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IntroductionCraniopharyngioma is a brain tumor arising in the region of the hypothalamic-pituitary axis. Children and adolescents with craniopharyngioma have high survival rates, but often experience significant morbidity, including high rates of sleep disorders. Vulnerabilities to circadian disruption are present in this population, but little is known about circadian health.MethodsWe present exploratory circadian findings from a prospective trial at a single center. Data presented here are from the baseline timepoint. Fifty-three patients between the ages of 7 and 20 years provided salivary melatonin samples, following surgical resection and prior to completion of proton therapy, when indicated. We estimated dim light melatonin onset (DLMO) and collected additional sleep data from actigraphy, overnight polysomnography, and the multiple sleep latency test.ResultsAlmost half of participants did not have a valid DLMO estimate during the sampling window, with most being above the threshold at the first sample timepoint. Those with greater disease severity variables (greater hypothalamic involvement and the presence of diabetes insipidus) were significantly more likely to have missed DLMO. For those with valid estimates, DLMO timing correlated with BMI and other sleep variables, including mean sleep latency values on the MSLT.DiscussionThese findings suggest that a subset of those with pediatric craniopharyngioma may experience a phase advance and that this may relate to poorer prognostic indicators. Furthermore, circadian timing correlates with other sleep and health factors. Further research with earlier sampling is needed to better understand circadian rhythms in pediatric craniopharyngioma and associations with other health and disease variables.

Pediatric oncology patients are increasingly being offered germline testing to diagnose underlying cancer predispositions. Meanwhile, as understanding of variant pathogenicity evolves, planned reanalysis of genomic results has been suggested. Little is known regarding the types of genomic information that parents and their adolescent children with cancer prefer to receive at the time of testing or their expectations around the future return of genomic results.Parents and adolescent children with cancer eligible for genomic testing for cancer predisposition were surveyed regarding their attitudes and expectations for receiving current and future germline results (ClinicalTrials.gov Identifier: NCT02530658).All parents (100%) desired to learn about results for treatable or preventable conditions, with 92.4% wanting results even when there is no treatment or prevention. Parents expressed less interest in receiving uncertain results for themselves (88.3%) than for their children (95.3%). Most parents (95.9%) and adolescents (87.9%) believed that providers have a responsibility to share new or updated germline results indefinitely or at any point during follow-up care. Fewer parents (67.5%) indicated that they would want results if their child was deceased: 10.3% would not want to be contacted, 19.3% were uncertain.Expectations for return of new or updated genomic results are high among pediatric oncology families, although up to one third of parents have reservations about receiving such information in the event of their child's death. These results underscore the importance of high-quality pre-and post-test counseling, conducted by individuals trained in consenting around genomic testing to elicit family preferences and align expectations around the return of germline results.

OBJECTIVE Youth with craniopharyngioma experience weight gain, fragmented sleep, excessive daytime sleepiness (EDS), fatigue, and psychosocial problems that negatively impact their overall health-related quality of life (HRQoL). Greater hypothalamic tumor involvement (HI) may be associated with higher rates or severity of these impairments; however, the direct and indirect impact of HI on the physical and psychosocial consequences associated with pediatric craniopharyngioma remain unclear. The purpose of the current study was to examine relations between HI, body mass index (BMI), fragmented sleep, EDS, fatigue, psychosocial problems, and HRQoL among youth with craniopharyngioma. METHODS Eighty-four youth with craniopharyngioma (Mage = 10.27 ± 4.3 years, 53.6% female, 64.3% White) were assessed with actigraphy, nocturnal polysomnography, and multiple sleep latency tests prior to proton therapy, when indicated. Caregivers completed measures of fatigue, psychosocial functioning, and HRQoL. RESULTS Hypothalamic tumor involvement was associated with greater BMI (Est. = 2.97, p = 0.003) and daytime sleepiness (Est. = 2.53, p = 0.01). Greater fatigue predicted more psychosocial problems (Est. = 0.29, p

To examine self-reported (30-day) sleep versus nightly actigraphy-assessed sleep concordance in long-term survivors of childhood cancer. Four hundred seventy-seven participants enrolled in the St. Jude Lifetime Cohort (53.5% female, median (range) age 34.3 (19.3–61.6) years, 25.4 (10.9–49.3) years from diagnosis) completed the Pittsburgh Sleep Quality Index and ≥ 3 nights of actigraphy. Participants had neurocognitive impairment and/or a self-reported prolonged sleep onset latency (SOL). Self-reported 30-day sleep and nightly actigraphic sleep measures for sleep duration, SOL, and sleep efficiency (SE) were converted into ordinal categories for calculation of weighted kappa coefficients. General linear models estimated associations between measurement concordance and late effects. Agreements between self-reported and actigraphic measures were slight to fair for sleep duration and SOL measures (kw = 0.20 and kw = 0.22, respectively; p

PURPOSE Clinical genomic testing is increasingly being used to direct pediatric cancer care. Many centers are interested in offering testing of tumors and paired germline tissues at or near the time of cancer diagnosis. We conducted this study to better understand parent preferences surrounding timing and content of consent conversations for clinical germline genetic testing of their children with cancer as a part of real-time cancer care. PATIENTS AND METHODS A seven-question survey developed by the Division of Cancer Predisposition and collaborators at St Jude Children's Research Hospital (St Jude) was distributed to members of the St Jude Patient Family Advisory Council, which included parents of childhood cancer survivors and bereaved parents whose children with cancer had died. Parents were asked to provide free text comments after each question. Qualitative methods were used to derive codes from parent comments, and survey results were depicted using descriptive statistics. RESULTS The survey was completed by 172 parents. Ninety-three (54%) endorsed an approach for consent conversations ≥ 1 month after cancer diagnosis, whereas 58 (34%) endorsed an approach at 1-2 weeks and 21 (12%) at 1-2 days. Needing time to adjust to a new or relapsed cancer diagnosis and feeling overwhelmed were frequent themes; however, parents acknowledged the urgency and importance of testing. Parents desired testing of as many cancer-related genes as possible, with clinical utility the most important factor for proceeding with testing. Most parents (75%) desired germline results to be disclosed in person, preferably by a genetic counselor. CONCLUSION Parents described urgency and benefits associated with germline testing, but desired flexibility in timing to allow for initial adjustment after their child's cancer diagnosis.

Clinical advancement programs (CAPs) benefit direct care nursing staff, patients, and healthcare institutions. As standards in nursing practice and workforce evolve, changes in CAPs are necessary. In this quality improvement project, nursing leadership and a 15-member nurse committee redesigned a CAP using Benner's Novice to Expert Model. Changes included self-selection of specialty tracks by shadowing senior nurses in direct patient care, education, and leadership. CAPs increase satisfaction, collaboration, and generation of innovative knowledge.

