Experiences of Children With Osteogenesis Imperfecta in the Co-design of the Interactive Assessment and Communication Tool Sisom OI: Secondary Analysis of Qualitative Design Sessions

Background Children with osteogenesis imperfecta (OI) experience a diversity of symptoms that expose them to difficult physical, mental, and social challenges. Sisom (DHealth) is an interactive assessment and communication tool designed to help children aged 6-12 years with chronic conditions express their symptoms. Recently, the co-design of the Sisom OI paper prototype was launched by seeking the perspectives of end users, including children with OI and their clinicians. Objective The aim of this study is to describe the experiences that children with OI were prompted to share with researchers during the co-design of the Sisom OI paper prototype. Methods A secondary analysis of qualitative data was conducted at a university-affiliated, pediatric, orthopedic hospital. The data sources consisted of interview transcripts, drawings, field notes, and observations derived from interviewing 12 children with OI who participated in the co-design of the Sisom OI paper prototype. The themes and subthemes identified from the data sources were generated using qualitative description. Results Three themes were identified. The first, Relating to Others, described the balance between feeling different versus feeling similar to other children. The subthemes were Common OI Experience, Feeling Different, and Feeling Just Like Others. The second, Relating to Their Condition, described children’s positive and negative interactions with their own condition and health care. The subthemes were Understanding Their Condition, Special Relationship with the Hospital, and Difficult Treatments and Procedures. The third, Reflecting on Capabilities, described children’s recognition of their strengths and limitations. The subthemes were Perceiving Limitations, Overcoming Isolation, and Celebrating Strengths. Conclusions This co-design process provided children with OI the space to not only contribute to the development of the end product but also eloquently describe their experiences. These findings, based on the descriptions given by the children themselves, offer us a unique understanding of what it means to grow up with OI.


Background
Osteogenesis imperfecta (OI), otherwise known as brittle bones disease, is the most common inherited chronic bone disorder [1][2][3][4]. The prevalence of OI has been estimated at 1 in 13,500 and 1 in 9700 in 2 recent population-based studies from Scandinavia [5,6]. Its principal clinical feature is bone fragility, for which there is no cure. Thus, health services focus on the prevention and treatment of fractures to maximize mobility [7]. However, pain, fatigue, and varying degrees of physical limitations may hinder participation in daily activities and acceptance by peers and lead to feelings of fear, otherness, and isolation [4,[8][9][10][11][12][13]. Relatively little attention has been directed toward understanding the day-to-day experiences of children with OI specifically and from their own perspective.
Children living with other chronic conditions have a unique understanding of their experience that cannot be conveyed through a proxy [14,15]. A growing body of knowledge demonstrates a gap between what children view as significant and what their parents perceive [15,16]. Children with chronic conditions are described as having an "intense embodied understanding of [their] disease" [17] and being "historians in their own right" [18]. As clinicians, we have a moral duty to elicit these children's voices and partner with them to truly understand their perspective. This notion has been reinforced in the United Nations' (1989) Convention on the Rights of the Child, which states that every child has the right to express themselves and be heard by others in matters that affect their well-being [19].
The concept of authentic listening is derived from the moral duty to treat children as competent moral agents [20]. It implies their active involvement in decision-making to help them foster a strong moral order and capacity in future decision-making. The concept of authentic listening goes beyond simply allowing a child to express himself/herself: it is about considering a child's viewpoint as unique and valuable rather than immature and underdeveloped. Research that seeks to elicit the voices of children and include them as experts in decision-making is thus of great importance.
One strategy to elicit a child's voice is to transform a child's place (a place for children that is run by adults and their rules) into a child's space (a place where children can take the lead) [21]. Efforts to create this space were made during the participatory co-design of a paper prototype called Sisom OI [13]. This was the first step our research team embarked on to address the absence of interactive computerized tools designed to assess the unique needs of children living with OI. Sisom (DHealth; Norwegian acronym derived from Si det som det er, meaning Tell it as it is) is an award-winning, rigorously tested, interactive, computerized tool that helps children aged 6-12 years with chronic conditions to express their symptoms [22]. It can be downloaded on a phone, tablet, laptop, or desktop computer. Sisom, originally designed for children with cancer, was adapted for children with congenital heart disease, and in the process of being adapted for other conditions, it also has the potential to engage children with other chronic conditions in their own care [23]. It is also considered a creative system that helps clinicians better understand children's perspectives [22]. Sisom uses spoken text, sound, and animations to depict symptoms that are each represented by an animated scene within 1 of the 5 symptom islands (Figure 1). After first creating an Avatar, one is then prompted by the Sisom Navigator Mary, the nurse, to indicate the presence and severity of symptoms displayed by using a 5-point Likert-type scale. Upon completion, Sisom generates a child-friendly symptom report that can be shared with the family and clinicians [22,24].
To date, the development, redesign, validation, and evaluation of Sisom demonstrate the ability of this tool to create a unique environment to elicit children's perceptions of their condition and engage them in their health care [22,23,[25][26][27][28].

