Right Heart Remodeling After Pulmonary Valve Replacement in Patients With Pulmonary Atresia or Critical Stenosis With Intact Ventricular Septum.

Background Patients with pulmonary atresia or critical pulmonary stenosis with intact ventricular septum (PA/IVS) and biventricular circulation may require pulmonary valve replacement (PVR). Right ventricular (RV) remodeling after PVR is well described in tetralogy of Fallot (TOF); we sought to investigate RV changes in PA/IVS using cardiac magnetic resonance imaging. Methods and Results A retrospective cohort of patients with PA/IVS who underwent PVR at Boston Children's Hospital from 1995 to 2021 with cardiac magnetic resonance imaging before and after PVR was matched 1:3 with patients with TOF by age at PVR. Median regression modeling was performed with post-PVR indexed RV end-diastolic volume as the primary outcome. A total of 20 patients with PA/IVS (cases) were matched with 60 patients with TOF (controls), with median age at PVR of 14 years. Pre-PVR indexed RV end-diastolic volume was similar between groups; cases had higher RV ejection fraction (51.4% versus 48.6%; P=0.03). Pre-PVR RV free wall and left ventricular (LV) longitudinal strain were similar, although LV midcavity circumferential strain was decreased in cases (-15.6 versus -17.1; P=0.001). At a median of 2 years after PVR, indexed RV end-diastolic volume was similarly reduced; cases continued to have higher RV ejection fraction (52.3% versus 46.9%; P=0.007) with less reduction in RV mass (Δ4.5 versus 9.6 g/m2; P=0.004). Post-PVR, RV and LV longitudinal strain remained unchanged, and LV circumferential strain was similar, although lower in cases. Conclusions Compared with patients with TOF, patients with PA/IVS demonstrate similar RV remodeling after PVR, with lower reduction in RV mass and comparatively higher RV ejection fraction. Although no differences were detected in peak systolic RV or LV strain values, further investigation of diastolic parameters is needed.

A case of neonatal sweet syndrome associated with mevalonate kinase deficiency.

BACKGROUND: Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopathologic variant of SS. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with H-SS. CASE PRESENTATION: Through a multidisciplinary approach, this patient was also found to have very early onset inflammatory bowel disease (VEO-IBD) and Mevalonate kinase-associated disease (MKAD). While prior case studies have characterized an association between VEO-IBD and MKAD, there is no literature describing the association of all three diagnoses this case: H-SS, VEO-IBD and MKAD. Initiation of canakinumab in this patient resulted in successful control of the disease. CONCLUSIONS: This case highlights the importance of a multidisciplinary approach to rare diagnoses, and collaboration during cases with significant diagnostic uncertainty.

The COVID-19 pandemic and its potential enduring impact on children.

PURPOSE OF REVIEW: The COVID-19 pandemic has ushered in great and rapid change in our society. Although children are somewhat less likely to get infected or have severe symptoms from COVID-19, they are being adversely affected by this global public health emergency in many direct and indirect ways. This review focuses on the major areas in which children and adolescents are suffering, and how pediatricians can anticipate and optimize child healthcare and support as the COVID-19 pandemic and its aftermath continues. RECENT FINDINGS: This review provides preliminary insights into the physical, psychological, educational, developmental, behavioral, and social health implications of the pandemic on the pediatric population, highlighting both the pandemic's current and potential future impact on children. SUMMARY: The COVID-19 pandemic has and will likely continue to adversely affect many different aspects of children's health and well-being. It is important for pediatricians to be aware of these consequences of COVID-19 and take steps to help their patients now and in the future. Because the virus continues to ravage many parts of the world, continued research is needed to identify and evaluate any additional COVID-related challenges and concerns that adversely impact the growth and development of children.

Native Bicuspid Pulmonary Valve in D-Loop Transposition of the Great Arteries: Outcomes of the Neo-Aortic Valve Function and Root Dilation After Arterial Switch Operation.

