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We examined care and communication factors that affect physical and mental health for patients with complex vascular anomalies (VAs). VA patients (N = 135) completed an online survey with measures of ability to navigate healthcare, quality of information exchange, perceived stigma, and demographic variables. We performed linear regression to determine if these variables were associated with mental and physical health. Physical and mental health were associated with information exchange (β =.41, 95% CI=.12 −.69; β =.33, 95% CI=.04 −.62), stigma (β = −.49, 95% CI=−.74 to −.24; β = −.63, 95% CI=−.89 to −.38), and education (β = 4.00, 95% CI=.63 - 7.38; β = 3.44, 95% CI=.06 to 6.82). Ability to navigate healthcare was associated with health outcomes in our bivariate model, but not significant in a multivariate model. The results underscore the importance of effective information exchange. Poor information exchange was associated with worse physical and mental health. VA patients with lower education levels and higher perceived stigma reported poorer health outcomes and likely face many struggles accessing care. Patient-centered information exchange between clinicians and patients is needed to address unmet information needs. Clinicians can also reduce perceived stigma by validating patients, and should provide resources to reduce disparities related to education. • Patients with vascular anomalies (VAs) face many challenges accessing expert care. • Effective information exchange with clinicians is associated with better physical and mental health. • VA patients who perceive more stigma report poorer physical and mental health. • Education level is associated with VA patients' physical and mental health. • Interventions are needed to improve information exchange, reduce stigma, and reduce disparities related to education. [ABSTRACT FROM AUTHOR]

Objective: Families affected by rare diseases face many challenges finding adequate care and often report poor communication with clinicians. In the current study, we explore patient and caregiver advice for families and clinicians in the context of complex vascular malformations (VMs), a condition that is frequently misunderstood and misdiagnosed.Methods: We performed semi-structured interviews with 21 adult patients with complex VMs and 24 caregivers of children with VMs. We analyzed the transcripts using thematic analysis.Results: Participants advised patients and caregivers to advocate for care, address mental and emotional well-being, seek social support, and promote self-management and self-care. Participants advised clinicians to show care and concern, show commitment, empower and validate, communicate information clearly, address mental/emotional well-being, acknowledge the broad impact of disease and treatment, acknowledge your limitations, work as a team, and commit to learning.Conclusion: Participants' advice revealed challenges related to family-centered communication and patient and caregiver quality of life and demonstrated the importance of self-advocacy and social support.Practice Implications: The result of this study can help newly-diagnosed families overcome challenges related to care and communication. Clinicians can also use the results to support families by offering them our accompanying handout to validate families' experiences and relay this advice. [ABSTRACT FROM AUTHOR]

Background: Vascular malformations (VMs) are a collection of rare diseases that result from abnormal overgrowth of vascular tissue and can lead to pain, infection, disfigurement, and decreased mobility. Vascular malformations can invade or impinge on nearby structures, including nerves, causing pain, numbness, and/or functional impairment. If nonsurgical therapy fails, surgical resection is considered. However, VMs' thin walls and tendency to infiltrate and/or recur makes resection difficult or impossible. Rather than resecting, we have found that patients can still benefit from surgery in the form of nerve decompression. Here, we present a series of patients with VMs causing symptomatic nerve compression that were successfully treated with nerve decompression, with or without VM resection. It is the purpose of this study to demonstrate that VM patients with nerve pain and/or sensorimotor dysfunction should be referred for evaluation by a peripheral nerve surgeon, regardless of whether the VM is considered "resectable.", Methods: Retrospective chart review of patient undergoing nerve decompression surgery for symptoms of peripheral nerve compression (pain and/or motor/sensory dysfunction) attributable to a nearby VM (confirmed on preoperative magnetic resonance imaging), with or without VM resection., Results: Six patients with peripheral nerve compression attributable to VM were treated with nerve decompression. One also underwent VM resection. Five of the 6 patients had undergone previous surgical intervention (resection or sclerotherapy) without clinical improvement in radiculopathy. Following intervention, all patients demonstrated improvement of their pain and motor/sensory dysfunction., Conclusions: Patients with VM nerve pain and/or sensorimotor dysfunction should be referred for evaluation by a peripheral nerve surgeon, regardless of whether the VM is deemed resectable. Even when unresectable, patients may still benefit from surgery in the form of nerve decompression., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: BS has consulting relationships with Novartis Pharmaceuticals, Relay Therapeutics, and Kaken Pharmaceuticals. MP has consulting relationships with Vioptix Inc, KLIS Bio. He has received research funds from 3M and Kent Imaging. AU has consulting relationships with Anigodynamics Inc. and Beckton Dickinson Inc. CS, LH, and LS have no conflicts of interest.

Objective: We investigated communication experiences of caregivers of children with medical complexity (CMC) during emergency care., Methods: Fifteen caregivers of CMC participated in semi-structured interviews regarding communication while seeking care for their child in the emergency department (ED). Thematic analysis was applied using a previously established functional communication model as an a priori framework., Results: Each of the previously established 8 core functions of communication were identified in this population. "Building relationships" manifested as clinicians soliciting caregiver input and responding to the needs of the caregiver. "Exchanging information" included clearly explaining next steps to caregivers. "Responding to emotions" acknowledged the inherent distress of seeking emergency care. "Providing validation" included recognizing caregivers as the expert of their own child. "Enabling self-management" manifested as identifying caregiver needs and directing toward resources. "Making decisions" manifested as involving caregivers in the decision-making process and recognizing caregiver decision fatigue. "Managing uncertainty" involved developing plans with caregivers. "Supporting hope" was the least commonly described function and manifested as emphasizing positive aspects of a child's condition. Many caregivers described negative communication experiences in which their concerns and insights were unheard or invalidated., Conclusion: Caregivers of CMC described communication experiences and goals that align with 8 communication functions that were identified in other serious childhood illnesses with unique operationalizations., Practice Implications: This communication framework can support ED clinicians in better understanding the communication needs of caregivers of CMC during emergency care., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Published by Elsevier B.V.)

