Parent-child communication surrounding genetic testing for Li-Fraumeni syndrome: Living under the cloud of cancer.

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

Factors supporting cardiomyopathy screening among at-risk adult survivors of pediatric malignancies.

PURPOSE: Anthracyclines and chest irradiation place adult survivors of childhood cancer at risk of cardiomyopathy; many survivors do not obtain the recommended screening. Based on our recent clinical trial, the addition of telephone counseling to a printed survivorship care plan more than doubled survivors' risk-based screening. Here, we sought to measure the impact of specific factors targeted in the intervention for their impact on survivors' screening participation. METHODS: Study population-survivors participating in a randomized longitudinal intervention trial. Survivor questionnaires and medical records at baseline and 1-year follow-up provided the data. Within- and between-group differences in factors were assessed at baseline and follow-up; structural equation modeling (SEM) identified direct and indirect effects on screening participation. RESULTS: Of the 411 survivors, 55.3% were female, 89.3% white, 38.9% college graduates, and age 26-59 years (mean = 41 years, SD = 7.68 years). At follow-up, the counseling group demonstrated higher scores for intent to undergo screening (p < 0.001), adherence determination (p < 0.001), autonomous regulation (p < 0.001), competency (p = 0.03), perceived effort warranted for screening (p < 0.001), and perceived value of screening (p = 0.02). SEM identified four factors that directly influenced screening participation (n = 411, RMSEA = 0.02 [90% CI = 0.000-0.05]; CFI = 0.99; TLI = 0.99; WRMR = 0.63): the counseling intervention (p < 0.0001), intrinsic motivation (p < 0.0001), competency (p < 0.0001), and decisional control (p = 0.001); intrinsic motivation was also a mediator (p = 0.002) of screening participation. CONCLUSIONS: Direct interpersonal interaction that focused on multiple modifiable, autonomy-supportive factors powerfully enhances the efficacy of a print survivorship care plan in increasing survivors' screening participation. This finding challenges providers to reach beyond the disease treatment focus and embrace these strategies in their behavior change efforts.

Investigating the relationship between COMT polymorphisms and working memory performance among childhood brain tumor survivors.

BACKGROUND: Survivors of childhood brain tumors are at increased risk for neurocognitive impairments, including deficits in abilities supported by frontal brain regions. Catechol-O-methyltransferase (COMT) metabolizes dopamine in the prefrontal cortex, with the Met allele resulting in greater dopamine availability and better performance on frontally mediated tasks compared to the Val allele. Given the importance of identifying resiliency factors against the emergence of cognitive late effects, the current study examined the relationship between COMT genotype and working memory performance among childhood brain tumor survivors. PROCEDURE: Children treated for a brain tumor with conformal radiation therapy (N = 50; mean age at irradiation = 7.41 ± 3.41; mean age at assessment = 13.18 ± 2.88) were administered two computerized measures of working memory (self-ordered search verbal and object tasks). Buccal (cheek) swabs were used to provide tissue from which DNA was extracted. RESULTS: Findings revealed an association between COMT genotype and performance on the self-ordered verbal (P = 0.03) but not object task (P = 0.33). Better performance was found for the Met/Val group compared to either Met/Met or Val/Val. CONCLUSIONS: COMT may indicate a potential resiliency factor against neurocognitive effects of cancer and its treatment; however, there is a need for replication with larger samples of childhood brain tumor survivors.

Utilization of Photobiomodulation for the Prevention and Treatment of Oral Mucositis.

Background: Oral mucositis is a significant and common toxicity experienced by patients who receive high-dose chemotherapy as a preparatory regimen for a hematopoietic cell transplant (HCT). Photobiomodulation (PBM) has been found to be feasible with significant efficacy in preventing the progression of oral mucositis in adult patients undergoing HCT. The purpose of this study was to determine the feasibility and efficacy of PBM in pediatric oncology patients undergoing HCT. Method: Forty children and adolescents admitted to the transplant unit for an allogeneic HCT for acute lymphoblastic leukemia or acute myeloid leukemia were treated daily at six sites until day + 20 or engraftment. Results: There were 1,035 patient encounters, with successful treatment of four or more sites during 979 patient encounters for a feasibility 93.3% CI [0.926, 0.039]. We had estimated a meaningful effect size of 20% for PBM and estimated 51% of patients treated with PBM would have at least one day or more of Grade 3 mucositis. The rate of patients who received PBM and developed Grade 3 mucositis was 20% CI [0.091, 0.356]. Patients treated with PBM had fewer days of hospitalization (p = .009) and less severe mucositis in comparison to the matched control group (p = .03). Conclusion: PBM is feasible and effective in preventing and treating oral mucositis and is now supported by the Children's Oncology Group for prevention and treatment of oral mucositis in patients undergoing an allogeneic HCT or receiving head/neck radiation.

