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Purpose: With the increased survival of retinoblastoma (RB) patients, it is important to evaluate the quality of life (QoL) of RB survivors as well as caregivers to provide comprehensive care to the children and caregivers. This study aims to assess the QoL of survivors of RB, as perceived by parents and the self-report by patients, through a pediatric QoL (PEDs-QoL) questionnaire., Methods: The study cohort included 86 RB survivors, 86 age-matched controls, and their primary carers. PedsQL 4.0 generic core scale and structured interview were administered. QoL in physical, social, emotional, and school health was evaluated and correlated with clinical and sociodemographic parameters., Results: The mean age of the RB survivors was 5.7 years with an M:F ratio of 1.1:1. Disease was bilateral in 79% of cases. About 45% (39/86) underwent enucleation, while others received combination therapy (16; 18%), chemotherapy (30; 34%), and radiation (1; 1%). As reported by parents, the QoL of physical health domain of RB survivors was 70.2 SD ± 27.8 and 96.15 ± 13 SD, emotional health was 72.1 ± 27.4 SD and 94.4 ± 12.5 SD, social health was 80.4 ± 24.9 SD and 98.6 ± 6.2 SD; and school health was 71.9 ± 2 6.5 SD and 96.1 ± 12.2 SD. As per the self-report perception, the QoL of physical health was 68.2 ± 27.8 SD and 96.2 ± 13 SD, emotional health was 66.2 ± 28.4 SD and 95.3 ± 12.5 SD, social health was 69.5 ± 24.9 SD and 98.7 ± 6.2 SD, and school health was 63.5 ± 26.5 SD and 95.1 ± 12.2 SD. There was a significant relationship between enucleation and QoL domains, where χ2 = 67.75, degrees of freedom (df) = 36, and P < 0.01. There was a significant association between vision in the better eye (6/18 or better = 8, 6/18-6/60 = 8, 3/60 or worse = 42) and QoL scores (χ2 = 95.36, df = 62, P < 0.01). There was a substantial association between socioeconomic status and QoL domains, where χ2= 88.5, df = 56, P < 0.01., Conclusion: The results of the study showed that the QoL of parents of RB survivors and self-proxy reports were negatively affected in many ways, including physical, social, emotional, and school-related dimensions. Despite the small differences, self-proxy reports indicated a lower QoL than the parents' group. The study findings indicate that there are notable correlations between enucleation and visual acuity less than 6/18, as well as socioeconomic status, with various aspects of QoL domains among individuals who have survived RB., (Copyright © 2024 Copyright: © 2024 Indian Journal of Ophthalmology.)

Objective and Design: Retinoblastoma (Rb) is a rare childhood eye cancer, with 45% of individuals impacted by heritable disease and the remainder impacted non-heritably. The condition can leave survivors with life-long psychological and social challenges. This qualitative study examined the psychosocial needs of teenagers and young adults living beyond Rb., Setting: A qualitative, exploratory study was conducted using focus groups with teenagers and interviews with young adults. Participants were recruited via the Childhood Eye Cancer Trust and the two national Rb treatment centres in the UK. Reflexive thematic analysis was used to analyse data using exploratory and inductive methods., Participants: 32 young survivors of Rb (10 heritable, 21 non-heritable, 1 unknown; 23 unilateral, 9 bilateral) aged between 13 and 29 years (12 male, 20 female)., Results: Data were rich and spanned the life course: three key themes were generated, containing eight subthemes. Theme 1 describes participants' experiences of childhood and trauma, including survivor guilt, memories from treatment and impact on personality. Theme 2 focuses on the challenges of adolescence, including the psychological impact of Rb, the impact on identity, and the sense of normality and adaptation to late effects. The third theme considered adulthood and the development of acceptance, a state of being widely considered unachievable during childhood, as well as the 'work' needed to feel supported, including seeking out information, peer support and therapeutic strategies., Conclusions: This study provides in-depth insight into the experiences of life beyond Rb. Findings highlight the need for specific psychosocial interventions informed by codesign., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Retinoblastoma treatment and follow-up is complex and varies between patients. Pathways of care can enhance the quality of care, patient outcomes, safety, satisfaction, and resource optimization. Developing a pathway of care for retinoblastoma was identified as a top research priority by the retinoblastoma community. This study aimed to co-design and pilot a pathway of care called the "Retinoblastoma Journey Map" tailored for caregivers of newly diagnosed children with retinoblastoma., Methods: A working group of patients, health professionals and researchers used human-centered design to ideate, prototype and refine the Retinoblastoma Journey Map. Caregivers of affected children were recruited to use and evaluate the Map. Mixed-methods data was collected on feasibility, acceptability, usability and perceived impact on communication, self-efficacy, anxiety, depression, and the quality of physician-patient interaction., Results: The Retinoblastoma Journey Map consisted of an illustrated roadmap with 25 child-friendly stickers covering clinical treatment, medical education and milestones. Quantitative analysis revealed that the Map was feasible, acceptable, and useable; however, no significant effect on communication, self-efficacy, anxiety, depression or quality of physician-patient interaction was observed. Qualitative analysis identified 6 themes: Primary Use; Challenges; Impact; Limitations; Feasibility, Acceptability, and Usability; and Unmet Needs., Conclusion: A pathway of care for retinoblastoma, co-designed by researchers, health professionals, and patients, was useable, acceptable, and feasible by caregivers of children with retinoblastoma. While significant effects on communication and physician-patient interaction were not observed, "legacy building" - documentation of the pathway of care by families for later education of their child - emerged as an unanticipated yet important use of the Retinoblastoma Journey Map., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Purpose: To (1) Compare vision-related quality of life (VRQOL) in adolescent and young adult (AYA) unilateral versus bilateral retinoblastoma (RB) survivors using a vision-targeted measure and a generic health-related quality of life (HRQOL) measure and (2) Assess associations among VRQOL and generic HRQOL domains and overall QOL and estimate associations of the VRQOL and HRQOL domains with overall QOL., Methods: The National Institute for Health (NIH) Toolbox® VRQOL instrument, PROMIS®-29 Profile v 2.1, and a single-item QOL measure were administered in a cross-sectional study of 101 RB survivors. Reliability for multi-item scales was estimated. Product-moment and Spearman rank correlation coefficients and stepwise ordinary least squares were used to measure associations of other variables with overall QOL., Results: Significantly worse VRQOL was reported by bilateral than unilateral RB survivors. Cronbach's alpha coefficients for all VRQOL scales ranged from 0.83 to 0.95. Medium to large correlations were found between all NIH Toolbox® VRQOL scales and the PROMIS®-29 measures. Depression and ability to participate in social roles and activities from the PROMIS®-29 Profile accounted for 38% of the variance in overall QOL with the psychosocial domain of the NIH Toolbox® VRQOL explaining 16% of the variance., Conclusion: VRQOL is impaired in bilateral RB survivors. VRQOL is associated substantially with the PROMIS-29 generic HRQOL measure but has significant unique associations with overall QOL. The NIH Toolbox® VRQOL measure provides important information about the vision-related effects on daily life of AYA RB survivors., (© 2022. The Author(s).)

