Search

The event-free survival rates of children diagnosed with acute lymphoblastic leukemia (ALL) demonstrate the advances that have been made in pediatric oncology and, more specifically, the improved understanding of the disease's heterogeneous characteristics. Understanding of the heterogeneity, differences between ALL cases, has been propelled through genetic classification of the leukemia, molecular monitoring of treatment response based on the genetic classification of the leukemia, and an understanding of the individual patient's variability in response to treatment. It is the culmination of the genetic profile of the leukemia, the individual patient, and molecular monitoring that has led to the earlier identification of patients who respond less favorably to treatment and thus prompts their treatment intensification and improved outcomes. It is imperative that nurses become knowledgeable of leukemic genetic markers and molecular monitoring and their clinical significance when caring for the child and their family. © 2009 Elsevier Inc. All rights reserved. [ABSTRACT FROM AUTHOR]

Improved laboratory techniques now allow a more sensitive detection of leukemia cells at designated intervals throughout therapy. Using flow cytometry and polymerase chain reaction, it is possible to detect 1 leukemic cell among 10 4 normal cells (1 leukemia cell in 10,000 normal cells), representing a 100-fold greater sensitivity than morphological examination in acute lymphoblastic leukemia (ALL). Recently, it has been shown that the molecular presence of persistent acute lymphoblastic leukemia at the end of remission therapy is a poor indicator of clinical outcome. Now similar studies are being performed in acute myeloid leukemia (AML). While the sensitivity using flow cytometry is less in AML than in ALL (able to detect 1 leukemic cell among 1000 normal cells in AML), persistent or minimal residual AML provides the clinician guidance with future treatment recommendations. Minimal residual disease (MRD) is now considered an important indicator response of disease response to treatment. As such, MRD once considered a research variable is now influencing treatment decisions. Therefore, it is imperative that the nurse have an understanding of the newer techniques to study residual leukemia and their clinical implications for patients and their families. © 2006 by Association of Pediatric Oncology Nurses [ABSTRACT FROM AUTHOR]

Over the last 25 years, significant advances have been made in supportive care of the immunocompromised patient. One significant advance is the use of trimethoprim-sulfamethoxazole (TMP-SMZ) in the prevention of Pneumocystic carinii pneumonia (PCP). Although TMP-SMZ remains the drug of choice for PCP prophylaxis, children who develop or have a history of adverse reactions must be prescribed an alternative treatment. In these instances, medications such as dapsone, aerosolized pentamidine, or atovaquone are prescribed. This report discusses four children with sulfa allergy who were prescribed dapsone and later developed methemoglobinemia. Although methemoglobinemia is associated with dapsone, there was no reference found regarding this link in the pediatric oncology literature. The purpose of these clinical examples is to alert the pediatric nurse and advanced practitioner to the association of dapsone and methemoglobinemia. [ABSTRACT FROM AUTHOR]

BACKGROUND: Dexamethasone contributes to high cure rates in paediatric acute lymphoblastic leukaemia (ALL) but significantly and adversely alters sleep and fatigue. Herein we explored three mechanisms (pharmacokinetics, serum albumin and pharmacogenetics) through which dexamethasone may cause debilitating fatigue and disrupted sleep. METHODS: We enrolled 100 patients on a 10-d study: 5-d of no dexamethasone (OFF DEX) followed by 5-d of dexamethasone (ON DEX) during continuation chemotherapy. Sleep variables were collected with continuous actigraphy on days 1 through 5, both OFF DEX and ON DEX. On days 2 and 5 of each 5-d period, parents and patients 7 years of age and older completed a sleep diary and Fatigue Scale questionnaire. Blood was collected at 0 (pre-dexamethasone), 1, 2, 4 and 8 h after the first oral dexamethasone dose for pharmacokinetic analysis. Serum albumin concentration was retrospectively analysed in stored samples. Patient DNA was genotyped for 99 polymorphic loci in candidate genes associated with glucocorticoid metabolism. RESULTS: Dexamethasone clearance was significantly greater in younger patients than in older ones and in lower risk patients. In multiple regression models, risk group was significantly related to pharmacokinetic parameters. We found that polymorphisms in three genes (AHSG, IL6, POLDIP3) were significantly associated with sleep measures but not with fatigue. CONCLUSION: Risk group had the most significant relationship with disrupted sleep in patients while on dexamethasone. Serum albumin levels had neither a direct relationship with sleep or fatigue variables nor an indirect relationship through systemic exposure to dexamethasone. We identified candidate genes that may help explain the adverse events of disrupted sleep in paediatric patients receiving dexamethasone. [ABSTRACT FROM AUTHOR]

