International recommendations for screening and preventative practices for long-term survivors of transplantation and cellular therapy: a 2023 update.

As hematopoietic cell transplantation (HCT) and cellular therapy expand to new indications and international access improves, the volume of HCT performed annually continues to rise. Parallel improvements in HCT techniques and supportive care entails more patients surviving long-term, creating further emphasis on survivorship needs. Survivors are at risk for developing late complications secondary to pre-, peri- and post-transplant exposures and other underlying risk-factors. Guidelines for screening and preventive practices for HCT survivors were originally published in 2006 and updated in 2012. To review contemporary literature and update the recommendations while considering the changing practice of HCT and cellular therapy, an international group of experts was again convened. This review provides updated pediatric and adult survivorship guidelines for HCT and cellular therapy. The contributory role of chronic graft-versus-host disease (cGVHD) to the development of late effects is discussed but cGVHD management is not covered in detail. These guidelines emphasize special needs of patients with distinct underlying HCT indications or comorbidities (e.g., hemoglobinopathies, older adults) but do not replace more detailed group, disease, or condition specific guidelines. Although these recommendations should be applicable to the vast majority of HCT recipients, resource constraints may limit their implementation in some settings.

Social adjustment in children diagnosed with sickle cell disease: A retrospective study.

BACKGROUND: Children with sickle cell disease (SCD) are at risk for physical, psychological, and social adjustment challenges. This study sought to investigate social adjustment and related factors in children living with SCD. METHODS: Data from 32 children (50% male, mean age = 10.32 years, SD = 3.27) were retrospectively collected from a neuropsychology clinic at a tertiary care pediatric hospital. Social adjustment was measured using the Behavior Assessment System for Children (BASC-3) parent-proxy, withdrawal subscale, and the Pediatric Quality of Life Inventory (PedsQL) Generic Module Social Functioning self- and parent-proxy subscales. Other measures captured executive functioning (i.e., Behavior Rating Inventory of Executive Function, Second Edition (BRIEF-2) Parent Form) and non-disease-related associations with social adjustment, including number of years in Canada and family functioning (i.e., PedsQL Family Impact Module). RESULTS: Sixteen percent of patients reported elevated social adjustment difficulties. Multiple linear regression found better family functioning [B = .48, t = 2.65, p = .016], and higher executive functioning [B = -.43, t = -2.39, p = .028] were related to higher scores on the PedsQL parent-proxy ratings of social adjustment [F(4,18) = 5.88, p = .003]. Male sex [B = .54, t = 3.08, p = .005], and having lived more years in Canada [B = .55, t = 2.81, p = .009], were related to higher PedsQL self-reported social adjustment [F(4,23) = 3.75, p = .017]. The model examining the BASC-3 withdrawal subscale was not statistically significant [F(4,16) = 1.63, p = .22]. IMPLICATIONS: Social adjustment in children diagnosed with SCD warrants future research to understand the influence of executive function, and non-disease-related factors, particularly focusing on sociocultural factors.

Alberta Childhood Cancer Survivorship Research Program.

Adverse outcomes after childhood cancer have been assessed in a range of settings, but most existing studies are historical and ascertain outcomes only after 5-year survival. Here, we describe the Alberta Childhood Cancer Survivorship Research Program and its foundational retrospective, population-based cohort of Albertan residents diagnosed with a first primary neoplasm between the ages of 0 and 17 years from 1 January 2001 to 31 December 2018. The cohort was established in collaboration with the Alberta Cancer Registry and Cancer in Young People in Canada program and has been linked to existing administrative health databases and patient-reported outcome questionnaires. The cohort comprised 2580 survivors of childhood cancer, 1379 (53.4%) of whom were 5-year survivors. Approximately 48% of the cohort was female, 47% of the cohort was diagnosed between 0 and 4 years of age, and the most frequent diagnoses were leukemias (25.4%), central nervous system tumors (24.0%), and lymphomas (14.9%). Detailed treatment information was available for 1741 survivors (67.5%), with manual abstraction ongoing for those with missing data. By the study exit date, the median time since diagnosis was 5.5 years overall and 10.4 years for 5-year survivors. During the follow-up time, 82 subsequent primary cancers were diagnosed, 20,355 inpatient and 555,425 ambulatory/outpatient events occurred, 606,773 claims were reported, and 437 survivors died. The results from this research program seek to inform and improve clinical care and reduce cancer-related sequelae via tertiary prevention strategies.

