Transitioning to adulthood with a progressive condition: best practice assumptions and individual experiences of young men with Duchenne muscular dystrophy

Abstract

Purpose: Youth with progressive conditions are living longer, and there is increased health care focus on assisting them with “transitioning” to adult services and adult life. The purpose of this investigation was to examine key discourses and normative assumptions underpinning transitions best practices and how they are reflected in the experiences of young men with Duchenne muscular dystrophy (DMD). Methods: Using a critical perspective, we qualitatively analyzed influential transitions best practice documents to identify their underpinning discursive assumptions. We compared these to the analysis of qualitative interviews and diary data from a study of 11 young men with DMD. Results: Transitions best practices are underpinned by discourses of developmental progression. They reproduce notions that associate successful transitions with becoming as independent as possible, approximating normal life trajectories, and planning for future adulthood. The accounts of youth with DMD both reflected and resisted these future-oriented discourses in creative ways that maintained positive personal identities. Conclusions: Normal developmental progression towards typical adult roles constitutes the generally accepted aims of transitions practices. Such aims may not be appropriate for all youth with disabilities. We suggest that alternative understandings of the life course and approaches to care need to be considered alongside dominant practices.

Implications for Rehabilitation

• Children and youth with progressive conditions, such as DMD, are living longer and there is increased interest in designing programs that will assist them with “transitioning” to adulthood.

• Transitions best practices reflect dominant social values and assumptions about what constitutes a successful adulthood, embedded in goals such as independent living, self-management and obtaining work.

• Rehabilitation professionals should be aware of both positive (e.g. feelings of achievement) and negative (e.g. anxiety about the future) consequences of transitions practices that emphasize normal social developmental trajectories and milestones.

• Discussions with youth should offer multiple possibilities for living a good life in the present and provide support to address negative feelings and the progressive effects of DMD.

• Development
• Duchenne muscular dystrophy
• life course perspective
• transitions
• young men

Introduction

In response to increasing life expectancies for children with chronic conditions, there is growing recognition within health care of the need to support these young people as they transition to adult life and adult services [1–3]. Best practice guidelines for transition to adulthood have been published by notable organizations such as the American Academy of Pediatrics, Society of Adolescent Health and Medicine, Canadian Pediatric Society, and CanChild Center for Childhood Disability Research. These influential best practice documents have been widely accepted and adopted by pediatric health and rehabilitation organizations in Canada and beyond. Their common aim is to prepare a broad population of youth with chronic conditions for adult life (life stage transitions) and for the process of transfer from pediatric to adult health services (service transitions).

Transitions best practices, as well as the evidence informing their advancement, are based on particular and often unexamined understandings of development and adulthood. These understandings are reflected in discourses that become taken for granted ways of thinking and talking about what is a “successful” transition to adulthood, which may not reflect the experiences of all young people with disabilities or meet their needs. By “discourses,” we refer to a “group of ideas or patterned ways of thinking which can both be identified in textual and verbal communications and located in wider social structures” [4, p. 145]. Unpacking discourses can illuminate normative social assumptions embedded in textual and oral communications in transitions practices, and prompt examination of their influences on individuals and social change [4].

In this article, we examine key discourses and assumptions underpinning influential transitions best practice documents and compare them to the transition experiences of young men with Duchenne muscular dystrophy (DMD) investigated in a recent study [5]. DMD is a genetic condition, affecting boys almost exclusively, and is characterized by progressive muscle weakness, leading to increasing motor impairments and an uncertain lifespan. With medical advances, young men with DMD can now live beyond childhood into their 30s and 40s, but face profound uncertainty regarding their lifespan. We were particularly interested in examining how transitions practices and policies reflect their circumstances, and how they experienced and understood what it means to transition with a shortened and shifting life expectancy.

The focus on transitions for youth with disabilities grew out of recognition that services for them were scarce or non-existent [6]. Youth who had access to multidisciplinary services and supports as children, fell off a service cliff once they hit the age of majority and were poorly prepared to manage their health and other needs [7]. Transitions programs were created to address this gap by preparing youth for increasing independence in managing their health-related needs, directing their attendants, and navigating the health care system. Increasingly, transitions programs also focus on preparing children to transition to social roles associated with adulthood, including attending post-secondary education or training, finding work, managing finances and personal care, and living independently. With the rise of transitions programs there has also been an increased focus on research and scholarship in this area and the creation of best practice principles, guidelines and recommendations [1,3,8–10].

