Development of a Patient-Centered Pathway of Care for Retinoblastoma Patients: A Mixed-Methods Pilot Study

Retinoblastoma, a curable childhood eye cancer, is commonly diagnosed before the age of two [1]. About half of all retinoblastoma survivors carry a germline RB1 pathogenic variant. There is a 50% chance of passing germline RB1 pathogenic variants onto offspring and these variants are associated with a predisposition for second cancers later in life [1].

Retinoblastoma treatment and follow-up are complex, often spanning several years, and are unique for each child. Patients undergo repeat eye examinations under anesthesia to treat active disease and monitor for recurrence. As disease is controlled and children grow older, they are transitioned to awake clinic visits and are monitored annually. Currently, there is no uniformity in how patients receive information about their care and follow-up. Families may obtain information through discussion with their healthcare providers or tangible resources while others do their own research to learn about retinoblastoma management and what to expect [2].

The Canadian Retinoblastoma Research Advisory Board (CRRAB) [3, 4] – composed of patients (defined as individuals with lived experience of retinoblastoma, including informal caregivers [5]), researchers, health professionals, and policy makers – was established to enhance opportunities for meaningful patient engagement in retinoblastoma research. To support the activities of CRRAB, we developed an online research registry to identify patients “beyond the usual suspects” across Canada [6]. A major initiative of CRRAB was to determine the top 10 retinoblastoma research priorities in Canada [2]. One of the identified retinoblastoma research priorities was, “How to provide a detailed pathway of care or plan, outlining treatment and follow-up, to retinoblastoma patients and families?” [2].

Pathways of care have been shown to enhance quality of care, improve patient outcomes, promote patient safety, increase patient satisfaction, and optimize the use of healthcare resources [7]. The Get Well Map [8] is an example of a child-friendly pathway of care that is used to help visualize treatment, follow-up, and associated milestones. Consisting of a 12″ × 18″ illustrated board depicting a roadmap or timeline, patients apply stickers to mark each stage of the treatment and follow-up in real time. The end of the roadmap or timeline is marked by a goal, chosen by the patient (e.g., finish chemotherapy). Once the goal is achieved, a new goal can be set, and tracking of the pathway continues. Get Well Maps have been used for a variety of conditions, and aim to reduce stress and anxiety, improve clarity for families, and promote effective communication with healthcare teams [8]. We hypothesized that adapting the Get Well Map for use in retinoblastoma, might improve physician-patient communication, self-efficacy, anxiety, depression, physician-patient interaction, and potentially reveal additional uses and benefits (e.g., facilitate communication between parent and child, serve as a keepsake).

The purpose of this study was to adapt the Get Well Map for retinoblastoma, by partnering with patients at every stage. Here we describe the process of co-designing, with patients, researchers and health professionals, a pathway of care called the “Retinoblastoma Journey Map,” and the results of its evaluation among parents of newly diagnosed children.

The first aim of the study was to co-create with patients, researchers, and health professionals, a pathway of care for retinoblastoma (Retinoblastoma Journey Map). The second aim was to evaluate the feasibility, acceptability, usability and perceived impact of the Retinoblastoma Journey Map among parents of children newly diagnosed with retinoblastoma.

The Retinoblastoma Journey Map was developed as a clinical innovation and evaluated as a quality improvement project at the Hospital for Sick Children (Toronto, Canada), thus was exempt from research ethics board approval. The project was aligned with the Guidance for Reporting Involvement of Patients and the Public (GRIPP) 2 short form (online suppl. Information File 1; for all online suppl. material, see https://doi.org/10.1159/000540055) [9] and the Consolidated Criteria for Reporting Qualitative Research (COREQ) 32 item checklist (online suppl. Information File 2) [10].

The adaptation of the Get Well Map was coordinated, implemented, and documented by a Parent in Research, a member of the team with lived experience as a mother of a child with retinoblastoma (I.R.). CRRAB is co-led by the Parent in Research and a research scientist with expertise in retinoblastoma (H.D.).