Subcutaneous panniculitis-like T-cell lymphoma in two unrelated individuals with BENTA disease.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous non-Hodgkin lymphoma involving CD8+ T cells, the genetic underpinnings of which remain incompletely understood. Here we report two unrelated patients with B cell Expansion with NF-κB and T cell Anergy (BENTA) disease and a novel presentation of SPTCL. Patient 1 presented early in life with recurrent infections and B cell lymphocytosis, linked to a novel gain-of-function (GOF) CARD11 mutation (p.Lys238del). He developed SPTCL-like lesions and membranoproliferative glomerulonephritis by age 2, treated successfully with cyclosporine. Patient 2 presented at 13 months with splenomegaly, lymphadenopathy, and SPTCL with evidence of hemophagocytic lymphohistiocytosis. Genetic analysis revealed two in cis germline GOF CARD11 variants (p.Glu121Asp/p.Gly126Ser). Autologous bone marrow transplant resulted in SPTCL remission despite persistent B cell lymphocytosis. These cases illuminate an unusual pathological manifestation for BENTA disease, suggesting that CARD11 GOF mutations can manifest in cutaneous CD4+and CD8+ T cell malignancies.

Treatment of breakthrough and prevention of refractory chemotherapy-induced nausea and vomiting in pediatric cancer patients: Clinical practice guideline update.

This clinical practice guideline update provides recommendations for treating breakthrough chemotherapy-induced nausea and vomiting (CINV) and preventing refractory CINV in pediatric patients. Two systematic reviews of randomized controlled trials in adult and pediatric patients informed the recommendations. In patients with breakthrough CINV, escalation of antiemetic agents to those recommended for chemotherapy of the next higher level of emetogenic risk is strongly recommended. A similar recommendation to escalate therapy is made to prevent refractory CINV in patients who did not experience complete breakthrough CINV control and are receiving minimally or low emetogenic chemotherapy. A strong recommendation to use antiemetic agents that controlled breakthrough CINV for the prevention of refractory CINV is also made.

Pediatric Hematopoietic Cell Transplantation: A Longitudinal Assessment of Health-Related Quality of Life of Pediatric Donors.

Pediatric donors may be at increased risk of psychological and social challenges following hematopoietic cell transplantation (HCT). Through a retrospective chart review, we evaluated the health-related quality of life (HRQL) of pediatric donors over time and examined facilitators and barriers to implementing a longitudinal psychosocial assessment. Fifty-one pediatric donors (M = 10.7 years, SD = 3.7) completed an HRQL questionnaire across six time points (T1 to T6) from prior to donation to 2 years after. Change in mean scores was assessed using a linear mixed-effect model for repeated measures design. Facilitators and barriers to implementation were examined. HRQL of pediatric donors improved between T1 and T6 with significant change in physical, emotional, and overall functioning. Facilitators to retention included the support of a clinical coordinator. Barriers to implementation included the absence of infrastructure to maintain contact with pediatric and their families. HRQL of pediatric donors of HCT improved steadily over time. Pattern of results suggests a need to further explore factors that contribute to change across time. Development of a longitudinal standardized assessment protocol that can be prospectively and feasibly implemented is integral to supporting the well-being of this group.

Adapting the HCT-CI Definitions for Children, Adolescents, and Young Adults with Hematologic Malignancies Undergoing Allogeneic Hematopoietic Cell Transplantation.

