Adult Survivors Of Pediatric Cancer Research Articles

Longitudinal associations between peripheral neuropathy and neurocognitive decline in long-term survivors of pediatric cancer treated without central nervous system (CNS) directed therapy.

10054 Background: Survivors of pediatric cancer treated without CNS-directed therapy are at risk of neurocognitive impairment and peripheral neuropathies. Using the St. Jude Lifetime Cohort, we investigated longitudinal associations with neuropathy to inform contributors to long-term cancer-related neurocognitive decline. Methods: Survivors of pediatric cancer (N=899 mean age 32 [SD 12], 24[10] years from diagnosis) treated without CNS-directed therapy completed neurocognitive testing and medical assessments at two timepoints (T1, T2 4.4[2.3] years apart). Peripheral sensory and motor neuropathy subscales combined with functional tasks were severity graded using a modified CTCAE (no neuropathy = none or grade 1 at T1 and T2; new onset = new grade 2 or 3 at T2; persistent = grade 2 or 3 at T1 & T2). Linear regression models estimated change in neurocognitive Z-score associated with neuropathy groups, adjusted for diagnosis age, time between visits, sex, race/ethnicity, high-dose intravenous methotrexate & baseline neurocognitive Z-score. Results: New onset sensory (n=33, 3.6%) and motor (n=35, 3.9%) neuropathy was associated with a 0.33 standard deviation (SD) decline in visual-motor processing speed (Table). New onset motor neuropathy was associated with a 0.50 SD decline in sustained attention as well as significant declines in verbal recall, working memory, and cognitive flexibility (p’s<0.05). Persistent sensory neuropathy (n=25, 2.8%) was associated with large declines in focused attention and cognitive flexibility (p’s<0.05). Persistent motor neuropathy (n=18, 2.0%) was associated with a 0.50 SD decline in sustained attention, verbal recall, and working memory (p’s<0.05). Conclusions: Peripheral neuropathies are associated with future neurocognitive decline in adult survivors of pediatric cancer. Research is needed to understand shared mechanisms (e.g. pain, inflammation) to inform targeted interventions to improve neuropathic symptoms and neurocognitive function. [Table: see text]

Are Young Adult Survivors of Pediatric Cancer Being Overlooked? Cognitive Testing Results and Referrals in Child, Adolescent, and Young Adult Survivors.

Treatment gaps in meeting the neuropsychological needs of young adult (YA) cancer survivors can be attributed to several clinical and systemic reasons. Access to neurocognitive care can be increased through the effective integration of neuropsychological monitoring and intervention in survivorship care. In this brief report, we aim to compare the efficacy of a brief neuropsychological screener (DIVERGT) in meeting the assessment and referral needs of pediatric and YA cancer survivors (n = 40) as part of a wellness and survivorship clinic. Participants (n = 40) were patients who presented to a pediatric oncology survivorship clinic over the span of 15 months.

Parental Perspectives on Health Care Transition in Adolescent and Young Adult Survivors of Pediatric Cancer.

The health care transition (HCT) from pediatric to adult care is pivotal for childhood cancer survivors (CCS) and their parents. However, there is little research examining parental needs during HCT, despite this being a key predictor of successful HCT. The goal of this study was to investigate the needs of parents of CCS during HCT. Using an integrative review of the literature structured around the social-ecological model (SEM) of CCS transition readiness yielded 454 articles, including three hand-searched articles. Six articles were included in the final analysis. Data were extracted into nine factors derived from SEM. Articles were published within the last decade, largely qualitative, and mainly examined parents and CCS together. Parents most frequently mentioned relationships with their practitioner and CCS as contributing to HCT readiness, while abstract factors, such as goal-setting and expectations around HCT, were not mentioned. Our results are limited by the dearth of research on this topic, the homogeneity of samples, and joint presentation of CCS and parent data. Nonetheless, our results indicate that parents do not weigh all aspects of SEM equally, with macrolevel barriers, such as sociodemographic factors being viewed as less salient for HCT readiness. Parents mostly focused on interpersonal factors, such as their relationships with practitioners and CCS, indicating that practitioners should emphasize these in preparing parents for HCT.

Premature aging as an accumulation of deficits in young adult survivors of pediatric cancer.

