Stress and Coping Factors Affecting Health-Related Quality of Life in Parents of Children with Congenital Heart Disease: An Integrative Review.

Parents of children with congenital heart disease experience unique stressors that affect their psychological well-being and health-related quality of life. A parent's ability to cope effectively influences how they adjust to stressful situations. The purpose of this review is to synthesize the factors influencing HRQoL in parents of children with congenital heart disease under the lens of the Transactional Model of Stress and Coping framework to identify areas for intervention and future research. An integrative literature review was conducted by keyword search of online databases PubMed, PsycINFO, Cumulative Index to Nursing and Allied Health Literature, and Medline from 2011 to 2022. Inclusion and exclusion criteria were applied to search results and studies were assessed for quality using the Mixed Method Appraisal Tool. The initial search yielded 1026 unique papers, and 24 were included based on the predefined criteria. The Transactional Model of Stress and Coping was applied to organize results and generate themes. Results showed a child's hospitalization is a time of uncertainty and distress for parents of children with congenital heart disease. Effective problem-focused and emotion-focused coping strategies can lead to reduced stress and improved health-related quality of life for parents. Additionally, multiple variables moderate the relationship between a parent's perceived stress and adjustment. Health care teams should focus on targeted education, faith, coping, and social support interventions. Further research is needed that examines the effect of mental health factors, cultural differences, and socioeconomic variables on the health-related quality of life of parents of children with congenital heart disease.

Barriers and facilitators to accessing pediatric specialty care for rural-dwelling children with complex chronic conditions: An integrative review.

PROBLEM: Pediatric specialty services are often geographically concentrated in urban areas, leaving up to 1 in 5 rural-dwelling children in the United States without access to advanced care. The purpose of this review was to identify and review extant literature related to barriers and facilitators to accessing specialty care for rural-dwelling children with complex chronic conditions. ELIGIBILITY CRITERIA: The Whittemore and Knafl (2005) integrative review method guided the review which included a critical appraisal and analysis of relevant articles published between 2012 and 2023. SAMPLE: Twenty-three studies were identified for inclusion in the integrative review. RESULTS: Using the domains of the Levesque et al. (2013) conceptual framework, findings were categorized according to the access to care continuum. Barriers included broadband access, transportation, and inadequate care coordination. Facilitators included telehealth, social support, and outreach clinics. CONCLUSIONS: To improve access to pediatric specialty care for rural-dwelling children, nurses, physicians, and policymakers will need to consider how the social determinants of health impact the healthcare access continuum from diagnosis to continuing healthcare. IMPLICATIONS: The findings of this integrative review will aid researchers in developing interventions to improve access to pediatric specialty care for rural-dwelling children.

Hospital-based ovarian cancer patient traceback program results in minimal genetic testing uptake.

OBJECTIVE: To determine the feasibility of hospital-based genetic counseling and testing (GC/T) Traceback for Ovarian Cancer (OC) patients, as proposed by the Division of Cancer Prevention and the Division of Cancer Control and Population Sciences, National Cancer Institute. METHODS: Living patients with OC were sent a letter explaining the availability of guideline-supported GC/T for at least BRCA1/2 and surrogates of deceased patients were called on the telephone. Outcomes of contact attempts were systematically recorded and statistically described. RESULTS: 598 Traceback-eligible OC patients diagnosed from 2006 to 2016 were identified (163 presumed-living and 435 deceased). Two living patients called our office and scheduled an appointment for GC/T after receiving a letter. For surrogates of prior patients, successful contact occurred in 25% of call attempts. Fourteen individuals (2 living patients and 12 surrogates) underwent genetic counseling. Of those 14, 10 individuals consented to genetic testing and 5 followed through with sample collection. None of these individuals had pathogenic variants (PVs). When surrogate call notes were reviewed, 58% reflected positive responses to contact, however 42% were noted to have negative or indifferent responses, which were most common among spouses. Total time spent for hospital-based Traceback was 109 h. CONCLUSIONS: Overall, hospital-based Traceback via letter and telephone contact of surrogates is time-intensive and results in minimal uptake of GC/T. To practically execute this type of outreach program, health systems should consider collection of alternative contact information to allow for electronic communication of patient surrogates. Our study also underscores the importance of timely GC/T while patients are in active cancer care.