Allogeneic hematopoietic cell transplantation is a curative procedure for hematologic malignancies but is associated with a significant risk of non-relapse mortality (NRM). The Hematopoietic Cell Transplantation-Comorbidity Index (HCT-CI) is a prognostic tool that discriminates this risk in all age groups. A recent survey of transplant physicians demonstrated that 79% of pediatric providers used the HCT-CI infrequently, and most reported concerns about its applicability in the younger population. We conducted a retrospective study using the Center for International Blood and Marrow Transplant Research database to examine the impact of expanded HCT-CI definitions on NRM in pediatric and young adult patients with hematologic malignancies. We included 5790 patients <40 years old receiving allogeneic transplants between 2008 and 2017 to examine broader definitions of comorbidities in the HCT-CI, including history of mechanical ventilation and fungal infection, estimated glomerular filtration rate, and body mass index (BMI) percentiles. Multivariable Fine-Gray models were created to determine the effect of each HCT-CI defining comorbidity and its modification on NRM and were used to develop 2 novel risk scores. We next developed the expanded HCT-CI for children and young adults (youth with malignancies; expanded ymHCT-CI), where 23% patients had an increased comorbidity score, compared to the HCT-CI. Comorbidities with hazard ratio < 1.2 were then removed to create the simplified HCT-CI for children and young adults (youth with malignancies; simplified ymHCT-CI), which demonstrated higher scores corresponded to a greater risk of NRM (P < .001). These novel comorbidity indexes with broader definitions are more relevant to pediatric and young adult patients, and prospective studies are needed to validate these in the younger patient population. It remains to be seen whether the development of these pediatric-specific and practical risk indexes increases their use by the pediatric transplant community.

Long-Term Organ Function After HCT for SCD: A Report From the Sickle Cell Transplant Advocacy and Research Alliance.

Hematopoietic cell transplantation (HCT) is an established cure for sickle cell disease (SCD) supported by long-term survival, but long-term organ function data are lacking. We sought to describe organ function and assess predictors for dysfunction in a retrospective cohort (n = 247) through the Sickle cell Transplant Advocacy and Research alliance. Patients with <1-year follow-up or graft rejection/second HCT were excluded. Organ function data were collected from last follow-up. Primary measures were organ function, comparing pre- and post-HCT. Bivariable and multivariable analyses were performed for predictors of dysfunction. Median age at HCT was 9.4 years; the majority had HbSS (88.2%) and severe clinical phenotype (65.4%). Most received matched related (76.9%) bone marrow (83.3%) with myeloablative conditioning (MAC; 57.1%). Acute and chronic graft-versus-host disease (GVHD) developed in 24.0% and 24.8%. Thirteen patients (5.3%) died ≥1 year after HCT, primarily from GVHD or infection. More post-HCT patients had low ejection or shortening fractions than pre-HCT (0.6% â†’ 6.0%, P = .007 and 0% â†’ 4.6%, P = .003). The proportion with lung disease remained stable. Eight patients (3.2%) had overt stroke; most had normal (28.3%) or stable (50.3%) brain magnetic resonance imaging. On multivariable analysis, cardiac dysfunction was associated with MAC (odds ratio [OR] = 2.71; 95% confidence interval [CI], 1.09-6.77; P = .033) and severe acute GVHD (OR = 2.41; 95% CI, 1.04-5.62; P = .041). Neurologic events were associated with central nervous system indication (OR = 2.88; 95% CI, 2.00-4.12; P < .001). Overall organ dysfunction was associated with age ≥16 years (OR = 2.26; 95% CI, 1.35-3.78; P = .002) and clinically severe disease (OR = 1.64; 95% CI, 1.02-2.63; P = .043). In conclusion, our results support consideration of HCT at younger age and use of less intense conditioning.

A Population-Based Study of the Long-Term Risk of Infections Associated With Hospitalization in Childhood Cancer Survivors.

PURPOSE: Infections pose a significant risk during therapy for childhood cancer. However, little is known about the risk of infection in long-term survivors of childhood cancer. METHODS: We performed a retrospective observational study of children and adolescents born in Washington State diagnosed with cancer before age 20 years and who survived at least 5 years after diagnosis. Survivors were categorized as having a hematologic or nonhematologic malignancy and were matched to individuals without cancer in the state birth records by birth year and sex with a comparator:survivor ratio of 10:1. The primary outcome was incidence of any infection associated with a hospitalization using diagnostic codes from state hospital discharge records. Incidence was reported as a rate (IR) per 1,000 person-years. Multivariate Poisson regression was used to calculate incidence rate ratios (IRR) for cancer survivors versus comparators. RESULTS: On the basis of 382 infection events among 3,152 survivors and 771 events among 31,519 comparators, the IR of all hospitalized infections starting 5 years after cancer diagnosis was 12.6 (95% CI, 11.4 to 13.9) and 2.4 (95% CI, 2.3 to 2.6), respectively, with an IRR 5.1 (95% CI, 4.5 to 5.8). The survivor IR during the 5- to 10-year (18.1, 95% CI, 15.9 to 20.5) and > 10-year postcancer diagnosis (8.3, 95% CI, 7.0 to 9.7) periods remained greater than comparison group IRs for the same time periods (2.3, 95% CI, 2.1 to 2.6 and 2.5, 95% CI, 2.3 to 2.8, respectively). When potentially vaccine-preventable infections were evaluated, survivors had a greater risk of infection relative to comparators (IRR, 13.1; 95% CI, 7.2 to 23.9). CONCLUSION: Infectious complications continue to affect survivors of childhood cancer many years after initial diagnosis. Future studies are needed to better understand immune reconstitution to determine specific factors that may mitigate this risk.