We aimed to characterize premature aging as an accumulation of deficits in survivors of pediatric cancer compared with community controls and examine associations with host and treatment factors, neurocognition, and mortality. Pediatric cancer survivors (n = 4000, median age = 28.6, interquartile range [IQR] = 23-35 years; 20 years postdiagnosis: IQR = 15-27), and community participants without a history of cancer serving as controls (n = 638, median age = 32, IQR = 25-40 years) completed clinical assessments and questionnaires and were followed for mortality through April 30, 2020 (mean [SD] follow-up = 7.0 [3.4] years). A deficit accumulation index (DAI) score was calculated from 44 aging-related items including self-reported daily function, psychosocial symptoms, and health conditions. Items were weighted from 0 (absent) to 1 (present and/or most severe), summed and divided by the total yielding a ratio (higher = more deficits). Scores less than 0.20 are robust, and 0.06 is a clinically meaningful difference. Linear regression compared the DAI in survivors and controls with an age*survivor or control interaction. Logistic regression and Cox-proportional hazards estimated the risk of neurocognitive impairment and death. Models were minimally adjusted for age, sex, and race and ethnicity. The adjusted mean DAI among survivors at age 30 years was 0.16 corresponding to age 63 years in controls (33 years premature aging; β = 0.07, 95% confidence interval [CI] = 0.06 to 0.08; P < .001). Cranial and abdominal radiation, alkylators, platinum, and neurosurgery were associated with worse DAI (P ≤ .001). Higher scores were associated with increased risk of neurocognitive impairment in all domains (P < .001) and increased risk of death (DAI = 0.20-0.35, hazard ratio = 2.80, 95% CI = 1.97 to 3.98; DAI ≥ 0.35, hazard ratio = 5.08, 95% CI = 3.52 to 7.34). Pediatric cancer survivors experience clinically significant premature aging. The DAI may be used to identify survivors at greatest risk of poor health outcomes.

Development of a Self-management and Peer-Mentoring Intervention to Improve Transition Readiness Among Young Adult Survivors of Pediatric Cancer: Formative Qualitative Research Study.

BackgroundChildhood cancer survivors require lifelong risk-based follow-up care. It should be noted that less than one-third of adult survivors of childhood cancer report any survivor-focused care, and fewer than 1 in 5 obtain risk-based follow-up care. It is thought that this may be due to inadequate transition readiness, including low levels of knowledge, skills, motivation, and resources to make the transition to independent self-management of follow-up care. Interventions that focus specifically on improving the transition from parent-managed to self-managed care are needed. Theory and prior research suggest that targeting self-management skills and using peer mentoring may be innovative strategies to improve transition readiness.ObjectiveThis study aims to identify the content of a self-management intervention to improve transition readiness among adolescent and young adult (AYA) survivors.MethodsIntervention development occurred in 3 stages: formative research with AYA survivors to identify barriers and facilitators to obtaining risk-based survivorship care, content development using feedback from multiple stakeholders (AYA survivors, parents, and providers), and content refinement (usability testing) of the initial proposed educational modules for the program. Content analysis, guided by the social-ecological model of AYA readiness for transition, was used to identify themes and develop and refine the content for the intervention.ResultsA total of 19 AYA survivors participated in the formative research stage, and 10 AYA survivors, parents, and health care providers participated in the content development and refinement stages. The major barrier and facilitator themes identified included knowledge of cancer history and risks; relationships with health care providers; relationships with family members involved in care; emotions about health, follow-up care, and transfer of care; and lifestyle behaviors and life transitions. These themes were translated into 5 self-management modules: understanding treatment history and the survivorship care plan, managing health care logistics and insurance, communicating with health care providers and family members involved in care, dealing with emotions, and staying healthy in the context of life transitions. Feedback from the key stakeholders indicated that the content was relevant but should include participative elements (videos and tailored feedback) to make the intervention more engaging. The AYA survivors were receptive to the idea of working with a peer mentor and expressed a preference for using SMS text messaging, telephone calls, or videoconference to communicate with their mentor.ConclusionsIncorporating AYA survivors, parents, and providers in the design was essential to developing the content of a self-management and peer-mentoring intervention. AYA survivors confirmed the important targets for the intervention and facilitated design decisions in line with our target users’ preferences. The next step will be to conduct a single-arm trial to determine the feasibility and acceptability of the proposed intervention among AYA survivors of childhood cancer.

Neuropathy and neurocognitive impairment in long-term survivors of pediatric cancer treated without central nervous system (CNS) directed therapy.