The bereavement experiences of children following sibling death: An integrative review.

PROBLEM: Sibling bereavement is one of the most distressing adverse life events during childhood but has received less attention in research than other forms of childhood bereavement. This integrative review identifies potential risk and protective factors for maladaptive coping following sibling bereavement and the influence of these factors on adjustment to loss. ELIGIBILITY CRITERIA: Articles were limited to peer-reviewed studies, published in English in 2000 and beyond. Target population was bereaved siblings 0-18 years, and outcomes examined grief experiences by child self-report or parent-proxy report. SAMPLE: The Whittemore and Knafl integrative framework was applied. Multidimensional Grief Theory guided the review with twenty-five studies synthesized across its domains: Separation Distress, Existential/Identity Crisis, and Circumstance-Related Distress. RESULTS: Adjustment following sibling death is a complex process associated with a host of risk and protective factors that contribute to the bereavement process for this population. Age, sex, circumstance-related factors, continuing bonds, parental distress, and limited social support were critical influencing factors. CONCLUSION: Definitive predictor variables were not identified but a combination of variables that influence the adjustment of bereaved siblings are discussed. IMPLICATIONS: Future research is needed to explore the risk and protective factors of maladaptive coping to inform intervention development to promote individual and family adjustment following sibling death.

Changes in Care Delivery for Children With Sickle Cell Anemia During the COVID-19 Pandemic.

BACKGROUND: Specialty care for children with sickle cell disease (SCD) may be disrupted during the coronavirus (COVID-19) pandemic. Our DISPLACE consortium includes 28 pediatric SCD centers. METHODS: In May 2020, we surveyed the consortium on the impact of COVID-19 on their practice focusing on transcranial Doppler ultrasound, chronic red cell transfusions, telehealth, and COVID-19 testing. OBSERVATION: Twenty-four DISPLACE providers completed the survey. Transcranial Doppler ultrasound screening decreased to 67% but chronic red cell transfusions remained at 96%. Most investigators (92%) used telehealth and 40% of providers had patients test positive for COVID-19. CONCLUSION: The COVID-19 pandemic has affected routine care and necessitated changes in practice in SCD.

Transcranial Doppler Screening in a Current Cohort of Children With Sickle Cell Anemia: Results From the DISPLACE Study.

Stroke prevention guidelines for sickle cell anemia (SCA) recommend transcranial Doppler (TCD) screening to identify children at stroke risk; however, TCD screening implementation remains poor. This report describes results from Part 1 of the 28-site DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study, a baseline assessment of TCD implementation rates. This report describes TCD implementation by consortium site characteristics; characteristics of TCDs completed; and TCD results based on age. The cohort included 5247 children with SCA, of whom 5116 were eligible for TCD implementation assessment for at least 1 study year. The majority of children were African American or Black, non-Hispanic and received Medicaid. Mean age at first recorded TCD was 5.9 and 10.5 years at study end. Observed TCD screening rates were unsatisfactory across geographic regions (mean 49.9%; range: 30.9% to 74.7%) independent of size, institution type, or previous stroke prevention trial participation. The abnormal TCD rate was 2.9%, with a median age of 6.3 years for first abnormal TCD result. Findings highlight real-world TCD screening practices and results from the largest SCA cohort to date. Data informed the part 3 implementation study for improving stroke screening and findings may inform clinical practice improvements.

The Experiences of Parent Dyads in the Neonatal Intensive Care Unit: A Qualitative Description.