Background: Retinoblastoma is the most common intraocular childhood cancer and is typically diagnosed in young children. With increasing number of survivors and improved medical outcomes, long-term psychosocial impacts need to be explored. Thus, the current study sought to assess functioning in school-aged survivors of retinoblastoma., Procedure: Sixty-nine survivors of retinoblastoma underwent a one-time evaluation of psychosocial functioning. Survivors (M age = 10.89 years, SD = 1.07 years; 49.3% male; 56.5% unilateral disease) and parents completed measures of quality of life (QoL; PedsQL) and emotional, behavioral, and social functioning (PROMIS [patient-reported outcome measurement information system] Pediatric Profile, BASC-2 parent report). Demographic and medical variables were also obtained., Results: On the whole, both survivors and caregivers indicated QoL and behavioral and emotional health within the typical range of functioning. Survivors reported better physical QoL compared to both parent report and a national healthy comparison sample, whereas caregivers reported that survivors experienced lower social, school, and physical QoL than a healthy comparison. Regarding behavioral and emotional health, survivors indicated more anxiety than a nationally representative sample. Parents of female survivors endorsed lower adaptive scores than parents of male survivors., Conclusions: Results indicated that survivors of retinoblastoma reported QoL and behavioral and emotional health within normal limits, although parents appear to perceive greater impairment across several assessed domains. Understanding both survivor and parent reports remains important for this population. Future research should explore psychosocial functioning of these survivors as they transition to adolescence and early adulthood, given the increased independence and behavioral and emotional concerns during these developmental periods., (© 2022 Wiley Periodicals LLC.)

Purpose: Retinoblastoma is an aggressive pediatric eye cancer. Patient-reported outcome measures reveal important insights into how patients perceive their own health. Currently, there is no widely used or validated measure for assessment of retinoblastoma outcomes. The purpose of this research was to uncover which treatment outcomes that retinoblastoma survivors and their parents value, to inform the development of a future measure., Methods: This qualitative, cross-sectional study included retinoblastoma survivors age 6 years and older and parents of retinoblastoma survivors. Participants who did not demonstrate fluency in English were excluded. Study participants participated in semistructured interviews or focus groups, either in person at The Hospital for Sick Children, Toronto, Canada, or through secure videoconference, between March 3, 2019, and January 25, 2020. Iterative rounds of opening coding, codebook development, and coresearcher analysis were used to identify key emergent themes and subthemes., Results: Seventeen adults participated in six focus groups. Nine pediatric survivors participated in individual interviews. Four common themes emerged from all participant groups: (1) definition of treatment success, (2) enucleation-acceptance and challenges, (3) treatment outcomes to measure, and (4) need for outcome reporting. An additional, unique theme was identified in all pediatric discussions: worries and coping mechanisms. Treatment outcomes deemed valuable were related to the following domains: psychosocial outcomes, daily functioning, functional vision, retinoblastoma education, cosmetic outcomes, and secondary eye conditions., Conclusion: This study represents the first stage in the development of a retinoblastoma-specific patient-reported outcome measure. The findings reveal insight into what outcomes are valued by survivors after treatment and offer promise to improve outcomes assessment for retinoblastoma., Competing Interests: Ajoy VincentConsulting or Advisory Role: Novartis Canada Pharmaceuticals Inc Helen DimarasConsulting or Advisory Role: Santen PharmaceuticalResearch Funding: NovartisNo other potential conflicts of interest were reported.

