Health-related and cancer risk concerns among siblings of childhood cancer survivors: a report from the Childhood Cancer Survivor Study (CCSS).

OBJECTIVE: To characterize the prevalence and predictors of concerns regarding future health and cancer risk among siblings of childhood cancer survivors. METHODS: This study reports longitudinal data (baseline and follow-up) from 3969 adult siblings (median age = 29 [range 18-56] years) of long-term survivors of childhood cancer (median time since diagnosis 19.6 [9.6-33.8] years). Self-reported future health and cancer risk concerns (concerned vs not concerned) were assessed. Demographics and health data reported by both the siblings and their matched cancer survivors were examined as risk factors for health concerns using multivariable logistic regression. RESULTS: Percentage of siblings reporting future health and cancer risk concerns, respectively, decreased across decade of survivors' diagnosis: 1970s (73.3%; 63.9%), 1980s (67.2%; 62.6%), and 1990s (45.7%; 52.3%). Risk factors associated with future health concerns included sibling chronic health conditions (grade 2 Odds Ratio [OR]=1.57, 95% CI: 1.12-2.20; grades 3-4 OR=1.86, 95% CI: 1.18-2.94; compared to less than grade 2). Risk factors associated with future cancer concerns included sibling chronic health conditions (grade 2 OR=1.43, 95% CI: 1.05-1.94; grades 3-4 OR=1.64, 95% CI: 1.09-2.47; compared to less than grade 2). CONCLUSIONS: Sibling concerns regarding future health and cancer have diminished in recent decades. There are subgroups of siblings that are at-risk for future health and cancer risk concerns. IMPLICATIONS FOR CANCER SURVIVORS: Routine screening of concerns in at-risk siblings of survivors of childhood cancer may benefit the siblings of cancer survivors. These individuals may benefit from early interventions during diagnosis and treatment of their siblings.

The Role of Social Media in Providing Support from Friends for Adolescent and Young Adult (AYA) Patients and Survivors of Sarcoma: Perspectives of AYA, Parents, and Providers.

Purpose: The aims of the current study were to better understand, from the perspective of adolescents and young adults (AYAs) with sarcoma, parents, and providers, the friendship support needs of AYAs with bone and soft tissue sarcoma and the role of social media in facilitating social support for AYAs with sarcoma. Methods: Semistructured interviews were conducted with 21 participants. AYA (n = 10) ranged in age from 14 to 23 years (mean 19.3, standard deviation 3.4 years; 50% female). All AYAs reported a current or past diagnosis of sarcoma, except for one patient who had another cancer diagnosis but was receiving treatment through the sarcoma clinic. Five parents of the adolescent participants were interviewed, as well as six health care providers. Data analysis was conducted using theory-driven immersion/crystallization, incorporating the Resilience in Illness Model as a framework to guide interpretation of the data. Results: Four main themes associated with social support from friends and social media were identified: (1) Social media provides a way to feel normal and connected to friends; (2) Social media accentuates the frustration of being left behind; (3) Social media facilitates the need to be understood by peers who have experienced sarcoma, and (4) Social media can lead to despair, and also provide hope for the future. Conclusions: Connecting with peers through social media can play an important role in providing support for AYAs with sarcoma, but it may also amplify feelings of frustration and anxiety. Future work is needed to determine intervention components that can maximize the benefits of social media for social support of AYAs with sarcoma. Clinical Trial Registration number: NCT03130751.

Longitudinal pain and pain interference in long-term survivors of childhood cancer: A report from the Childhood Cancer Survivor Study.

