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.

Health-related quality of life of Brazilian children and adolescents with benign and malignant solid tumours: A prospective cohort study during the first year after hospital admission.

INTRODUCTION: This study aims to assess the impact of paediatric benign and malignant solid tumours and its treatment on the health-related quality of life of children and adolescents who were followed up in a Reference Center in Pediatric Oncology in Rio de Janeiro. METHODS: It is a prospective cohort study. Quality of life assessment was performed using the PedsQL™ 4.0 Generic Core Scales and PedsQL™ 3.0 Cancer Module protocols three times: during hospital admission (T1), 6 months after admission (T2) and 1 year after admission (T3). RESULTS: We evaluated 132 patients, 59 men and 73 women, aged 2-17 years. In PedsQL™4.0, the Emotional Functioning scale was the one with the worst scores, while the scores on the Social Functioning scale was the best. In PedsQL™ 3.0, the worst domains were Procedural Anxiety and Worry. Patients with malignant bone tumours had the worst health-related quality of life. The group who received only surgery had better results. Total scores of PedsQL™4.0 and PedsQL™ 3.0 improved between T1 and T3. CONCLUSION: Children and adolescents with malignant and benign neoplasms undergo changes in quality of life as a result of the disease and treatment, but an improvement has been observed over time.

Parental coping with retinoblastoma diagnosis.

OBJECTIVE: To characterize coping and distress among parents of children with retinoblastoma, and to uncover their association with perceived health literacy, self-efficacy, and social support. METHODS: This was a cross-sectional study performed in the retinoblastoma clinics of Beijing Children's Hospital, Jilin Eye Hospital and Changchun Hospital in China. Parents of children with retinoblastoma (n = 104) completed a print Mandarin language questionnaire consisting of four sections: (i) demographic information, (ii) mini-mental adjustment to cancer scale, (iii) hospital anxiety and depression scale, and (iv) perceived health literacy, self-efficacy, and social support scales. Scores were tabulated for each measure and analyzed by bivariate correlation. RESULTS: Moderate anxiety affected 59.2% of parents, and 77.7% experienced low, moderate, or high levels of depression. Combined anxiety and depression was positively correlated with helplessness/hopelessness (R = 0.42, p < .01) and anxious preoccupation (R = 0.247, p < .05), and negatively correlated with perceived self-efficacy (R = -0.228, p < .05). Perceived social support from a partner was negatively correlated with depression (R = -0.207, p < .05) and helplessness/hopelessness (R = -0.271, p < .01). CONCLUSIONS: Knowledge of how parents cope with their child's cancer diagnosis can help healthcare teams understand how best to support their psychosocial needs.

"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.

A longitudinal investigation of parenting stress in caregivers of children with retinoblastoma.

BACKGROUND: Retinoblastoma is typically diagnosed in young children and may present unique parenting challenges. Qualitative research suggests that parents experience distress related to the initial diagnosis and treatment that subsequently resolves. The objectives were to systematically assess parenting stress over time in parents of young children with retinoblastoma and to examine associations between parenting stress and child outcomes. PROCEDURES: Parents of children with retinoblastoma completed the Parenting Stress Index (PSI) during serial psychological assessments scheduled based on the child's age (6 months to 5 years). Caregivers of 92 patients (85.9% mothers) completed the assessments. Child outcomes included developmental functioning and parent-reported adaptive functioning. RESULTS: At baseline and age 5, all subscales on the PSI were within normal limits, and most were significantly below normative means (i.e., demonstrating low levels of stress). All domains remained relatively stable over time. Associations between parenting stress and child outcomes were much stronger at age 5 than at baseline. Child-directed parenting stress was a small but significant contributor to declines in child functioning over time. CONCLUSIONS: Parents of children with retinoblastoma report normal levels of parenting stress while their children are young. However, baseline parenting stress appears to contribute to changes in child functioning over time. Future studies should assess illness-related aspects of adjustment to further understand the parenting experience of young children with cancer and/or having a visually impaired child.

Female Sex, Bilateral Disease, Age Below 3 Years, and Apprehension for Enucleation Contribute to Treatment Abandonment in Retinoblastoma.

Treatment abandonment is a major obstacle for treating retinoblastoma in developing countries. The aim was to evaluate plausible causes, outcome, and rate of treatment abandonment in patients with retinoblastoma. The study was retrospective and conducted in a University hospital in North-India. Two cohorts of patients were studied. One was a larger cohort (n=602) of all patients with retinoblastoma in the institution from 2000 to 2014. Limited variables (age, sex, and laterality) were examined in this cohort due to incomplete data/lack of contact. A detailed interview was conducted with contactable caregivers in a smaller cohort (n=104). Noncompliance was observed in 170/602 (28%) patients. Patients below 3 years were more likely to be noncompliant (65.1%), compared with older patients (34.9%) (P=0.003). Compliance was greater among male individuals (64%) than in female individuals (36%) (P=0.017) and in those with unilateral disease (72%) compared with those who had bilateral disease (28%) (P=0.009). Apprehension for enucleation (64% vs. 8%, P=0.0001) and difficulty in attending outpatient services of different departments (61.4% vs. 13.3%, P=0.003) for multimodality treatment were expressed more frequently in the noncompliant than in the compliant group, respectively. The mortality in compliant and noncompliant patients was 7% and 68%, respectively (P=0.0001). The rate of treatment abandonment in 602 patients over a 15-year period was a sizable 28%. The factors contributing to noncompliance included female sex, bilateral disease, age below 3 years, apprehension for enucleation, and difficulty in attending outpatient services of different departments.