Background: Retinoblastoma is the most common malignant tumor in infancy and early childhood. Due to the high incidences of intracranial metastasis and distant metastasis, retinoblastoma not only threatens the life of affected children, but also brings heavy mental stress to their parents. A strong mental stress often leads to anxiety, depression, and other adverse emotions, which is very unfavorable to the treatment and prognosis by generating great psychological pressure and reducing the quality of life of the family. Reducing the psychological stress of the parents and improving the quality of life of the family are beneficial to the treatment and prognosis of retinoblastoma in children. However, there are no recommended non-pharmacological therapies to reduce the psychological stress and improve the quality of life of the parents of children with retinoblastoma. This study aims to evaluate the effects of non-pharmacological therapies on psychological stress and quality of life of parents of children with retinoblastoma through a meta-analysis, thus providing clinical evidence., Methods: Randomized controlled trials reporting the effects of non-pharmacological therapies on psychological stress and quality of life of parents of children with retinoblastoma published before 2021 November will be searched in online databases, including the China National Knowledge Infrastructure, Wanfang, Chinese Scientific Journal Database, China Biomedical Literature Database, PubMed, Embase, The Cochrane Library, and Web of Science databases. The Cochrane Quality Assessment Manual will be used to assess the quality of the included literatures. Meta-analysis will be performed using Revman 5.4 software., Results: This study will evaluate the effects of non-pharmacological therapies on psychological stress and quality of life of parents of children with retinoblastoma via grading anxiety scores, depression scores, and quality-of-life scores., Conclusion: This study will provide a reliable evidence-based basis for non-pharmacological interventions on parents of children with retinoblastoma., Ethics and Dissemination: Ethical approval was not required for this study. The systematic review will be published in a peer-reviewed journal, presented at conferences, and shared on social media platforms. This review would be disseminated in a peer-reviewed journal or conference presentations.OSF REGISTRATION NUMBER., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Retinoblastoma is a malignant tumor of the eye that typically presents in early childhood and occurs in approximately 1 in 20,000 births. While active treatment of the tumor is typically completed in childhood, survivors often suffer from long-term effects from treatment including visual impairment, facial deformities, and fear of recurrence or secondary cancer. However, little is known how these long-term effects affect their health-related quality of life (HRQOL). Purpose: To review the literature on HRQOL in retinoblastoma survivors. Method: We searched three electronic databases from January 2005 to December 2018 for original research articles reporting on HRQOL or individual domains such as function, cognition, and psychosocial outcomes in retinoblastoma survivors. Results: A total of 59 articles were reviewed and 15 were identified as eligible. Five of the studies reported worse HRQOL in retinoblastoma survivors than controls or general population norms. Parent-proxy ratings were worse than survivors' self-reports. Conclusion: Our findings confirm the need for further HRQOL research to assess the factors influencing long-term outcomes associated with treatment in adolescent and young adult retinoblastoma survivors. By identifying any potential deficits in specific domains of HRQOL, early interventions might be developed to improve HRQOL in retinoblastoma survivors.

Purpose: To assess depression, anxiety, and stress in parents of patients with retinoblastoma and to evaluate the impact of unifocal vs multifocal retinoblastoma., Methods: A cross-sectional, self-reported psychological assessment of parents of patients with retinoblastoma at a tertiary care ocular oncology center was performed. The Beck Depression Inventory-II (BDI), Beck Anxiety Inventory (BAI), The Parental Stress Index 4-Short Form, and a retinoblastoma Knowledge Assessment questionnaire were administered. Descriptive statistics for outcomes and comparative analyses were made., Results: There were 138 parents of children with retinoblastoma (unifocal: n = 77, multifocal: n = 61). Overall, parents displayed mild, moderate, or severe depression (BDI) (n = 37, 26.7%); mild, moderate, or severe anxiety (BAI) (n = 49, 35.8%), and stress scores within normal limits (n = 138, 100%). A comparison (unifocal vs multifocal) revealed parents of children with multifocal retinoblastoma with severe depression (1.4% vs 10.2%, P < .02), and no differences in anxiety or stress. Factors associated with moderate or severe parental depression included previous history of depression (30.0% vs 3.9%, P < .001) and factors for moderate or severe anxiety included previous history of depression (33.3% vs 8.6%, P < .001), parent highest level of education at high school or less vs college or beyond (29.2% vs 10.9%, P = .031), and parental report of "child developmental delay" (31.5% vs 11.3%, P = .019)., Conclusions: The majority of parents displayed minimal depression (73.3%), anxiety (64.2%), or stress (100%). However, severe depression is more often found in those whose children have multifocal disease, and previous history of depression and less education can impact psychological function. NOTE: Publication of this article is sponsored by the American Ophthalmological Society., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Monocular vision has been found to have a negative effect on children's motion processing and motor functions. Yet, knowledge of motor function of survivors of retinoblastoma (RB) with monocular vision (due to enucleation, for example) is limited. This study examined motor function and its relationship to visual-related and health-related quality of life (HRQOL) in survivors of RB with monocular vision., Procedure: Parents of 27 survivors of RB, who underwent an enucleation of one eye resulting in monocular vision, and of 21 typically developing children between the ages of 6 and 12, were administered questionnaires relating to their children's motor function (DCDQ), as well as vision-related function (CVFQ) and HRQOL (PedsQL)., Results: Of the 27 survivors of RB, 7 (25.6%) were found to have difficulties in motor functions, compared with 1 (4.8%) child in the control group. The difficulties were faced mainly in daily function requiring control during movement, including jumping, running, and ball playing. Additionally, significant correlations were found between motor functions and children's QOL. Finally, survivors of RB with monocular vision were found to have lower QOL, specifically physical- and school-related QOL., Conclusion: Survivors of RB who have monocular vision have a higher rate of decreased motor function and lower QOL. These results point to a need for ongoing assessment of survivors of RB to allow timely detection of motor deficits and to institute appropriate therapeutic interventions., (© 2019 Wiley Periodicals, Inc.)