Purpose: United States healthcare systems have enacted varied adolescent online patient portal policies. No prior work has established whether these policies are acceptable to adolescents and parents., Methods: Mixed-method Delphi approach with 18 parents and 18 adolescents with and without chronic illness. We presented 19 policies related to (1) who can access different types of information through the portal; (2) timing of results release; and (3) portal messaging policies, such as whether individuals are charged for portal messages. Panelists voted on whether they "supported," "could live with," or "opposed" each policy., Results: Thirty-six panelists completed surveys with no attrition. For access-related policies, panelists opposed prohibiting parent or adolescent access, and they accepted policies that provided transparent access to sensitive and nonsensitive information for parents and adolescents. Panelists failed to reach consensus about whether adolescents should have the granular ability to determine which parts of the medical record their parents can see. For policies related to timing of release, panelists found either immediate or delayed access to results to be acceptable, although more panelists supported immediate access. For portal messaging policies, panelists supported the ability of parents and adolescents to send messages and opposed being charged for portal messages. Panelists found it acceptable for adolescents to see messages written by their parents, but failed to reach consensus on whether parents should see messages written by adolescents., Discussion: Some healthcare systems have implemented adolescent portal policies that panelists opposed or found concerning. Hospital leaders should consider these data as they re-evaluate institutional policies., (Copyright © 2024 Society for Adolescent Health and Medicine. Published by Elsevier Inc. All rights reserved.)

Conventional understanding and research regarding prognostic understanding too often focuses on transmission of information. However, merely overcoming barriers to patient understanding may not be sufficient. In this article the authors provide a more nuanced understanding of prognostic awareness, using oncological care as an overarching example, and discuss factors that may lead to prognostic discordance between physicians and patients. We summarize the current literature and research and present a model developed by the authors to characterize barriers to prognostic awareness. Ultimately, multiple influences on prognostic understanding may impede acceptance by patients even when adequate transfer of information takes place. Physicians should improve how they transmit prognostic information, as this information may be processed in different ways. A model of misunderstandings in awareness, ranging from patient understanding to patient belief, may be useful to guide future discussions. Future decision-making studies should consider these many variables so that interventions may be created to address all aspects of the prognostic disclosure process.

Purpose: The 21st Century Cures Act Final Rule mandates increased transparency and accessibility of the Electronic Health Record (EHR). This mandate has exacerbated tensions in adolescent healthcare between transparency and confidentiality. This study evaluates clinicians' perspectives on how well the EHR maintains confidentiality for adolescents in the post-Cures Act era., Methods: A web-based survey was distributed via the national listserv of the Society for Adolescent Health and Medicine in March-April 2022. English-speaking members practicing in the Unites States who used an EHR to document clinical visits with minors were eligible. The survey included questions about EHR training, features, and approach to information sharing with adolescent patients and proxies., Results: Participants included 97 respondents from 32 states. Most participants were physicians (98%) and identified themselves as attending (89%). Several EHR vendors were represented, although the majority used Epic (76%). As has been seen in prior studies, there remains significant variability in approaches to patient portal access for adolescent patients and their adult proxies. Respondents report that training around adolescent-specific privacy issues remains infrequent and is perceived as inadequate. Adolescent providers, despite feeling confident in navigating the EHR generally, continue to report low rates of confidence in how well their EHR may protect adolescent privacy., Discussion: Clinicians have persistent concerns about adolescent confidentiality postimplementation of the 21st Century Cures Act. Sharing sufficient information while protecting adolescent confidentiality might require standardization by EHR vendors to improve granularity of proxy information sharing. Healthcare institutions must also commit to training providers on management of adolescent confidentiality., (Copyright © 2023 Society for Adolescent Health and Medicine. Published by Elsevier Inc. All rights reserved.)

Background: Vascular anomalies (VAs) are a spectrum of rare pediatric disorders that require coordinated care from multiple subspecialists. Parents often struggle to coordinate care for their child's complex rare disorder. Even when they do access expert care, parents of children with VAs report high levels of stress and uncertainty. While previous research has explored parents' experiences navigating care for VAs, we know very little about how parents cope with stress together. Given the effect that dyadic coping can have on individual, couple, family, and child outcomes, we aimed to gain a better understanding of dyadic coping in the context of VAs., Procedures: We collected data using semi-structured interviews with 27 parents (13 dyads and one individual parent). Data were analyzed using dyadic thematic analysis., Results: Parents experienced stress related to medical, personal, logistical, and financial aspects of their child's healthcare. They relied on eight coping strategies: active coping, seeking emotional support, seeking informational support, cognitive avoidance, distraction, cognitive reframing, acceptance, and internalization. When analyzed together, we found evidence of five dyadic coping dynamics: collaborative, supportive, delegated, separate, and negative., Conclusion: Dyadic coping is complex and multilayered for parents of children with VAs. While the child's diagnosis is considered a shared stressor, both parents may not share preferred coping strategies. Parents of the same child may also be coping with different medical, relational/social, personal, or logistical stressors altogether. Psychosocial interventions designed to facilitate parental coping should address these complex coping dynamics., (© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Competing Interests: Declaration of Competing Interest No relevant conflicts of interest to disclose.