A working group was developed by recruiting CRRAB members. Patients, researchers and health professionals were selected to represent a diverse range of lived experience of retinoblastoma and professional expertise (i.e., research methods, oncology, ophthalmology).

Using human-centered design methods [11], working group members ideated, prototyped, and refined the Retinoblastoma Journey Map (Fig. 1).

The Get Well Map Foundation donated Get Well Maps and the working group co-designed stickers that could be used to customize a pathway of care. Working group members were sent a toolkit created by the Parent in Research and asked to generate ideas for 5–10 stickers. The Parent in Research led a brainstorming session, grouped the sticker ideas into categories and then ranked the stickers by frequency.

An in-person workshop for the working group members was held in conjunction with a CRRAB annual general meeting to develop 10 prototype stickers. Working group members were provided with the ranked list of potential sticker ideas. The Parent in Research facilitated a discussion about the stickers, and additional items were suggested. Working group members worked in pairs to generate sticker prototypes. The prototypes were shared with and discussed by the group. Stickers were sketched in real time by an illustrator and decisions on purpose, design, and language were decided upon by group vote. A Child Life Specialist (M.L.) shared designs with children attending a Child Life program running in parallel with the workshop, allowing young survivors to provide input on sticker designs. One young survivor chose to remain in the main working group session, offering suggestions on language and style (Fig. 1).

Participants contemplated how the Get Well Map could be used and its intended impact (i.e., physician-patient communication, knowledge, etc.). These insights were collected to inform pilot testing of the Get Well Map.

The first set of prototype stickers were circulated online to the working group members using an online survey tool which collected feedback. The feedback was used to guide the illustrator revisions. In a second round of refinement, working group members provided feedback on sticker clarity, design, descriptions and intended use.

Feedback from the rapid prototyping phase was used to draft and refine additional documents to accompany the Get Well Map and sticker set. Validated questionnaires were selected that aligned with the suggestions for potential impact of the Get Well Map. Patient working group members were sent the final intervention package and validated questionnaires from the Parent in Research, they provided feedback and final revisions were made.

Recruitment for the evaluation of the Retinoblastoma Journey Map began in October 2020 on a rolling basis using purposive sampling. No sample size was calculated; as a pilot project aiming for rapid feedback, the team aimed for 10–15 participants. Participants were eligible if they were (1) parents or primary caregivers of children with retinoblastoma within 2 years of diagnosis and (2) receiving care at SickKids. The Parent in Research met with interested participants to demonstrate the Retinoblastoma Journey Map and explain the purpose of the evaluation (Fig. 2).

Parents or caregivers who agreed to participate received a package consisting of the Retinoblastoma Journey Map, a full set of stickers, a feedback journal, and instructions.

A mixed-methods approach was used to evaluate the Retinoblastoma Journey Map (Fig. 2). Impact of the Retinoblastoma Journey Map was measured at baseline and at 6 months using the Distress Thermometer (DT) [12], Hospital Anxiety and Depression Scale (HADS) [13], Communication and Attitudinal Self-Efficacy scale for cancer (CASE-cancer) [14], and Questionnaire of the Quality of Physician-Patient Interaction (QQPPI) [15]. Feasibility, acceptability, and usability were evaluated at 6 months with the Feasibility of Intervention Measure (FIM) [16], Acceptability of Intervention Measure (AIM) [16], and System Usability Scale (SUS) [17]. All questionnaires were administered online via REDCap [18, 19].

Qualitative data on the same measures was collected via semi-structured interviews at 6 months. Interviews were conducted by the Parent in Research online over Zoom and recorded with audio consent; a team member (M.A.) attended to record field notes. Participants were asked to refer to their journals to answer questions. Some participants submitted photographs of their Retinoblastoma Journey Map to provide further insight on its use and impact. Interviews lasted 30–60 min. No repeat interviews were conducted. Data saturation was not a goal of the evaluation, as the aim was to interview all those who utilized the map.