Background Neo-aortic root dilation and neo-aortic regurgitation (AR) are common after arterial switch operation for D-loop transposition of the great arteries. We sought to evaluate these outcomes in patients with bicuspid native pulmonary valve (BNPV). Methods and Results A retrospective analysis of patients with transposition of the great arteries undergoing arterial switch operation at Boston Children's Hospital from 1989 to 2018 matched BNPV patients 1:3 with patients with tricuspid native pulmonary valve by year of arterial switch operation. Kaplan-Meier analyses with log-rank test compared groups for time to first neo-aortic valve reoperation, occurrence of ≥moderate AR, and neo-aortic root dilation (root z score ≥4). A total of 83 patients with BNPV were matched with 217 patients with tricuspid native pulmonary valve. Patients with BNPV more often had ventricular septal defects (73% versus 43%; P<0.001). Hospital length of stay (11 versus 10 days) and 30-day surgical mortality (3.6% versus 2.8%) were similar. During median 11 years follow-up, neo-aortic valve reoperation occurred in 4 patients with BNPV (6.0%) versus 6 patients with tricuspid native pulmonary valve (2.8%), with no significant difference in time to reoperation. More BNPV had AR at discharge (4.9% versus 0%; P=0.014) and during follow-up (13.4% versus 4.3%; hazard ratio [HR], 3.9; P=0.004), with shorter time to first occurrence of AR; this remained significant after adjusting for ventricular septal defects. Similarly, neo-aortic root dilation was more common in BNPV (45% versus 38%; HR, 1.64; P=0.026) with shorter time to first occurrence. Conclusions While patients with BNPV have similar short-term arterial switch operation outcomes, AR and neo-aortic root dilation occur more frequently and earlier compared with patients with tricuspid native pulmonary valve. Further long-term studies are needed to determine whether this results in greater need for neo-aortic valve reoperation.

Impact of immigration status on health behaviors and perceptions in cancer survivors.

BACKGROUND: Health behaviors including smoking cessation, physical activity (PA), and alcohol moderation are key aspects of cancer survivorship. Immigrants may have unique survivorship needs. We evaluated whether immigrant cancer survivors had health behaviors and perceptions that were distinct from native-born cancer survivors. METHODS: Adult cancer patients from Princess Margaret Cancer Centre were surveyed on their smoking, PA, and alcohol habits and perceptions of the effects of these behaviors on quality of life (QoL), 5-year survival, and fatigue. Multivariable models evaluated the association of immigration status and region-of-origin on behaviors and perceptions. RESULTS: Of the 784 patients, 39% self-identified as immigrants. Median time of survey was 24 months after histological diagnosis. At baseline, immigrants had trends toward not meeting Canadian PA guidelines or being ever-drinkers; patients from non-Western countries were less likely to smoke (aORcurrent  = 0.46, aORex-smoker  = 0.47, P = 0.02), drink alcohol (aORcurrent  = 0.22, aORex-drinker  = 0.52, P < 0.001), or meet PA guidelines (aOR = 0.44, P = 0.006). Among immigrants, remote immigrants (migrated ≥40 years ago) were more likely to be consuming alcohol at diagnosis (aOR = 5.70, P < 0.001) compared to recent immigrants. Compared to nonimmigrants, immigrants were less likely to perceive smoking as harmful on QoL (aOR = 0.58, P = 0.008) and survival (aOR = 0.56, P = 0.002), and less likely to perceive that PA improved fatigue (aOR = 0.62, P = 0.04) and survival (aOR = 0.64, P = 0.08). CONCLUSIONS: Immigrants had different patterns of health behaviors than nonimmigrants. Immigrants were less likely to perceive continued smoking as harmful and were less likely to be aware of PA benefits. Culturally tailored counselling may be required for immigrants who smoke or are physically sedentary at diagnosis.

Features of Catatonia in a 12-Year-Old Boy with Autism Spectrum Disorder.