BACKGROUND: The objective of this study was to characterize the prevalence and risk of pain, pain interference, and recurrent pain in adult survivors of childhood cancer in comparison with siblings. METHODS: This study analyzed longitudinal data from survivors (n = 10,012; 48.7% female; median age, 31 years [range, 17-57 years]; median time since diagnosis, 23 years) and siblings (n = 3173) from the Childhood Cancer Survivor Study. Survivors were diagnosed between 1970 and 1986 at 1 of 26 participating sites. Associations between risk factors (demographics, cancer-related factors, and psychological symptoms) and pain, pain interference, and recurrent pain (5 years apart) were assessed with multinomial logistic regression. Path analyses examined cross-sectional associations between risk factors and pain outcomes. RESULTS: Twenty-nine percent of survivors reported moderate to severe pain, 20% reported moderate to extreme pain interference, and 9% reported moderate to severe recurrent pain. Female sex, a sarcoma/bone tumor diagnosis, and severe/life-threatening chronic medical conditions were associated with recurrent pain. Depression and anxiety were associated with increased risk for all pain outcomes. Poor vitality mediated the effects of anxiety on high pain and pain interference (root mean square error of approximation, 0.002). CONCLUSIONS: A large proportion of adult survivors report moderate to severe pain and pain interference more than 20 years after their diagnosis. Increased screening and early intervention for pain interference and recurrent pain are warranted.

A systematic review of self-reported pain in childhood cancer survivors.

AIM: The aim of this systematic review was to examine the frequency and risk factors of pain among long-term childhood cancer survivors based on self-reported questionnaire studies. Participants aged 21 years or younger at the time of their cancer diagnosis were included. METHODS: The Medline (OVID), PubMed and PsycINFO databases were searched for manuscripts published between January 1, 1990, and August 31, 2018, following the PRISMA statement for reporting systematic reviews. RESULTS: In total, 25 studies, including five prospective cohort studies measuring pain up to 32 years post-diagnosis, were identified. Female sex, young age at diagnosis, older age at assessment, greater time since diagnosis, specific diagnoses (such as central nervous system [CNS] tumours, bone tumours and sarcoma), fatigue and persistent emotional distress were associated with cancer-related pain. The most common pain types were migraines, headaches and back pain. CONCLUSION: A subset of childhood cancer survivors reported clinically significant self-reported pain. Identifying survivor subgroups at risk for pain could be essential for developing tailored intervention and prevention strategies. Prospective studies that use standardised and psychometrically sound tools to evaluate pain are needed.

Parent proxy assessment of sibling quality of life following pediatric hematopoietic cell transplantation.

BACKGROUND: When a child undergoes hematopoietic cell transplantation (HCT), the impact extends to the entire family, including siblings. Assessment of the quality of life (QoL) of siblings is challenged by their general lack of availability for regular assessment by clinical providers. Thus, the use of parent proxy reporting may be useful. Our aim was to describe the QoL of siblings of HCT survivors, as reported by their parents, as well as to identify parent and family factors associated with lower sibling QoL. METHODS: A cross-sectional study was utilized to assess parent-reported QoL of the HCT recipient's sibling (Short Form (SF)-10 Health Survey for Children and the Pediatric Symptom Checklist (PSC)-17). Parent QoL was assessed using the SF-12. Multivariable linear regression was used to explore hypothesized predictors of sibling QoL, including parent QoL, family impact/function (Impact on Family Scale, Family Adaptability and Cohesion Evaluation Scales, IV, and a question asking about financial problems) while adjusting for demographic and HCT characteristics. RESULTS: Ninety-seven siblings (55% males) with a mean age of 12 years (standard deviation [SD] 4 years) were assessed, representing HCT survivors, who were an average of 5 years (SD 4 years) post-HCT. Neither sibling psychosocial (mean 49.84, SD 10.70, p = 0.87) nor physical health scores (mean 51.54, SD 8.42, p = 0.08) differed from norms. Parent proxies reported behavioral/emotional problems (PSC-17 total score > 15) in 24% of siblings. While parental ratings of their own physical health (SF-12 were higher than norms (mean 53.04, SD 8.17, p = 0.0005), mental health scores were lower (mean 45.48, SD 10.45, p < 0.0001). In multivariable analysis, lower parent emotional functioning and adverse family function were associated with lower sibling QoL, as reported by parents. CONCLUSIONS: While proxy-reported QoL of siblings did not differ significantly from normative data, both parent QoL and family function were associated with sibling QoL. Future research is needed to understand how siblings themselves perceive their QoL following HCT.