Fatigue is one of the most debilitating conditions associated with cancer and anticancer therapy. The lack of reliable and valid self-report instruments has prevented accurate assessment of fatigue in pediatric oncology patients. The purpose of this study was to identify the most sensitive and specific score, that is, the "cut score," on the Fatigue Scale-Child (FS-C) to identify those children with high cancer-related fatigue in need of clinical intervention. We first used Rasch methods to identify the items on the FS-C that distinguished children with high cancer-related fatigue from other children; our findings indicated that the FS-C needed to be reduced from 14 items to 10 items. We then assessed the 10-item FS-C for its psychometric properties and applied the receiver operating characteristics curve analysis to the FS-C responses from 221 children (aged 7-12 years) receiving anticancer treatment. The cut score identified with 75% sensitivity and 73.5% specificity was 12; 73 (33%) patients scored 12 or higher. Findings from this validated instrument provide a needed guide for clinicians to interpret fatigue scores and provide clinical interventions for this debilitating condition to their pediatric patients with cancer. [ABSTRACT FROM AUTHOR]

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: Measles is reemerging as a public health threat, raising important questions about disease vulnerability among childhood cancer survivors. This secondary analysis assessed the seroprevalence of anti-measles immunoglobulin G (IgG) antibodies as a marker of immune status in survivors of childhood cancer and associated demographic/treatment variables. Method: Participants were childhood cancer survivors who were free of active disease, having routine blood studies drawn, and could provide documentation of having received two doses of measles, mumps, and rubella vaccine before their cancer diagnosis. Patient record review documented demographic and treatment variables. Antimeasles (rubeola) IgG antibody seroprevalence was assessed by enzyme immunoassay for vaccine-specific antibodies. Results: Of 270 survivors evaluated, 110 (42%) were female, 196 (75%) were White, and 159 (61%) were leukemia/lymphoma survivors. Of these 262, 110 (42%) had negative measles seroprevalence, suggesting loss of immunity. Conclusion: Measles antibody surveillance and the need for reimmunization for survivors of childhood cancer survivors outside the transplant setting remains controversial. Our analysis indicates that a substantial proportion of survivors lose vaccine-related immunity to measles. Pediatric oncology nurses play important roles in educating cancer survivors regarding their risk of measles infection, evaluating the need for reimmunization, correcting misinformation about vaccine safety and effectiveness, and working to optimize community herd-based immunity., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., Competing Interests: Declaration of Conflicting InterestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Genomic testing is becoming increasingly common in the care of pediatric patients with cancer. Parental understanding of germline results and their intent and timing of results disclosure to their child and family may have significant implications on the family unit. The purpose of this study was to examine parental understanding of germline genomic results and plans for disclosure to their child and other relatives. Semi-structured interviews were conducted with 64 parents of children with cancer, approximately eight weeks after parents had received their child's results. Parents of children with negative results (n = 20), positive results (n = 15), or variants of uncertain significance (n = 29), were interviewed. Fifty-three parents (83%) correctly identified their child's results as negative, uncertain, or positive. Most parents had disclosed results to family members; however, only 11 parents (17%) acknowledged discussing results with their child. Most parents delayed disclosure due to the young age of their child at the time of testing. In summary, most parents appropriately described their child's germline genomic results, yet few discussed the results with their child due to age. Families should be followed with supportive counseling to assist parents in the timing and content of result disclosure to their children.

Purpose: To characterize parents' quality of life (QoL) after germline genomic sequencing for their children with cancer., Methods: Participants were n = 104 parents of children with cancer enrolled in a prospective study of clinical tumor and germline genomic sequencing. Parents completed surveys at study consent (T0), before disclosure of their child's germline results (T1), and again ≥5 weeks after results disclosure (T2). Bivariate associations with QoL were examined, followed by a multivariable regression model predicting parents' psychological distress., Results: At T2, parental distress significantly differed by their children's germline result type (positive, uncertain, negative; P = .038), parent relationship status ( P = .04), predisclosure genetics knowledge ( P = .006), and predisclosure worry about sequencing ( P < .001). Specifically, parents of children with positive (ie, pathogenic or likely pathogenic) results experienced greater distress than those of children with negative results ( P = .029), as did parents who were single, more knowledgeable about genetics, and with greater worry. In the adjusted regression model, a positive germline result remained significantly associated with parents' lower QoL at T2 follow-up ( F [4,92] = 9.95; P < .001; R 2 = .30; β = .19; P = .031)., Conclusion: Germline genomic sequencing for children with cancer is associated with distress among parents when revealing an underlying cancer predisposition among their affected children. Genetic education and counseling before and after germline sequencing may help attenuate this impact on QoL by addressing parents' concerns about test results and their health implications. Assessing parents' worry early in the testing process may also aid in identifying those most likely in need of psychosocial support.