10020 Background: Survivors of pediatric cancer, including those without CNS-directed treatment, are at risk of neurocognitive impairment and peripheral or autonomic neuropathies; conditions that co-occur in non-cancer populations. Using the St. Jude Lifetime Cohort, we investigated associations between these sequelae to inform contributors to long-term cancer-related neurocognitive impairment. Methods: Clinically assessed survivors of pediatric cancer (N=2,138, mean age 31 [SD 13], 23[11] years from diagnosis, 51% male) not treated with CNS-directed therapy completed neurocognitive testing. Modified total neuropathy score peripheral sensory and motor subscales were combined with functional tasks and severity graded using a modified NCI CTCAE. Cardiac autonomic neuropathy was defined as impaired heart rate reserve (&lt;80% age- sex-predicted or ≤62% with beta blocker). Separate log binomial regression models estimated risk of neurocognitive impairment (age adjusted z-score &lt;10th percentile) associated with grade 2+ sensory, motor or autonomic neuropathy. Path analysis examined if pain with daily interference mediated associations between motor or sensory neuropathy and neurocognitive impairment. Results: 838 (39%) survivors had autonomic, 115 (5.4%) had motor and 162 (7.6%) had sensory neuropathy. Compared to those with no neuropathy, survivors with motor or sensory neuropathy had a higher risk of impairment in sustained attention, visual-motor processing speed, short- and long-term memory, and executive function (Table, p’s&lt;0.01). Autonomic neuropathy was associated with a higher risk of impairment in visuo-motor processing speed (p=0.03). Pain partially mediated associations between sensory neuropathy and sustained attention (10% mediated, p=0.04), visual-motor processing speed (8% p=0.02), and executive function (14% p=0.02). Pain was not related to motor neuropathy. Conclusions: Peripheral and autonomic neuropathies are associated with significant risk of neurocognitive impairment in adult survivors of pediatric cancer. Research is needed to further understand shared mechanisms (e.g., pain, inflammation) to design targeted interventions to improve neuropathic symptoms and neurocognitive function. [Table: see text]

Cancer-Related Barriers to Health Behaviors Among Adolescent and Young Adult Survivors of Pediatric Cancer and Their Families.

Adolescent and young adult (AYA) survivors of pediatric cancer and their caregivers frequently do not meet national guidelines for physical activity (PA), nutrition, or sleep. Respondents from N = 73 AYA-aged (mean [M] = 19.0 years, range = 11-30 years) survivor families completed a survey on cancer-related barriers to health behaviors. Almost all respondents (68/73, 93.2%) endorsed ≥1 cancer-specific barrier(s) to current health behaviors (median = 5, standard deviation = 3.42, range = 0-16) in their family. Cancer-related barriers to PA, nutrition, and sleep were positively correlated (PA-nutrition: r = 0.39, p = 0.001; PA-sleep: r = 0.41, p < 0.001; nutrition-sleep: r = 0.52, p < 0.001). Wellness interventions are needed to address the unique needs of AYA-aged survivors and support multiple health behaviors simultaneously.

Psychometric Testing of Attitudes Toward Physical Activity Scale for Adolescent and Young Adult Survivors of Pediatric Cancer.

Purpose: Adolescent and young adult (AYA) survivors of pediatric cancer experience chronic health problems following treatment, many of which could be prevented through healthy lifestyle choices. This report describes the development of the AYA Cancer Survivor Attitude (AYA-CSA) Scale, an attitudinal scale that is associated with physical activity in AYA survivors of pediatric cancer. Methods: AYA survivors (n = 100) completed a survey to evaluate their attitudes toward engagement in physical activity. Internal consistency was calculated using Cronbach's alpha. Construct validity was assessed by exploratory factor analysis, correlation with physical activity intention and physical activity behavior, and prediction of physical activity, after adjusting for past physical activity, survivor age, gender, and family income. Results: Data demonstrated a single 6-item scale with excellent internal consistency (α = 0.82). Construct validity was supported by significant correlations with physical activity intention (r = 0.553, p < 0.001) and physical activity behavior (r = 0.489, p < 0.001). After controlling for past physical activity and demographic covariates, survivor age, attitude toward physical activity, and intention were significant predictors of physical activity, and the overall model (F(6, 77) = 7.722, p < 0.001) predicted 39.5% of the variability in physical activity. Conclusion: The AYA-CSA scale demonstrates good reliability and construct validity, most important of which was the ability to predict actual physical activity in AYA survivors of pediatric cancer. This reliable and valid measure is an important tool in the design of behavioral interventions to improve physical activity engagement in AYA survivors.

A survey of ovarian reserve and quality of life in female survivors of pediatric cancer.

