Lung Allocation Score Exceptions in Persons with Cystic Fibrosis Undergoing Lung Transplant.

Rationale: Lung transplantation can extend the lives of individuals with advanced cystic fibrosis (CF). Until March 2023, the Lung Allocation Score (LAS) was used in the United States to determine transplant priority. Certain clinical events or attributes ("risk events") that are not included in the LAS (e.g., massive hemoptysis) are relatively common and prognostically important in CF and may prompt an exception request to increase priority for donor lungs. The new Lung Composite Allocation Score (CAS) also allows for exceptions based on the same principles. Objectives: To evaluate the frequency of LAS exceptions in persons with CF (PwCFs) listed for lung transplantation and assess whether LAS exceptions are associated with improved waitlist outcomes for PwCFs compared with similarly "at-risk" individuals without LAS exceptions. Methods: A merged dataset combining data from the CF Foundation Patient Registry and the Organ Procurement and Transplantation Network (2005-2019) was used to identify PwCFs listed for lung transplantation. We compared waitlist outcomes between PwCFs with a LAS exception versus those without an exception despite having a risk event. Risk events were defined as an episode of massive hemoptysis, pneumothorax, at least three moderate/severe pulmonary exacerbations, and/or a decrease in forced expiratory volume in 1 second by ⩾30% predicted (absolute) in the prior 12 months. Analyses were performed using competing risk regression with time to transplantation as the primary outcome and death without a transplant as a competing risk. Results: Of 3,538 listings from 3,309 candidates, 2% of listings (n = 81) had at least one exception. Candidates with an exception and those with a risk event but no exception received lung transplants more slowly than people without an exception or risk event (subdistribution hazard ratio [95% confidence interval]: LAS exception cohort, 0.66 [0.52-0.85]; risk event cohort without exceptions, 0.79 [0.72-0.86]). There was no difference between those with LAS exceptions and those at risk without LAS exceptions: subdistribution hazard ratio, 0.84 (0.66-1.08). Conclusions: LAS exceptions are rare in PwCFs listed for lung transplantation. LAS exceptions resulted in a similar time to transplantation for PwCFs compared with similarly at-risk individuals. As we enter the CAS era, these LAS-based results are pertinent to improve risk stratification among PwCFs being considered for lung transplantation.

Race, ethnicity, and experienced racism are associated with adverse physical and mental health outcomes among cancer survivors.

INTRODUCTION: Survivors of cancer are at risk for adverse mental and physical health outcomes. It is not well understood, however, how these outcomes are differentially experienced according to an individual's exposure to racism. This study sought to evaluate associations of race/ethnicity, and experiences of racism, with adverse health outcomes in survivors of cancer. METHODS: Using the Behavioral Risk Factor Surveillance System database, data from 48,200 survivors between 2014 and 2020 were evaluated. Survey items included negative physical and emotional symptoms as a result of race-based treatment. Outcomes of interest included days of poor mental and physical health, activity limitations, depression, and inadequate sleep. Associations using prevalence ratios were evaluated. RESULTS: All historically marginalized racial/ethnic groups were more likely to experience at least one adverse health outcome compared with non-Hispanic White survivors. Those who physically experienced racism were 2.1 (95% CI, 1.64-2.69) times as likely to report poor physical health, 3.51 (95% CI, 2.61-4.71) times as likely to report poor mental health, 2.14 (95% CI, 1.77-2.58) times as likely to report inadequate sleep, 2.33 (95% CI: 1.91-2.83) times as likely to report depression, and 1.42 (95% CI, 1.04-1.93) times as likely to report activity limitations compared with those who have not experienced racism. Similar associations were observed for emotionally experienced racism. DISCUSSION: Racial inequities in health outcomes for survivors of cancer from marginalized racial/ethnic groups are well-established. Experienced racism contributes to adverse health outcomes and widens these disparities. Improving outcomes for survivors of cancer may require screening for experienced racism. PLAIN LANGUAGE SUMMARY: Survivors of cancer from marginalized racial/ethnic populations are more likely to have poor mental and physical health than their non-Hispanic White counterparts. Whether survivors from certain racial/ethnic populations of smaller size also have poorer health is less well understood. Generally, individuals who report experienced racism also report poor health, this association has not been studied in survivors of cancer. This study, from a national survey of survivors of cancer, describes disparities in health outcomes experienced by a variety of racial and ethnic populations. Our findings suggest racism is associated with poor mental and physical health in survivors of cancer.

Protocol for the promoting resilience in stress management (PRISM) intervention: a multi-site randomized controlled trial for adolescents and young adults with advanced cancer.