PURPOSE Brentuximab vedotin, an effective anti-CD30 antibody-drug conjugate approved for use in adults with classical Hodgkin lymphoma (HL), was introduced in this frontline trial to reduce prescribed radiation in children and adolescents with classical HL. METHODS Open-label, single-arm, multicenter trial for patients (age ≤ 18 years) with stage IIB, IIIB, or IV classical HL was conducted. Brentuximab vedotin replaced each vincristine in the OEPA/COPDac (vincristine, etoposide, prednisone, and doxorubicin/cyclophosphamide, vincristine, prednisone, and dacarbazine) regimen according to GPOH-HD2002 treatment group 3 (TG3); two cycles of AEPA and four cycles of CAPDac. Residual node radiotherapy (25.5 Gy) was given at the end of all chemotherapy only to nodal sites that did not achieve a complete response (CR) at the early response assessment (ERA) after two cycles of therapy. Primary objectives were to evaluate the safety and efficacy (complete remission at ERA) of this combination and the 3-year event-free (EFS) and overall survival (OS). The trials are registered at ClinicalTrials.gov (identifier: NCT01920932 ). RESULTS Of the 77 patients enrolled in the study, 27 (35%) achieved complete remission at ERA and were spared radiation. Patients who were irradiated received radiation to individual residual nodal tissue. At a median follow-up of 3.4 years, the 3-year EFS was 97.4% (SE 2.3%) and the OS was 98.7% (SE 1.6%). One irradiated patient experienced disease progression at the end of therapy and now remains disease free more than 6 years following salvage therapy, and one unexpected death occurred. Only 4% of patients experienced grade 3 neuropathy. CONCLUSION The integration of brentuximab vedotin in the frontline treatment of pediatric high-risk HL is highly tolerable, facilitated significant reduction in radiation exposure, and yielded excellent outcomes.

Background Parents of children newly diagnosed with cancer have increased anxiety, depression, and numerous questions. Many of these concerns can only be answered by parents who have undergone a similar experience. Local problem Our institution lacked a formal parent-to-parent mentoring program for parents of children newly diagnosed with cancer. Methods The Parent Family Advisory Council (PFAC) recommended implementation of a formalized program that would promote a structured approach to mentoring, with a formal referral process and designated parent mentors who had been vetted, trained, and supported by the institution. Intervention In 2010, a pilot parent-to-parent mentoring program was initiated. Results Since the programs initiation, 4062 encounters have been documented. This represents an annual average of 72 mentees paired with 20 mentors, averaging 677 annual encounters. Parents view the mentoring relationship as positive and report that they feel understood, less anxious, and less isolated. Conclusion We show that a parent-to-parent mentoring program is feasible and sustainable over time. Our model is now transitioning from a staff referral to self-referral, as well as incorporating real-time mentoring into the new commons area for patients and their families. A formal volunteer mentoring program can assist parents in navigating the trajectory of childhood cancer, decreasing isolation.

Craniopharyngioma is a histologically benign brain tumor that arises in the suprasellar region affecting critical neurovascular structures including the hypothalamic-pituitary-adrenal axis and optic pathways. Children with craniopharyngioma often experience excessive daytime sleepiness which may be compounded by anxiety and depression. The current study investigated disparate sleep profiles to better understand psychological adjustment among children diagnosed with craniopharyngioma. Method: Actigraphs recorded nightly sleep data, including measures of sleep onset latency and wake after sleep onset, in a cohort of 80 youth between the ages of 2 and 20 years (median age = 9). Parent reports of behavioral and emotional functioning were included in the analysis. A latent profile analysis examined disparate sleep profiles, and a multinomial logistic regression examined differences of anxiety and depression among the sleep profiles. Results: The latent profile analysis revealed three sleep profiles: "variable sleepers" (48.3%), "consistently poor sleepers" (45.4%), and "night wakers" (6.4%). Consistently poor sleepers had lower rates of anxiety (g = .76; p = .009) and depression (g = .81; p = .003) than variable sleepers and had significantly lower rates of anxiety than night wakers (g = .52; p = .05); all other differences were nonsignificant (ps > .05). Discussion: Youth with craniopharyngioma who have nightly variations in sleep may have worse psychological functioning than those with more consistent, albeit poor, sleep patterns. Patients with craniopharyngioma who report variable sleep should be assessed for anxiety and depression to prevent and intervene on emotional difficulties that may be reciprocally related to sleep.

PURPOSEFor the advances of pediatric oncology next-generation sequencing (NGS) research to equitably benefit all children, a diverse and representative sample of participants is needed. However, little is known about demographic and clinical characteristics that differentiate families who decline enrollment in pediatric oncology NGS research.METHODSDemographic and clinical data were retrospectively extracted for 363 pediatric patients (0-21 years) with cancer approached for enrollment in Genomes for Kids (G4K), a study examining the feasibility of comprehensive clinical genomic analysis of tumors and paired normal samples. Demographic and clinical factors that significantly differentiated which families declined were subsequently compared, for 348 families, with enrollment in Clinical Implementation of Pharmacogenetics (PG4KDS), a pharmacogenomics study with more explicit therapeutic benefit examining genes affecting drug responses and metabolism.RESULTSFifty-three families (14.6%) declined enrollment in G4K. Race/ethnicity was the only variable that significantly differentiated study refusal according to multivariable logistic regression, with families of black children more likely to decline enrollment compared with families of non-Hispanic or Hispanic white children. Reasons for declining G4K were generally consistent with other pediatric genomics research: feeling overwhelmed and insurance discrimination fears were most frequently cited. Families of black children were also more likely to decline enrollment in PG4KDS. Thirteen (3.7%) of the 348 families approached for both studies declined PG4KDS.CONCLUSIONRace/ethnicity differentiated study declination across two different pediatric oncology genomics studies, suggesting enrollment disparities in the context of pediatric oncology genomics research. Genomics research participant samples that do not fully represent racial and ethnic minorities risk further exacerbating health disparities. Additional work is needed to understand the nuances of parental decision making in genomic research and facilitate enrollment of diverse patient populations.

To describe existing guidance for qualifications of principal investigator s (PI s) of human subjects research and explore how they are operationalized for pediatric nurse scientists and clinical nurses in children's hospitals.After reviewing federal regulations, accreditation guidelines, and the literature, a convenience sample of members of the National Pediatric Nurse Scientist Collaborative (NPNSC). Participants completed a 33-item survey that included questions about Institutional Review Board (IRB), guidelines, and policies for PI status at their affiliated children's hospitals.The survey was electronically disseminated to 179 members of NPNSC through the Collaborative's listserv. Of the 39 members who responded, 90% hold a PhD and 80% practice in a free-standing children's hospital, nearly all of which (93%) are recognized as Magnet® hospitals. While the majority of respondents indicated that nurse scientists and other nurses were allowed to be PIs of research studies, educational requirements for PI status varied, with 3% requiring a PhD, 15% a baccalaureate degree, and 10% a graduate degree. 54% of respondents reported there was no degree requirement for PI status; however15% reported that even doctorally prepared nurse scientists cannot serve as PIs of research studies at their affiliated children''s hospitals.The survey identified substantial variability in requirements for PI status and potential barriers to pediatric nurses conducting independent research as PIs at children's hospitals.Operationalizing existing guidance will expand inclusion of nurse scientist expertise in human subjects research.