Objective: Disrupted sleep and fatigue are common symptoms in children with cancer, but little is known about this population's sleep health behaviors and how they may impact nighttime sleep. We aimed to describe the sleep health behaviors of children with newly diagnosed cancer and to determine if they changed over the next 8 weeks., Methods: Our sample included 169 children with cancer (86 males) who were aged 2-18 years (mean [SD] = 8.14 [4.4] y), with parent proxy report for 140 children (71 male) aged 2-12 years (mean [SD] = 6.67 [3.2] y) and self-report for 78 children (39 male) aged 8-18 years (mean [SD] = 12.0 [2.9] y). Parents and patients completed sleep hygiene questionnaires within 30 days of oncology diagnosis (T1); follow-up questionnaires were collected 8 weeks later (T2). Descriptive analyses characterized the sample by sociodemographic characteristics, cancer diagnosis, treatments received, and prescribed medications., Results: Age-related differences were found in sleep health behaviors, with adolescents reporting better overall sleep health behaviors than younger children at both time points. No differences in sleep health behaviors were found at T1 related to diagnosis, treatment, or medications. Some differences in sleep health behaviors were found at T2 related to gender, diagnosis, treatment, and medications. Sleep health behaviors and sleep problems remained relatively stable over 8 weeks. Fatigue was significantly associated with more pre-bedtime worries, insomnia, and lower rates of daytime sleepiness., Conclusions: These findings offer novel descriptive characteristics of sleep health behaviors in a pediatric oncology sample and show relatively stable yet somewhat poor sleep health behaviors across 8 weeks. Better understanding of sleep health behaviors as modifiable factors will help inform targeted interventions., Competing Interests: Declaration of Competing Interest The authors declare that there are no conflicts of interest., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: Sickle cell disease (SCD) is an inherited blood disorder that results in serious morbidity and early mortality. Novel therapies for SCD, most notably genetic therapies (GTs) and HLA-mismatched donor hematopoietic cell transplantation, are in clinical trials. While potentially curative, these interventions are some of the most intensive treatments for SCD and are associated with serious and life-altering side effects, which may manifest several years after treatment. Little is known about knowledge, beliefs, and attitudes of individuals with SCD, or their caregivers, toward existing and these emerging therapies., Methods: Patients with SCD at least 13 years of age (n = 66) and caregivers (n = 38) were surveyed about knowledge, attitudes, and beliefs surrounding treatments for SCD., Results: Only 4.8% felt "extremely knowledgeable" about GT for SCD while the majority (63.4%) reported little knowledge. Overall, health literacy was low among respondents. Most respondents had a neutral attitude regarding the safety of GT for SCD, and whether it was a good treatment for the disorder (56.7% and 58.6%, respectively). Only a few respondents endorsed the idea that GT was "unsafe" or "not a good treatment" (5.8% and 4.8%, respectively). There was an association between increasing knowledge about GT and agreement that it is safe (p = .012) and a good treatment for SCD (p = .031)., Conclusions: Given that very few patients with SCD feel knowledgeable about GT and a majority have neutral feelings about the safety and utility of this new approach, culturally appropriate patient-centered education is urgently needed as these treatments get regulatory approval and proceed to the clinic., (© 2023 Wiley Periodicals LLC.)

Objective: To evaluate attitudes toward vaccination and vaccine uptake regarding coronavirus disease 2019 (COVID-19) among pediatric patients with sickle cell disease (SCD) and their caregivers., Procedure: Adolescent patients and caregivers of children with SCD were surveyed during routine clinic visits; we then conducted a logistic regression analysis to understand differences in vaccine status, while qualitative responses were coded thematically., Results: Among respondents, the overall vaccination rate among adolescents and caregivers was 49% and 52%, respectively. Among the unvaccinated, 60% and 68% of adolescents and caregivers, respectively, preferred to remain unvaccinated, most commonly due to lack of perceived personal benefit from vaccination or mistrust in the vaccine. Multivariate logistic regression analysis showed that child's age (odds ratio [OR] = 1.1, 95% confidence interval [CI]: 1.0-1.2, p < .01) and caregiver education (measured by the Economic Hardship Index [EHI] score, OR = 0.76, 95% CI: 0.74-0.78, p < .05) were independent predictors of getting vaccinated., Conclusion: Despite the increased risk of severe illness due to COVID-19 in patients with SCD, vaccine hesitancy remains high in this population of families whose children have SCD. Fortunately, the reasons cited for deferring vaccination among those who are unvaccinated were largely due to barriers that may be overcome with quality communication around the utility of the vaccine and information about vaccine safety., (© 2023 Wiley Periodicals LLC.)