Background The hospitalization of an infant in the neonatal intensive care unit (NICU) is a distressing and often unexpected event for parents. Parents have risk for depression, anxiety, and posttraumatic stress, which can adversely impact them and their relationship. The hospitalization and subsequent stress may affect parents' capability to connect with and parent their child. Purpose Describe parents' experiences and ability to cope with the NICU; identify experiential and coping differences between mothers and fathers; examine the effect of the NICU hospitalization on the parent dyad relationship. Methods A qualitative descriptive design with dyadic interviews examined parents' experiences and coping skills, and subsequent effects on the parental relationship. Data collection continued until saturation was achieved and no new themes emerged. Through content analysis, an accurate description of parents' experiences in the NICU was rendered. Findings Nine themes from eight dyad interviews emerged and were categorized within the six domains of the transactional theory of stress and coping. The major themes were: Deeply Distressing, Unexpected and Unprepared, Expecting to Hear and Be Heard, Becoming Parents, Stronger Together, Support is Key, Parents Want Better Communication, and Adjusting to the NICU. Implications for practice Support from professionals and family, and clear and consistent communication from the treatment team helped alleviate parents' anxiety about their infant. Implications for research Research regarding the impact of a NICU hospitalization on the relationship between parent dyad members, specifically longitudinal studies, may lead to a better understanding of the long-term effects of this specific stressor on parents.

Practice patterns for stroke prevention using transcranial Doppler in sickle cell anemia: DISPLACE Consortium.

BACKGROUND: Children with sickle cell anemia (SCA) are at increased risk for stroke. In 2014, the National Heart, Lung, and Blood Institute (NHLBI) developed guidelines for stroke prevention in SCA informed by the Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP II) trials. The guidelines specify the use of transcranial Doppler (TCD) screening and intervention with chronic red cell transfusions (CRCT) in children with SCA who have TCD indication of high stroke risk. The purpose of this study was to describe real-world practice patterns of stroke risk screening and intervention in sites that participated in the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) Consortium. PROCEDURE: Site investigators completed a survey during the formative stages of the study to evaluate their TCD practices relative to the STOP studies. Descriptive statistics and analysis of free-text comments for more complex practices were evaluated. RESULTS: Results suggested universal acceptance of annual TCD screening and initiation of CRCT following an abnormal result among the DISPLACE Consortium, consistent with NHLBI recommendations. However, there was wide variation in methods for conducting TCD screenings (eg, dedicated Doppler vs TCD imaging), classifying TCD results, and actions taken for conditional and inadequate results. CONCLUSIONS: Annual TCD screening and initiation of CRCT are critical stroke prevention practices that were universally embraced in the consortium. Additional research would be beneficial for informing clinical practices for areas in which guidelines are absent or unclear.

Practice patterns for neuroimaging and transfusion therapy for management of neurologic complications in sickle cell anemia: DISPLACE consortium.

BACKGROUND: Children with sickle cell anemia (SCA) are at risk for neurologic complications (stroke and silent cerebral infarct). The 2014 National Heart, Lung, and Blood Institute (NHLBI) guidelines for sickle cell disease include recommendations for initiation and maintenance of chronic red cell transfusion (CRCT) therapy for children with SCA at risk for or with ischemic stroke. The guidelines do not include well-delineated recommendations for cerebral imaging for stroke screening. The purpose of this study was to evaluate current stroke risk screening, prevention, and intervention practices amongst the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) study sites. PROCEDURE: A survey was administered to DISPLACE site principal investigators to identify current stroke prevention practices relative to the Stroke Prevention Trial in Sickle Cell Anemia (STOP) study protocols and the 2014 NHLBI guidelines. Data were analyzed using descriptive statistics and line-by-line analysis of comments. RESULTS: Sites consistently applied NHLBI recommendations to initiate CRCT for ischemic stroke and abnormal transcranial Doppler ultrasound (TCD) results. Similarly, nearly all sites reported obtaining an magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) following an abnormal TCD result. There was wide variation for other indications for MRI/MRA, frequency of MRI/MRA, and other neurological indications for initiating CRCT. CONCLUSIONS: Guideline-based practices for preventing ischemic stroke through TCD and CRCT initiation were evident in nearly all sites. Wide variation in practices pertaining to MRI/MRA exists, potentially influenced by more recent stroke prevention trials. New guidelines from the American Society of Hematology were published in April 2020, which may reduce practice variation.

A Mixed-Methods Study to Investigate Barriers and Enablers to Nurses' Implementation of Nonpharmacological Interventions for Infants With Neonatal Abstinence Syndrome.

