at S2P. Wide variation suggests that site-specific practices impact feeding outcomes. HM and BF prevalence are suboptimal in this population, and identification of supportive institutional practices is needed.

Assessment of Youth Coping and Appraisals Through a Timelining Activity: Validation and Utility of a Tool for Pediatric Research and Practice.

INTRODUCTION: Many tools to assess coping in youth fail to engage youth meaningfully in the assessment process. This study aimed to evaluate a brief timeline activity as an interactive way to assess appraisal and coping in pediatric research and practice. METHOD: We used a mixed method convergent design to collect and analyze survey and interview data from 231 youths aged 8-17 years in a community-based setting. RESULTS: The youth engaged readily in the timeline activity and found the activity easy to understand. Relationships among appraisal, coping, subjective well-being and depression were in the hypothesized directions, suggesting the tool supports a valid assessment of appraisals and coping in this age group. DISCUSSION: The timelining activity is well-accepted by youth and supports reflexivity, prompting youth to share insights on strengths and resilience. The tool may augment existing practices for assessing and intervening in youth mental health in research and practice.

Participant-generated timelines as a novel strategy for assessing youth resilience factors: A mixed-methods, community-based study.

PURPOSE: The purpose of this study was to explore youth perceptions of resilience through participant-generated timelines as a potential new strategy for nursing research and practice. DESIGN AND METHODS: We used a concurrent triangulation design to collect and analyze qualitative and quantitative data from a statewide, community-based sample of 448 youth ages 8 to 17 years. We collected three sources of data during two waves of data collection in 2019 and 2021: a participant-generated timeline, a brief structured interview, and a PROMIS pediatric global health measure. RESULTS: The timeline activity was easy to administer and well accepted by youth in a community setting. Youth reporting an adverse experience or mental health challenge in the previous year had unique patterns of variability in the timelines, and more frequently reported certain resilience factors, including family support and regulatory, interpersonal, and meaning-making assets. Pandemic-related differences were noted between the two cohorts. CONCLUSIONS: The timeline drawing activity reflects and extends findings from a standard measure of global health. This activity benefits both the participant and the clinician or researcher by helping youth to communicate how they make sense of the world, honoring different ways of knowing and prompting recollection of coping appraisals and resilience factors. PRACTICE IMPLICATIONS: Participant-generated timelines are a developmentally appropriate and interactive strategy for assessing youth perceptions of their internal strengths. In conjunction with traditional assessments, this strategy may support the identification of malleable targets for intervention in practice with youth who have experienced adversity or mental health challenges.

Family-Centered Care: How Close Do We Get When Talking to Parents of Children Undergoing Diagnosis for Autism Spectrum Disorders?

Autism spectrum disorder (ASD) affects 1:59 children, yet little is known about parents' perceptions of family-centered care (FCC) during the diagnostic process leading up to diagnosis. This mixed-methods study explored key elements of FCC from 31 parents of children recently diagnosed with ASD using parallel qualitative and quantitative measures. Parents rated highly their receipt of FCC and discussed ways providers demonstrated FCC. However, the majority of parents indicated that the period when their child was undergoing diagnosis was stressful and reported symptoms of depression and anxiety. The study points to ways in which health care providers can enhance FCC provided to families when a child is undergoing ASD diagnosis.

Development and Testing of the Preparation for Labor and Birth Instrument.

OBJECTIVE: To develop and assess the reliability and validity of a new instrument used during the third trimester of pregnancy to measure women's confidence in their ability to achieve physiologic birth, the Preparation for Labor and Birth (P-LAB) instrument. DESIGN: Two-phase instrument development study that consisted of item generation and a prospective field test. SETTING: Field testing occurred in five midwestern U.S. prenatal clinics. PARTICIPANTS: Participants in the field test were 203 nulliparous and parous pregnant women who intended to give birth vaginally. METHODS: Psychometric testing consisted of test-retest reliability testing and assessments of content validity, face validity, and construct validity. We measured construct validity using exploratory factor analysis and correlation with the Sense of Coherence Scale. RESULTS: The 22-item P-LAB showed good content validity, good internal consistency, and stability over time. All items had content validity index scores greater than or equal to 0.8, and the total instrument content validity index was 0.95. We identified four factors related to women's confidence in their ability to achieve physiologic birth: Planned Use of Pain Medication, Relationship With Care Provider and Supportive Birth Environment, Beliefs About Labor, and Labor Support (social and professional). Cronbach's alpha coefficient for the four extracted factors were .93, .76, .73 and .74, respectively. Intraclass correlation [95% confidence interval] for the total questionnaire was .92 [.88, .94]. We found no linear association between total P-LAB scores and sense of coherence. CONCLUSION: Our findings demonstrate acceptable initial psychometric properties for the P-LAB instrument. Additional testing is required to evaluate the instrument's construct, convergent, and divergent validity.