Craniopharyngioma is a histologically benign brain tumor that arises in the suprasellar region affecting critical neurovascular structures including the hypothalamic-pituitary-adrenal axis and optic pathways. Children with craniopharyngioma often experience excessive daytime sleepiness (EDS) which may be compounded by anxiety and depression. The current study investigated disparate sleep profiles to better understand psychological adjustment among children diagnosed with craniopharyngioma. Method Actigraphs recorded nightly sleep data, including measures of sleep onset latency (SOL) and wake after sleep onset (WASO), in a cohort of 80 youth between the ages of 2 and 20 years (median age = 9). Parent reports of behavioral and emotional functioning were included in the analysis. A latent profile analysis examined disparate sleep profiles, and a multinomial logistic regression examined differences of anxiety and depression among the sleep profiles. Results The latent profile analysis revealed three sleep profiles: "healthy sleepers" (68.8%), "night wakers" (21.3%), and "prolonged onset sleepers" (10.0%). Contrary to expectations, sleep profiles were not associated with daytime anxiety (β = 2.26-4.30, p > .05) or depression (β = -5.87-4.74, p > .05). Conclusions Youth with craniopharyngioma demonstrate poor sleep and EDS. Those with delayed SOL and prolonged WASO are particularly vulnerable to disrupted nighttime sleep, which may significantly compound EDS. Disrupted sleep was not associated with anxiety or depression, which may be related to the overall poor sleep and daytime sleepiness or to timing, as patients were early in their treatment course. Further study should evaluate the factors underlying sleepiness and daytime function in patients with craniopharyngioma.

Background Adult survivors of childhood cancer are at risk of developing sleep and neurocognitive problems, yet few efficacious interventions exist targeting these prevalent late effects. Melatonin has known sleep-promoting effects; however, it has not been well studied among childhood cancer survivors. Method Survivors (n = 580; mean age = 33.5 years; 26 years post-diagnosis) from the St. Jude Lifetime Cohort were randomized (1:1) to a six-month double-blind placebo-controlled trial of 3 mg time-release melatonin within three strata (stratum 1: neurocognitive impairment only; stratum 2: neurocognitive and sleep impairment; stratum 3: sleep impairment only). Neurocognitive performance was assessed at baseline and post-intervention using standardized measures. Sleep was assessed via self-report and actigraphy. Independent sample t tests compared mean change scores from baseline to six months. Post-hoc analyses compared the prevalence of clinically significant treatment responders among melatonin and placebo conditions within and across strata. Results Intent-to-treat analyses revealed no statistically significant differences in neurocognitive performance or sleep from baseline to post-intervention. However, among survivors with neurocognitive impairment only, a larger proportion randomized to melatonin versus placebo demonstrated a treatment response for visuomotor speed (63% vs 41%, P = 0.02) and nonverbal reasoning (46% vs 28%, P = 0.04). Among survivors with sleep impairment only, a larger proportion treated with melatonin demonstrated a treatment response for shifting attention (44% vs 28%, P = 0.05), short-term memory (39% vs 19%, P = 0.01), and actigraphy-assessed sleep duration (47% vs 29%, P = 0.05). Conclusion Melatonin was not associated with improved neurocognitive performance or sleep in our intent-to-treat analyses; however, a subset of survivors demonstrated a clinically significant treatment response.

Objectives To qualitatively describe parent perspectives of next-generation genomic sequencing (NGS) for their children with cancer, including perceived benefits, risks, hopes/expectations, and decision-making process when consenting or not consenting to NGS and prior to result disclosure. Data Sources Qualitative interviews were used. Conclusion Altruism is an important factor in parents consenting to NGS testing, as well as making sense of their child's cancer and legacy building. Parents described realistic hopes and expectations associated with NGS participation. Although parents endorsed the likelihood of no medical benefit, those consenting to NGS felt there was no reason not to participate. Parents declining participation expressed avoidance of worry and parent guilt if a germline variant were to be disclosed. Implications for Nursing Practice As NGS evolves into a component of the routine diagnostic workup for pediatric cancer patients, genetic nurses play a role in conducting informed consent conversations and ensuring that patients and families have realistic hopes and expectations associated with NGS.

Background Workplace burnout can result in negative consequences for clinicians and patients. We assessed burnout prevalence and sources among pediatric hematology/oncology inpatient nurses, ambulatory nurses, physicians (MDs), and advanced practice providers (APPs) by evaluating effects of job demands and involvement in patient safety events (PSEs). Methods A cross-sectional survey (Maslach Burnout Inventory) measured emotional exhaustion, depersonalization, and reduced personal accomplishment. The National Aeronautics and Space Administration Task Load Index measured mental demand, physical demand, temporal demand, effort, and frustration. Relative weights analyses estimated the unique contributions of tasks and PSEs on burnout. Post hoc analyses evaluated open-response comments for burnout factors. Results Burnout prevalence was 33%, 20%, 34%, and 33% in inpatient nurses, ambulatory nurses, and MD, and APPs, respectively (N = 481, response rate 69%). Reduced personal accomplishment was significantly higher in inpatient nurses than MDs and APPs. Job frustration was the most significant predictor of burnout across all four cohorts. Other significant predictors of burnout included temporal demand (nursing groups and MDs), effort (inpatient nurses and MDs), and PSE involvement (ambulatory nurses). Open-response comments identified time constraints, lack of administrator support, insufficient institutional support for self-care, and inadequate staffing and/or turnover as sources of frustration. Conclusions All four clinician groups reported substantial levels of burnout, and job demands predicted burnout. The body of knowledge on job stress and workplace burnout supports targeting organizational-level sources versus individual-level factors as the most effective prevention and reduction strategy. This study elaborates on this evidence by identifying structural drivers of burnout within a multidisciplinary context of pediatric hematology/oncology clinicians.

BACKGROUND High-quality oncology care is marked by skillful communication, yet little is known about patient and family communication perceptions or content preferences. Our study sought to elicit pediatric oncology patient and parent perceptions of early cancer communication to establish whether informational needs were met and identify opportunities for enhanced communication throughout cancer care. METHOD An original survey instrument was developed, pretested, and administered to 129 patients, age 10-18 years, and their parents at 3 cancer centers between 2011 and 2015. Statistical analysis of survey items about perceived communication, related associations, and patient/parent concordance was performed. RESULTS A greater percentage of participants reported "a lot" of discussion about the physical impact of cancer (patients, 58.1% [n = 75]; parents, 69.8% [n = 90]) compared with impact on quality of life (QOL) (patients, 44.2% [n = 57]; parents, 55.8% [n = 72]) or emotional impact (patients, 31.8% [n = 41]; parents, 43.4% [n = 56]). One fifth of patients (20.9% [n = 27]) reported they had no up-front discussion about the emotional impact of cancer treatment. Parents indicated a desire for increased discussion regarding impact on family life (27.9% [n = 36]), long-term QOL (27.9% [n = 36]), and daily activities (20.2% [n = 26]). Patients more frequently than parents indicated a desire for increased physician/patient discussion around the impact on daily activities (patients, 40.3% [n = 52]; parents, 21.7% [n = 28]; P