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.

Cognitive function and social attainment in adult survivors of retinoblastoma: a report from the St. Jude Lifetime Cohort Study.

BACKGROUND: Retinoblastoma has a 5-year survival rate exceeding 95%, yet little is known about long-term functional outcomes for these patients. METHODS: Sixty-nine adult survivors of retinoblastoma (mean age, 33 years; mean years post-diagnosis, 31) who had enrolled in the St. Jude Lifetime Cohort Study completed clinical cognitive evaluations and questionnaires assessing adult social attainment. Scores on all cognitive measures were converted to z-scores (M = 0, SD = 1) using age-adjusted normative data. Multivariable linear regression analyses, adjusted for age at diagnosis and disease laterality, were used to examine associations between disease and treatment exposures and cognitive outcomes. RESULTS: Retinoblastoma survivors performed within normative expectations across most cognitive domains. In multivariable models, adjusted for disease laterality, survivors diagnosed at ≤1 year of age performed significantly better on measures of short-term verbal memory (ß = 0.87, P<.01), long-term verbal memory (ß = 0.66, P = .02), verbal learning (ß = 0.67, P = .02), and verbal reasoning abilities (ß = 0.79, P<.01) compared with survivors diagnosed at >1 year of age. In multivariable models, restricted to bilateral survivors and adjusted for age at diagnosis, whole brain radiation exposure was significantly associated with poorer performance on tasks of short-term verbal memory (ß = -0.003, P = .03) and long-term verbal memory (ß = -0.003, P = .01). Reported social attainment was consistent with adult developmental expectations. CONCLUSIONS: Adult survivors of retinoblastoma demonstrate few cognitive or social attainment deficits decades following diagnosis and treatment. Findings suggest the potential for neural reorganization following early insult to the visual system as well as vulnerability of the developing brain to low dose radiation exposure. Early intervention and rehabilitation will be important for these patients.

Stigma in Pediatric Cancer: An Exploratory Study of Osteosarcoma and Retinoblastoma in Guatemala, Jordan, and Zimbabwe.

PURPOSE: Stigma is an understudied barrier to health care acceptance in pediatric oncology. We sought to explore the stigma experience, including its impact on cancer treatment decision making, and identify strategies to mitigate stigma for patients with osteosarcoma and retinoblastoma in Guatemala, Jordan, and Zimbabwe. METHODS: Participants included caregivers, adolescent patients (age 12-19 years), and health care clinicians. A semistructured interview guide based on The Health Stigma and Discrimination Framework (HSDF) was adapted for use at each site. Interviews were conducted in English, Spanish, Arabic, or Shona, audio-recorded, translated, and transcribed. Thematic analysis focused on stigma practices, experiences, outcomes, drivers, mitigators, and interventions. RESULTS: We conducted 56 interviews (28 caregivers, 19 health care clinicians, nine patients; 20 in Guatemala, 21 in Jordan, 15 in Zimbabwe). Major themes were organized into categories used to adapt the HSDF to global pediatric cancer care. Themes were described similarly across all sites, ages, and diagnoses, with specific cultural nuances noted. Pediatric cancer stigma was depicted as an isolating and emotional experience beginning at diagnosis and including internalized and associative stigma. Stigma affected decision making and contributed to negative outcomes including delayed diagnosis, treatment abandonment, regret, and psychosocial fragility. Overcoming stigma led to positive outcomes including resilience, treatment adherence, pride, and advocacy. Identified stigma drivers and mitigators were linked to potential interventions. CONCLUSION: Participants describe a shared stigma experience that transcends geography, cultural context, age, and diagnosis. Stigma manifestations have the potential to impact medical decision making and affect long-term psychological outcomes. Stigma assessment tools and interventions aimed at stigma mitigation including educational initiatives and support groups specific to pediatric cancer should be the focus of future research.

Factors influencing parental fatigue in children with retinoblastoma based on the unpleasant symptoms theory.

Parents of children with retinoblastoma, the most common primary ocular malignant tumour in childhood, bear a heavy caregiving burden and are very susceptible to fatigue. However, little is known about their current status of fatigue and factors influencing fatigue; therefore,this study, based on the theory of unpleasant symptoms, and included 317 parents of children with retinoblastoma in China, whose general demographic data were collected, and on whom the fatigue severity scale (FSS), the generalized anxiety disorder (GAD-7) and 2-item patient health questionnaire (PHQ-2) were administered, from 9 March to 1 June 2020. The parents' FSS score and fatigue incidence were 4.41 ± 1.14 and 67.19%, respectively. The fatigue level was positively correlated with anxiety, depression, education, times of hospitalisations, and treatment types (r = 0.125-0.468, P < 0.05) and negatively correlated with health status, sleep quality, economic status, and family economic situation (r = - 0.120 to - 0.322, P < 0.05). Parent's anxiety level, being an only child and female sex of child influenced parents' fatigue. Healthcare personnel need to focus their attention on this high-risk fatigue group and implement appropriate interventions to reduce their fatigue level, promote their physical and mental health, and facilitate better care for children.

'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.