BACKGROUND: Adolescents and young adults (AYAs) with cancer are at high risk of poor psychosocial outcomes, and evidence-based interventions designed to meet their psychosocial and communication needs are lacking. The main objective of this project is to test the efficacy of a new adaptation of the Promoting Resilience in Stress Management intervention for AYAs with Advanced Cancer (PRISM-AC). METHODS/DESIGN: The PRISM-AC trial is a 2-arm, parallel, non-blinded, multisite, randomized controlled trial. 144 participants with advanced cancer will be enrolled and randomized to either usual, non-directive, supportive care without PRISM-AC ("control" arm) or with PRISM-AC ("experimental" arm). PRISM is a manualized, skills-based training program comprised of four 30-60 min, one-on-one sessions targeting AYA-endorsed resilience resources (stress-management, goal-setting, cognitive-reframing, and meaning-making). It also includes a facilitated family meeting and a fully equipped smartphone app. The current adaptation includes an embedded advance care planning module. English- or Spanish-speaking individuals 12-24 years old with advanced cancer (defined as progressive, recurrent, or refractory disease, or any diagnosis associated with < 50% survival) receiving care at 4 academic medical centers are eligible. Patients' caregivers are also eligible to participate in this study if they are able to speak and read English or Spanish, and are cognitively and physically able to participate. Participants in all groups complete surveys querying patient-reported outcomes at the time of enrollment and 3-, 6-, 9-, and 12-months post-enrollment. The primary outcome of interest is patient-reported health-related quality of life (HRQOL) and secondary outcomes of interest include patient anxiety, depression, resilience, hope and symptom burden, parent/caregiver anxiety, depression and health-related quality of life, and family palliative care activation. We will conduct intention-to-treat analysis to compare the group means of primary and secondary outcomes between PRISM-AC arm and control arm with regression models. DISCUSSION: This study will provide methodologically rigorous data and evidence regarding a novel intervention to promote resilience and reduce distress among AYAs with advanced cancer. This research has the potential to offer a practical, skills-based curriculum designed to improve outcomes for this high-risk group. TRIAL REGISTRATION: ClinicalTrials.gov Identifier NCT03668223, September 12, 2018.

Protocol for The Promoting Resilience in Stress Management (PRISM) Intervention: a multi-site randomized controlled trial for adolescents and young adults with advanced cancer.

Background Adolescents and young adults (AYAs) with cancer are at high risk of poor psychosocial outcomes, and evidence-based interventions designed to meet their psychosocial and communication needs are lacking. The main objective of this project is to test the efficacy of a new adaptation of the Promoting Resilience in Stress Management intervention for AYAs with Advanced Cancer (PRISM-AC). Methods/design: The PRISM-AC trial is a 2-arm, parallel, non-blinded, multisite, randomized controlled trial. 144 participants with advanced cancer will be enrolled and randomized to either usual, non-directive, supportive care without PRISM-AC ("control" arm) or with PRISM-AC ("experimental" arm). PRISM is a manualized, skills-based training program comprised of four 30-60 minute, one-on-one sessions targeting AYA-endorsed resilience resources (stress-management, goal-setting, cognitive-reframing, and meaning-making). It also includes a facilitated family meeting and a fully equipped smartphone app. The current adaptation includes an embedded advance care planning module. English- or Spanish-speaking individuals 12-24 years old with advanced cancer (defined as progressive, recurrent, or refractory disease, or any diagnosis associated with < 50% survival) receiving care at 4 academic medical centers are eligible. Patients' caregivers are also eligible to participate in this study if they are able to speak and read English or Spanish, and are cognitively and physically able to participate. Participants in all groups complete surveys querying patient-reported outcomes at the time of enrollment and 3-, 6-, 9-, and 12-months post-enrollment. The primary outcome of interest is patient-reported health-related quality of life (HRQOL) and secondary outcomes of interest include patient anxiety, depression, resilience, hope and symptom burden, parent/caregiver anxiety, depression and health-related quality of life, and family palliative care activation. We will conduct intention-to-treat analysis to compare the group means of primary and secondary outcomes between PRISM-AC arm and control arm with regression models. Discussion This study will provide methodologically rigorous data and evidence regarding a novel intervention to promote resilience and reduce distress among AYAs with advanced cancer. This research has the potential to offer a practical, skills-based curriculum designed to improve outcomes for this high-risk group. Trial registration: ClinicalTrials.gov Identifier NCT03668223, September 12, 2018.

Rectal suction biopsy versus incisional rectal biopsy in the diagnosis of Hirschsprung disease.