BACKGROUND To examine associations between phenotypes of short sleep duration and clinically assessed health conditions in long-term survivors of childhood cancer. METHODS Survivors recruited from the St. Jude Lifetime Cohort (n = 911; 52% female; mean age 34 years; 26 years postdiagnosis) completed behavioral health surveys and underwent comprehensive physical examinations. Sleep was assessed with the Pittsburgh Sleep Quality Index. Short sleep was defined as ≤6 h per night with phenotypes of short sleep including poor sleep efficiency (

PURPOSE: Cancer patients' belief about prognosis can heavily influence medical decision making and goals of care. It is known that parents of children with cancer tend to be optimistic regarding their child's prognosis; however, little is known about pediatric patients' prognostic beliefs, how physicians' prognostic communication is perceived, and how these perceptions are compared with actual prognoses. PATIENTS AND METHODS: An original survey was administered to 100 pediatric oncology patients, age 10-18 years, and their parents from 2013 to 2015, at St Jude Children's Research Hospital. Patients were eligible for inclusion if they had an oncologic diagnosis, were between 1 month and 1 year from diagnosis, and were English speaking. Survey responses regarding perceived prognosis were compared with actual prognoses as determined from the medical record review and published literature. Analysis included descriptive statistics and association tests. RESULTS: Nearly half of participants (patients = 48.9%, parents = 50.5%) displayed prognostic optimism as compared with the determined objective estimate of curative potential. The majority of both patients (78%) and parents (85%) reported belief in a very high chance of cure, although fewer reported that their physician communicated a very high chance for cure (patients = 57%, parents = 70%), and only 43% were determined to have a very high probability of cure. Significant differences were noted in prognostic optimism by cancer type ( P < .0001); patients with solid tumor were more often optimistic (n = 25, 83.3% optimistic; n = 5, 16.7% accurate), and patients with lymphoma were most often accurate (n = 2, 8.7% optimistic; n = 21, 91.3% accurate). CONCLUSION: Pediatric oncology patients and parents tend to be optimistic about their chance of cure, as compared to both perceived prognostic communication from physicians and objective estimated prognosis. Understanding the nature of prognostic optimism among patients with cancer and caregivers may empower clinicians to guide realistic decision making while supporting hope.

AIM To examine the impact of clinical factors, cognitive deficits, and sleepiness on health-related quality of life (HRQoL) among young people with craniopharyngioma. METHOD Seventy-eight patients (67% White; 41 males, 37 females; mean age 10y 8mo, SD 3y 11mo, range 6-20y) with craniopharyngioma were assessed for tumor extent and diabetes insipidus. All patients underwent overnight polysomnography and multiple sleep latency tests after surgical resection. Executive functioning was assessed using parent-reported measures. Patients and their parents completed measures of HRQoL. None had a history of previous radiation therapy. RESULTS Path analysis was used to test hypothesized relations while controlling for demographic and disease characteristics. Analyses revealed poorer parent-reported HRQoL among young people with greater executive functioning symptoms (estimate -0.83; p

To examine self-reported (30-day) sleep versus nightly actigraphy-assessed sleep concordance in long-term survivors of childhood cancer.Four hundred seventy-seven participants enrolled in the St. Jude Lifetime Cohort (53.5% female, median (range) age 34.3 (19.3-61.6) years, 25.4 (10.9-49.3) years from diagnosis) completed the Pittsburgh Sleep Quality Index and ≥ 3 nights of actigraphy. Participants had neurocognitive impairment and/or a self-reported prolonged sleep onset latency (SOL). Self-reported 30-day sleep and nightly actigraphic sleep measures for sleep duration, SOL, and sleep efficiency (SE) were converted into ordinal categories for calculation of weighted kappa coefficients. General linear models estimated associations between measurement concordance and late effects.Agreements between self-reported and actigraphic measures were slight to fair for sleep duration and SOL measures (kSelf-reported sleep and actigraphic sleep demonstrated discordance in our sample. Several prevalent late effects were statistically significantly associated with increased measurement discrepancy. Future studies should consider the impacts of late effects on sleep assessment in adult survivors of childhood cancer.

BackgroundPediatric oncology patients have a high rate of clinical deterioration frequently requiring critical care. Patient deterioration events are distressing for clinicians, but little is known about how Pediatric Early Warning Systems (PEWS) impact clinicians’ emotional responses to deterioration events.MethodsSemi-structured interviews were conducted with 83 nurses, pediatricians, oncologists, and intensive care clinicians who had recently participated in a patient deterioration event at two pediatric oncology hospitals of different resource-levels: St. Jude Children’s Research Hospital (N = 42 participants) in Memphis, Tennessee or Unidad Nacional de Oncología Pediátrica (N = 41 participants) in Guatemala City, Guatemala. Interviews were conducted in the participants’ native language (English or Spanish), transcribed, and translated into English. Each transcript was coded by two researchers and analyzed for thematic content.ResultsEmotions around patient deterioration including concern, fear, and frustration were reported across all disciplines at both hospitals. Concern was often triggered by an elevated PEWS score and usually resulted in increased attention, which reassured bedside clinicians that patients were receiving necessary interventions. However, persistently elevated PEWS scores, particularly at St. Jude Children’s Research Hospital, occasionally resulted in a false sense of relief, diminishing clinician attention and negatively impacting patient care. Nurses at both institutions described how PEWS amplified their voices, engendering confidence and empowerment, two of the only positive emotions described in the study.ConclusionClinicians experienced a range of emotions while caring for high-risk patients in the setting of clinical deterioration. These emotions have the potential to contribute to compassion fatigue and burnout, or to resilience. Acknowledgment and further investigation of the complex interplay between PEWS and clinician emotions are necessary to maximize the impact of PEWS on patient safety while simultaneously supporting staff wellbeing.

Several studies describe sleep–wake disturbances in pediatric craniopharyngioma, but none have determined the prevalence or associated predictors of excessive sleepiness in this group after diagnosis and prior to post-operative observation or adjuvant radiotherapy. In this study, we report sleep–wake disturbances in children and adolescents with craniopharyngioma and associated clinical and treatment variables. After surgery and prior to radiotherapy or observation, pediatric patients (n = 110) with craniopharyngioma ≥ 3 years old completed a baseline sleep clinic evaluation by a pediatric sleep specialist, polysomnography (PSG) and next-day multiple sleep latency test (MSLT). MSLT was limited to those ≥ 6 years old. Logistic regression models were used to determine the relationship between patient characteristics and the presence and type of hypersomnia. Amongst patients completing PSG and MSLT, 80% had polygraphic evidence of excessive daytime sleepiness. Hypersomnia due to medical condition was diagnosed in 45% and narcolepsy in 35%. Overweight or obese patients were more likely to be diagnosed with hypersomnia (P = 0.012) or narcolepsy (P = 0.009). Grade 2 hypothalamic involvement (HI) at diagnosis was associated with the diagnosis of narcolepsy (P = 0.0008). This study describes the prevalence and associated predictors of hypersomnia for patients with craniopharyngioma after surgical resection. HI was predictive of narcolepsy diagnosis, and a higher body mass index z-score was associated with hypersomnia due to medical condition and narcolepsy. We recommend that sleep assessment and intervention begin after surgical resection, especially in overweight or obese patients and those with extensive tumors.