Objective: To identify perceived benefits, problems, facilitators, and barriers to adolescent online patient portal use., Study Design: Qualitative, semi-structured interviews with dyads of parents and adolescents with or without chronic illness. The study team purposively sampled for racial and ethnic minorities and fathers. Three team members then performed thematic analysis of the transcripts, with subsequent dyadic analysis of themes represented by related parents and adolescents., Results: We performed 102 interviews with 51 dyads of parents and adolescents (26 with chronic illness, 25 without chronic illness). Nearly all participants believed that adolescents should be permitted portal access. We identified 4 themes related to portal benefits: improves adolescent's knowledge of health; supports medical self-management and autonomy; strengthens communication and relationships; and supports parental influence. We identified 4 themes related to portal problems: misunderstanding or confusion; emotional distress; strain on relationships; and irresponsible use of portal. Facilitators of portal use included severity of illness, adolescent's curiosity, and ease of technology use. Barriers included lack of awareness or interest, complexity of information, and access difficulties. Twenty adolescents (39%) did not know they could access the portal, and 23 (45%) lacked interest in portals. Parents and adolescents seldom used the portal as a collaborative tool, and instead were engaging with the portal independently., Conclusion: Parents and adolescents perceive several benefits and problems with portal use, but many adolescents lack interest in using portals. Adolescent portals represent an underutilized resource to engage adolescents in their care., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Objectives: Patient engagement is critical for the effective development and use of artificial intelligence (AI)-enabled tools in learning health systems (LHSs). We adapted a previously validated measure from pediatrics to assess adults' openness and concerns about the use of AI in their healthcare., Study Design: Cross-sectional survey., Methods: We adapted the 33-item "Attitudes toward Artificial Intelligence in Healthcare for Parents" measure for administration to adults in the general US population (AAIH-A), recruiting participants through Amazon's Mechanical Turk (MTurk) crowdsourcing platform. AAIH-A assesses openness to AI-driven technologies and includes 7 subscales assessing participants' openness and concerns about these technologies. The openness scale includes examples of AI-driven tools for diagnosis, prediction, treatment selection, and medical guidance. Concern subscales assessed privacy, social justice, quality, human element of care, cost, shared decision-making, and convenience. We co-administered previously validated measures hypothesized to correlate with openness. We conducted a confirmatory factor analysis and assessed reliability and construct validity. We performed exploratory multivariable regression models to identify predictors of openness., Results: A total of 379 participants completed the survey. Confirmatory factor analysis confirmed the seven dimensions of the concerns, and the scales had internal consistency reliability, and correlated as hypothesized with existing measures of trust and faith in technology. Multivariable models indicated that trust in technology and concerns about quality and convenience were significantly associated with openness., Conclusions: The AAIH-A is a brief measure that can be used to assess adults' perspectives about AI-driven technologies in healthcare and LHSs. The use of AAIH-A can inform future development and implementation of AI-enabled tools for patient care in the LHS context that engage patients as key stakeholders., Competing Interests: All authors have read and approved the final manuscript and do not declare any conflicts of interest., (© 2024 The Authors. Learning Health Systems published by Wiley Periodicals LLC on behalf of University of Michigan.)

Objective: We examined how parents experience and navigate open access to clinical notes ("open notes") in their child's electronic health record and explored their interactions with clinicians during an ICU admission., Methods: We performed a qualitative analysis using semistructured interviews of English-speaking parents who accessed their child's clinical notes during a pediatric ICU (general or cardiac) admission. We included patient-parent dyads with an ICU admission ≥48 hours between April 2021 and December 2022, note access by proxy timestamp during the ICU course, and either patient age <12 years or incapacitated adolescent ages 12 to 21 years. Purposive sampling was based on sociodemographic and clinical characteristics. Phone interviews were audio-recorded, transcribed, and analyzed using inductive thematic codebook analysis., Results: We interviewed 20 parents and identified 2 thematic categories, outcomes and interactions, in parents accessing clinical notes. Themes of outcomes included applied benefits, psychosocial and emotional value, and negative consequences. Themes of interactions included practical limitations and parental approach and appraisal. The ICU context and power dynamics were a meta-theme, influencing multiple themes. All parents reported positive qualities of note access despite negative consequences related to content, language, burdens, and lack of support. Parents suggested practice and design improvements surrounding open note access., Conclusions: Parental experiences with open notes reveal new, unaddressed considerations for documentation access, practices, and purpose. Parents leverage open notes by negotiating between the power dynamics in the ICU and the uncertain boundaries of their role and authority in the electronic health record., (Copyright © 2024 by the American Academy of Pediatrics.)

Over 50% of patients with systemic LCH are not cured with front-line therapies, and data to guide salvage options are limited. We describe 58 patients with LCH who were treated with clofarabine. Clofarabine monotherapy was active against LCH in this cohort, including heavily pretreated patients with a systemic objective response rate of 92.6%, higher in children (93.8%) than adults (83.3%). BRAF V600E+ variant allele frequency in peripheral blood is correlated with clinical responses. Prospective multicentre trials are warranted to determine optimal dosing, long-term efficacy, late toxicities, relative cost and patient-reported outcomes of clofarabine compared to alternative LCH salvage therapy strategies., (© 2024 British Society for Haematology and John Wiley & Sons Ltd.)

