Delayed diagnosis of congenital cataract in preterm infants: Findings from the IoLunder2 cohort study

BACKGROUND AND OBJECTIVES: Early detection is critical to achieving optimal outcomes in children with congenital cataract. We hypothesized that detection of congenital cataract in preterm infants would be delayed compared with term/post-term peers due to delayed delivery of whole population child health interventions. METHODS: Secondary analysis of data using a nested case-control study approach in a prospective population-based cohort study. Inclusion criteria comprised children diagnosed with congenital cataract requiring surgical intervention during the first two years of life in UK and Ireland in 2009 and 2010. Association between late detection (after eight weeks post-natal age, ie outside the neonatal and infant eye national screening programme) of cataract and preterm birth (gestational age less than 37 weeks) was assessed using multivariable logistic regression. RESULTS: Of 186 children with congenital cataract, 17 children were born preterm (9%, gestational age range 24–37weeks). Neonatal detection occurred in 64/186 (34%), and late detection in 64 children (34%). Late detection was independently associated with premature birth, specifically moderate/late preterm birth (adjusted odds ratio 3.0, 95%CI 1.1 to 8.5). CONCLUSIONS: Our findings suggest that, despite enhanced eye surveillance being recommended for those born moderate/late preterm (32+ weeks gestational age, ie not eligible for retinopathy of prematurity screening), congenital cataract is not being effectively detected through the routine screening programme for this vulnerable group. It is necessary to improve the effectiveness of the screening programme, and care must be taken to ensure that competing health care needs of preterm children do not prevent universal child health interventions

The role of screening and surveillance in the detection of childhood vision impairment and blindness in the UK

Objective: Understanding pathways to detection for childhood visual impairment (VI) is critical for planning services. We aimed to describe patterns of detection for childhood VI. // Design: and setting Cross-sectional study using data from British Childhood Visual Impairment and Blindness Study 2. // Patients: Children newly diagnosed with VI, severe vision impairment or blindness (SVI/BL)—that is, visual acuity worse than logMAR 0.5 in both eyes—were identified through active surveillance, with data collection at diagnosis and 1 year later. Outcome: measure Method of detection of vision/eyes problem. // Results: 784 children (45%, 356 girls) were identified, of whom 313 (40%) had VI, 471 (60%) had SVI/BL. Additional non-ophthalmic disorders or impairments (VI/SVI/BL ‘plus’), were diagnosed in 72% (559/784). Of the 784, 173 children were detected through routine screening (22%), 248 through targeted examinations (32%) and 280 through family self-referral (36%). Parents and carers had only reported symptoms in 55% of children who manifested them, with evidence that families living in socioeconomically deprived areas were less likely to report concerns. Paediatricians were the professionals most likely to raise initial suspicion of visual disability. // Conclusions: Our findings show that targeted screening and surveillance is important for the detection of full spectrum childhood visual impairment (VI/SVI/BL), as a significant proportion of children will not have symptoms, or their parents or carers will not report symptoms. As paediatricians were the professionals most commonly involved in detection, it would be helpful if their core competencies included the skills needed to undertake simple assessments of vision

Vision-related quality of life and mental health outcomes of children and young people with visual impairment and their carers [ISPOR Abstracts 07 May 2023 - 10 May 2023]

Objectives To investigate the associations between vision-related quality of life (VQoL) and mental health outcomes of children and young people with visual impairment (CYP-VI) and their parents/carers, as a first step towards development of family-centred intervention. Methods Cross-sectional study included 68 CYP-VI aged 8-18 years (visual acuity of logMAR 0.50 or worse, comprising moderate and severe visual impairment and blindness in ICD-11) and their carers. Families were recruited through two paediatric ophthalmology departments and relevant vision loss charities in the United Kingdom. Children’s outcomes included the overall scores on Vision-Related Quality of Life Questionnaire for Children and Young People (VQoL_CYP) as reported by the CYP-VI themselves, and Strengths and Difficulties Questionnaire (SDQ) as reported by their carers. Carers’ outcomes were overall scores on Satisfaction With Life Scale (SWLS), Patient Health Questionnaire (PHQ-9), General Anxiety Disorder Assessment (GAD-7), and Parental Stress Scale (PSS). Associations between children’s and carers’ outcomes were analysed using Spearman’s correlation coefficient. Results Lower VQoL was associated with more overall behavioural and emotional difficulties on SDQ (r=–0.485, p<0.001), lower carer’s satisfaction with life (r=0.395, p=0.002), worse carer’s depression (r=–0.390, p=0.002) and anxiety symptoms (r=–0.315, p=0.015). VQoL was not significantly associated with parental stress levels (r=–0.196, p=0.140). More overall child’s behavioural and emotional difficulties significantly correlated with higher parental stress (r=0.363, p=0.004),worse carer’s depression (r=0.436, p<0.001) and anxiety symptoms (r=0.422, p<0.001), but not with their satisfaction with life (r=–0.159, p=0.224). Visual acuity and gender were not related with any of the measured outcomes. Older age was associated with lower VQoL (r=–0.320, p=0.010). Conclusions Our findings show moderate correlations between mental health of CYP-VI and their carers, and indicate both should be considered when assessing VQoL outcomes of CYP-VI. Interventions targeting mental health of these families may promote better VQoL of CYP-VI

