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

Abstract

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.

Quality of Life and Psychosocial Well-Being in Youth With Neuromuscular Disorders Who Are Wheelchair Users: A Systematic Review

“…Hamdani et al 48 described four participants as NIV users, but no other descriptors of steroid, spinal fusion or NIV uptake were reported in any of these studies. Mid-adolescent boys identified practical and interpersonal barriers to independent socialisation including assisted toileting 42 .…”

“…One study was a survey conducted internationally 40 and three each were conducted in Australia [41][42][43] and the United States [44][45][46] . Nine studies recruited through tertiary clinics 43,44,[47][48][49][50][51][52][53] , five studies through multiple sources 40-42, 46, 54 and one study did not report their participant source 45 .…”

“…Three qualitative studies 42,45,48 reported positive and negative accounts of psychosocial wellbeing (Table 4). Hamdani et al 48 described four participants as NIV users, but no other descriptors of steroid, spinal fusion or NIV uptake were reported in any of these studies.…”

“…Five studies described all participants as wheelchair users 40,45,[47][48][49] and three recruited only boys with DMD of whom the majority were wheelchair users 41,44,46 . Two studies recruited by functional dependence on NIV 43,50 , one included only those with compromised sitting ability having undergone spinal fusion 52 and all three reported extremely low physical scores, hence they were assumed to include only non-ambulant participants.…”

“…In the eight studies that recruited participants with a larger age range, data of 12-22 year olds could be separately extracted 40,43,[46][47][48][49]54 . In the remaining 4 studies 41,44,50,52 participants' mean age fell within the specific age range of this review, with the age of outliers ranging from 8 44 to 25 years old 50 .…”