Transitions practices, like any field, are underpinned by basic assumptions that are reproduced and reinforced over time and place. Because transitions practices, are focused on the important work of helping young people thrive as they enter a new life phase, they reflect some basic assumptions regarding what constitutes a “good” or “successful” adulthood. Only a few studies have explored these assumptions to consider how they become accepted as the “right” ways of thinking about transitions, or examined their effects on various groups of young people [see e.g. 5,11,12]. Questions of a right and proper adulthood, i.e. what youth should or should not aim for in their lives, have important implications for health care and rehabilitation practice. For example, is “independence” the right goal for children with significant cognitive impairments? If so what does it look like? Similarly, how do we help youth with progressive conditions and uncertain adult lifespans, such as DMD, think about their future goals? If we only draw from understandings of a typical adulthood, are we missing opportunities to help youth with various impairments and life goals to thrive in potentially more fruitful ways?

There has been minimal critical reflection about how these assumptions shape transitions policies and programs. Left unexamined, best practices may not result in the intended outcome of successful transition to adult life and may inadvertently contribute to feelings of failure, low self-worth, or exclusion [5]. Examining the transitions experiences of disabled youth can potentially illuminate the effects of taken for granted assumptions underlying transitions discourses and provide new avenues for enhancing approaches to practice and research.

Little attention has been paid in the literature to the transitions experiences of youth with progressive conditions such as DMD [13]. Abbott, Carpenter, and Bushby [14] found that home and community supports have not increased for young men with DMD, although their life expectancies have increased due to health care advances. Reiss et al. [15] found that youth with conditions of a progressive nature and/or a shortened life expectancy and their parents were more resistant to health care service transitions than youth with relatively stable conditions. Gibson et al. [16] found that young men with DMD were marginalized by policies, services and inaccessible built environments, which limited their engagement in community life. Similarly, Bendixen et al. [17] suggested that future research should emphasize barriers and facilitators to participation and how participation changes throughout the course of DMD. As a whole, these findings indicate a need to better understand how tacit assumptions about disability and normal adulthood embedded in best practices influence the transition experiences and lives of young people with extended, yet uncertain, life expectancies.

In what follows, we critically examine key discourses underpinning transitions best practices. After outlining our conceptual approach, we provide details of our data sources and the methods of the analysis which sought to identify, describe and examine normative assumptions embedded in the best practice documents and in the accounts of young men with DMD. We then present the results of how these overlapped and diverged. We conclude with a discussion of possible directions for expanding transitions practices to encompass different impairment trajectories.

Conceptual approach

The analysis was guided by a critical social science perspective, which explicitly seeks to illuminate taken-for-granted assumptions that may be implied or unacknowledged in policy or practice [18]. A critical lens focuses on individuals' experiences and behaviors within larger socio-political contexts that contribute to forming people's values and perspectives [19]. We drew particularly on key ideas from Priestley's [20] life course approach to disability to examine the intersectionality of conceptualizations of disability and “generation” (e.g. childhood, adolescence, and adulthood). Priestley proposes that both disability and generational categories are constructed social phenomena. He emphasizes the importance of examining disability in the context of the life course according to “the ways in which disabled lives are understood, organized and governed within societies” [20, p. 4]. We used Priestley's life course approach as a lens for examining social assumptions of disability and of adulthood, critically interrogating transitions best practices and exploring how they were reflected in transitions experiences of young men with DMD.

Data sources

Transitions best practice documents

We included four influential transitions best practice documents in the analysis to provide a snapshot of key best practice principles, guidelines, and recommendations both internationally, and in Ontario, Canada where our study of young men with DMD was conducted. Each of these documents was developed by leading organizations in the field of child health to identify and guide best practices for transition to adulthood in the health and/or rehabilitation fields.