Background In noncancer populations, insomnia is known to affect neurocognitive processes. Although the prevalence of insomnia appears to be elevated in survivors of childhood cancer, relatively little is known about its association with neurocognitive performance in this at-risk population. Methods A total of 911 survivors (51.9% female; mean [SD] age, 34 [9.0] years; time since diagnosis, 26 [9.1] years) completed direct assessments of attention, memory, processing speed, and executive functioning and self-reported symptoms of sleep (Pittsburgh Sleep Quality Index), fatigue (Functional Assessment of Chronic Illness Therapy-Fatigue), and daytime sleepiness (Epworth Sleepiness Scale). Sex-stratified general linear models were used to examine associations between insomnia and neurocognitive performance, with adjustment for treatment exposures and chronic health conditions. All statistical tests were two-sided. Results Insomnia was reported by 22.1% of females and 12.3% of males (P Conclusions Insomnia was highly prevalent and contributed to the neurocognitive burden experienced by adult survivors of childhood cancer. Treatment of insomnia may improve neurocognitive problems in survivors.

The completion of the Human Genome Project revolutionized our understanding of precision health care. Currently, patients have numerous genetic and genomic testing options including predictive and diagnostic screening. Genetic/genomic screening provides precision medical care based on patient classification, such as disease susceptibility to understanding of disease etiology, as well as understanding an individual response to treatment. This chapter describes genetic and genomic precision health care, as well as the interaction of the individual’s environment, family, and lifestyle. This vastly changing care environment has a significant impact on nursing practice, as nurses promote potential benefit while respecting the patient and family decision-making process. Within the precision health care environment, we, as nurses, must take responsibility in supporting the well-being of our patients and families, while fostering new knowledge.

Background: Brentuximab vedotin is an anti-CD30 antibody-drug conjugate approved for use in adults with newly diagnosed and relapsed/refractory high-risk classical Hodgkin lymphoma (HL). Methods: Open label, single-arm, multicenter trial for patients (age < 18 years) with stage IIB, IIIB or IV classical HL. One dose of brentuximab vedotin (AdcetrisO) replaced each vincristine in the OEPA [vincristine, etoposide, prednisone, and doxorubicin]/COPDac [cyclophosphamide, vincristine, prednisone, and dacarbazine] regimen (2 cycles of AEPA and 4 cycles of CAPDac) according to GPOH-HD2002 treatment group 3 [TG3]. 1 Residual node radiotherapy (25·5 Gy) was given at the end of all chemotherapy only to nodal sites that did not achieve a complete response (CR) at the early response assessment (ERA) after 2 cycles of therapy. Primary objectives were to evaluate the safety and efficacy (complete remission at ERA) of this combination and the 3-year event-free and overall survival. Findings: Of the 77 patients enrolled in the study, 27 (35%) achieved complete remission at ERA and were spared radiation. Patients who were irradiated received radiation to individual residual nodal tissue. At a median follow-up of 3·4 years, the 3 year event-free survival was 97·4% (SE 2·3%) and overall survival 98·7% (SE 1·6%). One irradiated patient experienced disease progression at the end of therapy and now remains disease free more than 6 years following salvage therapy, and one unexpected death occurred. Only 4% patients experienced Grade 3 neuropathy. Interpretation: The integration of brentuximab vedotin in the frontline treatment of pediatric high-risk HL is highly tolerable, facilitated reduction in radiation exposure, and yielded excellent outcomes. Trial Registration: IND (#118603), The trial is registered with www.clinicaltrials.gov, NCT01920932. Funding Statement: Research reported in this publication was supported by the National Cancer Institute of the National Institutes of Health under Award Number P30CA021765. Declaration of Interests: None to declare. Ethics Approval Statement: The trial was approved by the institutional review board of each participating center and monitored by the SJCRH Data, Safety, and Monitoring Board. Informed consent was obtained as per institutional guidelines.

OBJECTIVE: Disrupted sleep is common in pediatric cancer, which is associated with psychological distress and may impact neural recovery. Information regarding sleep during pediatric brain tumor treatment is limited. This study aimed to describe objective sleep-wake patterns and examine the sleep-mood relation in youth hospitalized for intensive chemotherapy and stem cell rescue. METHODS: Participants included 37 patients (M age =9.6 ± 4.2 years) enrolled on a medulloblastoma protocol (SJMB03) and their parents. Respondents completed a mood disturbance measure on three days and patients wore an actigraph for five days as an objective estimate of sleep-wake patterns. General linear mixed models examined the relation between nocturnal sleep and next-day mood, as well as mood and that night’s sleep. RESULTS: Sleep duration was deficient, sleep efficiency was poor, and daytime napping was common, with large between-subjects variability. There were minimal mood concerns across all days. The sleep and next-day mood relationship was non-significant (p>0.05). Greater parent-reported child mood disturbance on day 2 was associated with decreased same-night sleep (p

The number of children, adolescents, and young adults transitioning into primary care is steadily increasing as the overall survival rates for childhood cancer improve. Survivors of childhood cancer are at significant risk for co-morbidities associated with their primary cancer and cancer therapy impacting their health-related quality of life. Sleep disturbances are now recognized as a public health concern and are common complaints among survivors of childhood cancer. This review will describe the most common sleep disturbances reported among survivors of childhood cancer, as well as appropriate evaluations to confirm sleep diagnosis and prescribed treatments.

BACKGROUND Evaluation of energy requirements is an important part of the nutrition assessment of pediatric oncology patients. Adequate provision of energy in this population is of extreme importance because of the prevalence of malnutrition and its effect on growth, development, quality of life, morbidity, and mortality. Numerous methods are used in clinical practice for estimating the resting energy expenditures (REE), specifically indirect calorimetry and predictive equations. A relatively new instrument used to assess REE is the hand-held indirect calorimeter. The purpose of this quality improvement project was to compare the accuracy of REE measurements taken by a hand-held indirect calorimeter and predictive equations to that of a standard indirect calorimeter metabolic cart. METHODS Patients receiving therapy for pediatric cancer, aged 7-18 years, and having a weight ≥15 kg and scheduled for a REE nutrition assessment were eligible. Sequentially, the patient's REE was assessed with the cart and the hand-held indirect calorimeter along with the predictive equation calculation. RESULTS Post hoc pairwise comparisons revealed that all 3 methods were significantly different from one another (P < .0001). When compared with the cart, the portable hand-held calorimeter was found to underestimate REE by 11.9%, whereas predictive equations overestimated REE by 12.4%. CONCLUSION Our quality improvement project suggests that the hand-held indirect calorimeter underestimated REE, and predictive equations overestimated REE in pediatric oncology nutrition assessment. Therefore, we recommend that these limitations in assessment be considered when assessing REE using a hand-held indirect calorimeter or predictive equations.