CASE: Thomas is a 12-year-old boy with autism spectrum disorder who presents to his primary care clinician with symptoms of worsening mood in the last 3 months. On review of his last school testing, his cognitive abilities are found to be within the average range, with a relative vulnerability with his processing speed. He can speak in sentences to communicate and answer questions, but he rarely picks up on conversational bids. He has had difficulties developing friendships and often prefers to play by himself.Thomas has a long history of some features of anxiety and depression for which it was recommended that he establish care with a therapist, but his family has had a hard time finding a provider for him. At this visit, the mother reports that for the past several months he has been more anxious, sad, and easily overwhelmed. He seems irritable at home and school and cries often. His family has been advocating for him to receive increased school supports, as school is a source of anxiety for him, but there are no recent changes in school services. There is a family history of both anxiety and depression. Given his worsening mood functioning, Thomas was started on selective serotonin reuptake inhibitor (SSRI) medication in addition to again recommending a therapist. Weekly phone call check-ins and an in-person clinic visit in 1 month are planned.About 1 month after starting the SSRI medication, he is still not showing any improvement in mood functioning, and his family reports he seems more "sluggish" than usual. There are no side effects reported with the medication, and the dose is increased to see whether it will help. However, about 2 weeks later, he is seen again in the clinic because there are increasing concerns. He continues to be "sluggish." During the clinic visit, he lies down on the examination table, sometimes holding his head off the edge of the table, which he has never done before. He responds very slowly to the questions and often says "I don't know, I don't know," almost in an automatic way. His mother reports that he is now engaging in some repetitive hand movements which he had not done previously. He is no longer able to shower independently. He is still eating and drinking adequately. What would you do next?

Patterns, perceptions, and perceived barriers to physical activity in adult cancer survivors.

PURPOSE: Physical activity (PA) during and after cancer treatment is associated with improved cancer- and non-cancer-related outcomes. We assessed for predictors of change in PA levels among cancer survivors. METHODS: Adult cancer survivors from a comprehensive cancer center completed a one-time questionnaire retrospectively assessing PA levels before, during, and after cancer treatment along with their perceptions of PA. Multivariable logistic regression models evaluated the association of clinico-demographics variables and perceptions of PA with changes in whether patients were meeting PA guidelines after cancer diagnosis. RESULTS: Among the 1003 patients, 319 (32%) met moderate to vigorous PA (MVPA) guidelines before diagnosis. Among those meeting guidelines before diagnosis, 50% still met guidelines after treatment; 12% not meeting MVPA guidelines initially met them after treatment/at follow-up. Among patients meeting guidelines before diagnosis, better ECOG performance status at follow-up, receiving curative therapy, and spending a longer time on PA initially were each associated with meeting guidelines at follow-up. After controlling for other variables, perceiving that PA improves quality of life (adjusted odds ratio, aOR = 11.09, 95%CI [1.42-86.64], P = 0.02) and overall survival (aOR = 8.52, 95%CI [1.12-64.71], P = 0.04) was each associated with meeting MVPA guidelines during/after treatment, in patients who did not meet guidelines initially. Only 13% reported receiving counseling, which was not associated with PA levels. Common reported barriers to PA included fatigue, lacking motivation, and being too busy. CONCLUSIONS: Patient perceptions of PA benefits are strongly associated with improving PA levels after a cancer diagnosis. Clinician counseling should focus on patient education and changing patient perceptions.

Oncology Nurses' Knowledge, Confidence, and Practice in Addressing Caregiver Strain and Burden

OBJECTIVES: To describe nurses' practices, confidence, and knowledge of evidence-based interventions for cancer caregiver strain and burden and to identify factors that contribute to these aspects. 
. SAMPLE & SETTING: 2,055 Oncology Nursing Society members completed an emailed survey.
. METHODS & VARIABLES: Pooled analysis of survey results. Variables included the baseline nursing assessment, intervention, confidence, knowledge, strategies used, and barriers encountered. 
. RESULTS: Nurses tend to overestimate the strength of evidence for interventions not shown to be effective and have moderate confidence in assessing and intervening with caregivers. Having been an informal caregiver and having received care from an informal caregiver were associated with higher reported practice and confidence. Major strategies used were referral to social workers and others. Barriers reported were financial, caregiver emotional responses, and distance. 
. IMPLICATIONS FOR NURSING: An opportunity exists to increase nurses' knowledge and confidence in assessment and intervention with caregivers. Greater use of technology may help nurses overcome some barriers to working with caregivers. Findings can be used to plan continuing education, develop clinical processes, and identify resources nurses need to address strain and burden among informal caregivers.