Objective
The aim of this study is to describe the experiences shared by children with OI when approached as experts in the co-design of Sisom OI, to further advance the prototype development, and to understand the children's experiences.

Design and Setting
Following the approval of the institutional review board (A06-B29-17B), a secondary analysis of qualitative data was conducted [29]. The data sources consisted of interview transcripts, drawings, field notes, and observations derived from interviewing 12 children with OI who participated in the study aimed to develop the Sisom OI paper prototype [13]. Both studies were conducted at a university-affiliated, nonprofit, pediatric, bilingual, orthopedic hospital in Montreal, Québec, specialized in OI care. On the basis of the average prevalence rate of 2 Scandinavian studies [5,6] of 1 in 11,600, we estimated that there were 3190 individuals with OI in Canada, of which the study site has seen 576 children or 18.06% (576/3190) of the population.

Participants
Purposive sampling was used to allow for maximum variation in age, self-identified gender, and type of OI for children. Children were eligible for the study if they (1) were diagnosed with any type of OI, (2) were aged between 6 and 12 years, (3) received health care at the study site, (4) had a consenting parent or legal guardian, and (5) spoke in either English or French. Sisom was designed with and for children aged 6-12 years to meet their varying cognitive and emotional capabilities [22]. Therefore, the age eligibility criterion was aligned with previous Sisom studies and was limited to this age group. Older children were excluded, as they think and express themselves differently than younger children [22,30]. Moreover, children aged ≥12 years, with enhanced readability, can use other tools [30,31].
The original study sample size estimate of 10-15 children and 5-10 clinicians was proposed based on leading experts [13,32,33]. These sample estimates were also aligned with previous Sisom studies with similar designs to successfully develop the prototype [22,23], translate [25], and establish the usability of the tool [27]. Although other Sisom studies have included larger samples, these studies were quantitative (N=100) [26] or a secondary analysis of 2 studies [25,34], which explicitly increased the sample size to target the multiple disease groups inherent in oncology from multiple sites (n=39) [35]. In this study, a secondary analysis of 12 interview transcripts was deemed sufficient for investigation, as the interviews were rich in children's verbal expressions of experiences. Finally, data saturation was reached, and a sense of closure was attained because the data derived from our sources yielded redundant information [36].

Recruitment
The children with OI were recruited by reaching out to clinicians who assisted by identifying, screening, and approaching families to determine if they were interested in hearing more about the study [13]. One member of the study team was charged with providing a verbal and written explanation of the study to those interested in obtaining written parental informed consent and child assent [13].

Data Collection and Procedures
Data were collected in the context of the participatory co-design of the Sisom OI paper prototype [13]. The early stages of the co-design of the Sisom OI paper prototype consisted of 3 feedback cycles with 2 to 6 semistructured, face-to-face, individual child interviews per cycle [13]. The length of the interview depended on the child's interest and varied from 20 to 60 minutes. It was the parent's or legal guardian's choice to be present during the interview. During the co-design of Sisom OI, the children were prompted in several ways. First, Sisom prompted the children both verbally, through the questions Mary, the nurse, was asking, and visually, through the different animations that appeared. Mary's questions prompted the children to share their experiences related to each symptom being assessed. Second, the interviewer verbally prompted the children to share and elaborate on their experiences when prompted. The interviewer would either directly ask the child a question or reflect something that the child shared in common with other participants to prompt a response. These prompts helped children to draw from their experiences to determine whether the Sisom symptoms, vignettes, or avatars were relevant or irrelevant, needed to be modified, required new additions, or remained unsure [13]. Third, if present, the parent or legal guardian also prompted their children to share their experiences (Multimedia Appendix 1). Field notes were recorded during and immediately after each audio-recorded interview, which included detailed descriptions of nonverbal data, other observations, impressions, and any drawings generated.