Effect of impaired vision on physical activity from childhood to adolescence

International physical activity (PA) guidelines are set irrespective of disabilities. Yet the levels of and changes in PA across transition from childhood into adolescence among those with impaired vision are not well understood due to the challenges of longitudinal population-based studies of rare conditions. Our study investigated whether children and adolescents with impaired vision can achieve PA levels equivalent to those without impaired vision

Do adolescents with impaired vision have different intentions and ambitions for their education, career and social outcomes compared to their peers? Findings from the Millennium Cohort Study

BACKGROUND/AIMS: To investigate if impaired vision adversely impacts the intentions/ambitions of adolescents concerning their future education, careers and social outcomes. METHODS: Population-based birth cohort study in the UK comprising 9273 participants from the Millennium Cohort Study who were followed up to age 17 years. Children were classified as having normal vision or unilateral or bilateral impaired vision caused by significant eye conditions based on detailed parental-structured questionnaire data on sight problems and treatment coded by clinicians. Ten domains covering education, career and social outcomes by age 30 were investigated. RESULTS: Adjusted regression models showed few differences by vision status. Bilateral impaired vision was associated with increased odds of intending to remain in full-time education after statutory school age (adjusted OR (aOR) 2.00, 95% CI 1.08 to 3.68) and of home ownership at age 30 (aOR 1.83, 95% CI 1.01 to 3.32). Impaired vision was not associated with intending to attend university. A significantly higher proportion of parents of children with bilateral or unilateral impaired vision thought that their child would not get the exam grades required to go to university than parents of those with normal vision (29% or 26% vs 16%, p=0.026). CONCLUSION: Adolescents with impaired vision have broadly the same intentions/ambitions regarding future education, careers and social outcomes as their peers with normal vision. The known significant gaps in attainment in these domains among young adults with vision impairment are therefore likely to be due to barriers that they face in achieving their ambitions. Improved implementation of existing interventions is necessary to ensure equality of opportunities

This is me: A qualitative investigation of young people’s experience of growing up with visual impairment

Background: Childhood visual impairment (VI) has a profound impact on many aspects of childhood and adolescence. This is well-documented in cross-sectional and/or quantitative studies utilizing self-report instruments which compare children with and without VI. Young people’s views on the experience of growing up with VI as a developmental, change-driven process remain largely unexplored. Methods: As part of our broader research programme on quality of life of visually impaired children and young people in the United Kingdom, in-depth, semi-structured interviews were conducted between March and June 2015, with a stratified sample of 17 young people with VI, aged 16–19 years. An age-sensitive, empirically-based topic guide encouraged retrospective reflections on participants’ experiences of growing up with VI, including age-normative and vision-specific challenges. Results: Descriptions of growing up with VI largely centered on an overarching higher-order theme labelled becoming me. Four themes representing everyday activities, attitudes, preferences and perceptions in relation to i) social relationships, ii) independence and responsibilities, iii) the future, and iv) rising to challenges emerged and were used by participants in their description of three stages in which they developed a sense of self: i) laying the foundations, ii) testing the waters, and iii) this is me. Differences in manifestation of VI influenced how young people made sense of their experiences and their sense of self. Conclusions: Findings are discussed in relation to normative and vision-specific changes in psychosocial development during adolescence, including the development of identity. They highlight the need for ongoing monitoring of subjective well-being in a clinical population with a unique early life course trajectory

Avoidable childhood blindness in a high-income country: findings from the British Childhood Visual Impairment and Blindness Study 2

BACKGROUND/AIMS: Addressing childhood visual disability is an international priority, with data on causes needed to plan, implement and evaluate public health and clinical care. We have examined the contribution of ‘avoidable’ blinding disorders to childhood visual impairment, severe visual impairment and blindness (VI/SVIBL) in the UK. METHODS: National prospective observational longitudinal study, the British Childhood Visual Impairment and Blindness Study 2 (BCVIS2), of children (aged 18 years or under) newly diagnosed with vision worse than 0.48 logMAR (logarithm of the minimum angle of resolution) or equivalent in both eyes. Proportions of children with an ‘avoidable’ disorder comprising either apotentially preventable(isolated disorders with an effective intervention which reduces disease incidence) ortreatable(isolated eye or vision disorders for which there are routinely available effective interventions able to improve vision or halt progressive visual loss) are reported. RESULTS: Of the 784 children within BCVIS2, isolated potentially preventable disorders were present in only 17% (132/784) and treatable disorders in an additional 13% (99/784). The most common treatable causes were cataract, retinopathy of prematurity and glaucoma. Of the 132 children with potentially preventable disease, 64 had hypoxic–ischaemic encephalopathy. Non-accidental injury accounted for almost two-thirds (11/16, 69%) of those with VI/SVIBL due to injury. CONCLUSION: Despite significant progress in the past decades in high-income countries, there remains a need to be vigilant about implementing preventive strategies and treatments. Attention to disorders that are currently neither preventable nor treatable remains a priority in these settings and will become increasingly important in lower-income and middle-income countries undergoing economic transition

