"A glint or a squint should make you think!": towards the earlier diagnosis of retinoblastoma

Abstract

Background: Affecting one or both eyes, retinoblastoma is the most common intraocular neoplasm occurring in children under the age of 5 years. The most frequent presenting signs of retinoblastoma include leukocoria and strabismus, whilst symptoms of pain or discomfort generally only manifest with advanced intraocular or extraocular disease and systemic spread. Although the management of retinoblastoma is largely determined by the stage of disease at presentation, fewer treatment options are available with late diagnosis. Moreover, diagnostic delay is associated with negative outcomes, including severe vision impairment, enucleation or death. As the keenest observers of their children, parents or primary caregivers are the most likely to notice the earliest signs of retinoblastoma. However, in Australia, parents are not currently provided with any information, nor are there any specific strategies in place, to alert them to the early signs of not just retinoblastoma but any paediatric eye disease.

Purpose: The overall aim of this doctoral research project is to develop and evaluate the effectiveness of an evidence-based, theory-informed pamphlet to improve parents’ awareness of the early signs of significant paediatric eye disease. A mixed methods approach, including both qualitative and quantitative methods, was utilised to achieve this aim.

It is hypothesised that providing parents with paediatric eye health information will improve knowledge and change help-seeking intention.

Research Design and Methods: Aim 1: Develop a brief information pamphlet educating parents to recognise and respond to notable signs of significant paediatric eye disease

An exploratory qualitative study investigated and identified existing knowledge gaps and determined factors that influence parents’ help-seeking behaviour for ocular signs or symptoms that they may observe in their child. Using an inductive thematic approach, qualitative data were analysed to ascertain overarching themes and concepts. The qualitative data supported the development of a paediatric eye health awareness information pamphlet for parents, which was guided by established guidelines for writing health information and a theoretical framework for behaviour change.

Aim 2: Evaluate the information pamphlet A single-centre, double-blind randomised controlled trial (RCT) was conducted to evaluate the effectiveness of the pamphlet developed in Aim 1. Pregnant women’s awareness and understanding of early signs of paediatric eye disease and their hypothetical help-seeking intentions were measured using a pre-/post-test design.

Results: Aim 1: Development of the information pamphlet Analysis of evidence gathered from 18 parents during five focus groups informed the development of a theory-driven information pamphlet specifically addressing knowledge gaps that were identified, as well as barriers and enablers for parents to seek advice for paediatric eye disease. Using a theoretical framework for behaviour change, persuasive messages were designed by linking information obtained from the exploratory qualitative research study specifically aimed at improving knowledge, boosting motivation and prompting parental monitoring of ocular health and action if they observed eye signs of concern.

Aim 2: Evaluation of the information pamphlet A total of 518 women consented to participate in the clinical trial and were randomised following completion of the baseline survey. Of these, 395 women completed the follow-up survey, which was sent two weeks after baseline. Using multiple logistic regression analysis, compared to the control group, participants in the intervention group were more likely to be concerned if leukocoria was observed, (OR 1.711, [CI 1.176-2.497] p=0.005) and less likely to delay seeking help (OR 0.560, [0.382-0.817] p=0.003). Whilst participants in the intervention group were no more concerned if they observed strabismus than the control group, they were less likely to delay help-seeking (OR 0.318, [0.125-0.806] p=0.016) at follow-up.

Conclusion: Motivated by delayed diagnosis of retinoblastoma reported in the literature and my own clinical experience in Victoria, this doctoral study explored a potential strategy to minimise such delays in the broader context of paediatric eye disease. If parents are provided with relevant information, messages to motivate and guidance on behaviour skills, their knowledge and help-seeking intention if they observe leukocoria or strabismus can improve. Systematic dissemination of useful health information to parents may reduce diagnostic delay for retinoblastoma, or indeed other, more common paediatric eye diseases.