Background: Fewer than 20% of patients with sickle cell disease (SCD) have access to curative matched sibling donor bone marrow transplantation (MSD-BMT). Development of novel therapies to ameliorate the serious morbidity and early mortality caused by SCD is a National Institute of Health research priority. Gene therapy for SCD has emerged as a promising approach in early-stage clinical trials approach with the potential to reach greater numbers of patients than MSD-BMT. Knowledge and attitudes of individuals with SCD towards this investigational new therapy and their willingness to participate in early-stage clinical trials have not been systematically described. Methods: Patients with SCD at least 13 years of age (n=66) and caregivers of children with SCD (n=38) were surveyed about knowledge, attitudes, and beliefs regarding gene therapy for SCD. Most questions utilized a 5-item Likert response scale while The Newest Vital Sign was used to assess health literacy. To explore attitudes around gene therapy in further detail, we identified a regionally diverse focus group comprised of 12 patients/caregivers with SCD from across the United States. We audio-recorded, transcribed verbatim, and analyzed five focus group discussions using thematic content analysis to identify salient themes related to attitudes towards gene therapy for SCD. Our goal was to identify the educational needs and preferences of this patient / caregiver stakeholder group. Results: Survey: Of the 104 survey respondents 96% identified as Black and 4% as Hispanic. Respondents were between 13-64 years (mean 29.6, Std Dev 11.9 years). HbSS and HbSC were the most common genotypes (61.5% and 24%, respectively). Only 4.8% of participants (N=5) felt "extremely knowledgeable" about gene therapy for SCD while most (63.4%) reported no/slight knowledge. We found no association between health literacy levels and gene therapy knowledge (p=0.361). Nearly 30% of participants reported that the risk of cancer as a potential side effect would "probably not prevent their enrollment" on a gene therapy trial while 48% said it "definitely/probably would preclude their participation". Most respondents had a neutral attitude regarding the safety of gene therapy for SCD and how good of a treatment it was (56.7% and 58.6%, respectively). Only a few respondents endorsed the idea that gene therapy was "unsafe" or "not a good treatment for SCD" (5.8% and 4.8% respectively). There was an association between increasing knowledge about gene therapy and agreement that it is safe (p=0.012) and a good treatment for SCD (p=0.031). Focus Groups: Among the focus group participants, there was consensus that communication about gene therapy is suboptimal. Participants noted that clinicians frequently use medical jargon, do not tailor their approach to the individual patient/caregiver, and fail to adequately disclose important details. Focus group members desired more information about side effects and risks associated with conditioning chemotherapy, immunosuppression, risk of infertility, and hair loss. Participants, particularly parents of children with SCD, were worried about future unknown risks and described the enormity of the decision to enroll in an early-phase trial. Focus group members verbalized the need for inclusion of patients with SCD as partners during the informed consent/assent process and ideally, at the outset of the clinical trial design. Conclusion: Very few patients with SCD described feeling knowledgeable about gene therapy for SCD; a majority have neutral feelings about the safety and utility ("good treatment for SCD") of this new approach. Patient/caregiver-centered education about gene therapy in a manner that meets stakeholder informational needs is urgently needed. Given the temporary hold on lentiviral gene therapy trials for SCD (after some participants developed a myeloid neoplasm on an industry sponsored trial in February 2021) and the risk of cancer as a probable or definite barrier to enrollment in a gene therapy trial for many patients, transparency of information about these risks is essential. For the successful execution of novel trials in SCD, a community-based participatory research approach is vital. Disclosures Sharma: Medexus Inc: Consultancy; Spotlight Therapeutics: Consultancy; Novartis: Other: Salary support paid to institution; Vertex Pharmaceuticals/CRISPR Therapeutics: Other: Salary support paid to institution; Vindico Medical Education: Honoraria; CRISPR Therapeutics: Other, Research Funding. Johnson: CRISPR Therapeutics: Research Funding.

Background: Asparaginase poses a substantial risk for hypersensitivity reactions during and after administration; however, these reactions vary by asparaginase formulation and administration route. It is imperative that nurses be knowledgeable of clinical symptoms associated with intravenous (IV) monomethoxypolyethylene glycol (PEG)-asparaginase reactions, as well as potential reaction timing. Purpose: This single institution retrospective study describes the clinical factors among patients with IV PEG-asparaginase hypersensitivity reactions. Methods: Reaction frequency and severity, dose, phase of treatment, and time between infusion initiation and reaction were collected on patients identified as having an IV PEG-asparaginase hypersensitivity reaction while undergoing acute lymphoblastic leukemia treatment. Results: Sixty-three patients (12.8%) developed a hypersensitivity reaction to IV PEG-asparaginase, with the reaction occurring during a median of 3 doses in both risk arms. Reactions were noted ≤60 minutes after infusion initiation in 98% of patients, and no reactions were fatal. Conclusion: Nurses should carefully observe patients throughout the infusion and anticipate adverse reactions, particularly during the first 3 doses and first 10 minutes of each infusion. Patient and family education should include the rare risk of delayed reactions.

In-home salivary collection quality and adherence to a prescribed collection methodology for evaluation of dim light melatonin onset (DLMO) is unknown in children. Primary aims of this study were to 1) describe a novel family centered methodology for in-home salivary collection; 2) determine the acceptance and feasibility of this methodology; 3) measure adherence to collection instructions; and 4) identify patterns between participants' age and quality of samples collected. After receiving instructional handouts from the study team, families utilized in-home salivary melatonin collection. Participants (N = 64) included 39 children (21 female, mean age 9.5 ± 1.61 years) and 25 adolescents (11 female, mean age 15.9 ± 2.12 years) with craniopharyngioma. Participants were 90% adherent to collection schedule, and 89% of the samples collected were of sufficient quantity and quality, with no differences found between age (child vs. adolescent) and melatonin sample quantity and quality. In-home saliva collection provides an acceptable and feasible method to collect salivary melatonin and biomarkers in children and adolescents.

9 Background: Integration of palliative care into cancer care is recognized as best practice by international oncology and pediatrics organizations. However, optimal strategies for integration of pediatric palliative care (PPC) within cancer care remain understudied. While the majority of PPC provision for cancer patients occurs through subspeciality consultation, growing evidence suggests that models embedding PPC within cancer care have the potential to improve quality of care. Embedded models rely on partnerships with multidisciplinary clinicians, whose perspectives regarding this model are not well known. Methods: We conducted 25 focus groups with 175 clinicians in an academic pediatric cancer center, with groups stratified by discipline (physicians, advance practice providers (APPs), nurses, psychosocial providers) and by care team (hematologic malignancy, bone marrow transplant, solid tumor, brain tumor). Focus groups were led by trained research facilitators and audio-recorded for subsequent targeted content analysis to identify clinician perspectives regarding embedded PPC models. Results: Across 4 physician, 5 APP, 12 nurse, and 4 psychosocial focus groups, 25 physicians, 30 APPs, 71 nurses, and 49 psychosocial providers participated, respectively. When asked to describe features of an “ideal” PPC model, physicians, APPs, and nurses predominantly discussed care delivery and identified early integration of PPC into cancer care as a core feature of an ideal model. Physicians, nurses, and psychosocial providers also emphasized the importance of having a formalized, structured care model. The need for collaboration was the dominant theme for psychosocial providers. Clinicians in all disciplines identified potential benefits from an embedded model, including access to PPC across care settings, normalization of PPC integration, and emphasis on collaboration, teamwork, communication, and earlier PPC involvement. Physicians, APPs, and nurses anticipated similar challenges with an embedded model, including possible reticence of oncology providers and inadequate PPC staffing. Nurses, APPs, and psychosocial providers also voiced concern about potential lack of clarity in delegation of roles and responsibilities between PPC and oncology providers. Conclusions: Pediatric oncology multidisciplinary providers recognize the potential value of an embedded model for integration of PPC in the care of children with cancer. Though providers at times identified similar themes with respect to ideal PPC provision and the benefits and challenges to an embedded model, some identified priorities varied by discipline. These findings highlight the importance of integrating varied interdisciplinary perspectives when developing an embedded care model to align with priorities of diverse pediatric cancer stakeholders.