A population-based analysis of the presentation and outcomes of pediatric patients with osteosarcoma in Canada: a report from CYP-C.

BACKGROUND: Frequently occurring in adolescents, osteosarcoma is the most common primary malignant bone disease, with a reported 15% of patients who present with metastasis. With advances in imaging and improvements in surgical care, an updated analysis is warranted on the outcomes of pediatric patients with osteosarcoma. METHODS: We completed a retrospective review of pediatric patients who presented with osteosarcoma between 2001 and 2017, using The Cancer in Young People in Canada (CYP-C) national database. Data on 304 patients aged younger than 15 years were analyzed. RESULTS: The proportion of patients who presented with metastasis was 23.0%. The overall 5-year survival (OS) for patients who presented with metastasis was 37.4%. Overall survival and event-free survival (EFS) were lower in these patients than in patients with localized disease (hazard ratio [HR] 4.3, p < 0.0001 and HR 3.1, p < 0.0001). For patients who presented with metastatic disease, the OS for those undergoing an operative intervention was 44.1% compared with 17.6% for those who did not undergo resection (p < 0.0001). CONCLUSION: The proportion of patients who presented with metastatic osteosarcoma in our population is higher than previously reported. Overall outcomes of patients with metastatic disease have not changed. Our data reaffirm a role for surgical resection in patients with metastasis with a need to explore new treatment strategies to improve the overall prognosis of these patients.

Pulmonary function in children and adolescents with sickle cell disease after nonmyeloablative hematopoietic cell transplantation.

BACKGROUND: Pulmonary complications are common in sickle cell disease (SCD). The use of standard myeloablative conditioning regimens may increase the risk of lung injury. We report serial pulmonary function testing (PFT) outcomes in children with SCD who underwent a matched-sibling donor hematopoietic cell transplantation (HCT) using nonmyeloablative (NMA) protocol. METHODS: This is a retrospective chart review describing pulmonary outcomes in pediatric patients post HCT. The conditioning regimen consisted of alemtuzumab and a single fraction of 300 cGy of total body irradiation (TBI), and sirolimus for graft-versus-host disease (GVHD) prophylaxis. Serial PFT testing was performed pre and post HCT. The evaluated pulmonary measures included: forced vital capacity (FVC), forced expiratory volume in the first second (FEV1 ), FEV1 /FVC, and forced expiratory flow (FEF25-75 ). RESULTS: Twelve subjects were included in the analysis. All had HbSS genotype, and five of the 12 patients had one or more episodes of acute chest syndrome prior to HCT. Serial PFT measures were completed per patient. No patient was diagnosed with chronic GVHD of any organ post HCT. The baseline median FVC, FEV1 , FEV1 /FVC, and FEF25-75 were within the normal range and remained relatively unchanged post HCT. A linear mixed effects model, adjusting for gender and time from HCT, suggested no significant relationship between HCT and PFT parameters, including FVC, FEV1 , and FEV1 /FVC. Interestingly, the FEF25-75 results exhibited a shift in the means post HCT (pre-HCT 86.2% predicted and post-HCT 93.05% predicted, p-value = .018). CONCLUSION: Our study suggests that HCT in children with SCD may prevent the anticipated decline in pulmonary function over time.

Neuropsychological, behavioral, and quality-of-life outcomes in children and adolescents with sickle cell disease treated with nonmyeloablative matched sibling donor hematopoietic cell transplantation: A case series.