BACKGROUND: The incidence of neonatal abstinence syndrome has increased significantly as a result of the opioid epidemic. A lengthy hospitalization is often required to treat the infant's withdrawal symptoms. A comprehensive understanding of factors that influence nurses' implementation of nonpharmacological interventions for infants with neonatal abstinence syndrome is needed. PURPOSE: To investigate barriers and enablers to nurses' implementation of nonpharmacological interventions for infants with neonatal abstinence syndrome through the lens of the Theoretical Domains Framework, which provides a structure to examine factors that influence healthcare providers' behavior related to the implementation of evidence-based practice and interventions in clinical practice. METHODS: A convergent parallel mixed-methods study was conducted. Qualitative data were collected using semistructured interviews and quantitative data were collected using a tailored Determinants of Implementation Behavior Questionnaire with neonatal nurses. Findings from the interviews and surveys were merged through joint review to compare and contrast themes. RESULTS: Enablers and barriers to nurses' implementation of nonpharmacological interventions included education, experience, ability to implement nonpharmacological interventions, parental participation, stigmatization, lack of managerial/organizational support, staffing ratios, internal and external resources, and stress. Knowledge, Skills, Beliefs About Capabilities, Social/Professional Role and Identity, Organization, and Emotion of the Theoretical Domains Framework aligned with these themes. IMPLICATIONS FOR PRACTICE AND RESEARCH: Findings from this study will inform the development of programs to improve nurses' implementation of nonpharmacological interventions and health and utilization outcomes in infants with neonatal abstinence syndrome. Furthermore, future work should focus on the development of programs to improve nurses' implementation of nonpharmacological interventions, with specific strategies aimed to mitigate marginalization of vulnerable patient populations.

An approach to revising mHealth interventions for children and families: A case example in sickle cell disease.

Frameworks for developing mHealth interventions that are informed by the end-user and improve accessibility are necessary to ensure interventions meet the needs of the intended population and advance the science of health behavior change. The approach described in this paper addresses a gap in the mHealth development literature by describing a step-by-step method for evaluating and revising the mHealth interventions for health behavior change in child-parent dyads. Furthermore, this approach introduces the Website Analysis and MeasureMent Inventory framework as an initial coding structure for analyzing qualitative data to gauge appeal and enhance engagement of intervention for end-users. This method uses specific considerations for child-parent dyads and details an example in the development and refinement of a mobile application for self-management in children with sickle cell disease. This approach is translatable to populations of children with other chronic conditions and to other health behaviors.

The Experiences of Parents in the Neonatal Intensive Care Unit: An Integrative Review of Qualitative Studies Within the Transactional Model of Stress and Coping.

Having a child hospitalized in the neonatal intensive care unit (NICU) is a deviation from the norms expected for pregnancy and childbirth. A NICU admission may be traumatic for some parents, causing psychological distress and altered parenting roles. The aim of this integrative review is to examine the experiences and perceptions of a NICU hospitalization from the perspective of both parents to inform clinical practice and future research. A systematic search of 3 databases was conducted and included studies were evaluated by the Critical Skills Appraisal Programme checklist for qualitative studies. The Whittemore and Knafl integrative review methodology and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis methodology were followed to provide a structure for searching and reporting findings. There were 248 participants (153 mothers and 95 fathers) from 9 countries and of varying socioeconomic backgrounds in the 16 primary qualitative studies included in this review. The resulting major themes included panic sequence, emotional upheaval, social support, faith, and adjusting. Interventions directed at managing parents' emotions, supporting their spiritual needs, facilitating parenting skills and infant attachment, and adapting the environment to parents' needs can help improve the NICU experience.

The Symptoms Self-Management Center (SSMC): Methods for developing and implementing technology-enhanced self-management interventions for pain and fatigue.