Cancer-Related Pain: Assessment and Management With Putting Evidence Into Practice Interventions
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Many evidence-based pharmacologic and nonpharmacologic strategies exist to manage acute, chronic, refractory, and breakthrough cancer pain. This supplement includes an overview of cancer pain assessment, which is the foundation of pain management. Following the overview are four systematic reviews covering 462 studies on cancer pain management. The Oncology Nursing Society Putting Evidence Into Practice (PEP) expert panel summarized the evidence and provided recommendations for practice based on PEP guidelines.

EQ-5D Health Utility Scores: Data from a Comprehensive Canadian Cancer Centre.

BACKGROUND: To improve the precision of health economics analyses in oncology, reference datasets of health utility (HU) scores are needed from cancer survivors across different disease sites. These data are particularly sparse amongst Canadian survivors. METHODS: A survey was completed by 1759 ambulatory cancer survivors at the Princess Margaret Cancer Centre which contained demographic questions and the EuroQol-5D (EQ-5D) instrument. Clinical information was abstracted from electronic records and HU scores were calculated using Canadian health state valuations. Construct validity was assessed through correlation of HU and visual analog scale (VAS) scores (Spearman) and by comparing HU scores between performance status groups (effect size). The influence of socio-demographic clinical variables on HU was analyzed by non-parametric between-group comparisons and multiple linear regression. RESULTS: Mean EQ-5D HU scores were derived for 26 cancers. Among all survivors, the mean ± standard error of the mean EQ-5D utility score was 0.81 ± 0.004. Scores varied significantly by performance status (p < 0.0001) and correlated with VAS (Spearman r = 0.61). The cancer sites with the lowest mean HU scores were acute lymphoblastic leukemia (0.70 ± 0.03) and pancreatic cancer (0.76 ± 0.03); testicular cancer (0.89 ± 0.02) and chronic lymphocytic leukemia (0.90 ± 0.05) had the highest mean scores. A multiple regression model showed that scores were influenced by disease site (p < 0.001), education level (p < 0.001), partner status (p < 0.001), disease extent (p = 0.0029), and type of most recent treatment (p = 0.0061). CONCLUSIONS: This work represents the first set of HU scores for numerous cancer sites derived using Canadian preference weights. The dataset demonstrated construct validity and HU scores varied by general socio-demographic and clinical parameters.

Cancer Patients? Willingness to Routinely Complete the EQ-5D Instrument at Clinic Visits.

BACKGROUND: Health utility (HU) scores play an essential role in pharmacoeconomic analyses. Routine clinical administration of the EuroQol-5 Dimensions (EQ-5D) can allow for HU and health related quality of life (HRQOL) assessments in the real-world setting. OBJECTIVES: The primary goals of this study were to evaluate whether patients were willing to complete the EQ-5D instrument on a routine basis and which clinical or demographic factors influence this willingness. METHODS: 618 adult cancer survivors across multiple cancer disease sites at the Princess Margaret Cancer Centre completed an acceptability survey after completing the EQ-5D instrument. RESULTS: The mean (SD) EQ-5D score was 0.81 (0.15). Among those surveyed, 88% reported that the EQ-5D was easy to complete. 91% took under 5 minutes and 88% were satisfied with its length. 85% were satisfied with the types of questions asked on the EQ-5D. Importantly, 92% reported that they would complete the EQ-5D, even if it were used solely for research purposes; 73% agreed with the notion of completing it regularly at their clinic visits. Patients with lower EQ-5D scores (p=0.0006), and non-Caucasians (p=0.0024; 60% willing) were less willing to complete the instrument on a regular basis. Curability of tumour, disease site, age, and gender did not influence willingness. CONCLUSIONS: The majority of cancer patients across disease sites are willing to complete the EQ-5D instrument regularly, even if it were solely for research purposes, but up to 39% declined participation in the first place.