Identifying and Communicating Postdischarge Goals for Hospitalized Children With Medical Complexity: A Process Improvement Pilot in a Specialty Pediatric Setting.

INTRODUCTION: Family-centered communication at transitions of care can decrease readmissions and costs for children with medical complexity (CMC). The purpose of this quality improvement project was to improve the communication of postdischarge goals for CMC in a pediatric specialty setting. METHODS: We used process improvement strategies to implement a Post-Hospitalization Action Grid (PHAG) and a standardized discharge handoff process. Families of hospitalized CMC at high risk for readmission received the pilot intervention over 3 months. Indicators of successful implementation included rates of use of the PHAG, perceptions of integrated care, and usability of the tool. RESULTS: The PHAG was implemented with 11 of 40 eligible CMC families. Most staff agreed that the new process could improve the communication of postdischarge goals; however, perceptions of integrated care in the organization changed only minimally. DISCUSSION: The PHAG facilitates family engagement in discharge transitions but requires organizational investment of resources to implement.

Teaching Systems Thinking for Advanced Family Nursing Practice: A Theory-Based Tool.

Systems thinking is essential for advanced family nursing practice, yet this skill is complex and not innate. The Family Nursing Assessment and Intervention Map (FN-AIM) was developed to support student development of systems thinking competencies for Family Systems Nursing practice (see Marigold Family Case Study). The FN-AIM is a pedagogical tool grounded in a family systems framework for nursing with a focus on core family processes as a foundation for interventions. The FN-AIM was implemented as an educational tool to support student skill development as part of a graduate family nursing course in the United States. Through a self-assessment of competence in family nursing practice, 30 students demonstrated an enhanced ability to articulate the distinction between family as context and family as system approaches to family nursing after using the FN-AIM mapping approach. The FN-AIM may be a useful strategy for supporting systems thinking in preparation for clinical skills development in graduate nursing students.

Outcomes research on children with medical complexity: A scoping review of gaps and opportunities.

BACKGROUND: There has been a recent, rapid increase in the number of studies of children with medical complexity (CMC) and their families. There is a need for attention to gaps and patterns in this emerging field of study. OBJECTIVES: The purpose of this scoping review was to identify patterns and gaps in the evidence related to classification systems, data, and outcomes in studies of CMC. DATA SOURCES: We searched peer-reviewed journals for reports of quantitative studies focused on CMC outcomes published between 2008 and 2018. On the basis of a structured screening process, we selected 63 reports that met our inclusion criteria. STUDY APPRAISAL AND SYNTHESIS: We used the methodological framework for scoping studies described by Arskey and O'Malley to map relevant literature in the field and the ECHO model to categorize studies according to three health outcome domains (economic, clinical, and humanistic). RESULTS: The terminology used to describe and classify CMC differed across studies depending on outcome domain. Two thirds of the reports focused on economic outcomes; fewer than a quarter included child or family quality of life as an outcome. A majority of studies used a single source of data, with robust analyses of administrative, payer, and publicly available data. CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: Research on CMC and their families would benefit from standardization of terms and classification systems, the use of measurement strategies that map humanistic outcomes as trajectories, and more attention to outcomes identified as most meaningful to CMC and their families.

School Nurse Perceptions of Nurse-Family Relationships in the Care of Elementary Students With Chronic Conditions.

The purpose of this study was to explore school nurse perceptions of the nurse-family relationship in the care of elementary students with asthma and attention-deficit hyperactivity disorder (ADHD). A cross-sectional survey design was used to collect data from 97 school nurses in Minnesota. The Family Nursing Practice Scale measured nurses' perceptions of their family nursing practice. Bivariate analyses were conducted to compare scores by factors at the community, school, nurse, and child levels. Results suggest that school nurses have positive appraisals of their family nursing practice, though scores were generally lower in the context of ADHD compared to asthma. Participants with a graduate degree reported greater skill in working with families, whereas novice nurses reported less confidence working with families and less comfort initiating family involvement in care. Results suggest that interventions at the nurse and school levels may support enhanced family nursing practice by nurses caring for students with chronic conditions.