Childhood cancer incidence is rising, affecting a growing proportion of elementary school students. For most of these children, school attendance can be limited by hospitalisations, treatments and side effects. However, little is yet known about the educational needs and experiences of this population. This phenomenological study explored the school experiences of 10 6- to 12-year-old children with cancer as they underwent chemotherapy. Results revealed perceptions that attending school in the hospital or home during cancer treatment is essentially lonely, confusing and “different”. These perceptions intertwined to illuminate five themes: (1) school should involve fun activities; (2) group educational formats are preferable; (3) old school is the “best school”; (4) being a “good student” is important during treatment; and (5) attending school is complicated during treatment. Therefore, hospital-based and homebound schooling programmes should integrate socially interactive and cognitively engaging ...

Whether children should be offered genetic testing for cancer risk is much debated but young voices are rarely heard in these conversations. The current study explored perspectives of genetic testing held by adolescents and emerging adults in families with Li Fraumeni syndrome (LFS). Twelve 12- to 25-year-olds in families with LFS completed qualitative interviews for this study. All believed that testing should be offered for children but many qualified this statement saying parental approval would be needed and testing should be optional. Genetic testing was seen as way to learn of risk status, allow for disease prevention efforts, and reduce uncertainty and anxiety. Perceived disadvantages included negative emotions associated with the testing result. Participants generally felt that children should be involved in the testing decision, but that parents could unilaterally decide to have a child tested in certain circumstances (e.g., young age, high risk). All who were aware of having been tested and of their test result (n = 7; 4 positive) said testing had no negative impact on their outlook and they agreed with the decision to undergo testing. Implications of these findings for clinical practice and future research are discussed.

BACKGROUND The incorporation of genomic testing to identify targetable somatic alterations and predisposing germline mutations into the clinical setting is becoming increasingly more common. Despite its potential usefulness, to the authors' knowledge physician confidence with regard to understanding and applying genomic testing remains unclear, particularly within the realm of pediatric oncology. METHODS Before initiating an institutional feasibility study regarding the integration of clinical genomic testing, the authors surveyed pediatric oncologists regarding their confidence around understanding of genomic testing, perceived usefulness of test results, preferences around the disclosure of germline test results, and possible risks and benefits of testing. RESULTS Among survey respondents (52 of 88 contacted; response rate of 59%), only a minority were confident in interpreting, using, and discussing somatic (35%) or germline (27%) genomic test results. Providers who were confident in interpreting somatic results were significantly more likely to anticipate using the results to plan the treatment of patients with relapsed or refractory cancers (P = .009). Similarly, providers who reported confidence in interpreting germline results were significantly more likely to discuss and use these results as part of clinical care (P

Introduction Sleep disturbances are prevalent among adult survivors of childhood cancer, though little is known about associations between sleep and health in this vulnerable population. Methods Survivors recruited from the St. Jude Lifetime Cohort (n=911; 52% female; mean age 34 years; 26 years post-diagnosis) completed surveys assessing habitual sleep patterns and mental health and underwent comprehensive physical examinations. A subset of survivors (n=491) completed sleep actigraphy. Short sleep duration was defined as sleeping Results Self-report and actigraphy-assessed short sleep was identified in 44% and 42% of survivors, respectively. However, these measures were weakly correlated (r=0.23). In adjusted multivariable models, self-reported short sleep was associated with higher risk of pulmonary conditions (RR=1.3, 95% CI=1.1-1.7), depression (OR=2.6, 95% CI=1.4-5.1) and anxiety (OR=3.4, 95% CI=1.6-6.8), while associations with cardiac (RR=1.10, 95% CI=0.94-1.30) and renal conditions (OR=1.30, 95% CI=0.79-2.13) were not significant. There were no significant associations between actigraphy-assessed short sleep and any of the health outcomes. Conclusion Habitual self-reported short sleep was associated with clinically ascertained adverse health outcomes. Although the temporality of these associations cannot be determined in this cross-sectional study, sleep is a modifiable health behavior and improving sleep may improve health in survivors. Measures of self-reported sleep may have unique value when assessing the relationship between sleep and health. Support CA225590, K. Krull Principal Investigator; CA195547, M. Hudson and L. Robison Principal Investigators; CA21765, C. Roberts, Principal Investigator

Introduction Patients with craniopharyngioma are at increased risk for hypersomnia/narcolepsy and circadian rhythm disruption, secondary to hypothalamic-pituitary involvement of the tumor. We assessed youth with craniopharyngioma to determine presence of the dim light melatonin onset (DLMO) and concurrent sleep disturbance. Methods Fifty-two patients (7-21 years; 51% female) enrolled on our institutional protocol for craniopharyngioma that included surgery, proton therapy, or both. In-home salivary melatonin was collected after surgery and hourly beginning 3 h before to 1 h after habitual bedtime to determine the DLMO, which was defined as the time that melatonin exceeded a 4 pg/mL threshold. Polysomnography and a next day multiple sleep latency test (MSLT) were also conducted. Results Hypersomnia/narcolepsy was indicated in 86% of patients. DLMO was detected in 29 (56%) patients and averaged 21:04 (±1:14). All but 2 patients with a DLMO had a concurrent sleep diagnosis (18 hypersomnia, 8 narcolepsy, 1 insomnia). In those we could not compute a DLMO, melatonin was above the 4 pg/mL threshold in 19 (37%), suggesting that the DLMO was likely earlier than the sampling window. Two (4%) did not reach threshold, suggesting that the DLMO was later than the window. For patients in which DLMO was not computed, all but 4 had a concurrent sleep diagnosis (7 hypersomnia, 9 narcolepsy, 1 MSLT not completed). Three (6%) participants showed a pattern of melatonin decreasing before bedtime (2 hypersomnia, 1 narcolepsy). Sleep disorder diagnosis was not associated with whether a DLMO was detected or not. Conclusion DLMO did not occur within the sampling window in 44% of patients with the majority due to the DLMO likely occurring before sampling started. Simultaneous assessment of both sleep-wake disturbance and circadian phase could provide more informed sleep interventions for excessive sleepiness and circadian misalignment in this patient population. Support This study was supported by cancer center grant (CA21765) from the National Cancer Institute, and ALSAC.