The National Institutes of Nursing Research provides funding via the P20 grant mechanism for research infrastructure and resources to develop nurse scientists with expertise in symptom self-management. The Medical University of South Carolina College of Nursing was awarded a P20 grant in 2016 to build the Symptoms Self-Management Center for technology-enhanced interventions to address pain and fatigue in individuals with chronic health conditions. Resources were derived from three key subcores: bioinformatics, mHealth and eHealth consultative services, and community engagement. This paper describes methods for deriving specific resources within each subcore, the application of subcore resources in two pilot studies, and lessons learned during the early phases of our Symptoms Self-Management Center implementation.

Baccalaureate Minority Nursing Students Perceived Barriers and Facilitators to Clinical Education Practices: An Integrative Review.

AIM: This integrative review synthesized baccalaureate minority nursing students' perceptions of their clinical experiences. BACKGROUND: The diversity of the nursing workforce does not mirror the United States population. Attrition rates of minority nursing students remain higher than rates for White students. Literature examining facilitators and barriers to minority student success predominantly focuses on academic factors, excluding those relevant to clinical education. METHOD: An integrative review using literature from nursing and education. FINDINGS: Three common perceived barriers were identified: discrimination from faculty, peers, nursing staff, and patients; bias in faculty grading practices; and isolation. CONCLUSION: Although little is known about the relationship between clinical failures and overall attrition, this review provides evidence that minority students encounter significant barriers in clinical education. To increase the diversity of the nursing workforce, faculty must address these issues and make modifications to ensure an equal opportunity at a quality education for all students.

Effect of Exercise Conditioning on Countering the Effects of Obesity and Insulin Resistance in Horses-A Review.

Obesity is an important health concern in horses, along with humans and companion animals. Adipose tissue is an inflammatory organ that alters the insulin-signaling cascade, ultimately causing insulin dysregulation and impaired glucose metabolism. These disruptions can increase the risk of metabolic disease and laminitis in horses and may also impact energy metabolism during exercise. A single bout of exercise, along with chronic exercise conditioning, increases insulin sensitivity and glucose disposal via both contraction- and insulin-mediated glucose uptake pathways. Regular exercise also increases calorie expenditure, which can facilitate weight (as body fat) loss. This paper explores the metabolic pathways affected by adiposity, as well as discusses the impact of exercise on insulin metabolism in horses.

Current Methods of Newborn Screening Follow-Up for Sickle Cell Disease Are Highly Variable and without Quality Assurance: Results from the ENHANCE Study.

Newborn screening (NBS) for sickle cell disease (SCD) has significantly improved childhood survival but there are still gaps resulting in delayed care for affected infants. As a state-run program, there are no national quality assurance programs to ensure each state achieves consistent, reliable outcomes. We performed this qualitative study of NBS follow-up practices to better evaluate and understand the multi-level, state-specific processes of how each state's public health department delivers the NBS results to families, how/if they ensure affected infants are seen quickly by sickle cell specialists, and to determine the close-out processes used in each state. This project used semi-structured interviews conducted with 29 participants across eight states to explore these NBS follow-up processes in each state. Participants included SCD providers, NBS coordinators, or personnel associated with state health departments and community-based SCD organizations (CBO). Our results show significant state-dependent variations in the NBS processes of information delivery and patient management. Specifically, programs differed in how they communicated results to affected families and which other organizations were informed of the diagnosis. There was also state-based (and intrastate) variation in who should assume responsibility for ensuring that infants receive confirmatory testing and are promptly started on penicillin prophylaxis. Case closure was also highly variable and poorly validated. Our results also yielded identifiable challenges and facilitators to NBS which were highly variable by state but potentially addressable in the future. This information suggests opportunities for systematic improvement in NBS follow-up processes.

DISPLACE study shows poor quality of transcranial doppler ultrasound for stroke risk screening in sickle cell anemia.