Background: There are notable differences in how adult patients with traumatic brachial plexus injuries (BPI) are evaluated and treated., Purpose: To better understand existing philosophies, we used the Delphi method to measure and foster consensus on routine use of electrodiagnostic testing, ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT) myelograms prior to surgery. Panelists were 10 peripheral nerve surgeons board certified in their respective specialties at 5 academic medical centers in the United States., Methods: We presented 2 cases (1 complete/pan-BPI and 1 upper trunk BPI) to panelists and asked how often they would order the following preoperative diagnostic tests: electrodiagnostic studies; ultrasound of the brachial plexus; MRI of the brachial plexus; CT myelogram. Our Delphi process included an initial survey with videoconference discussion after the first round. A second survey/videoconference round was conducted to further probe the items that did not reach consensus during the first round., Results: Among the 10 surgeons, there was consensus that prior to brachial plexus surgery electrodiagnostic studies and MRI should be routinely ordered and ultrasound of the brachial plexus should not be routinely ordered. The group did not reach consensus on whether CT myelogram should or should not be routinely ordered., Conclusions: Based on our panel's discussion, future work should focus on comparing the accuracy of CT myelogram and MRI for evaluation of cervical nerve root presence and viability., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Background and Objectives: Initially developed as immunosuppressive agents, mammalian target of rapamycin (mTOR) inhibitors are currently used widely in the management of vascular malformations and tumors. The incidence of infectious complications in the vascular anomalies (VA) population is not well defined. The goal of this systematic review was to better define the types and severity of reported infectious complications in patients with VAs treated with mTOR inhibition., Methods: This was a systematic review conducted following PRISMA guidelines evaluating all research articles focused on infectious complications in patients with VAs treated with sirolimus or everolimus. Thirty articles including 1182 total patients and 316 infections (in 291 unique patients) were ultimately included., Results: The majority of infections were viral upper respiratory (n = 137, 54%), followed by pneumonia (n = 53, 20%), and cutaneous infections (n = 20, 8%). There were six total infection-related fatalities, which all occurred in patients younger than 2 years. Two cases of Pneumocystis jirovecii pneumonia (PJP) were reported. These were infants with kaposiform hemangioendothelioma (KHE) who were also treated with steroids and did not receive PJP prophylaxis. Almost one-third (n = 96, 32%) of infectious complications were graded 3-4 according to Common Terminology Criteria for Adverse Events (CTCAE) criteria. Details of patient age, subtype of VA, and timing of infection were lacking from many reports., Conclusions: Most infectious complications reported in patients with VA on mTOR inhibitors were viral respiratory infections and non-severe. Bacteremia, infectious fatalities, and PJP are exceedingly rare. Future studies are needed to clarify the spectrum of infectious risks in VA patients and to provide guidance for infection prevention., (© 2023 Wiley Periodicals LLC.)

Background: High-quality communication in pediatric and adolescent cancer is the standard of care. Yet, we lack pediatric-specific communication measures. We designed self-report and caregiver-report communication measures for use in pediatric oncology settings., Methods: We recruited adolescent and young adults (AYAs; 12-24 years) with cancer and parents of children and AYAs between 1 month post-diagnosis and 2 months after completing treatment. Participants completed measures including 58 questions addressing eight previously characterized communication functions. For each function, we conducted factor analysis, and assessed reliability and construct validity. Based on psychometric properties, we created final long-form (31 items) and short-form (eight items) communication measures (PedCOM) for both self- and caregiver-report., Results: Participants included 200 parents and 88 AYAs. The final 31-item and eight-item PedCOM measures for parents and AYAs demonstrated good unidimensional model fit. Each communication function (e.g., building relationships) demonstrated high reliability, coefficient alphas ranged from .83 to .93 for parents and .85 to .93 for AYAs. The overall 31-item and eight-item PedCOM measures also demonstrated high reliability (alphas >.9). For construct validity, PedCOM-Parent-31 and PedCOM-Parent-8 correlated positively with satisfaction with care, trust, emotional self-management, and decisional satisfaction, and negatively with anxiety and caregiver burden. PedCOM-AYA-31 and PedCOM-AYA-8 correlated positively with satisfaction with care, trust, emotional self-management, symptoms self-management, and decisional satisfaction, and negatively with anxiety., Discussion: We developed valid and reliable measures of communication functions for parents and AYAs with cancer. These measures can support organizations and stakeholder groups that are striving to improve the quality of cancer care., (© 2023 Wiley Periodicals LLC.)

Objective: To characterize the current distribution, composition, and practice patterns of multidisciplinary vascular anomalies (VAs) teams in the US., Study Design: This is a cross-sectional survey of children's hospitals in the US offering VAs care. We approached 142 children's hospitals that provided care for VAs via email. The survey evaluated VA clinic location, medical staffing, research participation, and treatments offered. The survey was administered between October 2021 and July 2022., Results: Participants from 95 eligible hospitals responded to the survey (response rate = 67%). Large areas of the Midwest and Northwest US had no available multidisciplinary VA teams or clinics. Most respondents worked at academic centers (89%), with 66% at a freestanding children's hospital, and 56% reported having a multidisciplinary clinic. Most common physician participants in clinic included hematology-oncology (91%), interventional radiology (87%), dermatology (85%), plastic surgery (81%), and otolaryngology (74%). Only 38% of programs included medical geneticists. Smaller hospitals had fewer medical and ancillary staff and offered fewer therapeutic options. Research was available at most larger institutions (69%) but less commonly at smaller hospitals (34%)., Conclusions: Major portions of the US lack multidisciplinary VA care. Furthermore, VA programs vary in composition and geneticists are absent from the majority of programs. These findings should inform efforts to address disparate access and develop standards of care for multidisciplinary VA care in the US., Competing Interests: Declaration of Competing Interest The authors declare no conflicts of interests., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Purpose: Online patient portals represent widely available communication tools in pediatric oncology. Previous studies have not evaluated clinicians' perspectives on portal use, including issues related to access to adolescents' portals., Methods: We performed semistructured interviews with physicians and advanced practice providers (APPs) who care for children or adolescents with cancer. We performed thematic analysis of benefits, problems, and accommodations related to portal use in oncology., Results: We interviewed 29 physicians and 24 APPs representing 26 institutions. Participants described five themes of benefits provided by portals: (1) empowering adolescents, (2) improving efficiency and accuracy of communication, (3) promoting open and adaptive communication, (4) supporting parents in managing care, and (5) bolstering clinical relationships. Participants described eight themes of problems caused by portal access: (1) creating emotional distress and confusion, (2) increasing workload and changing workflows, (3) threatening adolescent confidentiality, (4) adolescents lacking interest to engage, (5) diminishing clinical relationship, (6) misusing portal messages, (7) diminishing quality of sensitive documentation, and (8) parents losing access to adolescents' records. Participants described three themes related to accommodations they made as a result of portal access: (1) modifying note writing, (2) providing anticipatory guidance about viewing results, and (3) adapting workflows. Some portal functions created either benefits or problems depending on the clinical context., Conclusion: Oncologists identified benefits and problems created by portal use, which were sometimes in tension, depending on the clinical context. To make portals useful, we must take steps to mitigate risks while preserving functionality for parents and adolescent patients.