A Mobile-Based Mindfulness and Social Support Program for Adolescents and Young Adults With Sarcoma: Development and Pilot Testing.

BACKGROUND: Approximately 70,000 adolescents and young adults (AYA) are diagnosed with cancer each year in the United States. Sarcomas carry a particularly high symptom burden and are some of the most common cancers among AYA. Recent work has documented significant levels of unmet needs among AYA with cancer, particularly the need for psychosocial support. Mobile technology may be a cost-effective and efficient way to deliver a psychosocial intervention to AYA with cancer and cancer survivors. OBJECTIVE: The two aims of this study were to (1) develop a pilot version of a mobile-based mindfulness and social support program and (2) evaluate program usage and acceptability. An exploratory aim was to examine change in psychosocial outcomes. METHODS: Thirty-seven AYA with sarcoma or sarcoma survivors, parents, and health care providers participated in the study. Semistructured interviews were conducted with 10 AYA, parents of five of the adolescents, and six health care providers. Themes from the interviews helped to inform the development of a mobile-based mindfulness pilot program and a companion Facebook-based social support group. Twenty AYA consented to participate in a single-arm pre-post evaluation of the program; 17 downloaded the app and joined the Facebook group. Seven of these participants had participated in the semistructured interviews. Six additional health care providers consented to participate in the evaluation stage. RESULTS: On average, participants completed 16.9 of the 28 unique sessions and used the mindfulness app for a mean 10.2 (SD 8.2) days during the 28-day evaluation period. The majority of participants (16/17) engaged in the social group and posted at least one reply to the moderator's prompts. The mean number of responses per person to the moderator of the social group was 15.2 of 31 (49%, range 0%-97%). Both AYA and health care providers responded positively to the Mindfulness for Resilience in Illness program and offered useful recommendations for improvements. Exploratory psychosocial analyses indicated there were no significant differences from pretest to posttest on measures of perceived social support, mindfulness, body image, or psychological functioning. CONCLUSIONS: This study offers preliminary support for the feasibility and acceptability of a mobile-based mindfulness and Facebook-based social support program for AYA with sarcoma. The feedback from AYA and health care providers will assist in creating a fully developed intervention. TRIAL REGISTRATION: ClinicalTrials.gov NCT03130751; https://clinicaltrials.gov/ct2/show/NCT03130751.

A prospective observational cohort study of exposure to womb-like sounds to stabilize breathing and cardiovascular patterns in preterm neonates.

PURPOSE: We exposed premature infants to womb-like sounds to evaluate such exposure on breathing and cardiovascular patterns. We hypothesized that these sounds would reduce apnea and intermittent hypoxemia, enhance parasympathetic outflow, and improve cardiovascular patterns. METHODS: A total of 20 cases and 5 control infants at ≤32-36 weeks corrected gestational age participated in a prospective observational cohort study. Twenty-four hours of continuous ECG, respiratory and oxygen saturation data were collected in all infants. Womb-like sounds were played intermittently in 6-hour blocks. Salivary samples were collected at study beginning and end for cortisol. Apnea, intermittent hypoxemia, and bradycardia were evaluated, and heart rate variability was assessed by time domain and spectral techniques. RESULTS: Intermittent hypoxemia and bradycardia significantly declined after sound exposure. No significant differences in apnea, cortisol levels, or heart rate variability were evident among the study infants. CONCLUSIONS: Exposing premature infants to womb-like sounds has the potential to reduce hypoxemic and bradycardic events, and be used as an intervention to stabilize breathing and cardiac control in preterm infants.

Health-related quality of life and psychological distress in young adult survivors of childhood cancer and their association with treatment, education, and demographic factors.