BACKGROUND/OBJECTIVES: Despite advances in the treatment of sickle cell disease (SCD), cerebrovascular and cognitive insults can have lifelong consequences. Hematopoietic cell transplantation (HCT) is an established curative therapy, and recent studies have demonstrated efficacy with reduced toxicity nonmyeloablative (NMA) regimens, but little is known about neuropsychological outcomes. The objective of this study was to describe neuropsychological, behavioral, and quality-of-life outcomes with medical correlates in children with SCD who received an NMA matched sibling donor (MSD) HCT. DESIGN/METHODS: Retrospective cohort analysis of nine recipients with hemoglobin SS SCD who underwent MSD HCT using the National Institutes of Health (NIH) NMA protocol. RESULTS: Mean full-scale intellectual functioning (FSIQ) was average pre-HCT (FSIQ = 92.1, SD 9.0; n = 8) and 2 years post-HCT (mean FSIQ = 96.6; SD 11.1; N = 9). Neuropsychological functioning was largely average across all cognitive domains, and no pre/post-HCT differences were found to be statistically significant given the small sample size. However, effect sizes revealed moderate improvements in processing speed (Cohen's d = .72) and verbal memory (Cohen's d = .60) post-HCT, and declines in measures of attention (Cohen's d = -.54) and fine motor speed and dexterity (Cohen's d = -.94). Parents endorsed better quality of life (Cohen's d = .91), less impact of SCD on their family, and less worry about their child's future (Cohen's d = 1.44). CONCLUSION: Neuropsychological functioning in a sample of children and adolescents treated uniformly with NMA MSD HCT remained stable or improved in most cognitive domains, and improvements in quality of life and family functioning were observed.

ADaPTS "(AD)olescents (P)ath through (T)ransplant (S)ickle cell disease".

BACKGROUND: Sickle cell disease is an inherited chronic hematological disorder with an average lifespan of fifty years. The human cost of sickle cell disease includes missed school days, occupational opportunities, social isolation, stigmatization, and psychological sequelae. Hematopoietic cell transplantation (HCT) is the only curative therapy available but comes with potential morbidity and mortality. Our study explores how quality of life (QoL) is affected from the perspective of an adolescent who has undergone a nonmyeloablative matched sibling donor HCT. METHODS: We employed multiple case study methodology with purposeful sampling by selecting information-rich cases. DATA SOURCES: 1) QoL inventories 2) patient interviews 3) parent interview 4) vital support interview 5) medical record analysis. DATA ANALYSIS: Intra-case analysis by assembling evidence within a single case and then analyzing the differences within cases to create a rich case description. Next, a time series analysis was completed to track changes in patients' QoL. We used multiple sources of data to compose a timeline and changes across time. Then, we employed pattern matching as an analytical technique allowing for examination of patterns across cases. Finally, we used cross case synthesis to review results of each case. RESULTS: Quality of life was reported across the physical, social and psychological domains for 5 participants. All had sickle cell HgSS genotype, 80% were male and 80% were born outside of Canada. Physical domain: pre-transplant, 100% of patients experienced pain, and the majority suffered from fatigue, insomnia, and fevers resulting in hospitalizations. Afterwards, participants reported improved physical wellbeing. Social domain: pre-transplant, QoL was poor characterized by stigma, social isolation, and parental absenteeism. Post-HSCT adolescents gained social acceptance in areas that had stigmatized and excluded them. They were able to participate freely in activities with peers and their social life vastly improved. Psychological pre-transplant life experiences were overshadowed by psychological stress. The majority commented that their future was bleak and may lead to premature death. Afterwards adolescents described a crisis free life with positive psychological outcomes. CONCLUSIONS: Adolescents with sickle cell disease who undertook HCT demonstrated improved QoL one year post transplant with regard to physical, social and psychological well-being.

Development of a functional and psychosocial evaluation toolkit using mixed methodology in a community-based physical activity program for childhood cancer survivors.