Genetic testing and counseling have become integral to the timely control of heritable cancers, like the childhood eye cancer retinoblastoma. This study aimed to determine attitudes, knowledge and experiences related to retinoblastoma genetics, among survivors and parents of children with retinoblastoma in Kenya. This qualitative study used focus groups as the primary data collection method, coupled with a brief demographic questionnaire. Study settings were Kenyatta National Hospital and Presbyterian Church of East Africa Kikuyu Hospital. Thematic analysis was used to identify key themes. Thirty-one individuals participated in five focus groups. Two main concepts emerged: (1) the origins of retinoblastoma are unclear, and (2) retinoblastoma is associated with significant challenges. The lack of clarity surrounding the origins of retinoblastoma was linked to limited knowledge of retinoblastoma genetics, and limited genetic counseling delivery and uptake. The challenges associated with retinoblastoma were discussed in terms of the impact of the diagnosis on individuals and families, and unmet healthcare needs related to the diagnosis. Next steps will incorporate these findings to develop evidence-informed and accessible cancer genetic services in Kenya.

Importance: Retinoblastoma survivors are at risk for adverse oculo-visual outcomes. Limited data are available regarding long-term vision-targeted health-related quality of life (HRQoL) of adult retinoblastoma survivors., Objective: To examine vision-targeted HRQoL as reported on the 25-item National Eye Institute Visual Field Questionnaire for overall and specific scale scores among adult survivors of retinoblastoma., Design, Setting, and Participants: The Retinoblastoma Survivor Study is a retrospective cohort of adult retinoblastoma survivors treated at 3 academic medical centers in New York between 1932 and 1994. Participants completed a comprehensive questionnaire between April 2008 and June 2010. Items were scored in January 2013 and preliminary analyses were performed in July 2015. Models were finalized in May 2017., Main Outcomes and Measures: Self-reported vision-targeted HRQoL as reported on the 25-item National Eye Institute Visual Field Questionnaire. Items are scored from 0 to 100, with 100 representing the highest quality of life., Results: Among 470 adult retinoblastoma survivors (53.6% with bilateral disease; 52.1% female; 86.4% white and non-Hispanic; mean age at study, 43.3 years; range, 18.0-77.0 years), 86% had at least 1 eye removed (1 eye, 74.5%; both eyes, 11.5%); 56.5% were previously treated with radiotherapy; and 61.3% rated their eyesight as excellent/good while 16.2% reported complete blindness. The overall mean (SD) VFQ composite score for all survivors was 81.1 (17.2) (mean [SD] score for unilateral retinoblastoma survivors, 91.4 [7.7]; bilateral retinoblastoma survivors, 72.3 [18.2]; difference between survivors with unilateral and bilateral disease, 19.1 [95% CI, 16.5-21.7; P < .001]). Prior exposure to radiotherapy was not associated with decreased overall VFQ (β = -0.08; 95% CI, -0.15 to 0.002; P = .06) but was related to a few specific subdomains of visual functioning., Conclusions and Relevance: These findings suggest retinoblastoma-related oculo-visual problems are associated with functional status and vision-targeted HRQoL of adult survivors, particularly among those with bilateral disease.

Clinical genetic services are increasingly providing a more nuanced understanding of genetic disease diagnostics and future risk for patients. Effectively conveying genetic information is essential for patients to make informed decisions. This is especially important for survivors of heritable cancers such as retinoblastoma (childhood eye cancer), where survivors who carry a germline mutation in the RB1 gene are at increased risk of second cancers in adulthood, and of passing on the disease risk to future offspring. We conducted focus groups with adult survivors of retinoblastoma and parents of children with retinoblastoma, to uncover their knowledge of, experiences with and attitudes about retinoblastoma genetics and related impacts of the cancer. Results revealed that participants understood that retinoblastoma was a genetic disease, but often misunderstood the implications of genetics on cancer phenotype and risk. Experiences with genetic testing and counseling were generally positive, however, participants reported challenges in accessing genetic information and psychosocial support. Participants suggested more educational resources, peer-to-peer counseling, and psychosocial support would enhance uptake of important genetic information. The results of the study will inform patient-oriented approaches to deliver comprehensive genetic healthcare.

Purpose: Because retinoblastoma typically arises at a very young age, children are particularly vulnerable to vision impairment, associated developmental delays, and functional limitations. Limited information is available describing developmental delay and functional limitations in this population, necessitating supportive services including rehabilitation. The aims of this study were to describe the participation of children with newly diagnosed retinoblastoma in an occupational therapy program that identifies children in need of rehabilitation services. We also identify indications for referral to rehabilitation services among children with newly diagnosed retinoblastoma and enumerate the likelihood of these children receiving the recommended services., Methods: Twenty-two children participated in longitudinal occupational therapy assessments during the first year after diagnosis., Results: We recommended 1 or more types of rehabilitation services for 16 of 22 (72.7%) participants. Twelve of 16 (75%) received services., Conclusions: The results of this pilot study indicate that implementing a prospective occupational therapy-screening program is feasible and results in identification and initiation of therapy services in some children with retinoblastoma. Developmental screenings and follow-up of children with retinoblastoma is strongly recommended.