BACKGROUND: Hirschsprung disease is one of the most common congenital anomalies that affect colorectal function. Rectal biopsy demonstrating the absence of ganglion cells in the affected bowel is the gold standard for diagnosis. Suction and incisional rectal biopsies are appropriate methods for obtaining diagnostic tissue. The goal of this study is to determine if any differences in adequacy exist between suction and incisional rectal biopsies at our institution. METHODS: We conducted a retrospective review of suction and incisional rectal biopsies for inadequacy per procedure at a tertiary pediatric hospital. Each procedure for rectal biopsy was also evaluated by a number of biopsies per procedure. We used a two-sample test of proportions to compare the inadequacy of suction vs. incisional biopsies. RESULTS: 133 rectal suction biopsy procedures (227 biopsies) and 125 incisional biopsy procedures (140 biopsies) were analyzed. In patients 6 months of age and older, the percentage of inadequate procedures was substantially higher in the suction biopsy group (24.1% vs 0.9%, p < 0.01). CONCLUSIONS: A substantially higher proportion of inadequacy was found in the suction rectal biopsy group compared to the incisional cohort among the older patient cohort, suggesting incisional biopsies should be strongly considered as the primary rectal biopsy method in patients older than 6 months.

Quality of Life in Adolescents and Young Adults: The Role of Symptom Burden.

CONTEXT: Adolescents and young adults (AYAs) with cancer report worse health-related quality of life (HRQOL) than other age groups. Symptom burden is a modifiable predictor of HRQOL. OBJECTIVES: The objective of this study was to identify which symptoms are most burdensome to AYAs with advanced cancer. METHODS: In this observational study, English-speaking individuals aged 12-25 years undergoing treatment for advanced cancer completed assessments of symptom burden (Memorial Symptom Assessment Scale) and HRQOL (Pediatric Quality of Life Inventory Generic Form and Cancer Module; minimal clinically important difference 4.4). We dichotomized participants as having low (<7) or high (≥7) symptom prevalence. Mixed regression models estimated HRQOL differences between groups. For individual symptoms, unadjusted mixed models estimated HRQOL reductions. RESULTS: N = 58 AYAs completed baseline surveys. The median age was 17 years (IQR 15-19), 58% were male, 59% identified as white, and 44% were diagnosed with leukemia/lymphoma. High symptom prevalence was associated with a mean generic HRQOL 7 points lower (95% CI: -11, -3; P < 0.01) and cancer-specific HRQOL score 12 points lower (95% CI: -17, -7; P < 0.01) than low symptom prevalence. The most prevalent symptoms were fatigue (71%), pain (58%), and difficulty sleeping (58%). Fatigue (-8), difficulty concentrating (-7), and mouth sores (-6) were associated with the greatest generic HRQOL score reductions. Dysphagia (-12), difficulty concentrating (-12), and sadness (-11) were associated with the greatest cancer-specific HRQOL score reductions. CONCLUSION: The symptom experience among AYAs with advanced cancer is unique. Separate evaluation of AYA's symptoms may optimize management and improve HRQOL.

Body Mass Index Recovery after Lung Transplant for Cystic Fibrosis.

Rationale: Many lung transplant recipients with cystic fibrosis (CF) have low preoperative body mass index (BMI); however, post-transplant BMI recovery is not well understood. Objectives: To evaluate BMI recovery (⩾18.5 kg/m2) among CF lung transplant recipients with low preoperative BMI and to investigate the association of survival with BMI recovery. Methods: The United Network for Organ Sharing and CF Foundation patient registries (June 2005-December 2016) were used to identify CF lung transplant recipients. Among recipients surviving ⩾1 year, Cox modeling compared post-transplant 1-year conditional survival between recipients with low (<17 and 17-18.49 kg/m2) versus normal preoperative BMI, stratified by BMI recovery. Results: Of 1,977 CF lung transplant recipients, 272 (14%) and 449 (23%) had a preoperative BMI of <17 and 17-18.49 kg/m2, respectively. For subgroups with a BMI of <17 and 17-18.49 kg/m2, 29% versus 49%, respectively, of those alive at 1 year recovered their BMI. Among recipients with low preoperative BMI, adjusted post-transplant 1-year conditional survival was worse than that in those with preoperative BMI ⩾ 18.5 kg/m2; however, BMI recovery mitigated this. Preoperative BMI < 17 kg/m2 had an adjusted hazard ratio of 1.29 (95% confidence interval [CI], 0.92-1.81) with BMI recovery versus 1.57 (95% CI, 1.09-2.25) without recovery, and preoperative BMI 17-18.49 kg/m2 had an adjusted hazard ratio of 1.28 (95% CI, 1.02-1.61) with BMI recovery versus 1.72 (95% CI, 1.14-2.59) without recovery. Conclusions: Patients with lower preoperative BMI were less likely to achieve BMI recovery within 1 year. However, for those who did, BMI recovery within 1 year after transplant was associated with longer survival.