Background: Communication between providers and patients' families is an integral part of clinical care. Family concern is a validated component of Pediatric Early Warning Systems (PEWS); however, little is known about the impact of PEWS on provider-family communication., Methods: Semi-structured interviews were conducted with 83 ward and Pediatric Intensive Care Unit (PICU) providers involved in the care of patients with deterioration at two pediatric oncology hospitals of different resource levels: St. Jude Children's Research Hospital (n = 42) in the United States and Unidad Nacional de Oncología Pediátrica (UNOP, n = 41) in Guatemala. Interviews were conducted in the participants' native language (English or Spanish), transcribed, and translated into English. Transcripts were coded by two researchers and analyzed for thematic content surrounding family communication and concern., Results: All participants recognized patients' families as a valuable part of the care team, particularly during events requiring escalation of care. Perceived barriers to communication included limited time spent at the bedside, and, at UNOP, language and literacy challenges which occasionally limited providers' ability to assess family concern and involve families in patient care. Despite these barriers, providers perceived PEWS improved communication by facilitating more interaction with families, allowing for relationship-building, anticipatory guidance, and destigmatization of the PICU. PEWS assessments also allowed families to contribute to identification of deterioration., Conclusions: PEWS improve the quality of communication between providers and families by providing more opportunities for interaction, building relationships, and trust. These findings further support the use of PEWS in the care of children with cancer in hospitals of all resource-levels., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Purpose: 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., Design and Methods: 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., Results: 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., Conclusions: 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., Practice Implications: Operationalizing existing guidance will expand inclusion of nurse scientist expertise in human subjects research., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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.

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., Competing Interests: Liza Marie JohnsonResearch Funding: Crispr Therapeutics (Inst) Kim NicholsStock and Other Ownership Interests: Incyte (I)Research Funding: Incyte/NovartisNo other potential conflicts of interest were reported.

Background: Bereavement after the death of a child is devastating and associated with worse physical and psychosocial well-being in parents. Evidence suggests that parents desire and benefit from support provided by other bereaved parents. To foster this peer support, an institutional peer-to-peer mentorship program for bereaved parents was established, through which trained bereaved parent mentors offer support for newly bereaved parents., Methods: Using a retrospective cohort design, we describe the characteristics of participants of the Bereaved Parent Mentorship program. Trained bereaved parent mentors documented encounters with newly bereaved parent mentees using a secure internet-based form. Mentors summarized each encounter including any concerns or need for professional psychosocial support. Descriptive statistics were used to describe mentor and mentee characteristics; free text from encounter summaries was qualitatively analyzed using content analysis., Results: A total of 1368 documented encounters occurred between 150 mentees and 39 mentors from January 1, 2014 to February 29, 2020. Only seven encounters (0.5%) were flagged as serious concern necessitating professional psychosocial support. Four key themes in the encounters between mentors and mentees emerged, including: descriptions of the grief experience, ways in which a mentor supported their mentee, challenges the mentor experienced in supporting the mentee, and personal benefit gained by the mentor from supporting their mentee., Conclusion: This structured Bereaved Parent Mentorship program fostered rich interactions between bereaved parent participants, with very few encounters requiring professional assistance. Future research will assess the impact of bereaved mentor programs on resilience and psychosocial, physical, and functional well-being of parents., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

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., Competing Interests: K.C.T. received funding from UTHSC College of Nursing Center for Community and Global Partnership for this project. E.N. declares no conflicts of interest. B.N.M. is receiving NIH grant funding NHLBI U01HL133996-04, R01CA239630-01, and R01 CA215405-01A1., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: 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., Methods: 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)., Results: 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., Conclusions: 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 (M age  = 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 < 0.001) and lower HRQoL (Est. = 0.23, p = 0.001). Psychosocial problems also predicted lower HRQoL (Est. = -0.34, p = 0.004). Fragmented sleep (Est. = 0.03, p = 0.04) and fatigue (Est. = 0.10, p = 0.02) indirectly predicted lower HRQoL through psychosocial problems., Conclusions: Youth with craniopharyngioma with greater HI may benefit from weight reduction interventions and management of excessive sleepiness. Patients should be prospectively monitored for sleep problems, fatigue, and psychosocial problems, as these patients may benefit from interventions targeting fatigue and psychosocial health to improve HRQoL., (© 2021 John Wiley & Sons Ltd.)