Background: With current modalities, cure rates of retinoblastoma are high and hence the number of survivors is increasing. However, data on quality of life (QOL) are minimal., Procedure: We analyzed QOL in 122 retinoblastoma survivors using the PedsQL(TM) 4.0 generic core scale. The self-reported questionnaire was filled by children of more than 5 years of age who had completed treatment for more than 12 months. The questionnaire consists of 23 questions on physical, social, emotional, and school domains on a scale from 0 to 4. This was converted to a scale from 0 to 100, where higher values represented better QOL. The QOL was compared with 50 siblings. Factors predicting the QOL were assessed., Results: The median age of retinoblastoma survivors was 98 months (range 60-247) and 68% were males. Overall QOL was significantly poorer in retinoblastoma survivors as compared with the controls. The emotional health domain of QOL was significantly affected. Difficulties in maintaining friendships and competing were reported in the social health domain. The school health domain showed significantly higher absenteeism. However, the physical health domain, including household work, exercise, and self-care, was similar in both the groups. Lower age at diagnosis (≤ 18 months) predicted better QOL (P = 0.05), whereas age at assessment, sex, IRSS stage, and previous surgery and radiotherapy were not predictive of poor QOL., Conclusions: We found a significantly poorer QOL in retinoblastoma survivors with the psychosocial health domain being more affected than the physical domain. Age less than 18 months at diagnosis predicted better QOL., (© 2015 Wiley Periodicals, Inc.)

Purpose: To identify the socioeconomic and psychosocial impacts of clinical treatment decisions for advanced unilateral intraocular retinoblastoma., Design: Retrospective observational case series., Setting: institutional study at Alexandria Main University Hospital., Study Population: records of 66 unilateral retinoblastoma cases treated from May 2005 to May 2013 were retrospectively reviewed. Sixty cases were eligible (International Intraocular Retinoblastoma Classification [IIRC] group C, D or E)., Procedures: two treatment groups were compared: enucleation vs. salvage treatment. Salvage treatment eyes were further subdivided based on IIRC group. Six socioeconomic parameters (financial burden, financial impact, psychological, social, medical and tumor impacts) were scored. Parameter scores ranged from 0 to 3, for overall score range 0 (no adverse impact) to 18 (severe adverse impact)., Main Outcome Measures: derived Socioeconomic scores were correlated with treatment and outcomes., Results: The enucleation group (28 eyes) had a median overall Socioeconomic score of 4/18, significantly lower than the salvage treatment group (32 eyes), median score 11/18 (P<0.01). Socioeconomic score varied with IIRC group. Attempted eye salvage failed in 25 children, due to uncontrolled tumor (44%) and socioeconomic impact of cumulative therapies (56%). Treatment duration and Socioeconomic score were higher for the 5 children in the salvage treatment group who developed metastatic disease compared to those without metastasis (P<0.01)., Conclusions: The socioeconomic and psychosocial impacts of attempted ocular salvage for unilateral intraocular retinoblastoma are severe, in comparison to primary enucleation. Primary enucleation is a good treatment for unilateral retinoblastoma., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

This study aimed to clarify the relationships among developmental characteristics of retinoblastoma (RB) infants, mother-infant interaction, and mental health of mothers. Prospective studies were conducted twice with 13 dyads of mothers and infants who were between one (Time 1) and two years old (Time 2). Cross-sectional and longitudinal analyses were performed. The mean Developmental Quotients (DQ) significantly decreased between Time 1 and Time 2 (p < .05). Four (30.8%) infants showed DQ p < 85 at Time 1. They showed a DQ below the borderline at Time 2 and their developmental changes were different from RB infants in the normal DQ range. Infant Behavior Checklist-R (IBC-R) scores negatively correlated with DQ (p < .05). The mothers' Japanese Nursing Child Assessment Teaching Scale (JNCATS) scores were significantly higher than the normative mean. The IBC-R scores negatively correlated with mothers' JNCATS scores at Time 1 (ρ = -.66, p < .05) and positively correlated with the child domain scores on the Parenting Stress Index at Time 2 (ρ = .62, p < .05). Some RB infants showed a developmental delay. Mother-child interactions were negatively affected and mothers recognized their infants' problem. The support needs to be provided in early infancy.

Purpose: To determine the developmental trajectory of early cognitive and adaptive skills in young children with retinoblastoma from diagnosis to 5 years of age., Patients and Methods: Ninety-four patients with retinoblastoma treated according to an institutional protocol underwent serial assessments of cognitive and adaptive functioning at age 6 months and 1, 2, 3, and 5 years. Data were analyzed by treatment strata, with patients with 13q deletion analyzed separately., Results: At baseline, across all patients (except those with 13q deletion), developmental functioning was comparable with the normative mean, with mean scores for all strata within the average range. However, at age 5 years, developmental functioning was in the low average range and significantly below normative means. The trajectories of developmental functioning demonstrated significant decline over time, although this varied by treatment group/strata. Patients treated with enucleation only evidenced the greatest decline in cognitive functioning; significant change was not observed in patients treated with other modalities. Notable declines in parent-reported communication skills were observed in the majority of patients. Patients with 13q deletion evidenced delayed cognitive functioning at baseline, but minimal declines were observed through age 3 years. However, significant decreases in adaptive functioning were demonstrated over time for the 13q deletion subset., Conclusion: The declines in functioning observed in this study were unexpected, as was the poorer performance of the enucleation-only group. This highlights the necessity of continuing to assess cognitive functioning in patients with retinoblastoma as they age. Additional research is necessary to determine the long-term trajectory of cognitive development in this population., (© 2014 by American Society of Clinical Oncology.)