Importance: Many health care systems offer adolescents access to health information through online patient portals, but few studies have explored how to engage adolescents in using and benefiting from online portals., Objective: To determine how US children's hospitals have attempted to encourage adolescent portal use, barriers to engaging adolescents, and ideal future goals for engagement., Design, Setting, and Participants: This qualitative study performed structured qualitative interviews with informatics administrators from children's hospitals across the US between February and July 2022. Informatics administrators were employed by US health care systems that managed a children's hospital with at least 50 dedicated pediatrics beds. Data analysis was performed from November 2022 to January 2023., Main Outcomes and Measures: This study used thematic analysis of (1) current steps that health care systems had taken to engage adolescents in using their online patient portals and (2) barriers to engaging adolescents and ideal future goals and outcomes of engagement., Results: Among 58 total interviews with 65 informatics administrators who represented 63 hospitals across 58 health care systems, 6 themes of approaches to engaging adolescents in portal use were identified: (1) promoting and educating adolescents about portal enrollment, (2) establishing workflows to support enrollment, (3) seeking and incorporating feedback, (4) creating a culture or environment supporting engagement, (5) increasing portal utility, and (6) limited efforts. Barriers to engaging adolescents in portal use related to either (1) stakeholder investment, interest, and capabilities or (2) intersecting technical, ethical, and legal factors. Participants identified 4 ideal future efforts to engage adolescents: (1) develop adaptable private means of communication with adolescents, (2) use adolescent-centric user design, (3) enhance promotion and education about portal use, and (4) simplify and adapt workflows to encourage enrollment. Participants described 3 ideal outcomes of this future engagement: (1) provide education about current health, (2) prepare for transition to adulthood, and (3) improve digital health education of adolescents., Conclusions and Relevance: In this qualitative study of informatics administrators, children's hospitals across the US were found to have varying degrees of efforts to engage adolescents in using their portals. Most of these efforts focused on supporting adolescent enrollment, but fewer efforts focused on making the portal useful and interesting to adolescents.

Objective: The aim of this study was to understand the influence of clinician encouragement and sociodemographic factors on whether patients access online electronic medical records (EMR)., Materials and Methods: We analyzed 3279 responses from the Health Information National Trends Survey 5 cycle 4 survey, a cross-sectional, nationally representative survey administered by the National Cancer Institute. Frequencies and weighted proportions were calculated to compare clinical encouragement and access to their online EMR. Using multivariate logistic regression, we identified factors associated with online EMR use and clinician encouragement., Results: In 2020, an estimated 42% of US adults accessed their online EMR and 51% were encouraged by clinicians to access their online EMR. In multivariate regression, respondents who accessed EMR were more likely to have received clinician encouragement (odds ratio [OR], 10.3; 95% confidence interval [CI], 7.7-14.0), college education or higher (OR, 1.9; 95% CI, 1.4-2.7), history of cancer (OR, 1.5; 95% CI, 1.0-2.3), and history of chronic disease (OR, 2.3; 95% CI, 1.7-3.2). Male and Hispanic respondents were less likely to have accessed EMR than female and non-Hispanic White respondents (OR, 0.6; 95% CI, 0.5-0.8, and OR, 0.5; 95% CI, 0.3-0.8, respectively). Respondents receiving encouragement from clinicians were more likely to be female (OR, 1.7; 95% CI, 1.3-2.3), have college education (OR, 1.5; 95% CI, 1.1-2.0), history of cancer (OR, 1.8; 95% CI, 1.3-2.5), and greater income levels (OR, 1.8-3.6)., Discussion: Clinician encouragement of patient EMR use is strongly associated with patients accessing EMR, and there are disparities in who receives clinician encouragement related to education, income, sex, and ethnicity., Conclusions: Clinicians have an important role to ensure that all patients benefit from online EMR use., Competing Interests: None declared., (© The Author(s) 2023. Published by Oxford University Press on behalf of the American Medical Informatics Association.)