Purpose: To examine self-reported (30-day) sleep versus nightly actigraphy-assessed sleep concordance in long-term survivors of childhood cancer., Methods: 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., Results: Agreements between self-reported and actigraphic measures were slight to fair for sleep duration and SOL measures (k w  = 0.20 and k w  = 0.22, respectively; p < 0.0001) and poor for SE measures (k w  = 0.00, p = 0.79). In multivariable models, severe fatigue and poor sleep quality were significantly associated with greater absolute differences between self-reported and actigraphy-assessed sleep durations (B = 26.6 [p < 0.001] and B = 26.8 [p = 0.01], respectively). Survivors with (versus without) memory impairment had a 44-min higher absolute difference in sleep duration (B = 44.4, p < 0.001). Survivors with, versus without, depression and poor sleep quality had higher absolute discrepancies of SOL (B = 24.5 [p = 0.01] and B = 16.4 [p < 0.0001], respectively). Poor sleep quality was associated with a 12% higher absolute difference in SE (B = 12.32, p < 0.0001)., Conclusions: Self-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., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: 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 ( p s > .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.

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., (© 2021 Wiley Periodicals LLC.)

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<0.001). Direct and indirect effects were found among diabetes insipidus, executive functioning, and parent-reported HRQoL. Diabetes insipidus directly predicted greater global executive functioning impairment (estimate 5.15; p=0.04) and indirectly predicted lower HRQoL through executive functioning impairment (estimate -4.25; p=0.049). No significant effects were found between excessive daytime sleepiness, tumor hypothalamic involvement, diabetes insipidus, executive functioning, and patient-reported HRQoL., Interpretation: These findings suggest that young people with craniopharyngioma presenting with diabetes insipidus may benefit from targeted neurocognitive and psychosocial screening to inform interventions. What this paper adds Children with craniopharyngioma and executive functioning impairment are more likely to have poorer health-related quality of life (HRQoL). Diabetes insipidus, a complication associated with surgery, predicted greater executive functioning impairment. Diabetes insipidus indirectly predicted lower parent-reported HRQoL through executive functioning impairment., (© 2021 Mac Keith Press.)

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., Competing Interests: Monika L. MetzgerResearch Funding: Seattle Genetics Michael P. LinkConsulting or Advisory Role: Incyte, ADC Therapeutics, Lilly, Steba Biotech, Mesoblast, GlaxoSmithKline, SyndaxResearch Funding: Seattle Genetics, Janssen Oncology Jamie FlerlageResearch Funding: Seattle Genetics Matthew J. EhrhardtHonoraria: Optum Torunn I. YockConsulting or Advisory Role: Huron Consulting Services, LLCResearch Funding: MIM Software Sue C. KasteStock and Other Ownership Interests: GE Healthcare Barry ShulkinConsulting or Advisory Role: Navidea Melissa M. HudsonConsulting or Advisory Role: Oncology Research Information Exchange Network, Princess Máxima Center Matthew J. KrasinConsulting or Advisory Role: Debiopharm GroupNo other potential conflicts of interest were reported.

Purpose: Burnout is a syndrome of emotional exhaustion, depersonalization, and reduced personal accomplishment because of chronic occupational stress. Approximately one third of pediatric hematology-oncology physicians experience burnout. The goal of this mixed methods study was to determine the prevalence and drivers of burnout among physicians caring for pediatric hematology-oncology patients at our institution., Materials and Methods: This mixed methods, cross-sectional study was conducted at a large academic cancer center. Validated survey instruments were used to measure burnout, job demands, experience with patient safety events, and workplace culture. Quantitative data informed development of a semistructured interview guide, and physicians were randomly selected to participate in individual interviews. Interviews were transcribed and analyzed via content analysis based on a priori codes., Results: The survey was distributed to 132 physicians, and 53 complete responses were received (response rate 40%). Of the 53 respondents, 15 (28%) met criteria for burnout. Experiencing burnout was associated with increased temporal demand. Twenty-six interviews were conducted. Qualitative themes revealed that frequent meetings, insufficient support staff, and workflow interruptions were key drivers of temporal demand and that temporal demand contributed to burnout through emotional exhaustion and reduced personal accomplishment., Conclusion: Nearly one-third of participating physicians met criteria for burnout, and burnout was associated with increased temporal demand. Qualitative interviews identified specific drivers of temporal demand and burnout, which can be targeted for intervention. This methodology can be easily adapted for broad use and may represent an effective strategy for identifying and mitigating institution-specific drivers of burnout.

