IntroductionPrecision medicine has become central to pediatric oncology, with germline genomic sequencing commonly integrated into routine care. Families must interpret complex genomic findings during emotionally vulnerable periods, generating mixed reactions ranging from clarity and relief to anxiety and uncertainty. Palliative care clinicians, genetic counselors, psychologists, social workers, and oncology providers may each contribute to supporting families as they interpret and integrate these findings over the course of a child’s cancer care and beyond. Little is known about the trajectory of parental emotional and cognitive responses after receiving germline sequencing results, limiting clinicians’ ability to anticipate support needs across the cancer care continuum. This study quantitatively examines parental emotional and cognitive responses across time following disclosure of germline sequencing results in a pediatric oncology setting.MethodsParents (n = 218) self-reported sequencing-related distress, positive feelings, intrusive thoughts, certainty, and self-efficacy using validated measures at two longitudinal follow-up points after disclosure of their child’s germline test results. Outcomes were compared across germline test result types (pathogenic/likely pathogenic [P/LP], n = 31 [14%]; variants of uncertain significance [VUS], n = 86 [39%]; and negative, n = 101[46%]).ResultsParents of children receiving P/LP or P/LP+VUS results reported significantly higher distress yet greater positive feelings than parents receiving negative results. Notably, certainty and self-efficacy increased from Timepoint 1 (median 254 days following return of results) to Timepoint 2 (median 537 days). Intrusive thoughts did not significantly differ by genetic result type or change over time; however, the factors contributing to intrusive thoughts could not be determined from the current study.DiscussionThese findings provide insight into how families adapt to germline genomic information following a pediatric cancer diagnosis. As precision medicine becomes increasingly embedded in pediatric oncology, structured follow-up and communication that address families’ evolving informational and psychosocial needs are essential to ensure care that is scientifically precise, emotionally attuned, and centered on the family experience.

Fear of cancer recurrence (FCR) is a significant but understudied concern among parents of childhood cancer survivors. This study quantitatively characterized parental FCR and explored potential demographic and clinical correlates among parents of children treated for cancer. Parents (N = 192) completed the Fear of Cancer Recurrence Inventory-Parent Short Form (FCRI-Parent) and provided demographic information. Clinical variables were obtained from medical chart review. Associations between FCR and demographic or clinical variables were analyzed using t-tests, ANOVAs, and Pearson's correlations. Parents reported a mean FCR score of 18.64 (SD = 8.73), with 42.2% of parents endorsing FCR above a score of 22. Parental FCR significantly varied by parent race, education, and spirituality. Higher FCR was also significantly negatively correlated with child age, time since diagnosis, and time since treatment completion. Parents of children with central nervous system tumors or hematological malignancies endorsed significantly higher FCR compared to parents of children with solid tumors. Findings build on previously identified psychosocial needs for parents of children treated for cancer by quantitatively describing parental FCR and exploring subgroups that may be at increased risk for FCR. Tailored interventions, including strategies that support spiritual coping, may help mitigate FCR among at-risk parents.

PURPOSE:To determine whether transitioning from a two-visit consent process led by an expert consenter to a decentralized model of consenting using an educational video and a clinical care provider translated into sustained decisional satisfaction and comparable levels of understanding about genomic sequencing for cancer predisposition. MATERIALS AND METHODS:A total of 150 parents of children with cancer agreed to participate in this study approximately 4 weeks (±2 weeks) after a consent conversation for genomic sequencing using a decentralized model to assess their genetic knowledge and decision satisfaction. Results were compared with a cohort of parents (n = 121) consented with an expert consenter using a two-visit process consisting of an informational session followed by knowledge reinforcement 5 weeks (±3 weeks) after diagnosis. RESULTS:Among parents in the decentralized consent process, 94% recalled providing consent. However, 53% could not recall who conducted the consent conversation. Less than half (44%) recalled reviewing a copy of the consent form, and only 17% viewed the educational video. Overall, parents endorsed consenting to sequencing as the right choice (mean 4.4/5-point Likert) without decisional regret (1.5 of 5). Parents who consented through the decentralized process did not significantly differ in genetic knowledge from the baseline preconsent genetic knowledge assessment for the comparison sample. Only 56% of parents who consented through the decentralized process answered at least 75% of the knowledge questions correctly (P < .001), compared with 82% of parents who consented through the expert consenter model. CONCLUSION:While the decentralized consenting processes translated into lower knowledge scores, parents maintained high levels of decisional satisfaction. Further research is warranted to maximize knowledge exchange and ensure values-aligned consent in decentralized models.