1. The Consensus Statement on Health Care Transitions for Young Adults with Special Health Care Needs [8] was developed collaboratively by three American Academies – Pediatrics, Family Physicians, and Physicians (internal medicine specialists) – for the purpose of maximizing “lifelong functioning and potential through the provision of high-quality, developmentally appropriate health care services that continue uninterrupted as the individual moves form adolescence to adulthood” [8, p. 1304]. This document is a policy statement that was published in Pediatrics – an influential journal published by the American Academy of Pediatrics with readership in North America and beyond. (This document is referred to as AAP hereafter.)

2. The Transition to Adult Health Care for Adolescents and Young Adults with Chronic Conditions [9] is a position paper of the Society of Adolescent Health and Medicine (SAHM). SAHM endorsed the AAP consensus statement, and thus does not differ significantly from AAP. This document was included in the analysis because SAHM is a multidisciplinary society with an international membership that has been a leading influence in adolescent health care since the 1960s. The analysis highlights principles shared across pediatric and adolescent medicine. (This document is referred to as SAHM hereafter.)

3. Transition to Adult Care for Youth with Special Health Care Needs was published by the Canadian Pediatric Society (CPS) to promote developmentally appropriate care and transition planning in adolescent medicine [21]. This Canadian specific document is oriented to pediatricians, family physicians, other health care professionals, parents and youth and is focused on both transfer of care and non-medical transitions to adult life. (This document is referred to as CPS hereafter).

4. “The Best Journey to Adult Life” for Youth with Disabilities was developed by the CanChild Center for Childhood Disability Research through a collaborative working group of youths, parents, service providers, community members and researchers [10]. CanChild is an interdisciplinary research center and knowledge hub focused on improving the lives of children and youth with disabilities and their families. The document was chosen in part because the organization's mandate and membership extend beyond medicine. It outlines an evidenced-based model and best practice guidelines for youth with disabilities. The model represents “the dynamic and ever-changing developmental process of a young person's life course, with the transition to adulthood depicted as an important ‘journey’” [10, p. 5]. The guidelines reflect current thinking about transitions best practices by key stakeholders in the childhood disability field in Canada. (This document is referred to as CanChild hereafter).

Accounts of young men with DMD

The accounts consisted of audio diaries and transcripts from photo-elicitation interviews that were generated as part of our recent study that investigated the intersections of disability, gender and transitioning to adulthood with 11 young men with DMD, ages 16 to 27 years living in Ontario, Canada [22]. Participants were recruited through a large urban pediatric rehabilitation hospital that provides services and supports to young people with childhood disabilities, including DMD. Ethics approval was obtained from the hospital's research ethics board. Six participants were in high school, one was in university, and four had completed college or university. Of the latter four participants, one was writing a book, another was engaged in volunteer work with an organization, another had just completed school and was looking for work, and one was completing qualifications for a professional certification. All participants lived at home with parents/guardians, with seven participants using paid attendants in the home. With respect to mechanical assistance for breathing, one participant used a ventilator full-time and three used a ventilator or BiPap machine at night only (either some or all of the time). Table 1 provides further information about the participants.

Table 1. Participants' characteristics.

Name (pseudonyms)	Age (years)	Education	Main mobility device
Emilio	16	In high school	Power wheelchair (w/c)
Joey	16	In high school	Power w/c
Mark	16	In high school	Manual w/c
Albert	17	In high school	Power w/c
Jean Marc	17	In high school	Manual w/c
Jeff	19	In high school	Power w/c
Sameer	21	In university	Power w/c
Peter	23	Completed college/university	Power w/c
Chris	24	Completed college/university	Power w/c
Shyam	24	Completed college/university	Power w/c
Michael	27	Completed college/university	Power w/c

Methods and primary results have been described in detail elsewhere [5,22]. The data analyzed for our purposes here included audio diaries and interview accounts. Briefly, each participant was provided with an digital camera and audio-recorder and asked to take photos and to audio-record thoughts that reflected daily life and experiences of transitioning to adulthood over a 2-week period. This was followed with a face-to-face interview that explored the diary entries, photos and broader issues of identity and transitions. The participants created between two and 20 audio entries each; interviews ranged from 45 to 120 min, with an average length of 80 min. Primary data analysis procedures included initial flexible coding and memoing that explored experiences and beliefs about disability, masculinity and adulthood [23]. For the purpose of this article, we conducted a secondary analysis that drew from the initial coding to examine how transitions best practice discourses and assumptions were reflected in participants' accounts. These analysis procedures are described below.