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 (<85%), prolonged sleep onset latency (SOL; ≥30 min), and wake after sleep onset (≥3 times per week). Covariates included childhood cancer treatment exposures, demographics, body mass index, and physical inactivity. Separate modified Poisson regression models were computed for each health category to estimate relative risks (RR) and 95% confidence intervals (CI). Multinomial logistic regression models examined associations between sleep and an aggregated burden of chronic health conditions., Results: Short sleep duration was reported among 44% (95% CI 41%-47%) of survivors. In multivariable models, short sleep duration alone was associated with pulmonary (RR = 1.35, 95% CI 1.08-1.69), endocrine (RR = 1.22, 95% CI 1.06-1.39) and gastrointestinal/hepatic conditions (RR = 1.46, 95% CI 1.18-1.79), and anxiety (RR 3.24, 95% CI 1.64-6.41) and depression (RR = 2.33, 95% CI 1.27-4.27). Short sleep with prolonged SOL was associated with a high/severe burden of health conditions (OR = 2.35, 95% CI 1.12-4.94)., Conclusions: Short sleep duration was associated with multiple clinically ascertained adverse health conditions. Although the temporality of these associations cannot be determined in this cross-sectional study, sleep is modifiable and improving sleep may improve long-term health in survivors., (© 2021 Wiley Periodicals LLC.)

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., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Children with cancer are at high risk for clinical deterioration and subsequent mortality. Pediatric Early Warning Systems (PEWS) have proven to reduce the frequency of clinical deterioration in hospitalized patients. This qualitative study evaluates provider perspectives on the impact of PEWS on quality of care during deterioration events in a high-resource and a resource-limited setting., Methods: We conducted semi-structured interviews with 83 healthcare staff (nurses, pediatricians, oncology fellows, and intensivists) involved in recent deterioration events at two pediatric oncology hospitals of different resource levels: St. Jude Children's Research Hospital (SJCRH; n = 42) and Unidad Nacional de Oncología Pediátrica (UNOP; n = 41). Interviews were conducted in the participant's native language (English or Spanish), translated into English, and transcribed. Transcripts were coded and analyzed inductively., Results: Providers discussed both positive and negative perspectives of clinical deterioration events. Content analysis revealed "teamwork," "experience with deterioration," "early awareness," and "effective communication" as themes associated with positive perception of events, which contributed to patient safety. Negative themes included "lack of communication," "inexperience with deterioration," "challenges with technology", "limited material resources," "false positive score," and "objective tool." Participants representing all disciplines across both institutions shared similar positive opinions. Negative opinions, however, differed between the two institutions, with providers at UNOP highlighting limited resources while those at SJCRH expressing concerns about technology misuse., Conclusion: Providers that care for children with cancer find PEWS valuable to improve the quality of hospital care, regardless of hospital resource-level. Identified challenges, including inadequate critical care resources and challenges with technology, differ by hospital resource-level. These findings build on growing data demonstrating the positive impact of PEWS on quality of care and encourage wide dissemination of PEWS in clinical practice., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Garza, Graetz, Kaye, Ferrara, Rodriguez, Soberanis Vásquez, Méndez Aceituno, Antillon-Klussmann, Gattuso, Mandrell, Baker, Rodriguez-Galindo and Agulnik.)

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., (Copyright © 2021 Elsevier Inc. All rights reserved.)

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.

Background: Pediatric 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., Methods: Semi-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., Results: Emotions 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., Conclusion: Clinicians 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., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Graetz, Giannars, Kaye, Garza, Ferrara, Rodriguez, Soberanis Vasquez, Mendez Aceituno, Antillon-Klussmann, Gattuso, Andes, Mandrell, Baker, Rodriguez-Galindo and Agulnik.)

Objective/background: 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., Participants: Fifty youth with craniopharyngioma (age 3-20 years) were assessed by overnight PSG and concurrent actigraphy after surgical resection and before proton therapy., Methods: 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., Results: 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., Conclusions: 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.