Assessment of the Promoting Resilience in Stress Management Intervention for Adolescent and Young Adult Survivors of Cancer at 2 Years: Secondary Analysis of a Randomized Clinical Trial.

Importance: Adolescents and young adults (AYAs) with cancer have a high risk of poor psychosocial outcomes. The Promoting Resilience in Stress Management (PRISM) intervention is one of few psychosocial interventions targeting younger people with cancer that has demonstrated efficacy in a randomized clinical trial. Objective: To explore 2-year trajectories of patient-reported well-being among AYA cancer survivors. The hypothesis was that AYAs who initially responded to PRISM would report sustained positive changes. Design, Setting, and Participants: This secondary analysis of data from a single-center, parallel, phase 2 randomized clinical trial was conducted from January 2015 to October 2016. Eligible participants were English-speaking AYAs between ages 13 and 25 years with cancer treated at a single, quaternary children's hospital in the US. Participants were randomly assigned (1:1) to PRISM or usual care. Data were analyzed between March 2021 and June 2021. Intervention: PRISM is a brief, skills-based coaching program targeting 4 resilience resources (stress management, goal setting, cognitive reframing, and meaning making). Main Outcomes and Measures: Patient-reported cancer-related quality of life (QoL) (PedsQL scale), hope (Hope scale), resilience (Connor-Davidson Resilience scale), and psychological distress (Kessler-6 scale) were collected at baseline, 6, 12, and 24 months. Data from AYAs who remained alive at 24 months were analyzed. Improvement was defined as a positive change in instrument scores between baseline and 6 months (ie, the end of the PRISM program) and used linear mixed effects regression to assess changes over time. Results: A total of 57 AYAs were included in analysis at 24 months (62% of 92 randomized). Twenty-six participants (46%) were female; 36 (63%) were between ages 13 and 17 years; and 20 (35%) identified as part of a racial or ethnic minority group (4 [7%] Asian, 11 [19%] Hispanic or Latino, 16 [28%] mixed race or other). PRISM was associated with improved QoL, hope, resilience, and distress between baseline and 6 months, and these associations were sustained for QoL over 12 months (ß = 7.4; 95% CI, 0.8 to 14; P = .03) and hope throughout the study period (12 months: ß = 6.2; 95% CI, 2.7 to 9.6; P < .001; 24 months: ß = 4.6; 95% CI, 1 to 8.3; P = .01). Of those whose scores improved between baseline and 6 months, PRISM participants more commonly reported sustained improvements in QoL (16 of 21 participants [76%] vs 8 of 17 [47%]), hope (14 of 24 participants [58%] vs 6 of 14 [43%]), resilience (9 of 18 participants [50%] vs 4 of 14 [29%]), and distress (14 of 19 participants [74%] vs 6 of 19 [32%]) at 24 months compared with usual care. Conclusions and Relevance: These results suggest that PRISM was associated with sustained improvements in psychosocial outcomes as reported by adolescents and young adults. Those who responded to PRISM in the first 6 months reported continued well-being 2 years later. Trial Registration: ClinicalTrials.gov Identifier: NCT02340884.

A novel combined resilience and advance care planning intervention for adolescents and young adults with advanced cancer: A feasibility and acceptability cohort study.

BACKGROUND: Few evidence-based psychosocial programs have been tested among adolescents and young adults (AYAs) with advanced cancer (AC), and early advance care planning (ACP) in this population is rare. The authors aimed to determine the feasibility and acceptability of 1) delivering an established resilience-coaching program, and 2) integrating ACP into that program, among AYAs with AC. METHODS: Eligible AYAs were 12 to 24 years old, diagnosed with advanced cancer (recurrent/refractory disease or a diagnosis associated with <50% survival) and fluent in English. The Promoting Resilience in Stress Management-Advanced Cancer (PRISM-AC) program included PRISM's standard sessions targeting stress-management, goal-setting, cognitive-restructuring, and meaning-making, delivered 1:1, 1 to 2 weeks apart, plus a new session involving elements of the AYA-specific Voicing My Choices ACP guide. Participants completed surveys at baseline and 12 weeks, and exit interviews following study completion. Feasibility was defined as ≥70% completion of 1) standard 4-session PRISM and 2) the new ACP session among those completing standard PRISM. Acceptability was defined qualitatively. Trajectories of patient-reported anxiety, depression, and hope were examined descriptively. RESULTS: Of 50 eligible, approached AYAs, 26 (52%) enrolled and completed baseline surveys. The AYAs had a mean age of 16 years (SD = 2.7 years), and the majority were male (73%) and White/Caucasian (62%). Twenty-two AYAs (85%) completed standard PRISM, and of those, 18 (82%) completed the ACP session. Feedback was highly positive; 100% and 91% described the overall and ACP programs as valuable, respectively. Anxiety, depression, and hope were unchanged after the program. CONCLUSIONS: Resilience coaching followed by integrated ACP is feasible and acceptable for AYAs with AC. Participating did not cause distress or decrease hope. LAY SUMMARY: Advance care planning (ACP) among adolescents and young adults (AYAs) with advanced cancer can be difficult to introduce. We investigated whether it is feasible and acceptable to integrate ACP into an existing resilience-coaching program for AYAs. In this cohort study of 26 AYAs with advanced cancer, we found the Promoting Resilience in Stress Management-Advanced Cancer program to be feasible (≥70% intervention-completion) and highly acceptable (positive post-participation feedback, no evidence of participant-distress). We conclude that an intervention integrating resilience coaching and ACP is feasible and acceptable among AYAs with advanced cancer.