Study Objectives Craniopharyngioma is a suprasellar brain tumor often associated with hypersomnia, which may be potentially due to alterations in sleep architecture. We performed cross-sectional and longitudinal assessments of sleep architecture in pediatric patients with craniopharyngioma. Methods We evaluated 94 patients (median age 9) enrolled in a clinical trial that included limited surgery with proton radiotherapy or radical surgery with observation. Nocturnal polysomnography and multiple sleep latency testing were performed at baseline and at 36 months after enrollment. Sleep architecture was compared to that of healthy children reported by Zhu et al., as well as longitudinally and across hypersomnia diagnostic groups using standard median comparison tests. Chi-squared tests assessed longitudinal changes in prevalence of hypersomnia disorders. Results Pediatric patients with craniopharyngioma exhibited 1.0% and 1.5% lower N1 and 9.21% and 9.59% lower N2 sleep (p < .001) at baseline and 36 months than healthy counterparts. N3 sleep was elevated by 11.84% and 13.47% respectively (p < .001). At 36 months, 2.63% less time was spent in REM (p = .005). Analysis of the entire cohort revealed no statistically significant longitudinal changes in sleep architecture (p > .05). However, in a subset of patients with advancing Tanner stage, N1 sleep decreased and REM sleep increased over 36 months. There were no longitudinal changes in prevalence of hypersomnia disorders (p > .05), nor differences in sleep architecture between patients with and without hypersomnia. Conclusions Children and adolescents with craniopharyngioma exhibit atypical sleep architecture following surgery, that persists with long-term follow-up. Further research is needed to determine if sleep architecture mediates hypersomnia.

BACKGROUND:Cancer-predisposing germline variants are increasingly identified and disclosed during pediatric oncology clinical care. To understand whether and how parents communicate about identified cancer predisposition syndromes (CPS) with their adolescent and young adult (AYA) children, this study qualitatively characterized the content and approach (e.g., timing) of parent-AYA communication about AYAs' CPS. PROCEDURE:AYAs with a CPS identified during clinical cancer care and their parents independently completed semi-structured interviews regarding their CPS-related communication. Interviews were completed 1.0-3.99 years following genetic test result disclosure and coded using inductive content analysis. RESULTS:Twenty-one AYAs (age 13-20 years) and 24 parents (17 mothers, 6 fathers, 1 grandmother) completed interviews. Although most parents reported repeatedly communicating with their child, several AYAs did not recall these conversations and nearly half described a one-time conversation. Parents described communicating to disclose genetic results, educate regarding cancer risk, attend to AYAs' emotions, and provide anticipatory guidance for future risk management. Parents whose AYA had a CPS conferring risk for adult-onset cancers (e.g., colon cancer) expected to continue to remind their AYA about cancer surveillance well into adulthood. Parents and AYAs described their CPS-related communication as "light," prompted by the AYAs' medical appointments and questions, and impacted by parents' understanding of the CPS. CONCLUSIONS:Our findings highlight areas for targeted genetic education and support. Parents may benefit from screening to ascertain their CPS understanding, education regarding AYAs' CPS-related information and emotional support needs, and support for transitioning AYAs to independently manage CPS-related health needs. TRIAL REGISTRATION:ClinicalTrials.gov identifier: NCT04848142.

