Patient knowledge, experiences and preferences regarding retinoblastoma and research: A qualitative study

Abstract Background We launched a patient engagement strategy to facilitate research involvement of the retinoblastoma (childhood eye cancer) community in Canada. To inform our strategy, we aimed to uncover the experiences with retinoblastoma, knowledge of retinoblastoma and research engagement among retinoblastoma survivors and parents. Methods Focus groups were held in Toronto and Calgary, including both in‐person and remote participants (via videoconference). Discussions centred on experience with retinoblastoma, knowledge of the disease and engagement with research. Focus group transcripts were evaluated by inductive thematic analysis. Results Four focus groups (3 in Toronto, 1 in Calgary) were held with a collective total of 34 participants. Retinoblastoma had a substantial impact on the life of participants, but overall, patients reported being able to adapt and persevere. Experiential knowledge of retinoblastoma was identified as distinct from the theoretical knowledge held by their clinicians. Participants indicated they often acted as a knowledge broker, communicating information about the cancer to their social networks. Participants were willing to engage in research as partners, but recognized barriers such as time and appropriate training. Conclusions Patients view their experiential knowledge of retinoblastoma as valuable to improving care and directing research. There is a unique role for research engagement in meeting the educational needs of patients.

retinoblastoma, necessitate not only extended clinical follow-up but also long-term engagement with the health and research community. At the Hospital for Sick Children (Toronto), a study was conducted that examined knowledge, attitudes and experiences of Canadian retinoblastoma survivors and their parents, with regard to the genetics of the cancer. 5 Unexpectedly, the study found that study participants were keenly interested in becoming more involved in retinoblastoma research.

Engagement of patients in other areas of health research has
shown to increase democratization of the research process, study enrolment and retention rates, 6 and broader community engagement. 7 Results from such studies are more credible and acceptable to stakeholders, [6][7][8] leading to improved clinical programmes. 9 The term 'patient' refers to individuals with lived experience of disease and informal caregivers 10 ; for retinoblastoma, this could include parents, guardians, survivors, spouses, siblings and unaffected carriers.
Given the documented value of patient engagement strategies to bolster research, [6][7][8][9][10][11][12][13][14] we launched the Canadian Retinoblastoma Patient Engagement Strategy in 2016. 15,16 To inform the further development of this Strategy, we sought to examine the perspectives of retinoblastoma patients in Canada regarding their experiences, knowledge and preferences regarding retinoblastoma and research engagement. We rationalized that this information would help tailor strategy recruitment and engagement efforts, and develop novel research activities to meet the needs and preferences of the Canadian retinoblastoma patient community.

| Study question and design
This qualitative cross-sectional study asked the question, 'What are the experiences with retinoblastoma, knowledge of retinoblastoma, and research engagement among retinoblastoma patients? ' We employed the definition of 'patient' suggested by the Canadian Institutes for Health Research: individuals with personal experience of a health issue, including informal caregivers. 10 Data were collected through focus group discussions. Research Ethics Board approval was received from the Hospital for Sick Children (REB# 1000054246).

| Study setting
Focus groups were held in Toronto and Calgary, the rationale being that these two cities have the largest retinoblastoma communities.

| Inclusion criteria
Participants were eligible to participate in the study if they were: (a) retinoblastoma survivors and/or the immediate family of someone diagnosed with retinoblastoma; (b) 18 years of age or older or considered an emancipated minor; and (c) residents of Canada.

| Participant recruitment
Participants were recruited non-randomly. Physicians, allied healthcare providers and advocacy groups were invited to disseminate a participant recruitment letter to their retinoblastoma networks. A participant recruitment poster was also disseminated through social media (Facebook, Twitter and Instagram) targeted to the Canadian retinoblastoma community.
Interested participants contacted the study team by email or telephone. A study team member confirmed eligibility criteria, noted availability and reviewed study objectives and consent form with the individuals. Participants were then asked to sign up for focus group time slots on a study-specific REDCap 17 electronic data capture tool hosted on the Hospital for Sick Children server. A unique study ID code was issued for each participant, and protected identifiable information was documented in a code-breaking log, which was stored separately from study data. Once dates for focus groups were set, an email was sent to all participants with a copy of the consent form for their review, location details for in-person participants and login details for remote participants.