Background: After enactment of the 21st Century Cures Act, many health care systems offer adolescents and parents access to electronic health information through online portals. Few studies have evaluated adolescent portal access policies since the implementation of the Cures Act., Methods: We performed structured interviews with informatics administrators in US hospitals with ≥50 dedicated pediatric beds. We performed thematic analysis of challenges to developing and implementing adolescent portal policies., Results: We interviewed 65 informatics leaders representing 63 pediatric hospitals, 58 health care systems, 29 states, and 14 379 pediatric hospital beds. Most hospitals provided portal access to adolescents (86%) and parents (95%). Filtering of results sent to parental portals ranged widely, with 14% providing unfiltered access, 31% performing minimal filtering for sensitive information, and 43% offering limited access. Portal access policies also varied widely within states. Challenges to developing policies included legislation and compliance issues, tension between confidentiality and usefulness, clinicians' preferences and concerns, limited understanding and investment of institutions in pediatric issues, and limited focus of vendors on pediatric issues. Challenges to implementing policies included technical challenges, educating end-users, potential for parental coercion, harms of bad news, complex enrollment processes, and informatics workforce limitations., Conclusions: Adolescent portal access policies vary widely across and within states. Informatics administrators identified multiple challenges related to developing and implementing adolescent portal policies. Future efforts should strive to develop intrastate consensus on portal policies and to engage parents and adolescent patients to better understand preferences and needs., (Copyright © 2023 by the American Academy of Pediatrics.)

Effective communication is integral to patient and family-centered care in pediatric and adolescent and young adult (AYA) oncology and improving healthcare delivery and outcomes. There is limited knowledge about whether AYAs and parents have similar communication preferences and needs. By eliciting and comparing communication advice from AYAs and parents, we can identify salient guidance for how clinicians can better communicate. We performed secondary analysis of semi-structured interviews from 2 qualitative communication studies. In one study, 80 parents of children with cancer during treatment, survivorship, or bereavement were interviewed. In the second study, AYAs with cancer during treatment or survivorship were interviewed. We asked AYAs and parents to provide communication advice for oncology clinicians. Using thematic analysis, we identified categories of advice related to three overarching themes: interpersonal relationships, informational preferences, and delivery of treatment, resources, and medical care. AYAs and parents provided similar advice about the need for compassion, strong connections, hopefulness, commitment, and transparent honesty However, AYAs placed additional emphasis on clinicians maintaining a calm demeanor.

Purpose: High-quality communication is a standard of palliative care for adolescents and young adults (AYAs) with cancer. Yet, few studies have characterized the negative communication experiences of AYAs near the end of life (EOL). Methods: We performed a secondary analysis of 27 qualitative interviews with bereaved caregivers of AYAs with cancer who died between 2013 and 2016 at 1 of 3 sites. Interviews focused on barriers to optimal EOL care for AYAs. We used thematic analysis using iterative consensus coding to analyze transcripts. Results: Participants were predominantly white (85%), non-Hispanic (93%), and female (74%). Half of the participants were bereaved parents, and 37% were bereaved partners or spouses. Overall, 23/27 (85%) caregivers described at least one negative communication experience related to one of three themes: (1) Insensitivity to patients' needs, preferences, and values; (2) Insufficient discussions of prognosis and/or EOL; and (3) Loss of support from the clinical team near EOL. Both clinician- and patient-related factors contributed to limited EOL discussions. Lack of care continuity related to both clinician factors and systems of care that required new or changing clinical care teams near the EOL. Conclusions: Caregivers report a desire for clinician sensitivity to their needs and values, information about the future, and longitudinal connections with individual clinicians. Clinicians might improve caregivers' EOL experiences by eliciting patient preferences, engaging in EOL discussions, adapting to the AYA's developmental and emotional needs, and demonstrating a commitment to AYAs and caregivers as they approach the EOL.

Background: Complex vascular malformations (VMs) are rare disorders that can cause pain, coagulopathy, disfigurement, asymmetric growth, and disability. Patients with complex VMs experience misdiagnosis, delayed diagnosis, delayed or inappropriate treatments, and worsened health. Given the potential consequences of delaying expert care, we must identify the factors that impede or facilitate this access to care., Results: We performed semi-structured interviews with 24 parents (21 mothers; 3 fathers; median age = 42.5 years) of children with complex VMs and overgrowth disorders living in the US, recruited through two patient advocacy groups - CLOVES Syndrome Community, and Klippel-Trenaunay Support Group. We performed thematic analysis to assess parental perspectives on barriers and facilitators to accessing expert care. We identified 11 factors, representing 6 overarching themes, affecting families' ability to access and maintain effective care for their child: individual characteristics (clinician behaviors and characteristics, parent behaviors and characteristics), health care system (availability of specialist multidisciplinary teams, care coordination and logistics, insurance and financial issues, treatments and services), clinical characteristics (accuracy and timing of diagnosis, features of clinical presentation), social support networks, scientific progress, and luck and privilege. Additionally, access to information about VMs and VM care was a crosscutting theme affecting each of these factors. These factors influenced both the initial access to care and the ongoing maintenance of care for children with VMs., Conclusion: Parents of children with VMs report multiple factors that facilitate or impede their ability to provide their child with optimal care. These factors represent possible targets for future interventions to improve care delivery for families affected by VMs., (© 2022. The Author(s).)

Objective: We aimed to evaluate the racial and ethnic diversity of study participants in recent pediatric cancer communication literature., Methods: We systematically searched for communication studies in pediatric oncology published between January 2018 and September 2020, limiting analysis to US studies. We considered race and ethnicity as separate categories in our analysis. Two authors screened studies and abstracted characteristics of race and ethnicity reporting and enrollment., Results: Of 98 articles included in this analysis, many studies failed to report participants' race (21/98) and ethnicity (40/98). Most studies ascertained race and ethnicity by self-report (51/98); 25 studies did not describe how they ascertained race and ethnicity. White participants were overrepresented in studies relative to the US population (median 80% in studies vs 72% in 2020 US census). Racial and ethnic minorities were underrepresented (Black: 7% vs 14%; Asian: 4% vs 7%; Pacific Islander: 0% vs 0.5%; Native American: 0.5% vs 3%; Hispanic 8% vs 19%)., Conclusion: Communication literature in pediatric oncology underrepresents all racial and ethnic minority populations and is inconsistent in the reporting of race and ethnicity., Practice Implications: Future work should follow best practices to ensure this literature adequately represents the experiences of all families in pediatric oncology., (Copyright © 2021 Elsevier B.V. All rights reserved.)