Data Analysis
The interviews were transcribed and read through, along with the field notes and observations taken by the interviewer and the 6 drawings created by the children [13], to gain a global picture of the data. A descriptive qualitative analysis was then applied to identify themes and patterns identified in the data sources [37]. First, open coding was performed diligently to remain close to the original data. Second, code reduction or clustering was performed to create categories and remove redundant codes [37]. Third, axial coding was performed to find the links between the main categories to generate overarching themes and subthemes. Finally, the original data were revisited to ensure that the child's experience was properly interpreted and described [37]. Through this process, themes and subthemes across the children's responses were identified. For each theme and subtheme generated, various examples were used to highlight particular points within the children's dialogue.
Ongoing meetings with the research team were held to discuss how to answer the research question, explore how the data could be best summarized, and check whether designated quotations fit the proposed themes and subthemes. The selected French quotes were subsequently translated into English. Throughout the entire process, an audit trail, composed primarily of methodological and analytical documentation, was kept, permitting the reproducibility and transferability of the process.

Sample Characteristics
A total of 12 children participated in this study ( Table 1). The participation rate was 92% (12/13). No child withdrew from the study.

Overview
In the co-design of Sisom OI, different prompts allowed children to open up about their experiences of life with OI. These prompts were from Sisom, the interviewer, and, if present, the parent or legal guardian. The dynamic between the child and their parent or legal guardian varied. Sometimes, the parent or legal guardian answered directly for the child. In these cases, the child would then agree, disagree, or remain silent. At other times, the parent or legal guardian gave the child space to answer on their own. The experiences shared in this analysis focused explicitly on the children's responses. Three themes and their respective subthemes were identified (Multimedia Appendix 2).

Relating to Others
Children with OI described how they saw themselves in relation to other children. They demonstrated that they were different but also very similar to any other child ( Figure 2).

Common OI Experience
Children with OI have a distinctive experience that unites them.

Feeling Different
Children were asked by Sisom if they felt different than other children ( Figure 2). They all responded that they did. One of them stated matter-of-factly: "Ya...because other kids are different than us." [P5, 10 years, type IV OI]. They also expressed that feeling different affected their lives in several ways. First, many of the children reflected that their differences led to being bullied. One child expressed how her peers at school teased her because she was different: "They often say, let's say, that I don't run fast, and they laugh at me." [P8, 7 years, type I OI].
Second, they reflected that their differences led others to stare at them, "the gaze of others" [P3, 11 years, type IV OI], which they acutely sensed, and caused their worries about feeling different to amplify. Third, they reflected that their differences led others to treat them differently. One child described how others were afraid to break her bones and that she was ostracized on a daily basis:

Feeling Just Like Others
All children demonstrated that they were more similar to others than they were different. The children shared their desires, wishes, and preferences just as any other child would. The goal was to engage children in the co-design of the Sisom OI paper prototype; however, OI was sometimes left out of the conversation altogether by the participants. For instance, when 1 child was asked what makes them feel different from other children, they commented on their age. When asked if there was anything else, they stated, "That's it" [P10, 9 years old, type VI OI]. Every interview included children laughing, playing, and sharing, just as any other child would.
They shared things about themselves that they found special, such as their likes: "We all love unicorns!" [P6, 6 years old, type I OI]; their dreams: "I want to be a vet." [P5, 10 years old, type IV OI]; and their hobbies:

Overview
In Relating to Their Condition, children described their positive and negative interactions with their own condition and the health care system (Figure 3).  It was also common for children to travel from great distances to receive treatment, as it was for one child, whose mother had to stay home, in another province: Children not only expressed that they felt discomfort during hospital procedures but also shared their pride in having gotten used to certain painful procedures. One child described being "Brave for everything" and doing a "Good job" by not crying during painful procedures, which she described as "another unhappy task" [P6, 6 years old, type I OI]. Another child justified his painful experiences with their purpose:

Reflecting on Capabilities
Overview By reflecting on their capabilities, children were able to identify some of their strengths and weaknesses ( Figure 4).