PURPOSE: The evidence demonstrating the benefits of exercise and PA in patients and survivors of childhood cancer has been translated into a handful of community-based programs, such as the Pediatric cancer patients and survivors Engaging in Exercise for Recovery Program (PEER). To support the translation of research to practice, the next step in knowledge translation is to evaluate program effectiveness. An evaluation must consider the goals of the PEER program, feedback from key stakeholders, and logistics of this program. Thus, the purpose of this study was to develop an evaluation toolkit with an algorithm for the implementation of the PEER program. METHODS: Semi-structured interviews were conducted with three different groups (stakeholders in pediatric oncology, PEER parents, and PEER participants). The interviews were transcribed and coded by two independent reviewers. RESULTS: Key themes extracted from the interviews were split into physical and psychosocial themes. The most reported psychosocial themes were quality of life (QOL), fatigue/energy levels, fun, and cs; and physical themes included motor skills, physical literacy, and physical activity levels. Tools were compiled into the evaluation based on key themes identified as well as logistics of PEER. An algorithm was developed to tailor the evaluation to participants based on age and mobility. CONCLUSION: To date, this is the first evaluation toolkit and algorithm developed for a specific community-based PA program, the PEER program. The next step in knowledge translation will be to implement the evaluation to assess feasibility and share the evaluation for adoption within other developing programs.

Health related quality of life in children with sickle cell disease: A systematic review and meta-analysis.

This review had three aims: 1) describe the measures used to assess health-related quality of life (HRQL) in pediatric patients diagnosed with sickle cell disease (SCD); 2) document the biopsychosocial factors related to HRQL in pediatric patients diagnosed with SCD; and 3) complete a meta-analysis comparing HRQL in pediatric patients diagnosed with SCD to healthy controls. Included studies were published in English, quantitatively assessed HRQL as a primary aim, in both SCD and controls, and included participants between 0 and 21 years of age. The final review included 66 articles, with a total of 8642 participants with SCD, 4 months-21 years of age, and 62,458 controls, 5-27 years of age. HRQL was predominately measured using the Pediatric Quality of Life Inventory Generic Core and Sickle Cell Disease Module. Meta-analyses revealed children with SCD had significantly worse HRQL compared to healthy controls (standardized mean difference = -0.93, 95% CI = -1.25, -0.61, p < 0.00001). Worse HRQL was associated with more severe SCD, female sex, and pain. The findings indicate that children with SCD are at risk for worse HRQL compared to their healthy peers and their HRQL may be impacted by several biopsychosocial factors. Future research is needed to examine how sociocultural factors uniquely impact this population and their overall quality of life.

Assessment of donor cell engraftment after hematopoietic stem cell transplantation for sickle cell disease: A review of current and future methods.

Hematopoietic stem cell transplantation (HSCT) is the only established curative treatment for sickle cell disease (SCD), a debilitating red blood cell (RBC) disorder with significant prevalence worldwide. Accurate assessment of RBC engraftment following HSCT is essential to evaluate the status of the graft and can enable early intervention to treat or prevent graft rejection. Currently, chimerism measurement is performed on whole blood samples, which mainly reflect white blood cell (WBC) chimerism. This approach has limitations in assessing engraftment in patients with SCD because RBCs engraft non-linearly with WBCs. Direct measures of RBC chimerism exist but are not routinely used. In this review, we critically examine the current methodologies for assessing donor engraftment; highlight the limitations of these different methods, and present emerging and novel technologies with the potential to improve clinical monitoring of RBC engraftment post-HSCT for SCD. Promising alternative methodologies include RBC-specific flow cytometry, RBC-specific RNA analysis, and quantification of plasma cell-free DNA derived specifically from nucleated RBCs.

Measuring fear of cancer recurrence in survivors of childhood cancer: Development and preliminary validation of the Fear of Cancer Recurrence Inventory (FCRI)-Child and Parent versions.