Trends in the long-term impact of childhood visual impairment on health and social outcomes in the UK: a cross-cohort study across three decades of disability-related legislation and policy implementation

BACKGROUND: Childhood vision impairment (VI) can adversely impact health and social outcomes and limit life chances. We investigated whether its adverse impacts into adult life changed during a period in which legislation, policy and services to address inequalities relating to disability were implemented. METHODS: Cross-cohort study comprising 14 247 participants from the 1946, 1958 and 1970 British birth cohorts (BC). Participants dichotomized as VI at age 15/16 (distance visual acuity was 6/12 or worse in the better-seeing eye) or normally sighted. Associations of childhood VI with health, well-being, socioeconomic and social participation outcomes in mid-adult life were investigated using regression models adjusted for participants' early life socioeconomic markers and sex. Change in adjusted odds ratios of >10% in the same direction in successive cohorts, or a > 20% difference between 1970BC and one older cohort were considered meaningful. RESULTS: Trends over time in impacts of childhood onset VI into mid-adult life were complex. This included worsening of odds of poorer physical health (odds ratio 1.47; 95% confidence interval 1.02-2.14), living in unsatisfactory (1.54; 1.03-2.29) or overcrowded (2.34; 1.26-4.06) households, being unemployed (2.19; 1.19-3.97) and not gaining additional educational qualifications during mid-adult life (1.61; 1.08-2.47). By contrast the odds of not participating in some social activities (e.g. seeing friends) improved over time. Associations with other outcomes were unchanged. CONCLUSIONS: Many adverse impacts of childhood VI do not appear ameliorated over time by legislation, policies and provision that would have been expected to reduce inequalities. Moreover, some were increased. Childhood VI continues to cast a life-long shadow

Data saves lives: optimising routinely collected clinical data for rare disease research

Necessity driven organisational change in the post-pandemic landscape has seen health care providers adopting innovations to manage and process health data. These include the use of ‘real-world’ datasets of routinely collected clinical information, enabling data-driven delivery. Rare disease risks being ‘left-behind’ unless our clinical and research communities engage with the challenges and opportunities afforded by the burgeoning field of health data informatics. We address the challenges to the meaningful use and reuse of rare disease data, and, through a series of recommendations around workforce education, harmonisation of taxonomy, and ensuring an inclusive health data environment, we highlight the role that those who manage rare disease must play in addressing them

Determinants of vision-related quality of life of children and young people with visual impairment

Purpose : To investigate the broader individual, family, and environmental factors associated with vision-related quality of life (VQoL) of children and young people with visual impairment (CYP-VI), in order to identify modifiable factors that could be targets for intervention. Methods : Cross-sectional study included 152 CYP-VI aged 7-18 years (visual acuity of logMAR 0.5 or worse, comprising moderate and severe visual impairment and blindness in ICD11) attending 22 Paediatric Ophthalmology Departments in the United Kingdom who participated in the final phase of our programme to develop two vision-specific patient-reported outcome measures (PROMs), Vision-Related Quality of Life (VQoL_CYP) and Functional Vision (FV_CYP) Questionnaires for Children and Young People. CYP-VI self-completed the two PROMs. Associations with family, sociodemographic characteristics (reported by their carers), and clinical factors (derived from their clinical records) were analysed using Spearman’s correlation coefficient, Kruskal-Wallis, Wilcoxon rank-sum tests and quantile regression models. Results : The median VQoL score was 55.58 (IQR=12.06, min=17.08, max=100.00), where higher scores indicate better VQoL. Better VQoL was associated with better FV overall (r=–0.516, p<0.001), parent-reported absence of additional chronic conditions (p=0.010, dCohen=0.457), attending mainstream (versus other) school (p=0.013, dCohen=0.439), higher socio-economic status (r=0.173, p=0.038) and higher parental education level (r=0.201, p=0.018). Other child (gender, ethnicity), clinical (severity of visual impairment, onset of vision loss, rate of vision deterioration), and family (parent’s age, ethnicity, employment status; number of siblings, birth order; siblings and parent’s vision and medical status; housing tenure, cars owned) were not associated with VQoL scores. Final quantile regression model included functional vision (FV) scores and presence of additional health condition. Conclusions : Both clinical characteristics as well as non-health-related factors should be considered when assessing vision-specific outcomes of CYP-VI. Our findings and further research on other potential determinants of VQoL are useful to develop recommendations and interventions to promote better VQoL and to support healthcare professionals to provide more personalised approach towards their patients’ care