Breastfeeding practices among childhood cancer survivors.

PURPOSE: This cross-sectional study compared breastfeeding outcomes among childhood cancer survivors to those of women in the general population and evaluated whether breastfeeding is adversely affected by cancer treatment or endocrine-related late effects. METHODS: A self-reported survey ascertained breastfeeding practices and incorporated items from the questionnaires used in the Infant Feeding Practices Study II (IFPS II) to allow comparison with the general population. Among 710 eligible survivors, 472 (66%) responded. The participants were predominantly non-Hispanic White (84%), married (73%), and had some college or less (60%). The mean maternal age at the time of birth of the first child after cancer treatment was 24 years (SD 24.3 ± 4.8). RESULTS: Fewer survivors planned to breastfeed than did IFPS II controls (67% vs. 82%, P < .0001), and fewer survivors initiated breastfeeding (66% vs. 85%, P < .0001). The median breastfeeding duration was shorter among survivors, with early undesired weaning occurring sooner in the survivor group (1.4 months, interquartile range (IQR) 0.5-3.5 months) than in the IFPS II group (2.7 months, IQR 0.9-5.4 months). A higher proportion of survivors reported an unfavorable breastfeeding experience (19% vs. 7.5%, P < .0001) and early, undesired weaning (57.5%, 95% CI 51-64) than did IFPS II participants (45.2%, 95% CI 44-47, P = .0164). Among survivors who expressed intention and chose to breastfeed, 46% endorsed disrupted lactation related to physiologic problems with high risk in those overweight/obese. CONCLUSIONS: Survivors are at risk of negative breastfeeding experiences; however, lactation outcomes were not significantly associated with cancer diagnosis, treatments, or endocrine complications. IMPLICATIONS FOR CANCER SURVIVORS: Prior research has not examined the association of cancer treatments and clinically validated late effects with lactation outcomes in a clinically diverse childhood cancer survivor cohort. Findings from this study suggest that childhood cancer survivors, especially those who are overweight/obese, are at risk of having negative breastfeeding experiences. Early undesired weaning, physiologic problems related to lactation and misconceptions about breastfeeding, especially fears of passing on cancer through breastmilk, highlight the need for counseling and specialized support to optimize lactation outcomes in this vulnerable population.

Reducing Compassion Fatigue in Inpatient Pediatric Oncology Nurses.

OBJECTIVES: To develop an evidence-based compassion fatigue program and evaluate its impact on nurse-reported burnout, secondary traumatic stress, and compassion satisfaction, as well as correlated factors of resilience and coping behaviors. SAMPLE & SETTING: The quality improvement pilot program was conducted with 59 nurses on a 20-bed subspecialty pediatric oncology unit at the St. Jude Children's Research Hospital in Memphis, Tennessee. METHODS & VARIABLES: Validated measures of compassion fatigue and satisfaction (Professional Quality of Life Scale V [ProQOLV]), coping (Brief COPE), and resilience (Connor-Davidson Resilience Scale-2) were evaluated preprogram and at two, four, and six months postprogram, with resilience and coping style measured at baseline and at six months postprogram. RESULTS: Secondary traumatic stress scores significantly improved from baseline to four months. Select coping characteristics were significantly correlated with ProQOLV subscale scores. IMPLICATIONS FOR NURSING: Ongoing organizational support and intervention can reduce compassion fatigue and foster compassion satisfaction among pediatric oncology nurses.

Increasing cardiomyopathy screening in at-risk adult survivors of pediatric malignancies: a randomized controlled trial.