Feasibility and diagnostic accuracy of the Patient-Reported Outcomes Measurement Information System (PROMIS) item banks for routine surveillance of sleep and fatigue problems in ambulatory cancer care.

BACKGROUND: Routine screening for problematic symptoms is emerging as a best practice in cancer systems globally. The objective of this observational study was to assess the feasibility and diagnostic accuracy of Patient-Reported Outcomes Measurement Information System (PROMIS) computerized adaptive testing (CAT) for fatigue and sleep-disturbance items compared with legacy measures in routine ambulatory cancer care. METHODS: Patients who attended outpatient clinics at the Princess Margaret Cancer Center completed PROMIS CAT item banks and legacy measures (the Functional Assessment of Chronic Illness Therapy [FACIT]-Fatigue scale and the Insomnia Severity Index [ISI]) using tablet computers during clinic visits. The completion rates, patient acceptability, and diagnostic accuracy of PROMIS CAT were evaluated against legacy measures using receiver operating characteristic (ROC) curve analysis. RESULTS: Participants consisted of 336 patients (mean age ± standard deviation, 57.4 ± 15.7 years; 55% females; 75% Caucasian). Over 98% of patients did not find symptom screening was burdensome, although only 65% were willing to complete the survey at every visit. PROMIS CAT scores were significantly correlated with both FACIT-Fatigue scores (r = -0.83) and ISI scores (r = -0.57; p < 0.0001 for all). Areas under the curve (AUC) by ROC analysis for fatigue were 0.946 using the FACIT-Fatigue cutoff ≤30, 0.910 for sleep disturbance, and 0.922 for sleep impairment using the ISI cutoff ≥15. The recommended T-score cut-off for PROMIS CAT Fatigue was 57, Sleep Disturbance was 57, and Sleep Impairment was 57. CONCLUSIONS: The current results support the feasibility and accuracy of PROMIS CAT and its potential for use in routine ambulatory cancer care. Future research will assess feedback of these data to clinicians and evaluate effects on earlier identification of and intervention for these problems. Cancer 2016. © 2016 American Cancer Society. Cancer 2016;122:2906-2917. © 2016 American Cancer Society.

Theoretical foundations of adherence behaviors: synthesis and application in adherence to oral oncology agents.

BACKGROUND: The use of theory can provide structure for understanding the dynamics of adherence to oral agents for cancer (OACs) and facilitate the development of strategies for patient assessment and care planning related to oral adherence. OBJECTIVES: The focus of this article is to identify common theories pertinent to medication-taking behavior, as well as their applications toward assessment and care planning regarding adherence to OACs. Approaches to apply these theories and their concepts in clinical practice are identified. METHODS: Theories applied to adherence behaviors were reviewed, and their concepts were compared and synthesized. These concepts were then applied to medication adherence behavior. FINDINGS: Various theories can help healthcare providers to better understand the influences and determinants of patients' adherence decisions and behaviors. They can then be used to structure assessment and plan related aspects of care.

The experience of implementing evidence-based practice change: a qualitative analysis.

The Oncology Nursing Society (ONS) and ONS Foundation worked together to develop the Institute for Evidence-Based Practice Change (IEBPC) program to facilitate the implementation of evidence-based practice (EBP) change in nursing. This analysis describes the experience of 19 teams of nurses from various healthcare settings who participated in the IEBPC program. Qualitative analysis of verbatim narratives of activities and observations during the process of implementing an EBP project was used to identify key themes in the experience. EBP implementation enabled participants to learn about their own practice and to experience empowerment through the evidence, and it ignited the spirit of inquiry, team work, and multidisciplinary collaboration. Experiences and lessons learned from nurses implementing EBP can be useful to others in planning EBP implementation.

Measuring oncology nurses' psychosocial care practices and needs: results of an Oncology Nursing Society psychosocial survey.