ALK-positive histiocytosis is a rare subtype of histiocytic neoplasm first described in 2008 in 3 infants with multisystemic disease involving the liver and hematopoietic system. This entity has subsequently been documented in case reports and series to occupy a wider clinicopathologic spectrum with recurrent KIF5B-ALK fusions. The full clinicopathologic and molecular spectra of ALK-positive histiocytosis remain, however, poorly characterized. Here, we describe the largest study of ALK-positive histiocytosis to date, with detailed clinicopathologic data of 39 cases, including 37 cases with confirmed ALK rearrangements. The clinical spectrum comprised distinct clinical phenotypic groups: infants with multisystemic disease with liver and hematopoietic involvement, as originally described (Group 1A: 6/39), other patients with multisystemic disease (Group 1B: 10/39), and patients with single-system disease (Group 2: 23/39). Nineteen patients of the entire cohort (49%) had neurologic involvement (7 and 12 from Groups 1B and 2, respectively). Histology included classic xanthogranuloma features in almost one-third of cases, whereas the majority displayed a more densely cellular, monomorphic appearance without lipidized histiocytes but sometimes more spindled or epithelioid morphology. Neoplastic histiocytes were positive for macrophage markers and often conferred strong expression of phosphorylated extracellular signal-regulated kinase, confirming MAPK pathway activation. KIF5B-ALK fusions were detected in 27 patients, whereas CLTC-ALK, TPM3-ALK, TFG-ALK, EML4-ALK, and DCTN1-ALK fusions were identified in single cases. Robust and durable responses were observed in 11/11 patients treated with ALK inhibition, 10 with neurologic involvement. This study presents the existing clinicopathologic and molecular landscape of ALK-positive histiocytosis and provides guidance for the clinical management of this emerging histiocytic entity., (© 2022 by The American Society of Hematology.)

Purpose: Communication is essential to providing family-centered care in pediatric oncology. Previously, we developed a functional model of communication between parents and clinicians. Prior research has not examined the domains and purposes of communication between children and clinicians. We explored parental perspectives to begin understanding this communication., Methods: Secondary analysis of semi-structured interviews with 80 parents of children with cancer across 3 academic medical centers during treatment, survivorship, or bereavement. We employed semantic content analysis, using the functional model of parental communication as an a priori framework., Results: We identified 6 distinct functions of communication in child-clinician interactions: building relationships, promoting patient engagement, addressing emotions, exchanging information, managing uncertainty, and fostering hope. These communication functions were identified by parents of older (> 13 years old) and younger (< 12 years old) children, although the specific manifestations sometimes differed by age. Notably, age was not always an indicator of the child's communication needs. For example, some parents noted older children who did not want to discuss difficult topics, whereas other parent described younger children who wanted to know every detail. Two functions from the previous parental model of communication were absent from this analysis: supporting family self-management and making decisions., Conclusion: Interviews with 80 parents provided evidence for 6 distinct functions of communication between children and clinicians. These functions apply to older and younger children, although specific manifestations might vary by age. This functional model provides a framework to guide clinicians' communication efforts and future communication research., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

Background: Healthcare is expected to increasingly integrate technologies enabled by artificial intelligence (AI) into patient care. Understanding perceptions of these tools is essential to successful development and adoption. This exploratory study gauged participants' level of openness, concern, and perceived benefit associated with AI-driven healthcare technologies. We also explored socio-demographic, health-related, and psychosocial correlates of these perceptions., Methods: We developed a measure depicting six AI-driven technologies that either diagnose, predict, or suggest treatment. We administered the measure via an online survey to adults (N = 936) in the United States using MTurk, a crowdsourcing platform. Participants indicated their level of openness to using the AI technology in the healthcare scenario. Items reflecting potential concerns and benefits associated with each technology accompanied the scenarios. Participants rated the extent that the statements of concerns and benefits influenced their perception of favorability toward the technology. Participants completed measures of socio-demographics, health variables, and psychosocial variables such as trust in the healthcare system and trust in technology. Exploratory and confirmatory factor analyses of the concern and benefit items identified two factors representing overall level of concern and perceived benefit. Descriptive analyses examined levels of openness, concern, and perceived benefit. Correlational analyses explored associations of socio-demographic, health, and psychosocial variables with openness, concern, and benefit scores while multivariable regression models examined these relationships concurrently., Results: Participants were moderately open to AI-driven healthcare technologies (M = 3.1/5.0 ± 0.9), but there was variation depending on the type of application, and the statements of concerns and benefits swayed views. Trust in the healthcare system and trust in technology were the strongest, most consistent correlates of openness, concern, and perceived benefit. Most other socio-demographic, health-related, and psychosocial variables were less strongly, or not, associated, but multivariable models indicated some personality characteristics (e.g., conscientiousness and agreeableness) and socio-demographics (e.g., full-time employment, age, sex, and race) were modestly related to perceptions., Conclusions: Participants' openness appears tenuous, suggesting early promotion strategies and experiences with novel AI technologies may strongly influence views, especially if implementation of AI technologies increases or undermines trust. The exploratory nature of these findings warrants additional research., (© 2021. The Author(s).)