PURPOSE: This study investigated health-related quality of life (HRQOL) and psychological distress among young adult (YA) survivors of childhood cancer and the association of these measures with treatment, education, and demographic factors ≥ 5 years post diagnosis. METHODS: Participants included cancer survivors (n = 91) recruited through the Cancer Registry of Norway (CRN) and healthy controls (n = 223) recruited from a student population. All participants completed self-report questionnaires, and the Pediatric Quality of Life Inventory (PedsQL™) 4.0 and the Hopkins Symptom Checklist-10 (HSCL-10) as a measure of HRQOL and distress, respectively. RESULTS: Survivors reported HRQOL at the same level as controls, except for poorer physical functioning. Survivors in general, and female survivors specifically, had higher odds than controls of reporting symptoms of distress above cut-off, but survivors did not have higher mean levels of distress compared to controls. Survivors reporting distress levels above the cut-off had significantly poorer HRQOL regarding physical functioning and lower total PedsQL scores than controls scoring above the cut-off. Age (for HRQOL only), female gender, low educational level, and perceived low economic status significantly predicted HRQOL and distress. Education interacted with the effect of cranial radiation in predicting HRQOL. CONCLUSIONS: Survivors reported similar mean levels of HRQOL and distress as controls, except for physical functioning. For cancer survivors, demographic variables predicted HRQOL and distress. Some groups of survivors require closer follow-up, and more attention should be paid to factors associated with poor HRQOL and psychological distress in survivors, including female gender, lower education level, and lower income. Survivors treated with cranial radiation may be particular vulnerable in combination with low education regarding HRQOL.

A Review of Demographic, Medical, and Treatment Variables Associated with Health-Related Quality of Life (HRQOL) in Survivors of Hematopoietic Stem Cell (HSCT) and Bone Marrow Transplantation (BMT) during Childhood.

Hematopoietic stem cell transplantation (HSCT) is a standard treatment after disease relapse and failure of conventional treatments for cancer in childhood or as a first line treatment for some high-risk cancers. Since hematopoietic stem cells can be found in the marrow (previously called a bone marrow transplantation) or periphery, we refer to HSCT as inclusive of HSCT regardless of the origin of the stem cells. HSCT is associated with adverse side effects, prolonged hospitalization, and isolation. Previous studies have shown that survivors of HSCT are at particularly high risk for developing late effects and medical complications, and thus, in addition to survival, quality of life in survivors of HSCT is an important outcome. This review summarizes and distills findings on the health-related quality of life (HRQOL) of long-term childhood cancer survivors of HSCT and examines significant sociodemographic, medical, disease and treatment correlates of HRQOL, as well as the methodology of the studies (instruments, type of studies, timing of assessment, type of transplantation). Because previous reviews covered the studies published before 2006, this review searched three databases published between January, 2006, and August, 2016. The search identified nine studies, including 2 prospective cohort studies and 7 cross-sectional studies. All studies reported a follow-up time of >5 years. The review found that HRQOL is significantly impacted over time following childhood HSCT, with salient correlates of HRQOL found to be presence of a severe chronic health or major medical condition, graft vs. host disease (GVHD), or pain. Continual evaluation of HRQOL must be integrated into long-term follow-up after childhood HSCT, and intervention should be offered for those survivors with poor HRQOL. Longitudinal studies should be emphasized in future research to allow for predictor models of resilience and poor HRQOL.

Long-term neurologic health and psychosocial function of adult survivors of childhood medulloblastoma/PNET: a report from the Childhood Cancer Survivor Study.