ABSTRACT: Children with sickle cell anemia (SCA) are at increased risk of stroke when compared with their age-based counterparts. The Stroke Prevention Trial in Sickle Cell Anemia (STOP) previously demonstrated that with the use of transcranial Doppler ultrasound (TCD; Sickle Stroke Screen) and chronic red cell transfusion, the risk of stroke is reduced by over 90%. The STOP criteria detailed the type and method of measurement required; the time-averaged mean maximum velocity (TAMMV). Unfortunately, it has been difficult to adhere to the appropriate TAMMV measurements. The objectives of this study were to assess the quality of TCD and transcranial Doppler imaging (TCDi) reports to determine the report quality and accuracy. This is a subanalysis of the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study. Over 12 000 TCD/TCDi reports were collected during this study from 28 institutions; 391 TCDs were reviewed for this subanalysis. There were significant variations in the vessels being assessed, the velocities used to define abnormal results, and who was interpreting the scans. In 52% of reports, it was impossible to identify whether the TAMMV was what was measured. Similarly, it was only clear in 42% of reports that the TAMMV was used to interpret the examination as normal/abnormal. Given this inconsistency, we strongly recommend standardization of TCD/TCDi reporting, specialized training for those performing and interpreting the scans in the use of TCD/TCDi in patients with SCA, internal quality assurance, and institutional quality improvement work to ensure appropriate use of this potentially lifesaving technology.

Phenotypic Clusters and Multimorbidity in Hypermobile Ehlers-Danlos Syndrome.

Objective: To perform a retrospective clinical study in order to investigate phenotypic penetrance within a large registry of patients with hypermobile Ehlers-Danlos syndrome (hEDS) to enhance diagnostic and treatment guidelines by understanding associated comorbidities and improving accuracy in diagnosis. Patients and Methods: From May 1, 2021 to July 31, 2023, 2149 clinically diagnosed patients with hEDS completed a self-reported survey focusing on diagnostic and comorbid conditions prevalence. K-means clustering was applied to analyze survey responses, which were then compared across gender groups to identify variations and gain clinical insights. Results: Analysis of clinical manifestations in this cross-sectional cohort revealed insights into multimorbidity patterns across organ systems, identifying 3 distinct patient groups. Differences among these phenotypic clusters provided insights into diversity within the population with hEDS and indicated that Beighton scores are unreliable for multimorbidity phenotyping. Conclusion: Clinical data on the phenotypic presentation and prevalence of comorbidities in patients with hEDS have historically been limited. This study provides comprehensive data sets on phenotypic presentation and comorbidity prevalence in patients with hEDS, highlighting factors often overlooked in diagnosis. The identification of distinct patient groups emphasizes variations in hEDS manifestations beyond current guidelines and emphasizes the necessity of comprehensive multidisciplinary care for those with hEDS.

Practice Patterns for Addressing Developmental-Behavioral Concerns in Sickle Cell Specialty Care.

Objective: Children with sickle cell disease (SCD) are at elevated risk for neurodevelopmental and behavioral disorders. This report describes developmental-behavioral practice patterns among sites who were part of the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) consortium in the context of current guidelines for addressing these concerns. Methods: An internal survey was developed for the Principal Investigators of the DISPLACE study to identify developmental-behavioral clinical practices across the 28-site consortium, including methods for identification, referral practices, access to psychologists, and barriers to services. Descriptive data were pulled from the survey to describe practice patterns. Results: Most sites used informal methods to detect developmental-behavioral concerns, though over 1/3 of sites were using a structured protocol. The most common referral indications for further developmental and neuropsychological evaluation were parent, provider, or school concerns or stroke. Evaluations were predominantly completed by pediatric neuropsychologists and pediatric psychologists. Despite most sites reporting access to a psychologist within the SCD clinic, sites also reported long waitlists and difficulty accessing providers for evaluation and treatment services. Insurance difficulties were also a common barrier. A range of additional barriers were reported at the patient, provider, organizational, and policy/socio-environmental levels. Conclusions: Many sites in the DISPLACE consortium were adhering to existing care guidelines for pediatric SCD; however, there was also wide variation in practices for which guidelines are absent or unclear. Additional work is needed to inform guidelines, to specify the role of psychology within specialty SCD care, and to overcome barriers to care. Implications for Impact Statement: Optimal developmental-behavioral care for children with SCD involves collaboration among specialty care providers and pediatric psychologists; however, these efforts may be hampered by dispersed or unclear guidelines and several barriers to care. Future studies and guidelines have the potential to improve care by providing clear, consistent, and unified care recommendations specific to psychologists and related care professionals who are supporting children with SCD.