BackgroundPrecision-based approaches are reshaping healthcare by tailoring assessments and therapies to a patient's individual needs. To ensure nursing remains integral to this evolving landscape, active collaboration with multidisciplinary teams is essential. Nurses must understand the clinical implications of genomic testing, be provided educational opportunities, and understand the patient and parent perspectives of precision-based care. Thus, the nurse is empowered to translate research into advancing patient-centered care.ObjectiveThis review presents our multidisciplinary team's approach to understanding the educational needs of nurses and physicians, as well as patient and parent perspectives of precision-based care, along with key nursing considerations.MethodThis is a process paper describing one institution's journey through in-depth inquiries identifying knowledge gaps related to genomic testing of tumor and germline tissues, consenting, pharmacogenetics, and gene therapy. We also explored expectations held by patients and their families surrounding testing and return of results.ResultsOur findings offer key insights, including the need for education of healthcare providers, equitable patient education and access to pharmacogenomic and cancer genomic testing, ethical complexities within the consenting process, and perspectives from parents and patients on clinical genomics and gene therapy for sickle cell disease.ConclusionThe findings from this institution's efforts may help guide or inform other institutions working to deliver precision-based oncology and hematology care. Nurses must be equipped with expertise to advocate effectively for patients and families. In doing so, they can guide them through the opportunities and challenges of precision-based care.

Survivors of pediatric cancer are at high risk of long-term cognitive dysfunction. This problem is exacerbated as this population often reports poor sleep quality, which can negatively affect well-being. Sleep-related brain activity is known to influence cognitive development by regulating memory consolidation and cognitive functioning. However, the differences in sleep-related brain activity between healthy controls and pediatric cancer survivors are unclear. This review examines how sleep disturbances and cognitive impairments converge in survivors of childhood cancer, and proposes electrophysiology as a possible mechanistic bridge between these domains. We synthesize what is known from the general population and pediatric samples, highlight the critical need to investigate electrophysiological anomalies in pediatric cancer survivors, and suggest that neuromodulation techniques such as transcranial direct stimulation, transcranial magnetic stimulation, and sensory stimulation may be viable therapeutic interventions to enhance both sleep quality and cognitive function once possible anomalies are characterized in this population. Our review emphasizes the need for additional research to understand the complex relationship between sleep and cognition in pediatric cancer survivors to improve patient care and quality of life.

Study Aim: Patients with high-risk Ewing sarcoma (ES) have dismal outcomes despite aggressive multimodal therapy. This phase II, single-institution study evaluated the response rate to two up-front cycles of irinotecan, temozolomide, and temsirolimus (ITT) and assessed the tolerability of maintenance therapy following standard treatment in high-risk ES. Methods: Eligible patients had newly diagnosed high-risk ES (age ≥14 years old, metastatic disease, or primary pelvic tumor). The therapy included two cycles of window therapy (ITT) followed by interval-compressed chemotherapy (vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide) and maintenance therapy (cyclophosphamide, sorafenib, and bevacizumab). A two-stage sequential design was employed to assess a >50% WHO response (CR or PR) with 80% power. Patients who required emergent radiation were excluded from receiving window therapy. Results: Sixteen patients (median age 12.2 years; range 4.8–23.6 years) were enrolled (12 evaluable for overall response, 10 for primary tumor response). Only three achieved a PR to window therapy, leading to study closure. All evaluable patients demonstrated a decline in their primary tumor volume (mean decline: 32.5%, standard deviation: 17.6%, p-value: 0.0005) and SUV peak (mean decline: 49.9%, standard deviation: 21.1%, p-value: 0.002). Maintenance therapy was well tolerated, with only 2/13 patients discontinuing due to toxicity. Conclusions: ITT did not achieve the prespecified response rate of 50%, according to WHO criteria; however, all patients exhibited decreased volume and metabolic activity, highlighting the limitations of conventional response assessments. Maintenance therapy was feasible and well tolerated. Although limited by small sample size, heterogeneous disease presentations, and the absence of a control arm, this study supports further evaluation of ITT and a maintenance approach in larger, randomized trials for high-risk ES.