Background: Medulloblastoma is the most common malignant childhood brain tumor, although long-term risks for chronic neurologic health and psychosocial functioning in aging adult survivors are incompletely characterized. Methods: The Childhood Cancer Survivor Study (CCSS) includes 380 five-year survivors of medulloblastoma/primitive neuroectodermal tumor (PNET; median age at follow-up: 30 y, interquartile range 24-36) and sibling comparison (n = 4031). Cumulative incidence of neurologic health conditions was reported. Cox regression models provided hazard ratios (HRs) and 95% CIs. Cross-sectional outcomes were assessed using generalized linear models. Results: Compared with siblings, survivors were at increased risk of late-onset hearing loss (HR: 36.0, 95% CI: 23.6-54.9), stroke (HR: 33.9, 95% CI: 17.8-64.7), seizure (HR: 12.8, 95% CI: 9.0-18.1), poor balance (HR: 10.4, 95% CI: 6.7-15.9), tinnitus (HR: 4.8, 95% CI: 3.5-6.8), and cataracts (HR: 31.8, 95% CI: 16.7-60.5). Temporal/frontal lobe radiotherapy of 50 Gy or more increased risk for hearing loss (HR: 1.9, 95% CI: 1.1-1.3), seizure (HR: 2.1, 95% CI: 1.1-3.9), stroke (HR: 3.5, 95% CI: 1.3-9.1), and tinnitus (HR: 2.0, 95% CI: 1.0-3.9). Survivors were less likely than siblings to earn a college degree (relative risk [RR]: 0.49, 95% CI: 0.39-0.60), marry (RR: 0.35, 95% CI: 0.29-0.42), and live independently (RR: 0.58, 95% CI: 0.52-0.66). Conclusions: Adult survivors of childhood medulloblastoma/PNET demonstrate pronounced risk for hearing impairment, stroke, lower educational attainment, and social independence. Interventions to support survivors should be a high priority.

Clinical Case Report: Yoga for Fatigue in Five Young Adult Survivors of Childhood Cancer.

PURPOSE: Cancer-related fatigue (CRF) is a distressing consequence of cancer and its treatment. CRF impacts many young adult (YA) survivors of childhood cancer, compromising work, social relationships, and daily activities. No satisfactory treatment exists. This pilot study aimed to assess the feasibility, safety, and preliminary efficacy of an 8-week twice/week Iyengar yoga (IY) intervention for treating persistent fatigue in YA survivors of childhood cancer. METHODS: Using a single-arm mixed-methods design, adult childhood cancer survivors aged between 18 and 39 years were recruited from a survivorship clinic at a single institution. Quantitative: The primary outcome was fatigue as measured by the Functional Assessment of Chronic Illness Therapy-Fatigue. Secondary outcomes included vitality, social functioning, multidimensional fatigue, mood, and sleep. Weekly self-report monitoring data were collected. Qualitative: Participants also completed a post-intervention interview, major themes evaluated. RESULTS: Five participants enrolled into the study and four completed the intervention. Attendance was 92% and there were no adverse events. Baseline mobility was highly varied, with one YA having had a hemipelvectomy. Quantitative data revealed significantly improved fatigue, social functioning, somatization, and general and emotional manifestations of fatigue following yoga. Qualitative data cross validated, clarified, and expanded upon the quantitative findings. CONCLUSIONS: The study suggests that a brief IY intervention is safe for YA survivors of childhood cancer, even for those with physical disabilities. Preliminary efficacy was demonstrated for the primary outcome of fatigue. Qualitative data elucidated additional improvements, such as work-related social functioning, and a sense of calm and relaxation.

Predictors of fatigue and poor sleep in adult survivors of childhood Hodgkin's lymphoma: a report from the Childhood Cancer Survivor Study.

PURPOSE: Survivors of pediatric Hodgkin's lymphoma (HL) are at risk for a number of debilitating late effects. Excessive fatigue and poor sleep quality are primary complaints of HL survivors. Understanding the emotional and physical factors that influence fatigue and sleep quality may provide opportunities for intervention to improve health-related quality of life for HL survivors. METHODS: Data from 751 adult survivors of childhood HL who participated in the Childhood Cancer Survivor Study (CCSS) from 2000-2002 were analyzed. Multivariable logistic regression analyses investigated the demographic, psychological, and physical variables that predicted clinically significant levels of poor sleep quality, fatigue, and excessive daytime sleepiness. RESULTS: Survivors' self-reported level of emotional distress, pain, and physical functioning limitations did not differ from population norms. Clinically elevated levels of emotional distress (OR 8.38, 95% CI 4.28-16.42) and pain (OR 3.73, 95% CI 2.09-6.67) increased the risk for endorsing elevated levels of fatigue. Survivors with elevated levels of emotional distress (OR 6.83, 95% CI 2.71-15.90) and pain (OR 5.27, 95% CI 1.78-15.61) were more likely to report poor sleep quality. Pain (OR 2.11, 95% CI 1.39-3.34) was related to excessive daytime sleepiness. CONCLUSIONS: Emotional and physical factors are associated with elevated levels of fatigue, excessive daytime sleepiness, and poor sleep quality in survivors of pediatric HL. This is consistent with findings from research conducted with non-cancer survivors. IMPLICATIONS FOR CANCER SURVIVORS: These results suggest that interventions designed to target sleep and fatigue difficulties in the general population may be well suited for pediatric HL survivors as well.