Analysis methods

All best practices documents were analyzed systematically to identify dominant transitions discourses and assumptions, drawing from both descriptive and interpretive methods described by Braun & Clarke [24]. First, the documents were analyzed to identify common discursive themes within and between texts. Second, we used guiding questions consistent with our aims and conceptual approach to interrogate the discursive themes [24,25] including: How are transitions defined and what is the scope of best practices? What practical problem are transitions best practices trying to fix? What assumptions underpin key discourses and what are the potential effects on disabled youth? What are the outcomes of interest and what constitutes a good or poor outcome? How are “disability,” “childhood” and “adulthood” discussed?

Further analytic questions, sensitized by the findings of the document analyses, were generated to examine the participants' data (audio diary entries and interview transcripts). We interrogated how the assumptions were or were not reflected in the accounts, how participants responded to these in the context of their lives and plans, and what other assumptions regarding transitions, adulthood and disability emerged. Y. H. conducted the initial line-by-line analyses of both data sets and the team met on several occasions to review the emerging findings, identify the dominant ideas, determine how they interrelated, and refine the analyses.

Results

In what follows, we discuss the results of the analyses organized by themes. The dominant idea that pervaded all documents was the notion of “normal development,” which was further manifested in the sub-themes of becoming “as independent as possible” and “approximating normal adult roles and milestones.” Within each of the themes we examine how these notions were reflected or not in the accounts of young men with DMD. All names used in the examples are pseudonyms.

Discourses of normal development

Developmental discourses were deeply entrenched in the four transitions best practice documents, aligning with dominant approaches to pediatric medicine and rehabilitation. All of the documents situate transition to adulthood as part of a normal developmental trajectory over the lifespan. Best practices are guided by principles of “normal, healthy development” (AAP, p. 1304), described as “appropriate for chronological age and developmental attainment” (SAHM, p. 309), and “mirror the young person's increasing maturity and emerging adulthood” (AAP, p. 1305). AAP describes the intended outcome for youth as the optimal “ability to assume adult roles and functioning” (p. 1304), whereas CanChild describes outcomes of “active citizenship and involvement in meaningful occupations” (p. 20). The AAP document states that:

The goal of transition in health care for young adults with special health care needs is to maximize lifelong functioning and potential through the provision of high-quality, developmentally appropriate health care services that continue uninterrupted as the individual moves from adolescence to adulthood (p. 1304).

What is meant by these ideas of development is not made explicit in these documents, but what they suggest is that the ideal outcome of transitions best practices is the acquisition of particular characteristics, abilities and responsibilities aligned with a “normal” adult life, such as increasing independence for managing health and activities of daily living, moving out of the family home, participating in work or post-secondary training, and forming intimate relationships [20]. Excluded are the potential effects of these expectations on young people whose physical and cognitive growth and maturation do not follow a typical trajectory. A normal social developmental trajectory is taken for granted as the “right” or only way to guide transitions best practices. No mention is made of other paths or perspectives that might align better with the experiences of youth with diverse and/or progressive impairments.

The exception is the CanChild document which provides an expanded understanding of developmental trajectories compared to traditional perspectives embedded in the other documents. This likely reflects the organization's focus on disabilities rather than medical conditions (e.g. asthma, juvenile diabetes, etc.). CanChild presents adulthood as an ongoing process not an endpoint in itself and adopts a metaphor of transition to adulthood as a “critical journey” (p. 10) because “there are significant changes occurring within the person and the environment” (p. 10). The journey to adulthood is described as potentially consisting of “multiple journeys” (p. 15) that “take place at different developmental stages and transitions” (p. 10), thus transition does not occur on a straight path. Moreover, CanChild's best practices address not only the skills and abilities of youth themselves, but also contextual factors such as cross-sector policies, information access, and supports for navigating service systems. Even so, the prevailing assumption is that ideal transitions should approximate as close to a “normal” trajectory as possible with assumed characteristics, abilities and responsibilities of an independent adulthood.