Sleep is a neurophysiologic and behavioral state essential for wellness. Pediatric cancer survivors are at elevated risk of developing sleep problems due to cancer and/or treatment-related factors, but their sleep health is understudied. We used unsupervised clustering to identify sleep health profiles in survivors and controls and evaluated differences in emotional function across sleep groups. Long-term survivors (> 5 years from diagnosis) of Hodgkin lymphoma (n=224, mean±SD age=40±9.5) and matched community controls (n=184, age=38±11.4) completed sleep questionnaires. Following the SATED model, sleep health components were derived from PSQI responses indicating Satisfaction, Alertness, Timing, Efficiency, and Duration. Continuous sleep metrics were computed as Euclidean distances of associated PSQI items, and Latent Profile Analysis identified sleep profiles. Parametric bootstrapped likelihood ratio tests estimated the number of profiles and robustness was assessed using Adjusted Rand Index. The health-related quality-of-life (SF36) and Brief Symptom Inventory (BSI-18) evaluated emotional function, and ANOVAs, t-tests, and χ2 assessed group differences. Three sleep profiles were identified: good-sleepers (PSQI=3.6±1.8), average-sleepers (PSQI=6.9±2.9), and poor-sleepers (PSQI=10.2±3.2). Profile distribution was significantly different between survivors and controls (χ2=19.2, p< 0.001) with a larger proportion of survivors in the poor-sleeper profile (33.9%vs15.2%). Average-sleepers had worse Alertness than good-sleepers, with no difference compared to poor-sleepers (F (2,405)=68.2,p< 0.001). Good-sleepers had better Timing than poor-sleepers, with no difference compared to average sleepers (F(2,405)=30.1,p< 0.001). Differences between all three profiles were found in Satisfaction (F(2,405)=85.7,p< 0.001), Efficiency (F(2,405)=110.7,p< 0.001), and PSQI disturbances (F(2,405)=71.4,p< 0.001), but no differences in Duration (F(2,405)=1.4,p=0.237). Considering quality-of-life, survivors have worse bodily pain, general health, and physical and role-physical limitations compared to controls. Poor-sleepers survivors reported worse mental (t(79)=-2.8,p< 0.001) and emotional health (t(78)=-2.5,p< 0.001). No sleep profile evidenced Survivor vs Control differences for depression or anxiety symptoms. Distinctive sleep health profiles were found in pediatric Hodgkin lymphoma survivors and community controls. Survivors reported more pain, poorer general health, and more physical and role-physical limitations, while poor-sleepers exhibited worse mental and emotional health compared to controls. We highlight the importance of understanding the dimensions of sleep health to inform targeted interventions focused on improving overall well-being in pediatric cancer survivors. NCI-NCI-NIH:1R01CA215405,T32CA225590;SRSF-Mentored-Collaboration-Grant(2024)