Childhood cancer is rare; the incidence in Sweden is approximately 16 new cases/100000 children each year. Reduced reproductive function and fertility are well-known side effects of cancer treatment. Anti-Müllerian hormone (AMH) has been shown to correlate well with antral follicle count in healthy women but is currently not recommended as the primary surveillance modality for evaluation of premature ovarian insufficiency in this patient group. Psychological wellbeing related to fertility could affect quality of life and should be included in long-term follow-up. The aim of the study is to present the baseline data from inclusion for a prospective follow-up study of fertility surveillance where both medical and psychological aspects of fertility in female childhood cancer survivors are considered. These are the first results from this longitudinal follow-up cohort study. Female adolescent and young adult survivors of pediatric cancer in Western Sweden were included from January 2016 to December 2018, a total of 54 participants. Median age at inclusion was 21 (15-29) years and median age at cancer diagnosis was 10 (1-17) years. AMH levels, antral follicle count, and data on fertility were recorded at inclusion and will be prospectively followed up. The study includes questionnaires and interviews concerning quality of life. This study is planned to continue until the participants reach the age of 40years. Eighteen of 54 (33%) participants had AMH levels below 1.0µg/L and were considered to have high or very high risk of infertility. Median AMH level was 2.50µg/L. Six women had immediate need of oocyte cryopreservation. Psychological assessment showed that more than one-third of participants (n=20) had elevated anxiety scores. One-third of female survivors of pediatric cancer in the study had high risk of low ovarian reserve, measured by a combination of AMH and antral follicle count, and many had signs of anxiety. The longitudinal study could contribute to better knowledge in the changes of AMH over time for this patient group. Psychological follow-up with questionnaires and interviews evaluating signs of depression and anxiety may serve as a model for future screening programs.

Clinical and genetic risk factors for radiation-associated ototoxicity: A report from the Childhood Cancer Survivor Study and the St. Jude Lifetime Cohort.

Cranial radiation therapy (CRT) is associated with ototoxicity, which manifests as hearing loss and tinnitus. The authors sought to identify clinical determinants and genetic risk factors for ototoxicity among adult survivors of pediatric cancer treated with CRT. Logistic regression evaluated associations of tinnitus (n=1991) and hearing loss (n=2198) with nongenetic risk factors and comorbidities among CRT-treated survivors in the Childhood Cancer Survivor Study. Genome-wide association studies (GWASs) of CRT-related tinnitus and hearing loss were also performed. Males were more likely to report CRT-related tinnitus (9.4% vs 5.4%; P=5.1×10-4 ) and hearing loss (14.0% vs 10.7%; P=.02) than females. Survivors with tinnitus or hearing loss were more likely to experience persistent dizziness or vertigo (tinnitus: P<2×10-16 ; hearing loss: P=6.4×10-9 ), take antidepressants (tinnitus: P=.02; hearing loss: P=.01), and report poorer overall health (tinnitus: P=1.5×10-6 ; hearing loss: P=1.7×10-6 ) in comparison with controls. GWAS of CRT-related tinnitus revealed a genome-wide significant signal in chromosome 1 led by rs203248 (P=1.5×10-9 ), whereas GWAS of CRT-related hearing loss identified rs332013 (P=5.8×10-7 ) in chromosome 8 and rs67522722 (P=7.8×10-7 ) in chromosome 6 as nearly genome-wide significant. A replication analysis identified rs67522722, intronic to ATXN1, as being significantly associated with CRT-related hearing loss (P=.03) and de novo hearing loss (P=3.6×10-4 ). CRT-associated ototoxicity was associated with sex, several neuro-otological symptoms, increased antidepressant use, and poorer self-reported health. GWAS of CRT-related hearing loss identified rs67522722, which was supported in an independent cohort of survivors. Hearing loss and subjective tinnitus (the perception of noise or ringing in the ear) are long-term side effects of cancer treatment and are common in children treated with radiation to the brain. These toxicities can affect childhood development and potentially contribute to serious learning and behavioral difficulties. This study's data indicate that males are at greater risk for hearing loss and tinnitus than females after radiation therapy to the brain. Those who develop these toxicities are more likely to use antidepressants and report poorer overall health. Health care providers can improve the management of survivors by informing patients and/or their parents of these risks.

Family and parent-child relationship correlates of pediatric cancer survivors' substance use.

The primary aims of this research were to examine substance use among adolescent and young adult survivors of pediatric cancer (AYA survivors) and AYA without a history of chronic or life threatening illness (AYA comparisons) and to explore links between demographic, medical, caregiver-AYA, and family system factors with AYA substance use patterns. Participants included 289 AYA (survivors, n = 171; comparisons = 118; 51% female; Mage = 17.15, SDage = 2.86) and their caregivers (Mage = 46.54, SDage = 6.81; 88% mothers). AYA and caregivers completed the family environment scale, and caregivers completed the parenting relationship questionnaire at the initial assessment. Two years later, AYA completed an assessment of substance use. Chi-square and frequency analyses were used to compare differences in substance use among AYA survivors and comparisons. Multivariate analysis of variance was used to examine links between AYA substance use patterns with family and caregiver-AYA system level factors. Patterns of substance use did not differ between AYA survivors and comparisons. AYA survivors were more likely to report polysubstance use if caregivers endorsed problematic caregiver-AYA relationship patterns. Family functioning and caregiver relationship patterns did not predict AYA comparison substance use. AYA survivors were just as likely as AYA comparisons to engage in substance use, increasing their vulnerability to problematic health outcomes. Findings indicate that the role caregiver-AYA relationship patterns may have on youth at risk for substance use and potential mechanisms for future intervention.