Pain in Fabry Disease: Practical Recommendations for Diagnosis and Treatment.

AIMS: Patients with Fabry disease (FD) characteristically develop peripheral neuropathy at an early age, with pain being a crucial symptom of underlying pathology. However, the diagnosis of pain is challenging due to the heterogeneous and nonspecific symptoms. Practical guidance on the diagnosis and management of pain in FD is needed. METHODS: In 2014, experts met to discuss recent advances on this topic and update clinical guidance. RESULTS: Emerging disease-specific tools, including FabryScan, Fabry-specific Pediatric Health and Pain Questionnaire, and Würzburg Fabry Pain Questionnaire, and more general tools like the Total Symptom Score can aid diagnosis, characterization, and monitoring of pain in patients with FD. These tools can be complemented by more objective and quantifiable sensory testing. In male and female patients of any age, pain related to FD can be an early indication to start disease-specific enzyme replacement therapy before potentially irreversible organ damage to the kidneys, heart, or brain occurs. CONCLUSION: To improve treatment outcomes, pain should be diagnosed early in unrecognized or newly identified FD patients. Treatment should include: (a) enzyme replacement therapy controlling the progression of underlying pathology; (b) adjunctive, symptomatic pain management with analgesics for chronic neuropathic and acute nociceptive, and inflammatory or mixed pain; and (c) lifestyle modifications.

Tobacco Use Among Siblings of Childhood Cancer Survivors: A Report From the Childhood Cancer Survivor Study.

BACKGROUND: Having a brother or sister with childhood cancer may influence health behaviors during adulthood. The aim of this study was to compare tobacco use in siblings of survivors with peers and to identify factors associated with sibling tobacco use. PROCEDURES: A retrospective cohort study was conducted using adult siblings (N = 1,974) of 5+ year cancer survivors in the Childhood Cancer Survivor Study (CCSS) and participants (N = 24,105, weighted to match CCSS) in the 2007 National Health Interview Survey. Self-reported tobacco use, sociodemographic, and cancer-related risk factors were analyzed. RESULTS: Siblings were equally likely to have ever smoked compared to their peers (odds ratio [OR] 1.02, 95% confidence interval [CI] 0.93-1.12). Siblings were less likely to be current smokers (OR 0.83, 95%CI 0.73-0.94), but more likely to be former smokers (OR 1.21, 95%CI 1.08-1.35). Siblings with low education were more likely to ever smoke (OR 1.51, 95%CI 1.15-2.00) and be current smokers (OR 1.67, 95%CI 1.24-2.26) compared to their peers. Among siblings, risk factors for current tobacco use included the following: low income <$20,000 (OR 1.66, 95%CI 1.09-2.54), low education (OR 6.68, 95%CI 4.07-10.97), psychological distress (OR 5.36, 95%CI 2.21-13.02), and heavy alcohol use (OR 3.68, 95%CI 2.50-5.41). CONCLUSIONS: Siblings of survivors take up smoking at similar rates to their peers, but are more likely to quit. Efforts are needed to address disparities by providing greater psychosocial support and education for the lowest socioeconomic status families facing childhood cancer.

Psychosocial and Neurocognitive Outcomes in Adult Survivors of Adolescent and Early Young Adult Cancer: A Report From the Childhood Cancer Survivor Study.