We turn now to outlining the particulars of these expectations and assumptions, and how they were reflected or resisted in the accounts of young men with DMD.

As independent as possible

One of the most pervasive ideas in the transitions documents was the notion of increasing independence. While the documents acknowledge that youth need support for part or all aspects of their lives, the implicit message is that independence is preferred over dependence. Independence appears throughout the documents and its relative value is revealed through concepts such as “independent living” (AAP, p. 1305), “interdependent” (CanChild, p. 12; CPS, p. 786), and “shared management” (CanChild, p. 15). The documents collectively promote the idea that a good transition is one that results in decreasing reliance on others and especially on family. They suggest that to the greatest extent possible, young people should be encouraged to gradually take over responsibility for managing their health and daily lives. Health care transition is described as a responsibility executed through partnerships between health care providers, youth, and families (AAP), which optimally leads to the development of “autonomous, independent consumer skills” (CPS, p. 785) for navigating the adult health system. While it is acknowledged that independent self-management is not always possible, the clear message is that the least amount of assistance is always preferred.

When independent self-management is not possible, the concept of “shared management” (CanChild, p. 15) is promoted as an alternative. CanChild describes shared management as a gradual shift in responsibility from parents and service providers to youth. Both CanChild and CPS emphasize the importance of youth learning to make independent decisions and to advocate for themselves with support of others. The notion that some forms of help are acceptable into adulthood is further expressed through discourses of “interdependence” (CanChild, CPS). CanChild explicitly proposes that interdependence is more realistic for many young people, and promotes a “‘do it on my own with supports’ approach” (p. 20) across the lifespan, emphasizing personal support networks and supportive environments as essential elements to successful transition to adult life. Interdependence is thus not presented as a form of reciprocal dependence as the term implies, but rather, as maximizing youth's independence with an acknowledged need for assistance. SAHM cautions that “adolescents with chronic conditions are at higher risk for unnecessary dependency” (p. 309), which can be prevented through practices that enhance autonomy and a sense of personal responsibility. The preference for independence and autonomy is obscured in the documents by ambivalent discourses about interdependence, in which dependence is presented as sometimes necessary but not ideal.

In the accounts of young men with DMD, participants for the most part reproduced discourses that valorize independence, but also creatively reformulated these discourses to accommodate their increasing needs for assistance. They presented themselves as highly knowledgeable about the skills and abilities needed to self-manage, while emphasizing the importance of care and assistance provided by their families. The accounts, moreover, reflected how participants negotiated identities of independence in the face of increasing physical dependencies. Younger participants, still in high school, justified dependency on family members in relation to their life stage as teenagers and as something that would decrease over time. They described how they were working towards directing their own care, such as learning to direct personal care attendants, and that dependence on parents would be less acceptable as they grew older. They, however, also expressed anxiety regarding this transition, and losing the high quality of diverse forms of care and assistance provided by parents. An excerpt from Jean Marc's (17 years old) audio diary reveals this tension:

The people I care about the most is family, because they're supportive and they help you go over challenges, and can help you with different – with your care and things like that, even if more and more you try to have attendants doing it. Which I think is a bit better because you're more independent. But it's family 'cause they do the caring, and they can help you with doing things that are not always easy to fix, like getting a wheelchair, that's not really easy if you're on your own…

Although Jean Marc preferred the care provided by his family, he took for granted that eventual independence would be better. Mark (16 years old), and Albert (17 years old) made similar comments regarding preference for family assistance with tasks such as being carried and transferred, rather than using mechanical lifts and personal care attendants. Albert discussed the emotional elements of good care and family support:

Interviewer: What is it about your family that you would want them to live with you?

Albert: I don't want to be alone.

Interviewer: What is it about being alone, is it scary or frightening, or just-

Albert: Yeah, just scary or frightening. I always need help a lot. I'm going to want someone to be there all the time.

Interviewer: And you're most comfortable with family.

Albert: Yeah.