Until the last two decades, indications that blind people would understand and create pictures were sparse. EW, a totally blind adult, who began making raised-line drawings in her thirties, created a portfolio of several hundred sketches in nine years. She selects her own topics and invents her treatments of the subjects. What is of special interest here is that two of her drawings, shown in the present paper, depict places but also use devices to indicate one is a sanctuary and the other concerns a tragic era, using metaphor. Lightness of line in a forest drawing indicates it is out of reality, enchanted, and a sanctuary. A tilted grid in a drawing of a Holocaust memorial shows the events at issue were twisted and crooked. The devices are metaphoric and novel. The drawings deal with an ontological category--values--for which metaphorical devices in raised-line depictions have not previously been considered.

The authors explored 12 couples' coping with their children's diagnosis and treatment of retinoblastoma using a semistructured interview, with qualitative, descriptive, narrative-interpretative analysis. Findings showed that the parents' experienced increased distress with the physician's first suspicion that something was seriously wrong. Distress was ameliorated when they arrived at a specialty treatment center but increased as they tackled treatment decisions. Distress decreased again after they consented to enucleation but increased after hospital discharge. The parents' strength, their ability together and individually, to separate and split between cognition and emotion contributed to coping. Parents need support from a multidisciplinary staff and parents who coped with retinoblastoma.

Purpose: To assess the participation and health-related quality of life (HRQOL) of survivors of childhood retinoblastoma (RB)., Patients and Methods: Parents of 46 survivors of childhood RB between the ages of 2-18 were administered questionnaires relating to their children's participation (CFFS) and HRQOL (CHQ and PedsQL) and children were administered the PedsQL. Results of the HRQOL were compared to population-based norms., Results: The overall QOL of survivors of RB was similar to that of age norms. However, parents' rating of their children's general and emotional health was lower than that of age norms, and survivors reported lower QOL related to school. Survivors of bilateral RB participated less in daily activities and had lower emotional QOL compared to those with unilateral RB, and parents of children who had an eye enucleated reported that their children had lower self-esteem. The level of participation was related to the perceived QOL., Conclusion: Our results indicate that children who are survivors of RB have an overall QOL that is similar to their age-peers. However, subgroups of survivors appear to have unique difficulties that require continued follow-up and intervention. Focus should be placed on their participation in daily activities both in the community and at school., (Copyright © 2010 Wiley-Liss, Inc.)

Background: Little is known about the impact of retinoblastoma (RB) on the health status of survivors in terms of disabilities and worries, both of which may restrict participation in activities of daily life., Methods: In this population-based cross-sectional study, content analysis was used to extract data on perceived restrictions and worries, from semi-structured interviews held with 156 RB survivors aged 8-35 years. The International Classification of Functioning Disabilities and Health (ICF) was used as a framework., Results: Of all survivors, 55% perceive RB-related restrictions in daily life activities (school, professional career, mobility, self-care, intimate relationships). Young/adolescent survivors (6%) and adult survivors (15%) frequently report anxiety about developing a second primary tumor (SPT). Compared with the general population, RB survivors did not differ in rates of employment or marital status. However, special educational services were more frequently offered, and the level of completed education was lower., Conclusion: RB has influenced the lives of most survivors and, even though their prognosis was good, illness-related restrictions are common. Especially fear of developing SPT and of further loss of vision are important life-long problems, and many survivors had special education needs. The ICF might serve as a bridge between families and professionals, because this classification may facilitate early detection of problems., (Copyright 2009 Wiley-Liss, Inc.)

Objective: To assess coping strategies of long-term retinoblastoma (RB) survivors and explore determinants of behavioural functioning, including medical, socio-demographic and coping variables., Methods: This population-based cross-sectional study included 117 RB survivors (12-35 years), registered in the Dutch national RB register. Survivors were asked to fill in coping, social support and behavioural questionnaires, and situational characteristics were obtained from medical archives and from an interview. Prevalence rates of coping strategies were computed based on self-reports. One-sample t-tests were applied to analyse differences in the use of coping strategies compared with healthy reference samples. Multiple regression analyses were performed to identify various determinants for behavioural problems within the RB sample., Results: RB survivors differed from their healthy reference group in one coping style, i.e. they showed significantly less emotion-oriented coping behaviour. Adolescents who came from a single-parent family and/or experienced lower social support and used more emotion-oriented coping reported more total problem behaviour. More internalizing problems were reported for adolescents who experienced less social support and less acceptance of the disease. For adults, more life events, emotion-oriented coping and lower social support explained more total problem behaviour, especially internalizing problems., Conclusion: RB survivors showed less emotion-oriented coping behaviour compared with the reference group. Behavioural problems are best determined by emotion-oriented coping, social support, life events other than RB and acceptance of the disease, and not by medical variables. Therefore, these variables should be taken into consideration during interventions for this group.

Objective: To assess behavioural problems in retinoblastoma (RB) survivors., Methods: This population-based cross-sectional study included 148 RB survivors (8-35 years), registered in the Dutch national RB register. Survivors and parents were asked to fill in behavioural questionnaires. Prevalence rates were computed, based on both self-reports and proxy reports. One-sample T-tests were applied to analyse differences compared with healthy reference samples. Multiple regression analyses were performed to identify predictors for behavioural problems within the RB sample., Results: Between-group differences varied across informants and across age groups. Parents reported significantly elevated total problem behaviour in 30% of their offspring (aged 8-17 years); this against 9% in adolescents (12-17 years) and 12% in adults (18-35 years) based on self-report. Parental reports showed significantly elevated rates of (1) internalising problems in boys and (2) somatic complaints in both girls and boys. Self-reports indicate significantly lowered levels of (1) externalising problems in adolescent and adult women and (2) thought problems in female adolescents and in adult men. Especially survivors who suffered hereditary RB, who had undergone more intensive treatment, and who came from a single-parent family were identified to be at most behavioural risk., Conclusion: Perception of severity and the nature of behavioural problems seem to differ between beholder, and to vary between age groups, if not between life stages. Health professionals should be aware that especially those who are confronted with hereditary RB and who subsequently undergo intensive treatment, and who grow up in broken families, run the risk of developing behavioural difficulties., ((c) 2008 John Wiley & Sons, Ltd.)