PURPOSE: To characterize psychological and neurocognitive function in long-term cancer survivors diagnosed during adolescence and early young adulthood (AeYA). METHODS: Six thousand one hundred ninety-two survivors and 390 siblings in the Childhood Cancer Survivor Study completed the Brief Symptom Inventory-18 and a Neurocognitive Questionnaire. Treatment and demographic predictors were examined, and associations with social attainment (employment, education, and living independently) were evaluated. Logistic regression models were used to compute odds ratios (ORs) and corresponding 95% CIs. RESULTS: Among survivors, 2,589 were diagnosed when AeYA (11 to 21 years old). Adjusted for current age and sex, these survivors, compared with siblings, self-reported higher rates of depression (11.7% v 8.0%, respectively; OR, 1.55; 95% CI, 1.04 to 2.30) and anxiety (7.4% v 4.4%, respectively; OR, 2.00; 95% CI, 1.17 to 3.43) and more problems with task efficiency (17.2% v 10.8%, respectively; OR, 1.72; 95% CI, 1.21 to 2.43), emotional regulation (19.1% v 14.1%, respectively; OR, 1.74; 95% CI, 1.26 to 2.40), and memory (25.9% v 19.0%, respectively; OR, 1.44; 95% CI, 1.09 to 1.89). Few differences were noted between survivors diagnosed with leukemia or CNS tumor before 11 years old versus during later adolescence, although those diagnosed with lymphoma or sarcoma during AeYA were at reduced risk for self-reported psychosocial and neurocognitive problems. Unemployment was associated with self-reports of impaired task efficiency (OR, 2.93; 95% CI, 2.28 to 3.77), somatization (OR, 2.29; 95% CI, 1.77 to 2.98), and depression (OR, 1.94; 95% CI, 1.43 to 2.63). CONCLUSION: We demonstrated that risk for poor functional outcome is not limited to survivors' diagnoses in early childhood. AeYA is a critical period of development, and cancer during this period can impact neurocognitive and emotional function and disrupt vocational attainment.

Sensory and Thermal Quantitative Testing in Children With Sickle Cell Disease.

Very little is known about pain processing in sickle cell disease (SCD). We examined the mechanical and thermal sensory patterns in children with SCD. Children ages 10 to 17 years (n = 48; mean 13.7 ± 2.0 y; 22 females) participated in quantitative sensory testing (QST) procedures and completed a quality of life (PedsQL) and anxiety and depression scale (RCADS). Thirteen children showed evidence of abnormal pain processing, indicated by decreased sensitivity to heat or cold sensations (hypoesthesia), and pain experienced with nonpainful stimuli (allodynia). Pain ratings associated with cold and warm sensations were significantly higher in the subgroup with abnormal QST compared with the 35 SCD children with normal QST (P = 0.01 and P < 0.0001, respectively). The presence of hypoesthesia and allodynia in children with SCD may represent abnormal changes in the peripheral and central nervous system. Clinicians need to be aware that sickle cell pain may not only be inflammatory or ischemic secondary to vasoocclusion and hypoxia, but may also be neuropathic secondary to nerve injury or nerve dysfunction. Neuropathic pain in SCD may be the result of tissue damage after vaso-occlusion in neural tissues, whether peripherally or centrally. Future studies are needed to determine the presence of neuropathic pain in children with SCD.

Psychosexual functioning among adult female survivors of childhood cancer: a report from the childhood cancer survivor study.