Quantitative data was analyzed by summary statistics. Interviews were transcribed in full, uploaded into NVivo QSR 12 [20] and analyzed by inductive thematic analysis [21]. Phenomenology was the methodological orientation used to underpin the evaluation. The interview transcripts were not returned to participants for comments and corrections. A pre-determined list of codes was created by reviewing the transcripts and noting repeating concepts and patterns. Two members of the team (I.R. and H.D.) coded 30% of the transcripts; all novel codes and disagreements were discussed until consensus was reached. The remaining transcripts were analyzed by a single coder (I.R.). Codes were grouped and sorted to reveal higher order themes. The list of themes with representative quotes was shared with working group members and discussed during a virtual workshop to verify the credibility of the conclusions.

The working group that created the sticker prototypes was led by the Parent in Research and additionally included 19 individuals: 10 patients (4 survivors, 6 parents), 8 health professionals (2 trainees), 1 researcher. The workshop was observed by 2 representatives from the Get Well Map Foundation. The working group that performed the data analysis was led by the Parent in Research and Scientist, and additionally included 2 health professionals and 2 patients (1 survivor, 1 parent).

A set of 25 stickers was developed, covering 3 themes: clinical treatment (13), medical education (1), and milestones (11) (Fig. 3). Consensus opinion was that clinical treatment stickers should match the visual treatment timeline used on a retinoblastoma electronic medical record [22], since the Canadian retinoblastoma community was expected to be familiar with those symbols. The one medical education sticker marked knowledge of genetic testing and counseling results. Much of the discussion centered around developing milestone stickers. Parent working group members were most vocal about using these stickers to celebrate achievements, whereas survivor working group members were keen to include stickers about connection and community. The youngest survivors suggested child-friendly language, new concepts, and design elements that reflected their unique lived experience (Box 1). The prototyping and refinement stages also led to the development of a sticker legend to accompany the Get Well Map and sticker set, as well as a set of instructions in both a video and print format.

Workshop participants suggested that the potential impact of the Map could be improved physician-patient communication and quality, reduced distress and anxiety. It was also suggested that implementation measures of feasibility, acceptability, and usability be measured. These suggested concepts were matched to existing validated questionnaires and an interview guide to be used in the evaluation (as described in section 2.4.3). A feedback journal was devised as an additional data collection method, to capture participant insights while using the Map.

A total of 25 patients were identified. Of these, 6 were not eligible (1 language barrier; 5 referred elsewhere for follow-up) and we attempted to contact the parents/caregivers of the remaining 19. Of these, 5 declined to participate (2 were too busy; 1 felt their child was the wrong age to benefit; 2 gave no reason), and 2 were unreachable. Of the 12 patients whose parents/caregivers agreed to participate (63% participation rate), 8 completed the full set of questionnaires (8/12, 67% completion rate), and 7 completed an interview (7/12, 58% completion rate). Interview data were supplemented with additional qualitative data obtained from written feedback journals provided by 8 participants, and photographs of final Retinoblastoma Journey Maps from 2 participants. Participant demographics are shown in Table 1.

Pre-and-post-test scores. Pre-test scores for distress were in the high range for 50% of study participants, and decreased to 38% in the post-test. The most common items reported in the problem list were “worry” (5/8) and “fears” (4/8). Depression scores were within normal limits for all participants in pre- and post-tests. Anxiety scores were within normal limits for 50% of participants during the pre-test and increased to 88% at post-test. Participants generally scored high on the 3 subscales of CASE-cancer (i.e., ability to seek and obtain information, understand and participate in care, and maintain positive attitude) with little fluctuation in the post-test. Mean participant scores for the QQPPI were similarly high, with a mean score of 65/70 in the pre-test and 66/70 in the post-test. Pre- and post-test scores are shown in Table 2.

Feasibility, Acceptability and Usability. At the project completion, the average score on the SUS was 77.5. FIM and AIM were rated on average 4.3/5 (Table 2).