Insurance Instability and Its Impact on Care of Young Adult Survivors of Pediatric Cancer: A Project Forward Study

By 2020, there will be more than 500,000 childhood cancer survivors (CCS) in the United States. CCS experience disparities in economic, social, and health‐related quality of life outcomes, and these disparities are magnified for CCS who are uninsured. Access to long‐term follow‐up (LTFU) care for surveillance, preventive care, and treatment of late effects for survivors are vital to improve long‐term outcomes. Inadequate insurance coverage, high out‐of‐pocket costs, and lack of perceived need for care have been shown to affect access to LTFU care among CCS. The objectives of this study were to (1) assess insurance instability longitudinally and describe patient factors that correlate with instability and (2) examine whether insurance instability and financial or patient factors influence access to LTFU care.Project Forward was a population‐based, observational study of CCS in Los Angeles County using California Cancer Registry (CCR) data to identify participants who completed a survey. Change in insurance coverage was assessed at diagnosis using CCR data and at survey and its impact on LTFU care. Those who experienced any change in insurance coverage (“insurance instability”) were set equal to one. Multivariable logistic regression models incorporating survey nonresponse weights were used to estimate the change in the marginal predicted probabilities of insurance instability and LTFU care, adjusted for demographic, socioeconomic, and clinical covariates and clustered by treating hospital.Study participants were diagnosed with cancer between the ages of 0 and 19 while living in Los Angeles County between 1996 and 2010 and were older than 21 at the time of survey, from 2015 to 2017 (N = 1106).Almost half (48%; N = 529) of participants experienced insurance instability from diagnosis to survivorship, while 577 did not. After adjusting for demographic, socioeconomic, and clinical covariates, the multivariable model predicting insurance instability indicated that being uninsured at diagnosis or at survey increased the probability of instability by 37% (P &lt; .001) and 58% (P &lt; .001), respectively, in comparison with those with private insurance. The multivariable model predicting LTFU care indicated that those who experienced insurance instability decreased the probability of LTFU care by 5% (P &lt; .05), in comparison with those who did not experience instability. When compared to those with private insurance coverage at diagnosis, participants who were covered by Medicaid, Medicare, or Indian Health Service plans at diagnosis were more likely to participate in LTFU care by 5% (P &lt; .05); however, those who were uninsured at the time of the survey were less likely to participate in LTFU care by 10% (P &lt; .05).CCS who were uninsured at diagnosis or survivorship were more likely to experience insurance instability. Insurance instability and being uninsured during survivorship were negatively associated with access to LTFU care; however, those with public insurance coverage at diagnosis were positively associated with access to LTFU care.Reducing insurance instability for CCS will improve access to LTFU care. This insight is key to improving health, reducing unnecessary and inappropriate health care use, and decreasing costs, while promoting services that can preserve and improve health for CCS.The study was funded by the National Institutes of Health.

Clinical and genetic risk factors for radiation-associated ototoxicity: A report from the childhood cancer survivor study and the St. Jude Lifetime Cohort.

