Psychosocial outcomes and quality of life among school-age survivors of retinoblastoma.

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 (Mage = 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.

Vision-related quality of life compared to generic measures in retinoblastoma survivors.

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.

The impact of monocular vision on motor function and quality of life in survivors of retinoblastoma.

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.

"Where Does it Come from?" Experiences Among Survivors and Parents of Children with Retinoblastoma in Kenya.

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.

Quality of Life Assessment in Retinoblastoma: A Cross-Sectional Study of 122 Survivors from India.

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.

'It's not meant to be for life, but it carries on': a qualitative investigation into the psychosocial needs of young retinoblastoma survivors.

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.

Quality of life of Retinoblastoma survivors in tertiary care eye hospital in South India.

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.

The coping experience of parents of a child with retinoblastoma-malignant eye cancer.

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.

Values of Retinoblastoma Survivors and Parents Regarding Treatment Outcomes: A Qualitative Study.

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.

Participation in daily activities and quality of life in survivors of 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.

The effect of non-pharmacological interventions on psychological stress and quality of life of parents of children with retinoblastoma: A protocol for systematic review and meta-analysis.

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.

Restrictions in daily life after retinoblastoma from the perspective of the survivors.

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.

A Review of Literature on Health-Related Quality of Life of Retinoblastoma Survivors.

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.

Behavioural functioning of retinoblastoma survivors.

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.

Coping strategies of retinoblastoma survivors in relation to behavioural problems.

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.

Depression, Anxiety, and Stress in Parents of Patients With Retinoblastoma.

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.

Mothers' explanations of communicating past health and future risks to survivors of childhood cancer.

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.

Knowledge, experiences and attitudes concerning genetics among retinoblastoma survivors and parents.

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.

Vision-Targeted Health-Related Quality of Life in Adult Survivors of Retinoblastoma.

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 (ß = -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.

Quality of life of adult retinoblastoma survivors in the Netherlands.

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.