Purpose: Hospitalized pediatric oncology patients are at high risk of deterioration and require frequent interdisciplinary communication to deliver high-quality care. Pediatric early warning systems (PEWS) are used by hospitals to reduce deterioration, but it is unknown how these systems affect communication about patient care in high- and limited-resource pediatric oncology settings., Methods: This qualitative study included semistructured interviews describing PEWS and subsequent team communication at 2 pediatric cancer centers, 1 in the United States and 1 in Guatemala. Participants included nurses, and frontline and intensive care providers who experienced recent deterioration events. Transcripts were coded and analyzed inductively using MAXQDA software., Results: The study included 41 providers in Guatemala and 42 providers in the United States (33 nurses, 30 ward providers, and 20 pediatric intensive care providers). Major themes identified include "hierarchy," "empowerment," "quality and method of communication," and "trigger." All providers described underlying medical hierarchies affecting the quality of communication regarding patient deterioration events and identified PEWS as empowering. Participants from the United States described the algorithmic approach to care and technology associated with PEWS contributing to impaired clinical judgement and a lack of communication. In both settings, PEWS sparked interdisciplinary communication and inspired action., Conclusion: PEWS enhance interdisciplinary communication in high- and limited-resource study settings by empowering bedside providers. Traditional hierarchies contributed to negative communication and, in well-resourced settings, technology and automation resulted in lack of communication. Understanding contextual elements is integral to optimizing PEWS and improving pediatric oncology outcomes in hospitals of all resource levels.

Purpose: 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., Methods: 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., Results: 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)., Conclusions: 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.

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 4-19 years wore an actigraph throughout their hospitalization (5 days). 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: 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  < .001). After adjustment for neurotoxic treatment exposures, insomnia (vs healthy sleepers with no daytime fatigue or sleepiness) was associated with worse neurocognitive performance in the domains of verbal reasoning, memory, attention, executive function, and processing speed (verbal reasoning: males β = -0.34, P  = .04, females β = -0.57, P  < .001; long-term memory: males β = -0.60, P  < .001, females β = -0.36, P  = .02; sustained attention: males β = -0.85, P  < .001, females β = -0.42, P  = .006; cognitive flexibility: males β = -0.70, P  =   .002, females β = -0.40, P  = .02). Self-reported sleep disturbance without daytime fatigue or sleepiness or daytime fatigue or sleepiness alone were not consistently associated with poorer neurocognitive performance., 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 Author(s) 2020. Published by Oxford University Press.)

Study Objectives: Children with craniopharyngioma are at risk for excessive daytime sleepiness (EDS). Multiple Sleep Latency Testing (MSLT) is the gold standard for objective evaluation of EDS; however, it is time and resource intensive. We compared the reliability, sensitivity, and specificity of the modified Epworth Sleepiness Scale (M-ESS) and MSLT in monitoring EDS in children with craniopharyngioma., Methods: Seventy patients (ages 6 to 20 years) with craniopharyngioma completed the M-ESS and were evaluated by polysomnography and MSLT. Evaluations were made after surgery, if performed, and before proton therapy., Results: MSLT revealed that 66 participants (81.8%) had EDS, as defined by a mean sleep latency (MSL) < 10 minutes, with only 28.8% reporting EDS on the M-ESS by using a cutoff score of 10. The M-ESS demonstrated adequate internal consistency and specificity (91.7%) but poor sensitivity (33.3%) with the established cutoff score of 10. A cutoff score of 6 improved the sensitivity to 64.8% but decreased the specificity to 66.7%., Conclusions: Patients with craniopharyngioma are at high risk for EDS, as documented objectively on the MSLT, but they frequently do not recognize or accurately report their sleepiness. Future sleep studies should investigate whether specific items or alternative self- and parent-reported measures of sleepiness may have greater clinical utility in monitoring sleepiness in this population., (© 2019 American Academy of Sleep Medicine.)

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., (© 2019 Wiley Periodicals, Inc.)

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 < .0001) in 121 parents participating in both the study introduction and consent visits. The percentage of parents correctly identifying the meaning of somatic and germline mutations increased significantly (from 18% to 59% [somatic] and from 31% to 64% [germline]; P < .0001). Nevertheless, these concepts remained unfamiliar to one-third of the parents. No relation was identified between the change in the overall percentage of correct answers and self-reported literacy, numeracy, or demographics., Conclusions: The use of a 2-visit communication model improved knowledge of concepts relevant to genomic sequencing, particularly differences between somatic and germline testing; however, these areas remained confusing to many participants, and reinforcement may be necessary to achieve complete understanding., (© 2019 American Cancer Society.)

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.

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.