10550 Background: Cranial radiation therapy (CRT) for pediatric cancer often results in ototoxicity in the form of hearing loss and tinnitus. We sought to identify clinical determinants and genetic risk factors for ototoxicity among adult survivors of pediatric cancer treated with cranial radiation. Methods: Relationships between age at last observation, sex, cumulative CRT dose and self-reported ototoxicity were evaluated for hearing loss and tinnitus among 1,991 (tinnitus) and 2,198 (hearing loss) survivors in the Childhood Cancer Survivor Study who received CRT. Logistic regression evaluated associations with non-genetic risk factors and comorbidities as well as SNP dosages in GWAS of CRT-related tinnitus (cases: 146; controls: 1,845) and hearing loss (cases: 270; controls: 1,928). Results: Males were more likely to report CRT-related tinnitus (9.4% vs. 5.4%; p = 5.81x10−4) and hearing loss (14.0% vs. 10.7%; p = 0.02) than females after adjusting for dose and age at last observation. Survivors with tinnitus or hearing loss were more likely to experience persistent dizziness or vertigo (tinnitus: p &lt; 2.00x10−16; hearing loss: p = 6.35x10−9), take antidepressants (tinnitus: p = 0.02; hearing loss: p = 0.01) and report poorer overall health (tinnitus: p = 9.40x10−7; hearing loss: p = 1.30x10−6) compared to survivors without tinnitus or hearing loss after age-adjustment. GWAS of CRT-related tinnitus revealed a prominent signal in chromosome 1 led by rs203248 (p = 1.50x10−9), while GWAS of CRT-related hearing loss identified rs332013 (p = 5.79x10−7) in chromosome 8 and rs67522722 (p = 7.78x10−7) in chromosome 6 as approaching genome-wide significance. Replication analysis in an independent cohort of pediatric cancer survivors (SJLIFE) indicated that rs67522722, intronic to ATXN1, a gene associated with the neurodegenerative disorder spinocerebellar ataxia type 1, was significantly associated with CRT-related hearing loss (p = 0.03). Enrichment analysis and LD score regression with previous GWAS results of cisplatin-related hearing loss and tinnitus in testicular cancer survivors showed no detectable enrichment in genetic architecture with CRT-related hearing loss and tinnitus, respectively. Conclusions: Radiation-associated ototoxicity was associated with sex, several neuro-otological symptoms, increased antidepressant use and poorer self-reported health. GWAS of CRT-related hearing loss identified rs67522722 that was replicated in an independent cohort of pediatric cancer survivors.

P1781 Cardio-protective role of dexrazoxane co-administered with anthracyclines: long-term echocardiographic follow-up in adult survivors of pediatric cancer

Abstract Background Cardiovascular disease is a well-recognized cause of increased late morbidity and mortality among survivors of childhood cancer treated with anthracyclines. A decrease in left ventricular (LV) ejection fraction (LVEF) and fractional shortening may be observed during follow-up. Previous studies reported non-negligible prevalence of subclinical systolic dysfunction assessed with deformation imaging at short-, mid- and long-term follow up. Co-administration of Dexrazoxane has been shown to significantly reduce short-term and mid-term cardiotoxicity. The usefulness of dexrazoxane in preventing late (&amp;gt;10 years) anthracycline cardiotoxicity remains under discussion. Purpose Aim of this study was to assess cardiac function in long-term (&amp;gt;10 years) survivors of childhood tumors treated with dexrazoxane/anthracycline association. Methods Twenty cancer survivors previously treated with co-administration of anthracyclines and dexrazoxane for childhood renal tumors or sarcoma and a control group of 20 healthy non-athletic subjects matched for age, sex and body surface area were enrolled in the study. Echocardiographic measurements included 3D LVEF and LV and right ventricular (RV) global longitudinal strain (GLS). Cancer survivors were evaluated at median follow-up time of 21.5 years (range 10-26). Results No evidence of cardiac toxicity, as defined by current guidelines, was reported in all survivors. None of survivors presented LVEF &amp;lt; 50% or abnormal longitudinal strain, defined as a value &amp;gt;2 SDs below the mean using sex-specific and age-specific strain values. No significant differences in standard and deformation imaging parameters were observed between survivors and controls (3D LVEF 58 ± 3 % vs 60 ± 5 % p = NS; LV GLS -21 ± 1 % vs - 21 ± 2 % p= NS; RV GLS - 23 ± 2 % vs - 23 ± 5 % p= NS). Moreover, considering subjects who received a cumulative dose of anthracyclines above the median (doxorubicin-equivalent dose ≥208 mg/m2) no significant differences were found as compared to the group receiving a lower dose. Conclusions No evidence of cardiac toxicity was detected in all survivors. Our findings support the cardio-protective role of dexrazoxane in children undergoing anthracycline-based treatment. Parameters Cancer survivors (n= 20) Controls (n= 20) P 3D LVEF (%) 58 ± 3 60 ± 5 NS LV GLS (%) -21 ± 1 -21 ± 2 NS RV GLS (%) -23 ± 2 -23 ± 5 NS 3 D LVEF: Three-Dimensional Left Ventricular Ejection Fraction, LV GLS: Left Ventricular Global Longitudinal Strain; LV; RV GLS: Right Ventricular Global Longitudinal Strain. Abstract P1781 Figure. LV strain analysis in a survivor

User-centered design and enhancement of an electronic personal health record to support survivors of pediatric cancers.