OBJECTIVE: Fear of cancer recurrence (FCR) is a common and distressing psychosocial concern for adult cancer survivors. Data on this construct in child survivors is limited and there are no validated measures for this population. This study aimed to adapt the Fear of Cancer Recurrence Inventory-Short Form (FCRI-SF) for survivors of childhood cancer aged 8-18 years (Fear of Cancer Recurrence Inventory-Child version [FCRI-C]) and their parents (Fear of Cancer Recurrence Inventory-Parent version [FCRI-P]) to self-report on their own FCR and to examine the initial psychometric properties. METHODS: The FCRI-SF was adapted through expert panel input and cognitive interviews with child survivors <18 years. The factor structure, internal consistency and construct and criterion validity of the FCRI-C and FCRI-P were examined in 124 survivors of childhood cancer (43% female; Mage  = 14.58 years, SD = 2.90) and 106 parents (90% mothers). RESULTS: All FCRI-SF items were retained for the FCRI-C with simplified language. The internal consistencies of the FCRI-C (α = 0.88) and FCRI-P (α = 0.83) were good. Exploratory factor analyses yielded one-factor structures for both measures. Higher scores on the FCRI-C and FCRI-P were associated with greater intolerance of uncertainty and pain catastrophizing. Higher child FCR was also related to more hypervigilance to bodily symptoms. Parents with higher FCR reported contacting their child's doctors and nurses and scheduling medical appointments for their child more frequently. Children reported significantly lower FCR compared to parents. CONCLUSIONS: The FCRI-C and FCRI-P demonstrated strong reliability and preliminary validity. This study offers preliminary data to support the use of the FCRI-C and FCRI-P to measure FCR in survivors of childhood cancer aged 8-18 years and their parents.

Psychosocial risk, symptom burden, and concerns in families affected by childhood cancer.

PURPOSE: The revised Psychosocial Assessment Tool (PATrev) is a common family-level risk-based screening tool for pediatric oncology that has gained support for its ability to predict, at diagnosis, the degree of psychosocial support a family may require throughout the treatment trajectory. However, ongoing screening for symptoms and concerns (e.g., feeling alone, understanding treatment) remains underutilized. Resource limitations necessitate triaging and intervention based on need and risk. Given the widespread use of the PATrev, we sought to explore the association between family psychosocial risk, symptom burden (as measured by the revised Edmonton Symptom Assessment System (ESAS-r)), and concerns (as measured by the Canadian Problem Checklist (CPC)). METHODS: Families (n = 87) with children ≤ 18 years of age (M = 11.72, male: 62.1%) on or off treatment for cancer were recruited from the Alberta Children's Hospital. One parent from each family completed the PATrev and the CPC. Participants 8-18 years of age completed the ESAS-r. RESULTS: Risk category (universal/low risk = 67.8%, targeted/intermediate risk = 26.4%, clinical/high risk = 5.7%) predicted symptom burden (F[2, 63.07] = 4.57, p = .014) and concerns (F[2, 82.06] = 16.79, p < .001), such that universal risk was associated with significantly lower symptom burden and fewer concerns. CONCLUSION: Family psychosocial risk is associated with cross-sectionally identified concerns and symptom burden, suggesting that resources might be prioritized for families with the greatest predicted need. Future research should evaluate the predictive validity of the PATrev to identify longitudinal concerns and symptom burden throughout the cancer trajectory.

Implementing compassion in pediatric healthcare: A qualitative study of Canadian patients', parents', and healthcare providers' perspectives.

BACKGROUND: Compassion has received significant scholarly attention over the past decade. Research has been largely theoretical, with interventions focused on self-care practices of healthcare providers (HCPs), rather than implementation at a systems level. This study aimed to identify how compassion can be operationalized within pediatric healthcare. DESIGN AND METHODS: Data was analyzed from a secondary dataset of a larger Straussian grounded theory study of perspectives and experiences of compassion in pediatric healthcare. Patients (n = 33); parents (n = 16); and HCPs (n = 17) were asked specifically how compassion could be implemented within the clinical culture and healthcare system. RESULTS: 66 participants generated an operational model of compassion indicating how compassion could be implemented across the organization and larger healthcare system. The data revealed four themes and associated subthemes: teach and train; recognize and reward; measure and report; and embed compassion across the healthcare system. CONCLUSIONS: Improving compassion in pediatric healthcare needs to extend beyond the efforts of individual HCPs. Compassion is the responsibility of the entire healthcare system and needs to traverse the patient and family experience. In addition to embedding compassion in policy, procedures, practice, and education, compassion should be considered a performance indicator that is measured and reported. PRACTICE IMPLICATIONS: This study provides a preliminary framework for organizational leaders to operationalize compassion across the services, structures, polices, procedures and practices of pediatric healthcare. This includes ongoing compassion training across the organization; assessing compassion, recognizing compassion as a performance indicator, and ensuring that the infrastructure and ancillary services of the organization reflect compassion.