Mothers of survivors of Retinoblastoma (Rb) experience unique challenges communicating with their child about the condition. Children are mostly diagnosed within their first year but the consequences continue into young adult life. Here 39 mothers of Rb survivors (23 males, mean age = 10.26 years) were interviewed about their experiences. Mothers were asked about communication with their children about Rb, and future health risks. Interviews were analysed using thematic analysis. Mothers reported that they had informed children about past diagnosis and treatment but had spoken less about genetic risk or risk of secondary cancer. The child's age and information-seeking behaviour were associated with mothers' disclosure, along with mothers' perceptions that information would facilitate child coping. Findings suggest that mothers may need more guidance during follow-up care in communicating about the disease and its consequences for future health. Medical staff should also take extra care to ensure that mothers are aware of genetic counselling services and how to access them before the child is discharged from specialist care.

Background: To assess health-related quality of life (HRQoL) in children (8-11 years) and adolescents (12-18 years) who survived retinoblastoma (RB), by means of the KIDSCREEN self-report questionnaire and the proxy-report version., Methods: This population-based cross-sectional study (participation rate 70%) involved 65 RB survivors (8-18 years) and their parents. Child/adolescents' and parents' perception of their youth's HRQoL was assessed using the KIDSCREEN, and the results were compared with Dutch reference data. Relations with gender, age, marital status of the parents, and visual acuity were analyzed., Results: RB survivors reported better HRQoL than did the Dutch reference group on the dimensions "moods and emotions" and "autonomy". Increased ratings of HRQoL in RB survivors were mainly seen in perceptions of the younger children and adolescent girls. RB survivors with normal visual acuity scored higher on "physical well-being" than visually impaired survivors. Age was negatively associated with the dimensions "psychological well-being", "self-perception" (according to the child and parent reports) and "parent relations and home life" (according to the child). "Self-perception" was also negatively associated with visual acuity (according to the child). Only parents of young boys surviving RB reported lower on "autonomy" than the reference group, and parents of low visual acuity and blind RB survivors reported higher on "autonomy" than parents of visually unimpaired survivors. Survivors' perceptions and parents' perceptions correlated poorly on all HRQoL dimensions., Conclusion: RB survivors reported a very good HRQoL compared with the Dutch reference group. The perceptions related to HRQoL differ substantially between parents and their children, i.e. parents judge the HRQoL of their child to be relatively poorer. Although the results are reassuring, additional factors of HRQoL that may have more specific relevance, such as psychological factors or coping skills, should be explored.

The following case report describes the expanding role of pediatric dentists in treating children with craniofacial pathology. Retinoblastoma is the most common intraocular malignancy in childhood and is approximately the tenth most common pediatric cancer in the United States. Treatment consists of enucleation, or removal of the entire globe followed by placement of orbital implants. Un-restored anopthalmic sockets exhibit growth retardation and can lead to facial disfigurement. Maxillofacial prosthetic (MFP) rehabilitation can be especially challenging in younger, pre-cooperative or behaviorally compromised children and requires the skills and participation of a pediatric dental specialist as part of the MFP team. The following case report involving a 3 yr-old girl with retinoblastoma describes such challenges. The objective of the maxillofacial prosthetic team was to provide custom-built, acrylic, bilateral ocular prostheses in as comfortable and atraumatic manner as possible. The case was a success and underscores the value of a multidisciplinary dental approach for the treatment of children with very special needs.

Background: To assess the quality of life (QoL) and predictors thereof in Dutch adult hereditary and non-hereditary retinoblastoma (RB) survivors., Methods: In this population-based cross-sectional study, a generic QoL questionnaire (SF-36) and a disease-specific interview were administered to 87 adult RB survivors aged 18 to 35 years. Their QoL data were compared with those of a Dutch healthy reference group. Among the RB hereditary/non-hereditary survivors, the QoL was compared and predictors for QoL were identified by linear multiple regression analyses., Results: As a group, RB survivors scored significantly lower than the reference group on the SF-36 subscale 'mental health' (t = -27, df = 86, p < 0.01). Hereditary RB survivors scored lower on the subscale 'general health' (t = 2.6, df = 85, p < 0.01) than non-hereditary RB survivors. Having experienced bullying, as a child was a predictor for the SF-36 subscales: 'physical functioning' (p < 0.05), 'role functioning physical' (p < 0.01), 'role functioning emotional' (p < 0.05) and 'social functioning' (p < 0.01). Having experienced bullying (p < 0.01), but also subjective experience of impairment related to RB (p < 0.05), was predictors for 'general health'. Subjective experience of impairment was a predictor for 'vitality' (p < 0.01) and 'bodily pain' (p < 0.01)., Conclusion: In this exploratory study, it appears that the group of adult RB survivors experience a relatively good overall but slightly decreased QoL compared with the reference group. However, they report more problems with regard to their mental health (anxiety, feelings of depression, and loss of control). Hereditary RB survivors differ significantly from non-hereditary RB survivors only in 'general health'. Bullying in childhood and subjective experience of impairment are the main predictors of a worse QoL. In order to prevent worsening of QoL, or perhaps to improve it, clinicians should make an inventory of these issues at an early stage. We recommend further research to assess the specific psychological factors that may lead to mental health problems in this population.