Feasibility and acceptability of a home-based resistance training intervention in adolescent and young adult hematopoietic cell transplant survivors.

BACKGROUND: Adolescent and young adult (AYA) hematopoietic cell transplantation (HCT) survivors are at increased risk of metabolic syndrome and lean body mass (LBM) deficits. Resistance training (RT) is a potential intervention to improve LBM, metabolic fitness, and reduce risk of cardiovascular disease. PROCEDURE: Eligible participants ages 13-39 years, 80-120 days post-HCT, transfusion independent, and prednisone dose ≤1 mg/kg/day were approached. Baseline assessments of body composition (DXA), anthropometrics, and strength testing were completed and participants were taught a 12-week, home-based RT intervention with weekly remote coaching. Follow-up assessments were at day +200 (FU1) and +365 post-HCT (FU2). Feasibility targets were (a) 60% enrollment of approached patients, (b) 80% completion of weekly phone calls, and (c) 80% completion of the RT intervention and FU1 assessments. Acceptability was based on positive responses in qualitative interviews. RESULTS: Twenty of 31 (65%) eligible AYAs enrolled. Three participants failed to complete baseline measurements (2 = scheduling barriers, 1 = passive refusal) and four participants who completed baseline assessments did not receive the intervention (1 = medical reasons, 2 = no longer interested). Of those who completed baseline assessments, 13 received the intervention, completed 88.5% of coaching calls, and 11 (65%) completed FU1. LBM (kg) increased or remained unchanged in nine of nine participants with complete body composition data at FU1 (mean 1.1 kg; 95%CI: 0.4, 1.9). All participants who completed FU1 reported they would recommend the intervention to an AYA HCT survivor. CONCLUSIONS: A home-based RT intervention in AYA HCT survivors early post HCT is both feasible and acceptable and may maintain or increase LBM.

Tincture of Time: The Evolution of Goals in Adolescents and Young Adults with Cancer.

Purpose: A diagnosis of cancer often derails an individual's goals. The purpose of this mixed-methods analysis was to describe the evolution of adolescent and young adult (AYA)-reported goals in the year after a diagnosis of new or advanced cancer. Methods: As part of the Promoting Resilience in Stress Management (PRISM) phase 2 randomized controlled trial, AYAs with cancer (ages 12-25 years) were asked at baseline, 6 months, and 12 months: "Please give an example of a goal you hope to accomplish over the next month/year." We used content analysis to categorize goals as follows: life milestones, physical health, mental/emotional health, cancer specific, and hobbies/interests. We summarized goal categories at each time point: for the entire cohort, by baseline diagnosis status (new vs. advanced cancer), treatment status (on-therapy vs. off-therapy), and baseline health-related quality of life (high vs. low). Results: Eighty-six participants completed the study and were included in the analysis (control: n = 41, PRISM: n = 45); 69 reported their goals at 6 months, and 54 at 12 months. Participants submitted a total of 169 goals at baseline, 148 at 6 months, and 126 at 12 months. At baseline, cancer-specific goals were most common for the short-term (35%), whereas milestone goals were most common for the long-term (46%). At 6 months, milestone goals were most common in both short- (38%) and long-term (51%) categories. A similar pattern was seen at 12 months. Conclusion: AYAs' goals change over the first year of their cancer experience, with a shift in short-term goals from cancer-specific goals at baseline to milestone goals at later time points. ClinicalTrials.gov (NCT02340884).

Parent Perspectives after the PRISM-P Randomized Trial: A Mixed-Methods Analysis.