OBJECTIVES To determine whether a sleep intervention compared with standard of care (SOC) was successful in preserving nighttime sleep in children with central nervous system cancers hospitalized for high-dose chemotherapy (HDCT) and autologous stem cell rescue, and to explore associations between sleep and fatigue during treatment. METHODS An unblinded, randomized, controlled, multicomponent intervention (NCT00666614) including evidence-based cognitive and behavioral strategies to improve sleep was implemented in 33 children (age 4-12 years) and adolescents (age 13-19 years) during hospitalization. Children wore an actigraph to measure sleep and wake, and reported fatigue scores daily. Parents concurrently kept a sleep diary and reported fatigue scores for their children. RESULTS The mean age was 9.5 ± 3.9 years, 81.8% were white, and 60.6% were male. Sleep in all children was seriously disturbed throughout the study. Children in the intervention group maintained their longest nighttime sleep across the study, while it declined in children receiving SOC (P = 0.009 for interaction). There were few other differences in sleep between groups. Controlling for age and baseline fatigue, higher nighttime activity score, and lower percent sleep were significantly associated with higher next-day adolescent-reported fatigue (P < 0.05); longest sleep was significantly positively associated with next-day child-reported fatigue (P = 0.018). CONCLUSION In this sample of children undergoing HDCT, a multicomponent sleep intervention modestly preserved nighttime sleep duration, although overall sleep was poor in both groups. Sleep is an integral component of health, and may influence outcomes of children receiving HDCT. Further investigation into methods of preserving sleep in children undergoing intensive cancer therapy is warranted.

The Children’s Oncology Group Nursing Discipline has identified the most concerning symptoms during childhood cancer treatment and the need for continued symptom assessment and intervention during treatment trajectory. To develop appropriate interventions, symptom science strategies must explore the biological mechanisms associated with symptoms of cancer and cancer treatment. To explore the associated biological mechanisms, biomarkers have been recommended for inclusion in symptom science studies, when applicable. The biomarker assessed, as well as the method of collection and storage, can affect the reliability and validity of the study results and clinical implication. This review will describe biomarkers that have been described in pediatric oncology symptom science research and provides special considerations for specimen collection and processing.

Youth with craniopharyngioma are at increased risk for excessive daytime sleepiness and narcolepsy. Polysomnography (PSG) is the gold standard for diagnosing sleep disorders, but is time-intensive, costly, and does not offer an in vivo measure of typical sleep routine. We determined the sensitivity, specificity, and accuracy of actigraphy compared with PSG in measuring nocturnal sleep in pediatric craniopharyngioma.Fifty youth with craniopharyngioma (age 3-20 years) were assessed by overnight PSG and concurrent actigraphy after surgical resection and before proton therapy.PSG and actigraphy data were synchronized utilizing an epoch-by-epoch comparison method. Sensitivity, specificity, and accuracy were calculated using measures of true wake, true sleep, false wake, and false sleep. Bland-Altman plots were conducted to further assess level of agreement.Actigraphy was 93% sensitive (true sleep [TS]) and 87% accurate (ability to detect TS and true wake) in measuring sleep versus wakefulness and was a reliable measure of sleep efficiency (SE) and sleep latency (SL). Specificity (true wake) was poor (55%) and total sleep time (TST) was underestimated by an average of 15.1 min. Wake after sleep onset (WASO) was overestimated by an average of 14.7 min.Actigraphy was highly sensitive and accurate and was a reliable measure of SE and SL. Although there were differences in TST and WASO measurements by actigraphy and PSG, our findings provide the basis for future studies on the use of actigraphy to monitor treatment response to wakefulness-promoting medications in youth with craniopharyngioma who demonstrate excessive daytime sleepiness.

Objectives To develop an evidence-based compassion fatigue program and evaluate its impact on nurse-reported burnout, secondary traumatic stress, and compassion satisfaction, as well as correlated factors of resilience and coping behaviors. Sample & setting The quality improvement pilot program was conducted with 59 nurses on a 20-bed subspecialty pediatric oncology unit at the St. Jude Children's Research Hospital in Memphis, Tennessee. Methods & variables Validated measures of compassion fatigue and satisfaction (Professional Quality of Life Scale V [ProQOLV]), coping (Brief COPE), and resilience (Connor-Davidson Resilience Scale-2) were evaluated preprogram and at two, four, and six months postprogram, with resilience and coping style measured at baseline and at six months postprogram. Results Secondary traumatic stress scores significantly improved from baseline to four months. Select coping characteristics were significantly correlated with ProQOLV subscale scores. Implications for nursing Ongoing organizational support and intervention can reduce compassion fatigue and foster compassion satisfaction among pediatric oncology nurses.

PURPOSE:Robust circadian rhythms are increasingly recognized as essential to good health. Adult cancer patients with dysregulated circadian activity rhythms (CAR) experience greater fatigue, lower responsiveness to chemotherapy, and shorter time to relapse. There is scant research describing circadian rhythms and associated outcomes in children with cancer. As part of a larger study examining whether a cognitive-behavioral intervention could preserve sleep in children and adolescents with central nervous system cancers hospitalized for high-dose chemotherapy (HDCT), this study aimed to compare CAR of these children to published values and to investigate the relationship between CAR and fatigue. METHODS:Participants aged 4-19years wore an actigraph throughout their hospitalization (5days). From activity counts recorded by actigraphy, six CAR variables were calculated: amplitude, 24-h autocorrelation (r24), dichotomy index (I < O), interdaily stability (IS), intradaily variability (IV), and acrophase. Parent-reported child fatigue and child/adolescent self-reported fatigue measures were collected daily. RESULTS:Thirty-three participants were included. Three CAR variables (amplitude, r24, and I < O) showed dysregulation compared to published values. Older age was significantly associated with later acrophase and greater dysregulation of all other CAR variables. Controlling for age, more dysregulated amplitude (p = 0.001), r24 (p = 0.003), IS (p = 0.017), and IV (p = 0.001) were associated with higher parent-reported fatigue; more dysregulated IV (p = 0.003) was associated with higher child-reported fatigue. CONCLUSIONS:Participants demonstrated dysregulated CAR during hospitalization for HDCT. Greater dysregulation was associated with greater fatigue. Research on circadian dysregulation and its relationship to health-related outcomes in children with cancer, and interventions to support circadian rhythmicity, is urgently needed.

BACKGROUND Patients with cancer are increasingly offered genomic sequencing, including germline testing for cancer predisposition or other disorders. Such testing is unfamiliar to patients and families, and clear communication is needed to introduce genomic concepts and convey risk and benefit information. METHODS Parents of children with cancer were offered the opportunity to have their children's tumor and germline examined with clinical genomic sequencing. Families were introduced to the study with a 2-visit informed consent model. Baseline genetic knowledge and self-reported literacy/numeracy were collected before a study introduction visit, during which basic concepts related to genomic sequencing were discussed. Information was reinforced during a second visit, during which informed consent was obtained and a posttest was administered. RESULTS As reflected by the percentage of correct answers on the pretest and posttest assessments, this model increased genetic knowledge by 11.1% (from 77.8% to 88.9%; P