We describe the Quality of Life (QoL) and IQ of survivors of retinoblastoma (Rb), both in relation to the normal population and between subgroups of Rb patients differing in relative risk (i.e. unilateral vs. bilateral disease). The sample included 54 children (28 males, age-range 8-16 years) and their mothers. Mothers completed standardized questionnaires to report their own QoL and that of their child. Children completed a brief IQ test. Compared with population norms, mothers reported lower levels of QoL for their child on total QoL and for sub-scales measuring Physical and Psychosocial function. Mothers reported their own QoL to be comparable or higher than norms on all but one of eight sub-scales (energy/vitality). Compared with population norms, children with no visual impairment scored in the normal range for tasks measuring Verbal IQ, but below the mean on tasks measuring Performance IQ. According to their mothers, survivors of Rb have excellent school attendance and take part in most school activities. However, based on standardized questionnaire, they show compromised QoL. We consider that excellent survival rates in Rb are matched with good QoL according to mothers' report.

Background: To assess the impact of providing research feedback to retinoblastoma survivors or their parents regarding the risk of second cancers., Procedure: A four-page survey was sent to 801 retinoblastoma survivors and 55 parents to ascertain their reactions to a "results-letter." The "results-letter" provided feedback from a study indicating that retinoblastoma survivors may be at increased risk of second cancers., Results: Three hundred and thirty-nine (339) retinoblastoma survivors and 43 parents responded to the survey. Eighty-four percent (84%) of respondents found the "results-letter" "very" to "extremely" understandable and 72% found it "very" to "extremely" useful. Participants scored "very" to "extremely" to the following emotions: frightened = 28%, anxious = 27%, sad = 25%, overwhelmed = 15%, angry = 11%, and guilty = 6%. Five (1.4%) respondents stated that they would have preferred not to receive the results. Responses did not vary significantly between survivors with different risks of second cancers. No significant differences were observed between males and females. However, parents were significantly more likely to report feelings of anxiety, guilt, anger, being overwhelmed, and frightened compared to adult retinoblastoma survivors (P < 0.05). Individuals with less than a college education were significantly more sad, angry, overwhelmed, and frightened by the information than individuals with a college degree or higher (P < 0.05). Eighteen percent (18%) of all respondents shared the feedback with their physician. The method of choice for receiving results was by letter with contact names and phone numbers., Conclusions: These findings indicate that research participants want feedback even when the information is upsetting. Additional studies are needed to identify individuals who experience greater levels of distress following feedback in an attempt to provide improved methods of feedback and support., (Copyright 2003 Wiley-Liss, Inc.)

We describe the psychological reactions after diagnosis and treatment of the malignant intraocular tumors: uveal melanoma and retinoblastoma. The chapter on uveal melanoma includes general consideration on the treatment of these tumors, the psychological effects on the patients, the professional and social problems, the follow-up after treatment and the results of recent studies on quality of life. For retinoblastoma we describe the treatments and results with the risk of second cancer, the follow-up of the patients, the psychological problems for parents and children and the specificity of the familial cases.

Retinoblastoma is a rare malignant tumour in the retina usually presenting before 3 years of age. In Sweden, 6-7 new cases are diagnosed each year. The medical treatment is highly specialized, very effective, with a survival ratio of almost 100%. Very little was known about the emotional and psychosocial effects on the parents and the patients themselves. This study includes all children diagnosed in Sweden between 1992 and 1994. The parents and children were included in our longitudinal support programme immediately after the diagnosis was made. This paper deals with the following topics: how the illness was detected and diagnosed, how the parents were handled by the health professionals, the parents' emotional reactions to the diagnosis and treatment and how the children reacted to the disease and treatment. Children's concepts of illness and acute loss are discussed.

This article describes the application of genetic testing of children for hereditary cancers and the resultant ethical and psychosocial implications. Basic cancer genetics concepts are reviewed. Specific hereditary cancers that may affect children are described along with case examples and recommendations for nursing practice.

Quality-of-life assessment is being used increasingly in clinical research. This is true particularly in the case of survivors of cancer in childhood, where improving survival rates have raised concern regarding the long-term effects of medical cure. Health-status assessment and quality-of-life instruments have been developed for the most part in the English language, thus necessitating their translation and cultural adaptation for use in non-English-speaking countries. Our purpose was to develop a set of Spanish-language questionnaires for application with a population of children with cancer in a tertiary-care center in Buenos Aires, Argentina. The Health Utilities Index (HUI), a conceptual framework for assessing health status, was chosen for this study. Three distinct questionnaires, based on the HUI, were used: a self-completed one for health professionals and teachers (15Q) to report assessments of children and 2 interviewer-administered ones, for child survivors (42Q) to report assessments about their own health status and parents (45Q) to report assessments about their children's health status. The original translations and reviews were accomplished with direct oversight by members of the HUI Group, to ensure conceptual equivalence. The instruments were then tested in Buenos Aires by application to staff of the hematology-oncology service, childhood cancer patients and the parents of childhood cancer patients. Several modifications were made based on these tests. We concluded that the translation and cultural adaptation of these instruments was adequate for use with the groups tested in a pilot survey of survivors of childhood cancer in Argentina., (Copyright 1999 Wiley-Liss, Inc.)