The objective of this article is to demonstrate how user-centered design theory and methods can be employed to develop and iteratively improve technologies to support survivors of childhood cancer. Focus groups and structured interviews with young adult survivors of pediatric cancer (N = 3), parents (N = 11), and healthcare providers (N = 14) were conducted to understand their needs as potential users and the contexts in which they would use an electronic personal health record (PHR) for survivors, Cancer SurvivorLinkTM (https://cancersurvivorlink.org/). Usability evaluations were conducted to assess the functionality of the PHR using think aloud protocol with survivors/parents (N = 4) and focus groups with providers (N = 12). Major themes identified through the needs assessment guided design of the PHR, including (1) education about the lifelong healthcare needs of pediatric cancer survivors ("Learn"), (2) secure electronic storage for healthcare documents to direct long-term follow-up care ("Store"), and (3) communication functionality to allow sharing of health documents with healthcare providers ("Share"). Usability evaluations identified challenges with the PHR design, which informed site enhancements to improve PHR usefulness and ease of use including a registration wizard and healthcare provider directory. User-centered design methods informed iterative enhancements to an untethered, patient-controlled PHR to address usability barriers and meet the self-identified needs of survivors of childhood cancer and their providers.

Neighborhood effect and obesity in adult survivors of pediatric cancer: A report from the St. Jude lifetime cohort study.

Survivors of childhood cancer are at risk for obesity, a condition potentially modifiable if dietary intake and physical activity are optimized. These health behaviors are likely influenced by neighborhood of residence, a determinant of access to healthy, affordable food and safe and easy exercise opportunities. We examined associations between neighborhood level factors and obesity among survivors in the St. Jude Lifetime cohort and community comparison group members. Persons with residential addresses available for geocoding were eligible for analysis (n = 2,265, mean age 32.5 [SD 9.1] years, 46% female, 85% white). Survivors completed questionnaires regarding individual behaviors; percent body fat was assessed via dual X-ray absorptiometry (obesity: ≥25% males; ≥35% females); neighborhood effect was characterized using census tract of residence (e.g., neighborhood socioeconomic status [SES], rurality). Structural equation modeling was used to determine associations between neighborhood effect, physical activity, diet, smoking, treatment exposures and obesity. Obese survivors (n = 1,420, 62.7%) were more likely to live in neighborhoods with lower SES (RR: 1.23, 95% CI: 1.10-1.38) and rural areas (RR: 1.22, 95% CI: 1.07-1.39) compared to survivors with normal percent body fat. Resource-poor neighborhoods (standardized effect: 0.06, p < 0.001) and cranial radiation (0.16, p < 0.001) had direct effects on percent body fat. Associations between neighborhood of residence and percent body fat were increased (0.01, p = 0.04) among individuals with a poor diet. Neighborhoods where survivors reside as an adult is associated with obesity. Interventions targeting survivors should incorporate strategies that address environmental influences on obesity.

Echocardiographic long-term follow-up of adult survivors of pediatric cancer treated with Dexrazoxane-Anthracyclines association

AimsCardiovascular disease is a well-recognized cause of increased late morbidity and mortality among survivors of childhood cancer treated with anthracyclines. Co-administration of Dexrazoxane has been shown to significantly reduce short-term and mid-term cardiotoxicity. Aim of this study was to assess cardiac function in long-term (>10 years) survivors of childhood tumors treated with dexrazoxane/anthracycline association. Methods and resultsTwenty cancer survivors previously treated with co-administration of anthracyclines-dexrazoxane for childhood renal tumors or sarcoma and a control group of 20 healthy subjects were enrolled in the study. Echocardiographic measurements included 3D left ventricular (LV) ejection fraction (LVEF) and LV and right ventricular (RV) global longitudinal strain (GLS). Among cancer survivors group the median age at diagnosis was 5 years (1–17) and they were evaluated at median follow-up time of 21.5 years (10–26).No evidence of cardiac toxicity, as defined by current guidelines, was reported in all survivors. No significant differences in standard and deformation imaging parameters were observed between survivors and controls (3D LVEF 58 ± 3% vs 60 ± 5% p = NS; LV GLS −21 ± 1% vs −21 ± 2% p = NS; RV GLS −23 ± 2% vs −23 ± 5% p = NS). No second tumor was registered in dexrazoxane-treated survivors. ConclusionsOur findings may support the role of dexrazoxane as a useful strategy for cardio-protection in children undergoing anthracycline based treatment. However, large randomized trials are needed to confirm the cardio-protective role of dexrazoxane in pediatric setting at long-term follow-up.

"There Is No Before Cancer… There Is Only Cancer." Perceived Late Effects of Pediatric Cancer on Survivors.