Analysis of participant discussions and written feedback resulted in the identification of 6 themes: (1) Primary Use; (2) Challenges; (3) Impact; (4) Limitations; (5) Feasibility, Acceptability, and Usability; and (6) Unmet Needs (Table 3, with supporting quotes).

Primary Use. The primary way participants reported using the Retinoblastoma Journey Map was to communicate with their child about their retinoblastoma journey; they thought it could serve as a tool to facilitate communication with their child in future. Some families used it to communicate aspects of retinoblastoma with family and friends. Another use was as a visualization tool to prepare for the end of treatment journey and intervening steps.

Challenges. The main challenges in using the Map were related to the timing of introduction of the Map. Some families felt that the stage in which they had been introduced to the Map was too late and would have preferred it closer to diagnosis. Others felt their child was too young at that time to benefit from the Map. The launch of the project during the onset of the pandemic was another challenge, with some families finding it difficult to focus on using the tool or bringing it to clinic.

Impact. Perceived benefits to using the Map were the psychosocial impacts, primarily helping users to stay positive and reduce stress. Some participants indicated increased confidence, particularly in their understanding of the treatment pathway and their child’s confidence in their diagnosis. Another perceived impact was that the Map was seen to support recollection of treatment and served as a written record of their child’s experience.

Limitations. The Map proved to be of limited use in recording a detailed account of the treatment journey; some participants indicated their level of required knowledge went above what the Map could cater to. Others felt that while the Map might alleviate some stress, the greater mental health needs of caregivers required other interventions. Similarly, participants often felt that their physician-patient interaction was quite good, and the Map was not necessarily a factor that improved that relationship.

Feasibility, Acceptability, and Usability. The Map was feasible for use as a tool to discuss retinoblastoma with the family, but not with members of the clinical team, mainly due to its size. The Map was acceptable, with participants noting that the stickers, graphic design choices and supporting instructional package supported its use. However, aspects of the Map that were not acceptable were its lack of portability and ability to tailor to their own unique experiences including having more space for additional stickers. In terms of usability, the participants pointed to the supplementary package with instructions that made it easy to use. However, they found that it was difficult to recall events from memory to complete the Map.

Unmet Needs. Participants indicated that the Map did not meet their need for supportive care elements of the retinoblastoma journey (e.g., community support groups, social work). Others indicated that they preferred a way to take more detailed notes on the retinoblastoma journey. An additional suggestion was to turn the Map into a tool that could be somewhat tailored and predictive according to what a family might expect, perhaps by adapting the Map into an electronic resource. Finally, the participants preferred to personalize the Map, for example to create their own stickers or language.

We describe the patient-partnered development and evaluation of a pathway of care for families affected by retinoblastoma. The “Retinoblastoma Journey Map” uniquely documented treatment, achievements, and educational milestones that patients encounter during their care.

Initially anticipated to serve as a tool to mark treatment progress in real time, potentially improving physician-patient communication during medical visits, the Map’s primary use became “legacy building,” capturing the journey for future communication between parent and child. While there were some minor challenges due to its size and lack of portability, enhanced during the peak phase of the COVID-19 pandemic, it was not used because parents preferred to ask more complex questions about treatment, and desired more detailed information than what could be recorded on the Map. Prior research has shown that parents seek guidance on how to communicate about childhood cancer with their children [23, 24]; the Retinoblastoma Journey Map appears to be a tool that may aid in this area.

Overall, the Retinoblastoma Journey Map was found to be acceptable and useable, and qualitative feedback indicated that some participants thought it helped reduce stress. Quantitative data indicated that anxiety scores were on average lower on the post-tests, but this also might be expected as parental anxiety is anticipated to ease with time. No effects on depression, self-efficacy, or quality of physician-patient interaction were observed (however, all participants were within normal limits on the depression scale, and most scored in the high range for self-efficacy and quality of physician-patient interaction at evaluation onset).