Background: Parents experience high distress following their child's diagnosis of cancer. We previously tested two delivery models (group and one-on-one) of the "Promoting Resilience in Stress Management for Parents" (PRISM-P) intervention in a randomized trial: one-on-one delivery improved resilience and benefit finding at three months when compared to usual care (UC). Objective: The objective of this analysis was to evaluate quantitative and qualitative outcomes at six months. Design: In this single-center, phase 2, parallel, 1:1:1 randomized trial conducted December 2016 to December 2018, English-speaking parents with a 2-24 year-old child diagnosed with new cancer were randomly assigned to UC, one-on-one, or group PRISM-P, a brief, skill-based curriculum targeting stress management, goal setting, cognitive reframing, and meaning making. We collected parent-reported outcomes (resilience, benefit finding, and psychological distress) at baseline and three and six months. We applied linear mixed-effects regression models to examine six-month outcomes among all participants and conducted directed content analyses of exit interviews with the first 12 parents to complete each study arm. Results: The 94 participating parents were median aged 35-38 years and predominantly white, college-educated mothers. At six months, there was no statistically significant difference in parent-reported outcomes. Exit interviews (n = 36) suggested that PRISM-P was highly valued: 100% of interviewed recipients recommended it for other parents. Most suggested more coaching would help them retain skills, and almost all endorsed a combined one-on-one and group program. Conclusions: Although the PRISM-P benefits observed at three months were not sustained for six months, all interviewed parents found it valuable. Additional opportunities to strengthen and sustain resilience resources include longer follow-up, flexible format, and skill reinforcement. Trial Registration: NCT02998086.

Adverse childhood experiences (ACEs) and medically traumatic events (TEs) in adolescents and young adults (AYAs) with cancer: a report from the Promoting Resilience in Stress Management (PRISM) randomized controlled trial.

OBJECTIVE: In adolescents and young adults (AYAs) with cancer, we examined (1) the distribution and type of traumatic events (TEs) experienced prior to baseline assessment and (2) how a resilience intervention, Promoting Resilience in Stress Management (PRISM), impacted changes in patient-reported outcomes (PROs) for AYAs with and without TEs. METHODS: AYAs (12-25 years) within 1-10 weeks of diagnosis of new malignancy or ever diagnosed with advanced cancer were enrolled and randomly assigned to usual care (UC) with or without PRISM. To assess TEs, we screened medical records for traditionally defined adverse childhood experiences (ACEs) and medical traumatic events. Age-validated PROs assessed resilience, benefit-finding, hope, generic health-related quality of life (QoL), cancer-specific QoL, depression, and anxiety at enrollment and 6 months later. We calculated effect sizes (Cohen's d) for PRISM vs. UC effect on PRO score change at 6 months for 1+ TEs and 0 TE groups. RESULTS: Ninety-two AYAs enrolled and completed baseline surveys (44-UC, 48-PRISM; N = 74 at 6 months, 38-UC, 36-PRISM); 60% experienced 1+ TEs. PROs at baseline were similar across groups. PRISM's effect on score change was greater (Cohen's d ≥ 0.5) for the 1+ TE group on domains of benefit-finding and hope; and similar (d < 0.5) on domains of resilience, depression, anxiety, and both generic and cancer-specific QoL. CONCLUSIONS: In AYAs with cancer, TEs occurred at similar rates as the general population. PRISM may be particularly helpful for improving benefit-finding and hope for those who have experienced TEs.

Creating a Resilient Research Program-Lessons Learned From a Palliative Care Research Laboratory.

Conducting palliative care research can be personally and professionally challenging. Although limitations in funding and training opportunities are well described, a less recognized barrier to successful palliative care research is creating a sustainable and resilient team. In this special report, we describe the experience and lessons learned in a single palliative care research laboratory. In the first few years of the program, 75% of staff quit, citing burnout and the emotional tolls of their work. To address our sustainability, we translated resilience theory to practice. First, we identified and operationalized shared mission and values. Next, we conducted a resilience resource needs assessment for both individual team members and the larger team as a whole and created a workshop-based curriculum to address unmet personal and professional support needs. Finally, we changed our leadership approach to foster psychological safety and shared mission. Since then, no team member has left, and the program has thrived. As the demand for rigorous palliative care research grows, we hope this report will provide perspective and ideas to other established and emerging palliative care research programs.

Sexual Activity and Substance Use Among Adolescents and Young Adults Receiving Cancer Treatment: A Report from the PRISM Randomized Controlled Trial.