Background: Advances in the application of genetic technologies reveal a growing number of heritable disorders associated with an increased risk to develop cancer during childhood. As genetic testing is increasingly employed in the clinical setting, it is essential to understand whether parents communicate with their children about test results and to elucidate the factors that influence the content and outcomes of these conversations., Methods: Semistructured interviews were conducted with 14 parents whose children tested positive for Li-Fraumeni syndrome (LFS). Semantic content analysis was performed on transcribed interviews, focusing on questions related to parent-child conversations about the genetic testing process and disclosure of positive test results., Results: All parents emphasized the importance of involving children in conversations about LFS. The majority (93%) identified as being part of "cancer families" in which prior experiences with cancer created opportunities for communication. While all had spoken with their children about cancer, only seven (50%) specifically disclosed to their children that they had tested positive for LFS. The most common reason cited for nondisclosure at the time of this study was the young age of the children., Conclusion: Parents of children with LFS desire open conversations about genetic testing and cancer risk. These conversations are challenging yet essential to enable child understanding of genetic risk status and enhance compliance with health-promoting and cancer surveillance measures. Development of age-appropriate educational materials and novel clinical models to facilitate parent-child conversations about genetic test results and risk status for cancer are needed., (© 2018 Wiley Periodicals, Inc.)

Purpose: To compare importance ratings of patient-reported outcomes (PROs) items from the viewpoints of childhood cancer survivors, parents, and clinicians for further developing short-forms to use in survivorship care., Methods: 101 cancer survivors, 101 their parents, and 36 clinicians were recruited from St. Jude Children's Research Hospital. Participants were asked to select eight items that they deemed useful for clinical decision making from each of the four Patient-Reported Outcomes Measurement Information System Pediatric item banks. These item banks were pain interference (20 items), fatigue (23 items), psychological stress (19 items), and positive affect (37 items)., Results: Compared to survivors, clinicians rated more items across four domains that were statistically different than did parents (23 vs. 13 items). Clinicians rated five items in pain interference domain (ORs 2.33-6.01; p's < 0.05) and three items in fatigue domain (ORs 2.22-3.80; p's < .05) as more important but rated three items in psychological stress domain (ORs 0.14-0.42; p's < .05) and six items in positive affect domain (ORs 0.17-0.35; p's < .05) as less important than did survivors. In contrast, parents rated seven items in positive affect domain (ORs 0.25-0.47; p's < .05) as less important than did survivors., Conclusions: Survivors, parents, and clinicians viewed importance of PRO items for survivorship care differently. These perspectives should be used to assist the development of PROs tools.

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., (© 2018 American Society for Parenteral and Enteral Nutrition.)

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.

Daytime sleepiness is recognized in childhood brain tumor survivors. Our objective was to determine prevalence, risk factors for PSG/MLST proven hypersomnia/narcolepsy, and response to stimulants in childhood brain tumor survivors. Standard PSG/MSLT criteria were used to diagnose hypersomnia/narcolepsy. Medical records of brain tumor survivors having undergone a PSG/MSLT were reviewed for the diagnostic code of hypersomnia/narcolepsy. Survivors with hypersomnia/narcolepsy were matched with 2-3 survivors without reported hypersomnia/narcolepsy by age at tumor diagnosis, gender, and time from tumor diagnosis. Between January 2000 to April 2015, 39 of the 2336 brain tumor patients treated at our institution were diagnosed with hypersomnia/narcolepsy for a prevalence rate of 1670/100,000. Hypersomnia/narcolepsy was diagnosed at a median of 6.1 years (range 0.4-13.2) from tumor diagnosis and 4.7 years (range - 1.5 to 10.4) from cranial radiation. Midline tumor location (OR 4.6, CI 1.7-12.2, p = 0.002) and anti-epilepsy drug (AED) use (OR 11, CI 2.4-54) correlated with hypersomnia/narcolepsy while radiation dose > 30 Gray trended towards significance (OR 1.8, CI 0.9-3.6); posterior fossa tumor location reduced the risk (OR 0.1, CI 0.04-0.5, p = 0.002). AED use also correlated with midline tumor location. Thirty-seven survivors were treated with stimulants and reported improved wakefulness and school performance [response rate CI 0.97 (0.86-0.99) and 0.83 (0.65-0.94)]. Prevalence of hypersomnia/narcolepsy among childhood brain tumor survivors was higher than the general population. Tumor location and radiation dose were possible risk factors, and stimulants were reported to be beneficial.

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., (© 2017 Wiley Periodicals, Inc.)