Perceiving Limitations
All children expressed a desire to do something they were physically incapable of because of a fear of getting hurt. For some children, this activity was eating. Some described not being able to eat tasty foods that were "Like, let's say, sticky things...and things that are too hard" [P8, 7 years old, type I OI] and "like [...] bars, for example, I can no longer have them..." [P3, 11 years, type IV OI] because of an orthodontic mouthpiece they were required to wear for their OI. One child explained that OI prevented her from playing with her friends:

Overcoming Isolation
Ideas of feeling left out were expressed by all children. When prompted by Sisom "Do you ever feel left out?" children answered: They overcame these feelings by finding independent activities and excelling in other areas, escaping through imagination, having good friends, and thinking positively.
First, the children asserted their independence in several areas such as reading and journaling and taking time for themselves to be alone: "I want to write something on here!" [P9, 6 years old, type III OI]. One child described how she turned to nature when her sister did not want to play with her: "So I just go outside and sit behind one of my trees" [P6, 6 years old, type I OI]. Another child described how while she was dependent on others in some areas, she was determined to find ways to maintain her independence: One child reflected how she did not feel bad when her friends played without her at recess but always appreciated the time that they spent with her:

Celebrating Strengths
All the children experienced feelings of otherness, exclusion, and physical limitations, but they also celebrated their strengths and asserted their independence in various ways. Children were excited about their mobility improvements: This confidence allowed them to attend special events and show off their skills. One child participated in special Paralympic tournaments: "I won [...] I got three gold medals" [P3, 11 years, type IV OI]. She also played soccer at her specialized school, where she felt comfortable being herself.
Another child also played soccer and hockey with his friends [P7, 9 years old, type III OI]. These adapted environments were important contributors in reinforcing feelings of inclusion and value.
The children also celebrated their academic accomplishments. They highlighted how well they do in school: "I'm one of the best readers in my class!" [P12, 10 years old, type IV OI]. The children described their accomplishments with pride and confidence. One child explained how she was able to excel even in an accelerated program: Not only did she celebrate her own strengths, but she also encouraged others to find their strengths and celebrate them.

Principal Findings
Efforts to authentically listen and bring to light what children with OI shared when approached as experts in their condition was made possible by conducting a secondary analysis of qualitative data sources collected for the co-design of the Sisom OI paper prototype [13]. This study demonstrates, as shown in other recent findings, that children who use Sisom are prompted to share their experiences [25,27]. Through the analysis of their dialogue, 3 themes were identified. The first, Relating to Others, described the balance between feeling different versus feeling similar to other children. Children with OI had a unique way of maintaining balance in their lives. This was demonstrated through all three of the themes. First, in Relating to Others, children described a balance between feeling different and feeling similar to other children. They also expressed a concern about the perception of others. This has previously been described in other studies of children with OI [8,38]. An important way that children with OI overcame this feeling of otherness was through building relationships with other children and clinicians in the OI community [8,39]. Another way children navigated between feeling different and feeling similar to other children was by sharing with others the special things that defined them and by participating in activities with other children as best as they could. This helpful strategy has been described in other populations of children with chronic conditions [17,40].
Second, in Relating to Their Condition, children described a balance between the positive and negative aspects of living with OI. Children's depth of understanding and moral awareness can range from rich sophistication to simplistic matter-of-factness [20]. Children are capable of such awareness, and their voice should be attended to without comparison with the adult perspective [20]. Indeed, in the data, there was a wide range of depth in the understanding of OI. All children were able to share what it meant to have OI. Being in the hospital was an experience that was painful, with numerous unpleasant procedures including surgeries, needle pricks, and extended hospitalizations. The fear of needles was encountered by children living with OI in this study as well as others [8,38]. Tolerating pain and getting used to painful procedures was something that children with OI have expressed in other studies [38]. This idea of getting used to pain was expressed by the children as a proud adaptation to a difficult aspect of living with OI. Another way that children overcame the negative aspects of being hospitalized was by reflecting on the positive relationships they had developed with clinicians and volunteers, which other children have reported [18,41]. It has been shown previously that children with OI form special relationships with people who understand OI, for instance, their physicians [8].
Finally, in Reflecting on Capabilities, children described a balance between perceiving their limitations and celebrating their strengths. Feeling left out because of the fear of fractures or physical limitations was a major element of this theme and has been explored in other studies [8,38,42]. Despite the presence of this feeling, children with OI have been found to have an immensely positive and strength-based outlook on life [8,39,42]. Our study showed 1 way that these children embraced positivity was to constantly strive to improve themselves physically and academically. This concept of a strong desire to move forward was supported by other studies; however, these studies also showed that children with OI held back due to the fear of pushing too hard and fracturing [8,39]. Children's perception of these physical limitations propelled them to take on and excel in their intellectual feats [8]. Interestingly, our study showed that, given the right environment and support, children with OI are also capable of excelling in physical feats. These children are taking life in stride and using their positive outlook to overcome feeling different and feeling left out and making the best of every day [42].
Sisom may contribute to transforming a child's place into a child's space [13] by inviting children to participate in their care [28] and showcasing how they can serve as advisors and partners in their care [21]. Sisom allows for the promotion of discussions about what children deem the most important. This essential information may help clinicians enhance children's participation in their own care and ultimately empower them to cope with the difficult physical, mental, and social challenges they face. However, Sisom OI is yet to be developed, tested, or integrated into practice. Therefore, these study findings offer only preliminary insight into the types of discussions that may unfold from the future use of Sisom OI in practice. As OI remains a rare disease with few clinicians encountering a child with OI over the course of their career, there may be some reluctance to use Sisom. Offering training and delineating potential responses to children's expressed concerns may help integrate this novel, interactive, computerized tool into practice and contribute to the transformation of children's rightful space in their health care systems [28,35].
In this study, the partnership created between the interviewer and the child successfully elicited and highlighted the child's perspective, placing the child in the center of their experience. These children had many things to share about their experience, as noted by other chronically ill children [43]. Although parental involvement, at times, made it difficult for the child to take the lead, our approach revealed the child's perspective. Parents highlighted the things with which children did not necessarily agree. These differences in perspective have been reported in previous studies [15,16]. Sisom's ability to highlight such differences is considered a key feature, as it strives to promote communication between children and adults so that they may engage in shared decision-making.