PURPOSE/OBJECTIVES: To develop and implement a survey of Oncology Nursing Society (ONS) members focused on their current practices and needs in relation to providing psychosocial care. DESIGN: Descriptive, cross-sectional. SETTING: Web-based survey of ONS members. SAMPLE: An invitation was e-mailed to 11,171 ONS members. Of those, 623 followed the link to the electronic survey and 64% of those (n = 401) completed the survey. METHODS: An ONS Psychosocial Project Team was convened in 2009. One of the team's goals was to develop a survey to assess members' needs. The final survey consisted of 24 items, including five items related to demographic characteristics. Response formats included Likert-type scale, yes and no, and open-ended questions. MAIN RESEARCH VARIABLES: Psychosocial care practices, education, and research. FINDINGS: Psychosocial concerns are assessed using a variety of methods. Nurse perceptions regarding primary responsibility for providing psychosocial services differ by group. Barriers to the provision of psychosocial care exist at the individual, institutional, and community levels. CONCLUSIONS: Although nurses assess patients' psychosocial needs, multiple barriers still exist related to interdisciplinary communication; knowledge of the Institute of Medicine's recommendations; and resources at the individual, institutional, and community levels. IMPLICATIONS FOR NURSING: The survey results were presented to the ONS Board of Directors, along with a three-year plan that included recommendations for future development of advocacy, practice, education, and research initiatives. Additional work is needed to effectively support RNs in their provision of psychosocial care to patients and families.

Improving cancer survivorship care: oncology nurses' educational needs and preferred methods of learning.

Oncology nurses are essential in all phases of cancer care; however, their role in survivorship care is unclear. To better understand the self-reported knowledge and educational needs on topics of survivorship care and oncology nurses' learning preferences, an online survey was conducted. Respondents self-reported knowledge level for 31 care topics, identified areas of most interest, topics needed to assist patients and address patient questions, and reported participation in continuing education and preferred learning methods. Knowledge was rated highest for topics of fatigue, anxiety, and fear of recurrence and lowest for issues related to finance, employment, and insurance. Nurses were most interested in late and long-term physical effects of cancer or treatment, managing emotional issues, cancer screening and surveillance, and complementary and alternative therapies. Study findings suggest that online learning methods would be feasible and well accepted by nurses to meet continuing education needs related to cancer survivorship.

Transfer of glass fragments when bottles and drinking glasses are broken.

Experiments have been carried out to determine if and how many glass fragments are transferred onto upper garments following breakage of bottles and drinking glasses. In all instances glass was transferred. The numbers of transferred fragments after a bottle is broken ranges from three to twenty five. The numbers of fragments transferred following the breakage of a drinking glass ranges from three to approximately one hundred and twenty. On average three times the amount of glass is transferred following breakage of a drinking glass as compared to breakage of a bottle.

Oncology nurses' perspectives on the state of cancer survivorship care: current practice and barriers to implementation.

PURPOSE/OBJECTIVES: to describe current survivorship care from the perspectives of oncology nurses. DESIGN: descriptive. SETTING: e-mail invitation to Web-based survey. SAMPLE: 399 Oncology Nursing Society members providing care for patients initially treated more than one year previously. METHODS: an online survey was used to evaluate current aspects of survivorship care. MAIN RESEARCH VARIABLES: practice settings, services provided, and barriers to delivering survivorship care. FINDINGS: few nurses (27%) worked in settings with a formal survivorship program. Several program components were provided significantly more often in outpatient settings, pediatric facilities, and workplaces with a formal survivorship program. At the transition from acute to follow-up care, the survivorship nursing care provided most often was scheduling for ongoing monitoring (71%) and the least likely was assistance for employment or legal issues (16%). The greatest barriers to providing survivorship care were lack of time and funding (46%). Among nurses new to oncology (fewer than five years), 49% indicated they lacked sufficient knowledge compared to 36% of nurses with more than five years of oncology experience. CONCLUSIONS: findings describe current aspects of survivorship care across practice settings. Nurses reported that the greatest barriers are lack of time, funding, and lack of knowledge about survivorship issues. IMPLICATIONS FOR NURSING: a need exists for education to enhance knowledge and skills of nurses who will provide survivorship care. Research is warranted to develop empirically supported guidelines and care-delivery models that address the barriers to providing survivorship services.