Communication in pediatrics is important for psychological well-being and health outcomes. However, consensus is lacking regarding the core functions of communication in pediatrics. The aims of this study were (1) to evaluate whether and how core communication functions from adult oncology apply to communication in pediatric medicine and (2) to examine whether any unique core communication functions exist in pediatric medicine. We analyzed 36 narratives written by parents (n = 23) or former patients (n = 13) describing pediatric communication experiences with clinicians that were published in Narrative Inquiry in Bioethics. Utilizing deductive and inductive coding, we evaluated narratives for evidence of core communication functions previously documented in adult oncology. We also evaluated for novel functions not represented in adult oncology. We identified the presence of all adult core communication functions in pediatric narratives. We also found evidence of a novel function, "engendering solidarity and validation." We report clinician behaviors that appeared to facilitate or impair these core functions. Core communication functions in adult oncology apply to communication in pediatric medicine, but we also identified a novel communication function in pediatrics.

Context: Cancer is a life-changing diagnosis accompanied by significant emotional distress, especially for children with advanced disease. However, the content and processes of discussing emotion in advanced pediatric cancer remain unknown., Objectives: To describe the initiation, response, and content of emotional communication in advanced pediatric cancer., Methods: We audiorecorded 35 outpatient consultations between oncologists and families of children whose cancer recently progressed. We coded conversations based on Verona Coding Definitions of Emotional Sequences., Results: About 91% of conversations contained emotional cues, and 40% contained explicit emotional concerns. Parents and clinicians equally initiated cues (parents: 48%, 183 of 385; clinicians: 49%) and concerns (parents: 51%; clinicians: 49%). Children initiated 3% of cues and no explicit concerns. Emotional content was most commonly related to physical aspects of cancer and/or treatment (28% of cues and/or concerns, present in 80% of conversations) and prognosis (27% of cues and/or concerns, present in 60% of conversations). Clinicians mostly responded to emotional cues and concerns implicitly, without specifically naming the emotion (85%). Back channeling (using minimal prompts or words that encourage further disclosure, e.g., uh-huh) was the most common implicit response that provided space for emotional disclosure (32% of all responses). Information advice was the most common implicit response that reduced space for further emotional disclosure (28%)., Conclusion: Emotional communication in advanced pediatric cancer appears to be a subtle process where parents offer hints and clinicians respond with non-emotion-laden statements. Also, children were seldom engaged in emotional conversations. Clinicians should aim to create an environment that allows families to express emotional distress if and/or when ready., (Copyright © 2019 American Academy of Hospice and Palliative Medicine. Published by Elsevier Inc. All rights reserved.)

Purpose: Parents of children with cancer make treatment decisions in highly emotional states while feeling overwhelmed with information. In previous work, 1 in 6 parents demonstrated heightened decisional regret regarding treatment at diagnosis. However, it is unclear how regret evolves over time. We aimed to determine whether parents of children with cancer experience decisional regret over time and to identify parental characteristics and clinician behaviors associated with longitudinal regret., Methods: Prospective, questionnaire-based cohort study of parents of children with cancer at two academic pediatric hospitals. Parents reported decisional regret at diagnosis, 4 months, and 12 months., Results: At baseline, 13% of parents (21/158) reported heightened regret, 11% (17/158) at 4 months (p = 0.43, McNemar's test relative to baseline), and 11% (16/158) at 12 months (p = 0.35 relative to baseline and p = 0.84 relative to 4 months). In multivariable analysis using generalized estimating equations adjusted for the time point of survey completion, heightened regret was associated with non-white race/ethnicity (OR 11.57, 95% CI 3.53 to 41.05, p < .0001) and high anxiety (OR 2.01, 95% CI 1.04 to 3.90, p = .04). Parents with high peace of mind (OR 0.24, 95% CI 0.09 to 0.62, p = .003) and those reporting high-quality information (OR 0.22, 95% CI 0.07 to 0.69, p = 0.01) had lower odds of heightened regret. We found no association between heightened regret and the time point of survey administration., Conclusions: A small, significant proportion of parents experience heightened regret throughout the first year of their child's cancer treatment; non-white parents are at higher risk. Effective communication may protect against regret.

Appropriate enrollment in early-phase clinical trials demands that potential research participants understand and appreciate critical study-related information, because discrepancies in understanding or appreciation can potentially invalidate informed consent to participate in research. Four terms were previously developed to categorize these discrepancies: therapeutic "misconception," "therapeutic misestimation," "therapeutic optimism," and "unrealistic optimism." In this article, we propose a continuous framework of therapeutic misperceptions, rather than discrete categorical concepts. One end of this continuum contains discrepancies in understanding, and at the other end are discrepancies in appreciation. Categorical terminologies represent points along this continuum. Discrepancies in understanding and appreciation each lead to unique ethical concerns and likely require different interventions. This framework highlights the dearth of empirical work on the appreciation end of the continuum, especially related to navigating persistent discrepancies in appreciation. Employing a continuous framework of therapeutic misperceptions supports a nuanced approach to the unique circumstances of each research subject, aiding researchers in supporting truly informed consent., (Copyright© 2018 The Hastings Center.)

Having children is a deeply held desire for many people. After a cancer diagnosis, fertility is often threatened by cancer treatments. The choice to pursue fertility preservation often necessitates a delay in starting cancer treatment which may negatively impact the patient. Fertility decisions are laden with heavy emotional burden and multiple ethical challenges. The narratives in this symposium provide rich emotional context, including allusions to failure, loss, guilt, fear, anger, hope, and acceptance. Given the important role of emotion in moral reasoning, exploration of these emotions supports further discussion of ethical challenges. Subsequent ethical themes include cost and unequal access, poor communication, and differing priorities between healthcare providers and patients. These stories draw us into the fearful intersection of cancer and infertility, yet they leave us with hope that many current challenges can be improved with open discussion and sensitivity to the needs of others.