Limitations
The children in this study were prompted to give their opinion on Sisom to co-design Sisom OI. Therefore, they were not directly prompted to share their OI experiences, unless prompted by the interviewer. The questions posed in Sisom were not tailored to specifically capture the experiences of children with OI. This may have led to significant data gaps. Future studies should be conducted to specifically aim to understand children's perspective on their OI. Furthermore, the involvement of parents and legal guardians in the interviews had, in some cases, a muting effect on the child. Instead of giving space for their child to answer the questions independently, some took it upon themselves to answer for their child. In some cases, this prompted the child to express their feelings and share their experiences, whereas in other cases, this caused the child's voice to be lost. Further studies should consider how to highlight the child's voice by emphasizing on the child's answers and constantly reemphasizing the purpose of the study to the parents involved.

Clinical Implications
This study highlights the richness of the experiences shared by children who are approached as experts in their own condition. These children had valuable information and insights to share regarding their day-to-day lives and the factors in their lives that both hinder their development and support their endeavors. The study findings offered a glimpse of what it means to grow up living with OI but also showcased a novel approach to gain their perspective. The children's inputs will be further used to advance the development of Sisom OI and our overall approaches to offer care that responds directly to their needs and empower them to be active partners in their care.

Research Implications
The study findings will be also used to inform the development, verification, validation, and evaluation of Sisom OI to ensure that meaningful aspects of life with OI are included in the app and attended to in practice. At present, the Sisom OI paper prototype has been developed [13]. Although many of the existing Sisom symptoms were deemed relevant for inclusion in the Sisom OI paper prototype, 57 new symptoms were generated. The relevant symptoms addressed children's thoughts and feelings about hospitalization and their wishes for participation in their own care. The new symptoms addressed fractures, body image, and social isolation related to difficulties with accessibility and intimidation. Therefore, these findings, in addition to the themes identified in this study, will be used to inform the next phase of Sisom OI development with end user partnerships, stakeholder buy-in, and the necessary funding. We expect to build the prototype; test for usability; pilot for feasibility; and evaluate the integration of Sisom OI into the hospital, home, and school settings.

Conclusions
This co-design process provided children with OI the space to not only contribute to the development of the end product but also to eloquently describe their experiences. These findings, based on the description given by the children themselves, offer us a unique understanding of what it means to grow up with OI.