PURPOSE: Childhood cancer survivors may be at risk for impaired psychosexual functioning as a direct result of their cancer or its treatments, psychosocial difficulties, and/or diminished quality of life. PATIENTS AND METHODS: Two thousand one hundred seventy-eight female adult survivors of childhood cancer and 408 female siblings from the Childhood Cancer Survivor Study (CCSS) completed a self-report questionnaire about their psychosexual functioning and quality of life. On average, participants were age 29 years (range, 18 to 51 years) at the time of the survey, had been diagnosed with cancer at a median age of 8.5 years (range, 0 to 20) and were most commonly diagnosed with leukemia (33.2%) and Hodgkin lymphoma (15.4%). RESULTS: Multivariable analyses suggested that after controlling for sociodemographic differences, survivors reported significantly lower sexual functioning (mean difference [MnD], -0.2; P = .01), lower sexual interest (MnD, -0.2; P < .01), lower sexual desire (MnD, -0.3; P < .01), lower sexual arousal (MnD, -0.3; P < .01), lower sexual satisfaction (MnD, -0.2; P = .01), and lower sexual activity (MnD, -0.1; P = .02) compared with siblings. Risk factors for poorer psychosexual functioning among survivors included older age at assessment, ovarian failure at a younger age, treatment with cranial radiation, and cancer diagnosis during adolescence. CONCLUSION: Decreased sexual functioning among female survivors of childhood cancers seems to be unrelated to emotional factors and is likely to be an underaddressed issue. Several risk factors among survivors have been identified that assist in defining high-risk subgroups who may benefit from targeted screening and interventions.

Health and well-being in adolescent survivors of early childhood cancer: a report from the Childhood Cancer Survivor Study.

OBJECTIVE: With the growing number of childhood cancer survivors in the US, it is important to assess the well-being of these individuals, particularly during the transitional phase of adolescence. Data about adolescent survivors' overall health and quality of life will help identify survivor subgroups most in need of targeted attention to successfully transition to adulthood. PARTICIPANTS AND METHODS: This ancillary study to the Childhood Cancer Survivor Study focused on children 15-19 years of age who had been diagnosed with cancer before the age of 4 years. A cohort of siblings of pediatric cancer survivors of the same ages served as a comparison sample. Adolescent health was assessed using the Child Health and Illness Profile-Adolescent Edition (CHIP-AE) survey. RESULTS: The teen survey was sent to 444 survivor teens and 189 siblings. Of these, 307(69%) survivors and 97 (51%) siblings completed and returned the survey. The overall health profiles of siblings and survivors were similar. Among survivors, females scored significantly below males on satisfaction, discomfort, and disorders domains. Survivors diagnosed with central nervous system tumors scored less favorably than leukemia survivors in the global domains of satisfaction and disorders. CONCLUSION: In general, adolescent survivors fare favorably compared to healthy siblings. However, identification of the subset of pediatric cancer survivors who are more vulnerable to medical and psychosocial disorders in adolescence provides the opportunity for design and implementation of intervention strategies that may improve quality of life.

Suicide ideation and associated mortality in adult survivors of childhood cancer.

BACKGROUND: Adult survivors of childhood cancer are at risk for suicide ideation, although longitudinal patterns and rates of recurrent suicide ideation are unknown. This study investigated the prevalence of late report (ie, after initial assessment) and recurrent suicide ideation in adult survivors of childhood cancer, identified predictors of suicide ideation, and examined associations among suicide ideation and mortality. METHODS: Participants included 9128 adult survivors of childhood cancer and 3082 sibling controls enrolled in the Childhood Cancer Survivor Study who completed a survey question assessing suicide ideation on one or more occasions between 1994 and 2010. Suicide ideation was assessed using the Brief Symptom Inventory-18 instrument. Mortality data was ascertained from the National Death Index. RESULTS: Survivors were more likely to report late (odds ratio [OR] =1.9, 95% confidence interval [CI] =1.5-2.5) and recurrent suicide ideation (OR=2.6, 95% CI=1.8-3.8) compared to siblings. Poor physical health status was associated with increased risk of suicide ideation in survivors (late report: OR=1.9, 95% CI=1.3-2.7; recurrent: OR=1.9, 95% CI=1.2-2.9). Suicide ideation was associated with increased risk for all-cause mortality (hazard ratio=1.3, 95% CI=1.03-1.6) and death by external causes (hazard ratio=2.4, 95% CI=1.4-4.1). CONCLUSIONS: Adult survivors of childhood cancer are at risk for late-report and recurrent suicide ideation, which is associated with increased risk of mortality. Routine screening for psychological distress in adult survivors appears warranted, especially for survivors who develop chronic physical health conditions.