| Sample size
Ethical approval was granted to conduct up to 4 focus groups in Toronto and 2 in Calgary, collectively including a maximum of 60 participants. This was based on the estimated patient population in each city, balanced against an educated guess of how many focus groups might be necessary to reach theoretical saturation.
Focus groups were conducted until a saturation of themes was reached.

| Focus group structure
Each focus group included 6-10 participants. Written informed consent was obtained from each participant prior to the beginning of each focus group. Participants completed a brief demographic questionnaire. Focus groups were video-and audio-recorded, and 1-2 study team members were present as note takers (CM, HD).
The facilitator (KF) directed the discussion using a focus group

| Data processing and analysis
Demographic data were summarized using descriptive statistics.
Recordings were transcribed verbatim from audio recordings and non-verbal communications referenced from written notes and video recording. Participants were de-identified using participant's study ID codes.
A codebook was developed iteratively by study team members (KF, CM, HD). Each researcher independently coded one transcript to test for consistency and compared across the others. Once consensus was reached, data were coded by one study team member (CM), using NVivo QSR 11. Inductive thematic analysis was employed to derive common themes, following the study framework. An audit trail using memos was used in the initial coding phase, and discussions were held with the rest of the team (HD, KF) to ensure that the findings logically flowed from the raw coded data. This study adheres to the Standards for Reporting Qualitative Research (SRQR) guidelines 20 (Supporting Information S2) and Guidance for Reporting Involvement of Patients and Public-2 (GRIPP2) 21 (Supporting Information S3).

| Relationship with retinoblastoma
Ten participants identified as survivors (10/34, 24%). Twenty-four were parents of an individual with retinoblastoma (24/34, 71%). One participant identified as both a survivor and a parent. One survivor was a grandparent of a child with retinoblastoma. One individual did not fall into a survivor or parent category, being an unaffected RB1 gene mutation carrier, inherited from a survivor parent and passed on to an unaffected carrier child (1/34, 3%) (

| Themes
Common themes were identified and related to three main categories: (a) experiences with retinoblastoma, (b) knowledge of retinoblastoma and (c) research engagement (Table 4).   Unemployed and looking for work 2 6 Unemployed and not looking for work 1 3

35
Number of prior research studies (Self) No response 23 68 Role in prior research studies (Self) (n = 11)

Participant 10 91
Study coordination 0 0 Other 1 9 Number of prior research studies (Child) Opportunities to participate in retinoblastoma research (n = 34) Usually unaware of any opportunities 12 35 Too few opportunities 11 32 Just enough opportunities 9 26 Too many opportunities 0 0 No response 2 6 Participants expressed that psychosocial support had been largely missing from their care. The issue of geographical location of residence as affecting quality of care was brought up by some. This was mainly a concern to those living outside Toronto.

| Knowledge of retinoblastoma
Theme: Patients rate their personal knowledge of retinoblastoma as above average Participants were asked to rate their knowledge of retinoblastoma on a scale from one (low) to ten (high) and explain their choices. Most perceived themselves as having higher than aver- Participants also expressed a concern that they had to constantly act as their own advocates when interacting with health-care providers outside of the retinoblastoma primary care team.