Treatment of craniopharyngioma, a rare brain tumor, may compromise sleep-regulating structures such as the suprachiasmatic nuclei and lateral hypothalamus. Patients commonly experience excessive sleep fragmentation, daytime sleepiness, and central disorders of hypersomnolence (CDH). This study objectively characterizes sleep health among pediatric craniopharyngioma patients by comparing nighttime actigraphy metrics to National Sleep Foundation age-based consensus guidelines for sleep duration (2014) and quality (2017). Study participants were pediatric craniopharyngioma patients enrolled in an institutional phase II proton therapy trial. Participants wore actigraphy for at least 3 nights and parents completed sleep diary data. Variables included total time in bed, total sleep time (TST), sleep efficiency (SE), sleep onset latency (SOL), and wake after sleep onset (WASO). CDH were diagnosed by standard AASM guidelines. Differences between ages, pubertal status, CDH status, hypothalamic tumor involvement, and BMI z-score were examined. Data from 65 patients ages 1-19 years (mean = 8.95±4.55) were included, 67.7% white, 50.8% female. Mean overnight TST was inappropriately short among toddlers (7.11 hours) and preschoolers (7.96 hours). School-age children (7.86 hours) and teenagers (7.28 hours) also slept less than the minimum recommended duration for their age. SE was within appropriate ranges for all age groups except preschoolers (84.02%) regardless of sleep disorder status and school age children with narcolepsy (83.62%). SOL was longer than appropriate among preschoolers (39.45 minutes) and school-aged children (31.69 minutes) overall, and among school-aged (37.73 minutes) and teenage (31.46 minutes) patients with narcolepsy. Mean WASO durations were longer than recommended for all age groups in our sample (range 50.05-91.10 minutes). Pediatric craniopharyngioma patients in our sample did not achieve recommended TST, SOL, or WASO durations in certain age groups. Preschoolers overall and school-age children with narcolepsy did not achieve recommended values by any metric assessed. Future studies including assessment of daytime sleep are warranted to further characterizing sleep health in pediatric craniopharyngioma. This work was funded by a St. Jude Auxiliary Cancer Center Support Grant for National Cancer Institute-designated cancer centers (CA21765) from the National Cancer Institute and by the ALSAC; American Lebanese Syrian Associated Charities. Alice Bai was supported by R25CA23944 from the National Cancer Institute.

ABSTRACT Purpose To assess the level of moral distress (MD) and perceptions of ethical climate among pediatric hematology/oncology (PHO) nurses and to identify bioethics topics where increased education was desired. Methods In this cross‐sectional study, we administered the 26‐item Swedish Moral Distress Scale‐Revised (sMDS‐R), specifically revised and validated for pediatric oncology, in conjunction with the Clinical Ethics Needs Assessment Survey (CENAS). Electronic surveys were sent to inpatient and outpatient PHO nurses. Analysis included descriptive statistics, Spearman correlation, and the Mann–Whitney U test. Results Of 123 nurse respondents, the overall mean MDS‐R score was 2.75 (range: 0–16). Distressing encounters occurred infrequently (frequency mean 0.96, range: 0–4), resulting in relatively low distress (mean 2.24, range: 0–4). The scenarios resulting in the highest overall MD included performing painful procedures on children (2.46), lack of meaningful conversations due to time constraints (2.41), and poor team communication (2.68). Inpatient nurses reported higher levels of MD compared to outpatient nurses ( p = 0.0002). The overall CENAS score was greater than 3 (range: 1–4), suggesting a positive ethical climate. There was a moderate negative relationship between sMDS‐R combined scores and CENAS scores ( p < 0.0001). Inpatient nurses reported a less positive ethical climate compared to outpatient nurses ( p = 0.01). Nurses expressed a need for additional ethics education around withholding/withdrawing of life‐sustaining treatments, providing end‐of‐life care, and working with challenging patients/families. Conclusions Although the ethical climate was overall positive, several clinical situations appear to result in higher moral distress despite their infrequency, especially on inpatient units. Additional resources and education in navigating ethical dilemmas were requested by the nurses.

Background: Oral mucositis is a significant and common toxicity experienced by patients who receive high-dose chemotherapy as a preparatory regimen for a hematopoietic cell transplant (HCT). Photobiomodulation (PBM) has been found to be feasible with significant efficacy in preventing the progression of oral mucositis in adult patients undergoing HCT. The purpose of this study was to determine the feasibility and efficacy of PBM in pediatric oncology patients undergoing HCT. Method: Forty children and adolescents admitted to the transplant unit for an allogeneic HCT for acute lymphoblastic leukemia or acute myeloid leukemia were treated daily at six sites until day + 20 or engraftment. Results: There were 1,035 patient encounters, with successful treatment of four or more sites during 979 patient encounters for a feasibility 93.3% CI [0.926, 0.039]. We had estimated a meaningful effect size of 20% for PBM and estimated 51% of patients treated with PBM would have at least one day or more of Grade 3 mucositis. The rate of patients who received PBM and developed Grade 3 mucositis was 20% CI [0.091, 0.356]. Patients treated with PBM had fewer days of hospitalization ( p = .009) and less severe mucositis in comparison to the matched control group ( p = .03). Conclusion: PBM is feasible and effective in preventing and treating oral mucositis and is now supported by the Children's Oncology Group for prevention and treatment of oral mucositis in patients undergoing an allogeneic HCT or receiving head/neck radiation.