Health-Related Quality of Life Outcomes of a Telehealth Care Coordination Intervention for Children With Medical Complexity: A Randomized Controlled Trial.

The purpose of this study was to explore health-related quality of life (HRQL) and family impact in the context of an advanced practice registered nurse-delivered telehealth care coordination intervention for children with medical complexity (CMC). This was a secondary outcomes analysis of a randomized controlled trial with 163 families of CMC in an existing medical home. HRQL and family impact were measured using the PedsQL measurement model. Bivariate and analysis of covariance analyses were conducted to explore associations at baseline and the intervention effect over 2 years. Significant predictors of Year 2 child HRQL were baseline HRQL and the presence of both neurologic impairment and technology dependence. There was no significant intervention effect on child HRQL or family impact after 24 months. Care coordination interventions for CMC may need to incorporate family system interventions for optimal outcomes in a range of quality of life domains.

Strategies for Developing Family Nursing Communities of Practice Through Social Media.

This discussion article presents communities of practice (CoPs) and bridging social capital as conceptual frameworks to demonstrate how social media can be leveraged for family nursing knowledge, scholarship, and practice. CoPs require a shared domain of interest, exchange of resources, and dedication to expanding group knowledge. Used strategically and with a professional presence, mainstream social media channels such as Twitter, Facebook, and YouTube can support the family nurse in developing and contributing to CoPs related to family nursing. This article presents four strategies-curate, connect, collaborate, and contribute-for establishing and growing a social media presence that fits one's professional goals and time availability. Family nurses who leverage social media using these strategies can strengthen existing CoPs and at the same time bridge networks to reach new audiences, such as family advocacy groups, policy makers, educators, practitioners, and a wide array of other extended networks.

The Impact of Telehealth and Care Coordination on the Number and Type of Clinical Visits for Children With Medical Complexity.

INTRODUCTION: The purpose of this analysis was to evaluate the effects of an advanced practice nurse-delivered telehealth intervention on health care use by children with medical complexity (CMC). Because CMC account for a large share of health care use costs, finding effective ways to care for them is an important challenge requiring exploration. METHOD: This was a secondary analysis of data from a randomized clinical trial with a control group and two intervention groups. The focus of the analysis was planned and unplanned clinical and therapy visits by CMC over a 30-month data collection period. Nonparametric tests were used to compare visit counts among and within the three groups. RESULTS: The number of unplanned visits decreased over time across all groups, with the greatest decrease in the video telehealth intervention group. Planned visits were higher in the video telehealth group across all time periods. DISCUSSION: Advanced practice registered nurse-delivered telehealth care coordination may support a shift from unplanned to planned health care service use among CMC.

Strategies to Maximize Data Collection Response Rates in a Randomized Control Trial Focused on Children with Medical Complexity.

INTRODUCTION: Obtaining complete and timely subject data is key to the success of clinical trials, particularly for studies requiring data collected from subjects at home or other remote sites. A multifaceted strategy for data collection in a randomized controlled trial (RCT) focused on care coordination for children with medical complexity is described. The influences of data collection mode, incentives, and study group membership on subject response patterns are analyzed. Data collection included monthly healthcare service utilization (HCSU) calendars and annual surveys focused on care coordination outcomes. MATERIALS AND METHODS: One hundred sixty-three families were enrolled in the 30-month TeleFamilies RCT. Subjects were 2-15 years of age at enrollment. HCSU data were collected by parent/guardian self-report using mail, e-mail, telephone, or texting. Surveys were collected by mail. Incentives were provided for completed surveys after 8 months to improve collection returns. Outcome measures were the number of HCSU calendars and surveys returned, the return interval, data collection mode, and incentive impact. RESULTS: Return rates of 90% for HCSU calendars and 82% for annual surveys were achieved. Mean return intervals were 72 and 65 days for HCSU and surveys, respectively. Survey response increased from 55% to 95% after introduction of a gift card and added research staff. CONCLUSIONS: High return rates for HCSU calendars and health-related surveys are attainable but required a flexible and personnel-intensive approach to collection methods. Family preference for data collection approach should be obtained at enrollment, should be modified as needed, and requires flexible options, training, intensive staff/family interaction, and patience.