| Discussion
The purpose of this study was to uncover the patient experiences with and knowledge of retinoblastoma and associated research.
The intention behind the study was to inform the development of the Canadian Retinoblastoma Patient Engagement Strategy. 15,16 The results reveal that the retinoblastoma patient community has a wealth of diverse experiential knowledge related to retinoblastoma from a variety of patient perspectives (eg heritable and non-heritable, unilateral vs. bilateral, parent vs. survivor). Yet, regardless of the type of experience, the effects of retinoblastoma were uniform: all-encompassing and lifelong. These experiences reflect important avenues on which future research could be based.
Participants' general satisfaction with their primary retinoblastoma care is likely reflective of the high cure rates in Canada. 2 Yet, the participants pointed out an urgent need for health-care teams to cater to their psychosocial care. This is consistent with other studies of paediatric rare diseases where parents report unmet social, informational, emotional and psychological needs. 22 A prior study on retinoblastoma indicated that multidisciplinary teams help parents with emotional support and coping with treatment, as do peer support groups, during times of stress. 23 Following the current study, we conducted a priority setting workshop with patients, researchers and clinicians, which revealed that improving psychosocial care for families affected by retinoblastoma is one of the top 3 Retinoblastoma Research Priorities in Canada (publication pending).
There was also a strong sentiment from participants on the need of long-term follow-up into adulthood, particularly for the detec- Parents and survivors indicated they often find themselves in the role of knowledge broker, to explain retinoblastoma to those outside the retinoblastoma community, such as extended family members or teachers. Participants recognized need for high-quality and reliable information, but in an understandable format. In a prior study, online patient education materials in retinoblastoma were analysed for readability and found to be written at a higher grade level than recommended for patients, possibly interfering with their interpretation. 29 Participants suggested that online materials were more prone to error, and preferred the information to come from a trusted source like their clinician. Consistent with this, a prior study revealed that patients rated online sources as least important for their learning and health-care providers as most important. 30  Advisory Board meets annually to govern the strategy, with working groups led by patient and non-patient pairs, who advance efforts to engage patients on projects that advance joint research priorities.
Working group activities are sustained via monthly teleconferences, and modest stipends are provided to patient partners as a token of appreciation for their contributions. These activities are currently being evaluated using the Public and Patient Engagement Evaluation Tool. 33 When parents are well informed, their children grow to be survivors who are well informed. 34 Yet, a distinct portion of our participants indicated they did not wish to know more about retinoblastoma, perceiving it to only add to their anxiety and discomfort.
Perhaps the desire for 'normalcy', expressed by some participants, is at odds with learning more about retinoblastoma, as it could be a reminder of having cancer, something perceived as 'abnormal'. This dichotomy in the patient population-those who wish to know more, and those who do not-will be challenging to navigate for medical and research teams. Individualized approaches may be necessary to provide only as much information as desired by the patient, while ensuring that they have the necessary knowledge to achieve the best possible outcome.
One limitation to this study is that the geographical location of participants was limited to just four Canadian provinces, all Englishspeaking. Recruitment aimed to reach patients and families nationwide, and while we did not achieve that, participants represented different types of retinoblastoma diagnoses and treatment experiences, and other demographic characteristics, including a higher participation of males than seen in other qualitative studies in our field. As the Canadian Retinoblastoma Patient Engagement Strategy grows and is strengthened, we expect to reach patients in all parts of the country and increase geographical representation in this respect.

| CONCLUSION
In conclusion, retinoblastoma has a substantial impact on the life of those it affects. Patients view their experiential knowledge of retinoblastoma as valuable to improving care and directing research as it is distinct from the theoretical knowledge of the cancer held by clinicians. Patients recognized that they have some knowledge gaps on retinoblastoma, and interestingly were divided on whether or not they wished to learn more or remain in the dark. For those who do wish to learn more, this study finds that there could be a unique role for research engagement in meeting educational needs of patients, in addition to informing unique and patient-centric directions for research.

ACK N OWLED G EM ENTS
The authors wish to acknowledge Ari Psihopedas, Helen Psihopedas, Leslie Low, Peggy Gronsdahl and Abby White for providing patient testimonials and being co-applicants on the grant application to request funding for this study.

CO N FLI C T O F I NTE R E S T
The authors declare that they have no competing interests.

AUTH O R S ' CO NTR I B UTI O N S
KF and HD conceived and designed the study; CM, KF and HD acquired, analysed and interpreted the data; CM drafted the manuscript; CM, KF and HD critically revised the manuscript for important intellectual content and approved the final version of the manuscript.

E TH I C A L A PPROVA L
Research Ethics Board approval was obtained from the Hospital for Sick Children (REB# 1000054246).

DATA AVA I L A B I L I T Y S TAT E M E N T
The data that support the findings of this study are available from the corresponding author upon reasonable request.