Purpose: Despite health implications, sexual activity and substance use among adolescents and young adults (AYAs) receiving cancer treatment are understudied. Methods: AYAs 12-25 years of age participated in a randomized controlled trial testing the efficacy of a resilience intervention. They were fluent in English and either diagnosed with new cancer (NC) or advanced cancer (AC). At baseline and 6 months, participants self-reported sexual activity and substance use. We describe the percentage of AYAs who endorsed each behavior and a count of total behaviors endorsed by each respondent. We describe frequencies by sex/gender (male/female), age (<18/≥18), and disease status (AC/NC). Results: Participants (N = 92) were majority white/Caucasian (57%), 12-17 years old (73%), and diagnosed with leukemia/lymphoma (62%); 32% had AC. Responses were not associated with the intervention; hence, we summarized data from the whole cohort. At both time points, median behavior endorsed was 2. At baseline and follow-up, 87% and 81% endorsed at least 1 behavior: 13% and 15% were sexually active, 75% and 73% of whom used birth control inconsistently; and 22% and 22% reported drinking alcohol, 31% and 27% using prescription opioids/sedatives, 19% and 22% using other drugs, and 9% and 7% using tobacco. Young adults engaged in most behaviors more frequently than adolescents (e.g., 48% vs. 12% alcohol at baseline); males engaged in sexual activity more frequently than females (e.g., 20% vs. 5% sexually active at baseline); and AYAs with NC engaged in most behaviors more frequently than those with AC (18% vs. 0% sexually active at baseline). Conclusion: AYAs engage in sexual activity and substance use during cancer treatment.

Underweight Patients With Cystic Fibrosis Have Acceptable Survival Following Lung Transplantation: A United Network for Organ Sharing Registry Study.

BACKGROUND: Reduced BMI is an absolute contraindication for lung transplantation (LTx) at most centers in the United States. The objective of this study was to quantify post-LTx survival of moderate to severely underweight patients with cystic fibrosis (CF) (BMI < 17 kg/m2) in the United States relative to normal-weight recipients with CF and other frequently transplanted patient cohorts. METHODS: Using United Network for Organ Sharing Registry data (undergoing transplant from June 2005-November 2015), Kaplan-Meier estimates of median posttransplant survival were calculated for all patients with CF, COPD, and idiopathic pulmonary fibrosis (IPF), as well as low and normal weight CF subgroups. Cox regression modeling stratified according to transplant center assessed risk of posttransplant mortality in recipients with CF and a BMI < 17 kg/m2 compared with recipients with COPD (reference). RESULTS: Median posttransplant survival (95% CI) for CF, COPD, and IPF was 7.9 (7.2-8.6), 5.9 (5.6-6.2), and 5.5 (5.2-5.8) years, respectively. Although an absolute decrease was noted in posttransplant survival for recipients with CF and a BMI < 17 kg/m2, compared with those with CF and a BMI ≥ 17 kg/m2 (7.0 years [4.5-7.9] vs 8.2 years [7.3-9.0]), Cox modeling found no increased mortality risk (adjusted hazard ratio, 1.09; 95% CI, 0.90-1.32; P = .38). There was no difference in posttransplant mortality between patients with CF and a BMI < 17 kg/m2 and recipients with COPD and all BMIs (adjusted hazard ratio, 1.04; 95% CI, 0.86-1.25; P = .71). CONCLUSIONS: Transplant recipients with CF and a BMI < 17 kg/m2 had posttransplant survival rates comparable to those of other groups frequently undergoing transplantation. BMI < 17 kg/m2 as a single risk factor in the CF population should not be treated as an absolute contraindication to LTx.

Examining key sociodemographic characteristics of adolescents and young adults with cancer: A post hoc analysis of the Promoting Resilience in Stress Management randomized clinical trial.

BACKGROUND: The "Promoting Resilience in Stress Management" intervention is a skills-based, early palliative care intervention with demonstrated efficacy in adolescents and young adults with cancer. AIM: Utilizing data from a randomized clinical trial of Promoting Resilience in Stress Management versus Usual Care, we examined whether response to Promoting Resilience in Stress Management differed across key sociodemographic characteristics. DESIGN: Adolescents and young adults with cancer completed patient-reported outcome measures of resilience, hope, benefit-finding, quality of life, and distress at enrollment and 6 months. Participants were stratified by sex, age, race, and neighborhood socioeconomic disadvantage based on home address (Area Deprivation Index scores with 8-10 = most disadvantaged). Differences in the magnitude of effect sizes between stratification subgroups were noted using a conservative cutoff of d > 0.5. SETTING/PARTICIPANTS: Participants were 12 to 25 years old, English-speaking, and receiving cancer care at Seattle Children's Hospital. RESULTS: In total, 92 adolescents and young adults (48 Promoting Resilience in Stress Management, 44 Usual Care) completed baseline measures. They were 43% female, 73% 12 to 17 years old, 64% White, and 24% most disadvantaged. Effect sizes stratified by sex, age, and race were in an expected positive direction and of similar magnitude for the majority of outcomes with some exceptions in magnitude of treatment effect. Those who lived in less disadvantaged neighborhoods benefited more from Promoting Resilience in Stress Management, and those living in most disadvantaged neighborhoods benefited less. CONCLUSION: The "Promoting Resilience in Stress Management" intervention demonstrated a positive effect for the majority of outcomes regardless of sex, age, and race. It may not be as helpful for adolescents and young adults living in disadvantaged neighborhoods. Future studies must confirm its generalizability and integrate opportunities for improvement by targeting individual needs.