Objective: The aim of this study was to describe fatigue, health-related quality of life (HRQOL) and brain tumor-associated symptoms after surgical resection and during proton radiotherapy, using latent class analysis (LCA), and to determine if there is class membership change among pediatric patients with craniopharyngioma. Methods: For all patients (n = 92), demographic and disease-related/clinical variables were attained, and patient reported outcomes were collected prior to proton therapy, at week three, and at the completion of proton therapy. The mean scores for fatigue, HRQOL, and brain tumor symptoms were compared over time and profiles were identified. Factors that influenced profile status and transition probability were examined. Results: Fatigue, HRQOL, and brain tumor symptoms improved over time during proton therapy; however, a subset remained in the lower profile, profile 1, associated with increased internalizing behaviors, compared to profile 2. Conclusions: Future study should explore the bidirectional relationship of sleep, worry and anxiety in the context of ongoing radiotherapy.

Background: Genomic testing is an increasingly important technology within pediatric oncology that aids in cancer diagnosis, provides prognostic information, identifies therapeutic targets, and reveals underlying cancer predisposition. However, nurses lack basic knowledge of genomics and have limited self-assurance in using genomic information in their daily practice. This single-institution project was carried out at an academic pediatric cancer hospital in the United States with the aim to explore the barriers to achieving genomics literacy for pediatric oncology nurses. Method: This project assessed barriers to genomic education and preferences for receiving genomics education among pediatric oncology nurses, nurse practitioners, and physician assistants. An electronic survey with demographic questions and 15 genetics-focused questions was developed. The final survey instrument consisted of nine sections and was pilot-tested prior to administration. Data were analyzed using a ranking strategy, and five focus groups were conducted to capture more-nuanced information. The focus group sessions lasted 40 min to 1 hour and were recorded and transcribed. Results: Over 50% of respondents were uncomfortable with or felt unprepared to answer questions from patients and/or family members about genomics. This unease ranked as the top barrier to using genomic information in clinical practice. Discussion: These results reveal that most nurses require additional education to facilitate an understanding of genomics. This project lays the foundation to guide the development of a pediatric cancer genomics curriculum, which will enable the incorporation of genomics into nursing practice.

Schizophrenia (SCZ) and schizoaffective disorder (SHZ) are psychiatric disorders commonly identified in individuals in their late adolescence or early adulthood. Comorbidities are common, though a concurrent diagnosis of leukemia, one of the most frequently occurring cancers of adolescence, has not yet been described in such cases. This case study outlines the clinical presentation, course, and treatment response of two 17-year-old male adolescents whose psychotic disorders complicated their leukemia treatment. The first patient was diagnosed with leukemia and subsequently with SCZ while undergoing leukemia treatment. The second patient was diagnosed with SHZ prior to the onset of leukemia. The case study will follow the methodology of Robert E. Stake (Abma & Stake, 2014), as the two cases share a leukemia diagnosis and the reported mental health impact connected with cancer-directed treatment. Early identification and treatment are critical for both psychotic disorders and cancers, often impacting the long-term prognosis. However, when co-occurring, their interplay can present unique challenges to care.

Germline genomic sequencing is increasingly integrated into pediatric cancer care, with pathogenic cancer-predisposing variants identified among 5–18