Despite trepidations regarding the loss of family supports, participants' accounts suggested that they took up discourses of becoming less reliant on parents as an indicator of adult achievement. This was particularly prevalent in the accounts of the older participants who finished secondary school. Sameer (21 years old), for example, discussed his independence as a point of pride. He described how his mother urged him to book his own appointments and attendants, take public transportation and direct his care at an early age:

She always would say, ‘you know what, one day you're going to be living on your own and you're going to have to be able to manage things on your own.’ So gradually, she gave me my own responsibilities and that kind of led me to eventually manage a lot of the stuff on my own. So I manage my schooling on my own, little things like that depending on [the availability of] attendant services, I would manage stuff that on my own. If need be, I would get her to come in and help, out with that.

Sameer's account highlights the ways he pursued independence in his daily life, but acknowledged the need for ongoing assistance from his mother in certain situations. Thus, the valorization of independence and the goal of “as independent as possible” that were reflected in the best practices documents were paralleled in the participants' accounts. Participants did not question the value of pursuing independence even if it might require compromising the interpersonal connection, trust, and quality of care they enjoyed with their family members. Rather, they mostly positioned this pursuit as “normal” for their life stage and presented themselves as working toward achieving independence to the extent possible. Although independence was discussed in positive terms, participants also resisted its normative demands through their actions of deferment. Living independently, when discussed, was portrayed as something that participants would pursue in the future. Thus, participants could claim they were on a right and proper path to independence while deferring the actual move and its consequences. We discuss this further below.

Approximating normal adult roles and milestones

Normal development, the staged achievement of physical and cognitive milestones underpinning children's rehabilitation practices, is accepted almost universally as uncontroversial [20,26]. Developmental principles were reproduced throughout the transitions documents and provided the underlying rationale for practices designed to maximize children's capacities in adulthood. The documents highlight assumptions about ideal roles and responsibilities of adulthood in almost every arena of human life. As such, they advocate for transitions interventions across diverse sectors, agencies, and providers within and outside of health care. AAP suggests that the health care sector cannot work alone to address transitions, but must collaborate and partner with education, social service, vocational and recreation sectors. CanChild proposes that best practices take a broader view of transitions beyond health care that encompasses participation in “daily community life” (p. 11) and “active citizenship” (p. 23). It recommends a navigator role to facilitate connection to community services and supports for adults; and calls for an integration of policies from multiple sectors to improve coordination of service. CPS recommends that transitions care address physical health, mental health, sexuality, acceptance by peers, social isolation and employment. Overall, the reach of best practice interventions extend into almost all aspects of daily life including employment, housing, social relationships and community participation.

Regardless of the focus of interventions, the documents all construct the optimal outcome of development qua transition as participation in particular adult roles and activities. These include work, post-secondary education, independent living, and intimate relationships. These normative standards are unquestioned as the best way for professionals to guide progression to adulthood.

The accounts of young men with DMD illuminated key normative assumptions regarding adult roles, particularly in relation to academic achievement and work. Participants described themselves as “normal” or “regular” people who had the same aspirations to work as other people their age. In his interview, Chris (24 years old) talked about feeling part of the “real” workforce in his volunteer job placement:

Interviewer: And how did that make you feel?

Chris: Great. I felt I was in the real workforce. Well I went twice a week only. But twice a week was good enough for me, for my condition, I mean it was good enough.

Chris further reflected on the significance of being paid as an indicator of adult status:

Interviewer: Why is that important, to say that you're getting paid?

Chris: I don't know. Just maybe from a family perspective, like you know, in Chinese culture maybe it's important you have a job. Someone will look up to you if you have a job. Like you know, you're like 20 something, you don't have a job [trailed off].

The purpose of pursuing school and work was at least in part oriented to establishing adult identities by engaging in the typical activities of youth peers. Working towards employment was an outcome in itself, and was consistent with a developmental trajectory towards normal adult life, regardless of whether or not the endpoint was achieved.