PURPOSE: To determine whether the addition of advanced-practice nurse (APN) telephone counseling to a printed survivorship care plan (SCP) significantly increases the proportion of at-risk survivors who complete cardiomyopathy screening. PATIENTS AND METHODS: Survivors age ≥ 25 years participating in the Childhood Cancer Survivor Study who received cardiotoxic therapy and reported no history of cardiomyopathy screening in the previous 5 years were eligible for enrollment. The 472 participants (mean age, 40.1 years; range, 25.0 to 59.0; 53.3% women) were randomly assigned to either standard care, consisting of an SCP summarizing cancer treatment and cardiac health screening recommendations (n = 234), or standard care plus two APN telephone counseling sessions (n = 238). The primary outcome-completion of cardiomyopathy screening within 1 year-was validated by medical records and compared between the two arms using adjusted relative risks (RRs) with 95% CIs. RESULTS: Participants in the standard and APN counseling groups were not statistically different by demographic or clinical characteristics. At the time of 1-year follow-up, 107 (52.2%) of 205 survivors in the APN group completed screening compared with 46 (22.3%) of 206 survivors in the non-APN group (P < .001). With adjustment for sex, age (< 30 v ≥ 30 years), and Children's Oncology Group-recommended screening frequency group (annual, 2 years, or 5 years), survivors in the APN group were > 2× more likely than those in the control group to complete the recommended cardiomyopathy screening (RR, 2.31; 95% CI, 1.74 to 3.07). CONCLUSION: The addition of telephone counseling to an SCP with cardiac health screening recommendations increases cardiomyopathy screening in at-risk survivors.

Noncancer-related mortality risks in adult survivors of pediatric malignancies: the childhood cancer survivor study.

PURPOSE: We sought to identify factors, other than cancer-related treatment and presence/severity of chronic health conditions, which may be associated with late mortality risk among adult survivors of pediatric malignancies. METHODS: Using the Childhood Cancer Survivor Study cohort and a case-control design, 445 participants who died from causes other than cancer recurrence/progression or non-health-related events were compared with 7,162 surviving participants matched for primary diagnosis, age at baseline questionnaire, time from diagnosis to baseline questionnaire, and time at-risk. Odds ratios (ORs) and 95 % confidence intervals (CIs) were calculated for overall/cause-specific mortality. Independent measures included number/severity of chronic conditions, medical care, health-related behaviors, and health perceptions/concerns. RESULTS: Adjusting for education, income, chemotherapy/radiation exposures, and number/severity of chronic health conditions, an increased risk for all-cause mortality was associated with exercising fewer than 3 days/week (OR = 1.72, CI 1.27-2.34), being underweight (OR = 2.58, CI 1.55-4.28), increased medical care utilization (P < 0.001), and self-reported fair to poor health (P < 0.001). Physical activity was associated with a higher risk of death among males (OR = 3.26, CI 1.90-5.61) reporting no exercise compared to those who exercised ≥3 times per week. Ever consuming alcohol was associated with a reduced risk of all-cause (OR = 0.61, CI 0.41-0.89) and other nonexternal causes of death (OR = 0.40, CI 0.20-0.79). Concerns/worries about future health (OR = 1.54, CI 1.10-2.71) were associated with increased all-cause mortality. CONCLUSIONS: Factors independent of cancer treatment and chronic health conditions modify the risk of death among adult survivors of pediatric cancer. IMPLICATIONS FOR CANCER SURVIVORS: Continued cohort observation may inform interventions to reduce mortality.

Protective effects of breastfeeding for mothers surviving childhood cancer.