Although "late effects" connotes experiencing effects later in life, they can emerge immediately after active treatment. The effects that survivors experience have been reported but rarely from the point of view of the survivors regarding their life after treatment. To examine the perceived late effects of pediatric cancer on survivors and their self-identified primary support persons in order to understand the multifaceted nature of living after a pediatric cancer diagnosis. Using a pragmatic interpretive phenomenology approach, 10 survivors of pediatric cancer (aged 21-28 years) and 9 of their support persons (aged 23-73 years) participated, completing background questionnaires and semistructured, one-on-one interviews. Interview transcripts were analyzed thematically. Both survivors and support persons acknowledged that survivors experienced negative physical and cognitive health outcomes that require follow-up care. Survivors acknowledged that their cancer experience and residual effects have changed the trajectory of their lives. Research on young adult survivors of pediatric cancer and the residual/late effects and emotional outcomes they experience is warranted. Longitudinal research can aid in understanding how effects develop or worsen over time. The findings of this study support The Children's Oncology Group Long-term Follow-up Guidelines for practitioners. As the frontline caring for these individuals and families, nurses' involvement in transitional care out of treatment is necessary. Continued involvement and understanding of long-term pediatric cancer survivorship for nurses are also imperative for continuity for survivors and families.

Neighborhood effects and obesity in adult survivors of pediatric cancer: A report from the St. Jude Lifetime Cohort Study.

e23051 Background: Survivors of childhood cancer are at risk for obesity and associated chronic health conditions - risks that are potentially modifiable if survivors adopt a lifestyle with adequate physical activity and a healthy diet. Neighborhoods where survivors reside may influence uptake of health behaviors. We examined associations between neighborhood factors and obesity in survivors. Methods: Adult survivors participating in the St. Jude Lifetime cohort with addresses available for geocoding were eligible for analysis [N = 2265, mean assessment age 32.5 (SD 9.1) years, 46% female, and 85% white]. Survivors completed questionnaires regarding individual behaviors; percent body fat was assessed via dual x-ray absorptiometry (obesity: ≥25% males; ≥35% females); and neighborhood effects were characterized using census tract of residence (e.g. neighborhood socioeconomic status (SES), rurality). Structural equation modeling (SEM) was used to determine associations between neighborhood effects, physical activity, diet, smoking, treatment exposures, and obesity. Results: Obese survivors (n = 1420, 62.7%) lived in neighborhoods with less access to exercise opportunities (63% vs 66%, p = 0.01) and lower SES (22% vs 18%, p &lt; 0.001); and were more likely to live in small town/rural areas (14% vs 10%, p = 0.04) compared to non-obese survivors. Obese survivors who lived in the lowest SES neighborhoods were more likely to be CNS tumor survivors (17% vs 12%, p = 0.02) and received higher mean doses of cranial radiation (CRT) (15Gy vs 11Gy, p = 0.02) than obese survivors living in higher SES neighborhoods. Resource poor neighborhoods (standardized effect: 0.09, p &lt; 0.001) and CRT (0.14, p &lt; 0.001) had direct effects on percent body fat. Associations between neighborhood of residence and percent body fat were attenuated (-0.02, p &lt; 0.001) among individuals with a better diet. Conclusions: The neighborhood in which a childhood cancer survivor resides as an adult is associated with obesity, and obese survivors treated with CRT are more likely to live in neighborhoods with lower SES. Interventions targeting survivors should incorporate strategies that address environmental influences on obesity.

Population-Based Survey Methods for Reaching Adolescent and Young Adult Survivors of Pediatric Cancer and Their Parents.

Contacting childhood cancer survivors (CCS) to assess reasons for declining receipt of follow-up care after treatment is difficult and participation in surveys may be low, resulting in biased results. We sought to demonstrate effective recruitment and population-based sampling methods to improve response and minimize bias. Four hundred and seventy CCS diagnosed between 2000 and 2007 at two hospitals in Los Angeles County were selected from the California Cancer Registry and were 15-25 years of age at the time of interview. Surveys of survivors and their parents were completed by multiple methods including mail, online, and telephone. Effectiveness of "plain" versus "designer" formatting of study materials was tested. Variables associated with response were analyzed using univariate and multivariable methods. Effort required for recruitment was quantified. Fifty percent of survivors (n = 235) and 36.5% of parents (n = 171) responded, and there were 160 parent-child dyads among them. Among located survivors, 61% participated. Response was higher for women, parents of younger survivors, and those from higher socioeconomic status areas. Among Hispanics, no variables were related to response. More effort was required to reach men and older survivors, but efforts beyond 15 calls and 7 remailings were unproductive. Formatting (i.e., plain vs. designer) did not affect response. Efforts to reach survivors must include multiple methods to be successful. Use of an intensive recruitment strategy and population-based sample resulted in a largely representative sample of CCS, especially for Hispanics. Expensive design efforts had little effect on recruitment, suggesting that plainer materials are sufficient. This example may inform similar studies.