As mentioned, within both the documents and the participants' accounts, independent living was also associated with successful transitions. Sameer (21 years old) preferred living at home because it was more practical and easier for him, but his interview account reflected assumptions that linked independent living to achieving an adult identity:

(I want) just to prove to myself that, over the years it develops, independence, and now I am, I'm pretty independent to be on my own. You know, to see if I've developed fully all the skills that I need, just for my own personal ideals

For the most part, the young men in the study were ambivalent about moving out on their own. Participants worried about the expense, the quality of care they would receive, and developing the skills and confidence to direct personal care attendants. Peter (23 years old) described not being “mentally ready” to move out:

Interviewer: You felt it [moving out] was something you could do?

Peter: Something I could, yeah, yes.

Interviewer: So what changed your mind?

Peter: [nervous laugh] I don't know maybe I got a little scared, I don't know. Maybe I got a little bit worried. I don't know if I could handle it or not.

Joey's (16 years old) audio diary entry provides an additional example of a deep ambivalence to pursuing independent living:

I think it is important to be able to move out on your own, because your parents aren't always going to be there. And they can't just hold your hand your entire life. So it is important to be able to move out on your own. It's not necessarily that you have to; you don't have to move out. But it's a good idea to be able to.

In stating that “you don't have to move out,” Joey resists the notion of a singular pathway to transition. He sees the practical advantage of having the skills to live alone but also presents moving out as a personal choice.

Best practice discourses imply that moving out of the family home is an important symbol of achieving adulthood. Nevertheless, none of the participants, of any age, had moved out of their family homes and none had immediate plans to do so. The real possibility that participants had only a few more years to live was not directly addressed in their accounts; yet, the reality of the progressive nature of DMD formed a backdrop to the discussions and was reflected in their accounts. Planning to move out whether or not they actually ever moved out helped signal their entry into adulthood and conformed with the expectations in which they were immersed. Within developmental discourses, there is little space to express a preference for staying home with parents indefinitely. For example Michael (27 years old) who was the oldest participant in the study stated he would move out “once he makes some money” and Chris (24 years old) stated he would wait until he had a wife to “look after him”. These participants were well aware that they could move out without these supports in place but that it would be difficult. They preferred living at home where they received physical and emotional support. In this way, they both reproduced notions of a normative path to adulthood that they were working towards, and resisted this path by deferring a move and continuing to live with their parents.

Transitions best practices are oriented not just towards a good transfer from child to adult services in the short-term, but to achieving long-term outcomes beyond adolescence. Thus, best practices are very much about imagining a future adult life and are reflected in terminology, such as “life phase” (CPS, p. 785),“life span” (AAP, p. 1304) and “life course approach” (CanChild, p. 11). Participants in the study both reflected and resisted the future-focused social discourses reproduced in the best practices documents. Notions of “staying positive” and focusing on the present were pervasive across the accounts, and dwelling on future decline and/or escalating needs was avoided or discussed as unhealthy or negative. In his audio diary, Mark (16 years old) stated:

Nothing stops me and gets in the way of my life. I just stay positive …and don't worry about the future. I just have a positive attitude.

An audio diary entry of Peter (23 years old) reflected his preference for thinking positively in the present and deflecting decisions related to DMD:

I have to make a decision about having a ventilator when I am older to help me live. I don't think worrying about it is really going to help. So I'm just thinking positive and being happy, and that is all I should think about.

Resisting talking and thinking about uncertain futures achieves particular ends for young men with DMD by helping them to maintain positive psychological states, but these behaviors contrast rather dramatically with best practice notions of preparing for the future.

Discussion

Assumptions about what constitutes a successful transition to adulthood, including notions of increasing independence and approximating typical adult roles are largely unquestioned guiding principles in transitions best practices. In this article, we applied a critical lens to illuminate, unpack, and compare the assumptions embedded in influential transitions best practice documents, and the accounts of young men with DMD. The latter group, because of their shortened but shifting lifespans, provided an example of youth who may not fit into dominant formulations of transitions. Dominant principles of normal development pervaded the four transitions best practice documents reviewed. Expectations for gradual independence and the adoption of normal adult roles and competencies were taken for granted goals. Our analysis revealed that young men with DMD internalized these pervasive discourses but also resisted them in creative ways. The uncertainty of living with DMD was manifested in practices such as the deferment of the pursuit of adult milestones, and philosophies oriented to living well in the present.