A Psychosocial Intervention's Impact on Quality of Life in AYAs with Cancer: A Post Hoc Analysis from the Promoting Resilience in Stress Management (PRISM) Randomized Controlled Trial.

Promoting Resilience in Stress Management (PRISM), a psychosocial intervention for adolescents and young adults (AYAs) with serious illness, enhances resilience resources via four skills-based training sessions. A recent randomized controlled trial showed PRISM improved health-related quality of life (HRQOL) compared to usual care (UC). This post hoc exploratory analysis aimed to better understand the effect of PRISM on HRQOL by describing changes in HRQOL subdomain scores. English-speaking AYAs (12-25 years) with cancer were randomized to PRISM or UC. At enrollment and six months later, HRQOL was assessed using the Pediatric Quality of Life Inventory (PedsQL) Generic Short Form (SF-15) and Cancer Module. Scores at each time point were summarized descriptively and individual HRQOL trajectories were categorized (<70 vs. ≥70). "Positive" trajectories indicate participants maintained scores ≥70 or improved from <70 to ≥70 during the study period. Baseline assessments were completed by 92 participants (48 PRISM, 44 UC); six-month assessments were completed by 74 participants (36 PRISM, 38 UC). For the SF-15, positive trajectories in psychosocial domains were more common with PRISM; trajectories in the physical subdomain were similar across groups. For the Cancer Module, positive trajectories were more common with PRISM in the following subdomains: nausea, treatment anxiety, worry, cognitive, physical appearance, and communication. From this, we conclude PRISM may improve HRQOL, especially in psychosocial domains of wellbeing.

Effect of the Promoting Resilience in Stress Management Intervention for Parents of Children With Cancer (PRISM-P): A Randomized Clinical Trial.

Importance: Parents of children with serious illness, such as cancer, experience high stress and distress. Few parent-specific psychosocial interventions have been evaluated in randomized trials. Objective: To determine if individual- or group-based delivery of a novel intervention called Promoting Resilience in Stress Management for Parents (PRISM-P) improves parent-reported resilience compared with usual care. Design, Setting, and Participants: This parallel, phase 2 randomized clinical trial with enrollment from December 2016 through December 2018 and 3-month follow-up was conducted at Seattle Children's Hospital. English-speaking parents or guardians of children who were 2 to 24 years old, who had received a diagnosis of a new malignant neoplasm 1 to 10 weeks prior to enrollment, and who were receiving cancer-directed therapy at Seattle Children's Hospital were included. Parents were randomized 1:1:1 to the one-on-one or group PRISM-P intervention or to usual care. Data were analyzed in 2019 (primary analyses from January to March 2019; final analyses in July 2019). Interventions: The PRISM-P is a manualized, brief intervention targeting 4 skills: stress management, goal setting, cognitive reframing, and meaning making. For one-on-one delivery, skills were taught privately and in person for 30 to 60 minutes approximately every other week. For group delivery, the same skills were taught in a single session with at least 2 parents present. Main Outcomes and Measures: Participants completed patient-reported outcome surveys at enrollment and at 3 months. Linear regression modeling evaluated associations in the intention-to-treat population between each delivery format and the primary outcome (Connor-Davidson Resilience Scale scores, ranging from 0 to 40, with higher scores reflecting greater resilience) and secondary outcomes (benefit finding, social support, health-related quality of life, stress, and distress) at 3 months. Results: In total, 94 parents enrolled, were randomized to 1 of the 3 groups, and completed baseline surveys (32 parents in one-on-one sessions, 32 in group sessions, and 30 in usual care). Their median (interquartile range) ages were 35 to 38 (31-44) years across the 3 groups, and they were predominantly white, college-educated mothers. Their children had median (interquartile range) ages of 5 to 8 (3-14) years; slightly more than half of the children were boys, and the most common cancer type was leukemia or lymphoma. One-on-one PRISM-P delivery was significantly associated with improvement compared with usual care in parent-reported outcomes for resilience (ß, 2.3; 95% CI, 0.1-4.6; P = .04) and for benefit finding (ß, 0.5; 95% CI, 0.2-0.8; P = .001). No significant associations were detected between either platform and other parent-reported outcomes. Conclusions and Relevance: When delivered individually, PRISM-P was associated with improved parent-reported resilience and benefit finding. This scalable psychosocial intervention may help parents cope and find meaning after their child receives a diagnosis of a serious illness. Trial Registration: ClinicalTrials.gov identifier: NCT02998086.