INTRODUCTION: Female childhood cancer survivors experience adverse health events secondary to cancer treatment. In healthy women, breastfeeding provides protection against many of these complications. Breastfeeding may be beneficial for mothers surviving childhood cancer by decreasing risks of, or ameliorating adverse late effects. Healthcare providers and survivors should be aware that successful lactation may be affected by previous cancer treatment. METHODS: The literature addressing lactation outcomes in cancer patients is reviewed, and processes that may disrupt breastfeeding are discussed. A summary of common late effects experienced by women is provided, and arguments are made for the potential amelioration of these complications by breastfeeding. RESULTS: Findings demonstrate that breastfeeding is beneficial for healthy mothers in regard to specific health conditions. There are limited data addressing breastfeeding outcomes among cancer survivors. Preliminary findings suggest that lactation is adversely affected among mothers treated for childhood cancer. CONCLUSIONS: Fundamental research is needed to determine rates of breastfeeding in childhood cancer survivors, identify specific cancer therapies and their effects on lactation, examine the efficacy of breastfeeding in risk reduction and/or amelioration of late effects, and develop interventions to increase breastfeeding among survivors of childhood cancer. IMPLICATIONS FOR CANCER SURVIVORS: As female childhood cancer survivors transition into adult medical care, it is important each patient be aware of her past medical history and the impact of treatment on her ability to successfully lactate. If lactation is possible, these women can choose to breastfeed their children, thereby engaging in a health behavior that may help protect them against many late effects of cancer treatment.

Long-term efficacy of methylphenidate in enhancing attention regulation, social skills, and academic abilities of childhood cancer survivors.

PURPOSE: Methylphenidate (MPH) ameliorates attention problems experienced by some cancer survivors in the short term, but its long-term efficacy is unproven. PATIENTS AND METHODS: This study investigates the long-term effectiveness of maintenance doses of MPH in survivors of childhood brain tumors (n = 35) and acute lymphoblastic leukemia (n = 33) participating in a 12-month MPH trial. Measures of attention (Conners' Continuous Performance Test [CPT], Conners' Rating Scales [CRS]), academic abilities (Wechsler Individual Achievement Test [WIAT]), social skills (Social Skills Rating System [SSRS]), and behavioral problems (Child Behavior Checklist [CBCL]) were administered at premedication baseline and at the end of the MPH trial while on medication. A cancer control group composed of patients who were not administered MPH (brain tumor = 31 and acute lymphoblastic leukemia = 23) was assessed on the same measures 12 [corrected] months apart. RESULTS: For the MPH group, repeated measures analysis of variance revealed significant improvement in performance on a measure of sustained attention (CPT indices, P < .05); parent, teacher, and self-report ratings of attention (CRS indices, P < .05), and parent ratings of social skills or behavioral problems (SSRS and CBCL indices; P < .05). In contrast, the cancer control group only showed improvement on parent ratings of attention (Conners' Parent Rating Scale indices; P < .05) and social skills (SSRS and CBCL indices; P < .05). There was no significant improvement on the academic measure (WIAT) in either group. CONCLUSION: Attention and behavioral benefits of MPH for childhood cancer survivors are maintained across settings over the course of a year. Although academic gains were not identified, MPH may offer benefits in academic areas not assessed.

Side effects of methylphenidate in childhood cancer survivors: a randomized placebo-controlled trial.

OBJECTIVES: To investigate the frequency and severity of side effects of methylphenidate among childhood survivors of acute lymphoblastic leukemia and brain tumors and identify predictors of higher adverse effect levels. METHODS: Childhood cancer survivors (N = 103) identified as having attention and learning problems completed a randomized, double-blind, 3-week, home-crossover trial of placebo, low-dose methylphenidate (0.3 mg/kg; 10 mg twice daily maximum) and moderate-dose methylphenidate (0.6 mg/kg; 20 mg twice daily maximum). Caregivers completed the Barkley Side Effects Rating Scale (SERS) at baseline and each week during the medication trial. Siblings of cancer survivors (N = 49) were recruited as a healthy comparison group. RESULTS: There was a significantly higher number and severity of symptoms endorsed on the SERS when patients were taking moderate dose compared with placebo or low dose, but not low dose compared with placebo. The number of side effects endorsed on the SERS was significantly lower during all 3 home-crossover weeks (placebo, low dose, moderate dose) when compared with baseline symptom scores. The severity of side effects was also significantly lower, compared with baseline screening, during placebo and low-dose weeks but not moderate-dose weeks. Both the number and severity of symptoms endorsed at baseline were significantly higher for patients compared with siblings. Female gender and lower IQ were associated with higher adverse effect levels. CONCLUSIONS: Methylphenidate is generally well tolerated by childhood cancer survivors. There is a subgroup at increased risk for side effects that may need to be closely monitored or prescribed a lower medication dose. The seemingly paradoxical findings of increased "side effects" at baseline must be considered when monitoring side effects and designing clinical trials.