Our findings regarding how youth took up or resisted developmental discourses resonate with other research. Studies of young people with cerebral palsy, cystic fibrosis (CF), and congenital heart disease have revealed a similar focus on academic achievement and employment [11,27]. Participants across these studies accepted implicitly that pursuit of employment was the proper goal for someone their age, even when they acknowledged that declining physical abilities and fatigue made working increasingly challenging to maintain. Philosophies of living well in the present reflected in our data, were also seen in Abbott, Carpenter and Bushby's [14] study of transitions for young men with DMD. The young men in their study avoided thinking about living with DMD too much because it made it difficult for them to stay positive. Similarly, Moola and Norman [11] found that youth with CF used several strategies to cope with their life-limiting condition, such as choosing to do things now rather than later, and enjoying the present without depending on the future.

In Western neoliberal societies, children's dependency is socially acceptable whereas dependent adults are seen as problems that do not conform to norms of ideal citizens, that is, self-sufficient, financially independent, employed, and productive [20]. These traits of adulthood, valued in able-bodied and adult-centered societies, shape understandings of what is normal and expected of young people and adults alike [20]. The aim of transitions practices is to move youth along a continuum until these milestones of adult status are achieved. In our analysis we have suggested that, while these efforts can have multiple positive effects in the lives of young people, they also may be inappropriate or even result in some unanticipated harms. The latter may include inadvertently deepening the exclusion of youth with complex and progressive impairments who cannot easily achieve adult milestones because of escalating loss of function and myriad external barriers to participation. Practices focused on evaluating milestones and timeframes may only serve to accentuate what is not possible for youth with DMD and increase their sense of exclusion [11].

Normal development or “developmentalism” as a basis for intervention has been widely critiqued across the fields of psychology, sociology, and disability studies [26,28–31]. Even though development is only one way among others to understand the life course, it is so dominant that it is rarely questioned in health and rehabilitative care practices. Critics assert that development has evolved into a powerful instrument of categorization that allows value-loaded judgments regarding what is best for children. This is not to say that the health and rehabilitation fields should abandon current transitions approaches for youth with disabilities. However, an unreflective pursuit of normal social roles and milestones may draw attention away from giving greater significance to other activities and ways of being and living [5]. Alternate pursuits might include activities oriented to living well in the present, such as getting together with friends, participating in individual or group recreational activities, and going on community outings, as alternatives to working full time work or even working at all [32]. Furthermore, understanding youth as a social and generational category in its own right, with its own expectations and possibilities, rather than being understood as “un-adult” or transitional, might promote development of interventions focused on maximizing well-being in the present [20].

Current best practice guidelines aimed at a broad population of youth with chronic conditions address generic concerns related to transition to adulthood and do not account for various etiologies nor the effects of these variations on the lived experiences of youth [33]. The paradox, faced by those with progressive conditions, of thinking and planning for the future within a precarious lifespan, needs to be more fully articulated and addressed in health care practices. This might mean finding sensitive ways to balance thinking about the present and the future, or particularizing goals and possible pathways to match individual circumstances and priorities. For example, presenting youth with a menu of options – some future focused (work), other focused on the present (daily activities) or near future (developing new relationships) – supporting their choices, and allowing them to move between these options as their priorities change. Importantly, the subtle and pervasive ways that “normal” roles are promoted as the only path, may need to be actively countered through ongoing dialogue, values clarification, and exposure to alternative ideas and role models. At a minimum, findings from this analysis highlight the need for health and rehabilitation professionals to be aware of the effects of implicit assumptions underlying transitions best practice discourses and to assess these effects in practice.

Declaration of interest

The “Becoming Men” study was supported by the Canadian Institutes for Health Research (grant number 103245). Barbara Gibson holds the Bloorview Children's Hospital Foundation Chair in Childhood Disability Studies. The other authors report no declarations of interest.

Acknowledgements

We would like to sincerely thank the study participants for sharing their experiences. We also thank Gail Teachman for providing feedback on a manuscript draft and Ian Barrett for assisting in manuscript formatting. We would like to acknowledge the other members of the research team involved in the “Becoming Men” study: Brett Smith, Karen Yoshida, David Abbott and Sally Lindsay.