This project was novel in that patient input influenced the Map’s design and project design stemming from its identification as a Top 10 retinoblastoma research priority [2]. CRRAB’s structure was instrumental in facilitating broad patient and non-patient collaboration. The Parent in Research role bridged the gap between patient and professional communities by encouraging reflection and incorporation of lived experience alongside clinical and scientific approaches [25, 26]. Human-centered design encouraged creativity and engagement, and facilitated the inclusion the youngest survivors, resulting in the elicitation of their unique insights (Box 1). The CRRAB group has experience using this approach, in development of the Retinoblastoma Research and You! booklet, a resource that guides patients in research involvement [25]; however, this was the first time in CRRAB’s history that young children had the opportunity to engage, advise and collaborate.

Involving patients in the analysis of the results led to interesting discussions for next steps for the Retinoblastoma Journey Map. One idea was to develop a guided activity to be used at a future Retinoblastoma Research Symposium, where patients could populate them alongside health professionals who are part of their circle of care, potentially facilitating quality physician-patient communication, or resulting in the elicitation of additional perspectives and new uses. While the project was successful in engaging patients broadly during the development and analysis of the Map, recruitment and enrollment was primarily led by a single patient (the Parent in Research). Additional patient involvement at this stage of the project may have assisted in an increased enrollment and retention rates in the project.

What the Retinoblastoma Journey Map was unable to achieve was to provide a more detailed and informative pathway of care, tailored to their child’s diagnosis. Some discussions centered around developing a pathway of care with predictive abilities (i.e., to help families understand all possible next steps in their child’s care plan based on their treatment and response history). The desire for such a feature may be reflective of the unpredictability of retinoblastoma; families are challenged with sudden changes in treatment and the uncertainty of attaining desired outcomes (e.g., vision or eye salvage). Communication about retinoblastoma care plans should ideally acknowledge this unpredictability and help families understand factors contributing to uncertain outcomes.

Some participants indicated that digitizing the Map or linking it to an electronic medical record could better meet patient informational needs. Studies on other childhood cancers suggest informational electronic resources should focus information to help patients with decision making and on how to access psychosocial support resources [27]. It is possible that the retinoblastoma-specific electronic medical record, DEPICT Health [22], could provide this functionality in future.

Patient involvement in co-design, implementation and evaluation was instrumental in producing the Retinoblastoma Journey Map, a patient-centered tool with a role in educating children and extended families about retinoblastoma through legacy building. The need remains for a more detailed pathway of care to empower patients to understand and successfully navigate the more challenging and complex details of retinoblastoma management.

We thank the Get Well Map Foundation for gifting the Maps used in this project and Sumreen Siddiqui for offering invaluable insights. We thank the participants of the 2023 Retinoblastoma Research Symposium Priority #9 research workshop for contributing to the development of the plain language summary.

The Retinoblastoma Journey Map was developed as a clinical innovation and evaluated as a quality improvement project at the Hospital for Sick Children (Toronto, Canada). As such, the Hospital for Sick Children Research Ethics Board granted an exemption from ethical review and written informed consent was not required from participants.

The authors declare that they have no conflict of interests. All authors have seen and agree with the contents of the manuscript and there is no financial interest to report.

Funding was provided by the SickKids Garron Family Cancer Clinical Innovation Fund and the University of Toronto Department of Ophthalmology and Vision Sciences Quality Improvement Fund.

Conception and design of work: Helen Dimaras and Ivana Ristevski. Drafting the work: Ivana Ristevski. Acquisition, analysis, or interpretation of data; revising the work critically for important intellectual content, final approval of the version to be published, and agreement to be accountable for all aspects of the work: Ivana Ristevski, Kaitlyn Flegg, Mawj Al-Hammadi, Morgan Livingstone, Taline Dorna, Leslie Low, Jill Robert, Alissa Ulster, Stephanie Kletke, Ashwin Mallipatna, Katherine Paton, and Helen Dimaras.

Ivana Ristevski, Taline Dorna, Leslie Low, and Jill Robert were patient partners.

The data that support the findings of this study are not publicly available due to their containing information that could compromise the privacy of research participants but are available from the corresponding author (H.D.) upon reasonable request.