Self-management and independence guidelines for the care of people with spina bifida

1. Introduction

Research suggests that more than 75% of individuals born with spina bifida [SB] will survive into adulthood [2] with evidence underscoring the critical importance of self-management in extending life expectancy. According to the Guidelines for the Care of People with Spina Bifida [3]:

Self-management for youth and emerging adults with Spina Bifida is an active daily and flexible process in which youth and their parents share responsibility and decision-making for managing their condition, health, and well-being through a wide range of knowledge, attitudes, activities, and skills. The goal of this increasing responsibility is to develop the self-management behaviors needed to achieve independence and transition to adulthood and independent living [4, 5].

Self-management may also be thought of as the interaction of health behaviors that affected individuals and families engage in to care for a chronic condition [6]. Within the SB population, effective self-management is particularly important for preventing co-morbidities and secondary complications such as skin breakdown, renal dysfunction, and bladder and bowel incontinence [7, 8]. Self-management is also central to a successful transition from adolescence into emerging adulthood, participating fully in society, and combating vulnerability, stigma, and discrimination. SB is a congenital condition; therefore the building blocks of self-management must begin early in childhood and in partnership with families and multi-disciplinary providers [9]. Therefore, these guidelines begin in infancy and progress through adulthood.

Child autonomy provides a critical foundation for developing self-management and independence. For all children, autonomy begins early and is fostered by opportunities to make choices and to develop a sense of mastery. Most children with SB ultimately achieve basic self-management and independence behaviors (e.g., dressing appropriately, planning activities with peers, or cooking pre-planned meals) yet often lag 2–5 years behind their typically-developing peers in these behaviors [1]. This gap may be due to the child’s difficulties performing common everyday motor and processing activities in efficient and independent ways [1]. Adaptation of performance and initiation of new steps may be especially challenging [10].

Social skills in children are also important building blocks for independence. Many children with SB need assistance building adaptive social behaviors in peer interactions, specifically basic social skills such as reading social cues, clarity of thought, and collaboration [10]. Monitoring self-management learning is needed for all with cognitive functioning challenges, especially those with executive functioning, inattention, and working memory issues [11, 12, 13, 14, 15]. Educational programs in the home, school, and broader community that offer opportunities to practice new behaviors are critical.

Youth with SB often do not enter adolescence with a comprehensive knowledge base of how to self-manage their condition (i.e. watching for signs of skin breakdown, bowel problems, shunt failure, and urinary tract infections) yet most develop this knowledge before age eighteen [1, 4, 16]. Advanced self-management behaviors for community living (i.e., managing a bank account, cooking independently) are typically achieved by peers without complex disabilities by age 18, but not as commonly by individuals with SB [1]. It is not clear if these difficulties reflect a delay in development for youth with SB or are due to a lack of expectations and support in the home, school, health system, or broader community. However, research suggests healthy family functioning characterized by open communication and shared decision-making is related to more optimal self-management outcomes across all developmental stages [13, 16].

Since there is evidence that responsibility in the home (e.g., chores and general decision-making) promotes self-management and skill-building, individuals and families should be encouraged to expand the range of everyday living skills and responsibilities for youth with SB [1, 16, 17, 18, 19, 20, 21]. These findings are complicated by longitudinal studies indicating that older school-age children with SB perceive themselves as being more independent relative to parent assessment [21]. Moreover, while health care providers expect school-age and older children to perform self-management behaviors related to bladder programs, bowel programs, skin checks, and prevention of other secondary conditions [22], older children have reported that self-catheterization and bowel programs were a challenge and often required parent involvement [4]. In addition, both the families and children have had difficulty carrying out diet recommendations, bowel programs, and skin care [23]. Thus, tailored interventions are needed to support growth in these areas [23].

Adolescents with SB transitioning to emerging adulthood are generally poorly prepared to self-manage their condition or live independently and are therefore at risk for preventable secondary conditions such as skin breakdown and UTIs [24]. This vulnerability appears to persist into adulthood [25]. Other research has identified associations between lower self-management behaviors and higher hospitalization rates in this population [7, 26]. However, there is evidence that improved self-management in young adults is associated with decreased depressive symptomology [27]. In addition, there is evidence in other conditions such as asthma that increased self-management improves health outcomes and healthcare utilization [28, 29, 30, 31]. Most adults with SB over 18 years of age have not achieved optimal independence milestones in education, employment, and independent living [32]. However, they were reported to have higher independence than those with other severe conditions such as fragile X syndrome or muscular dystrophy [33].

Self-management interventions for youth with SB and other chronic health conditions generally show significant improvement in at least one area, although a short workshop-based intervention for older children with SB yielded no significant differences in intervention and control groups [34, 35]. Family-oriented self-management interventions may be most effective in younger adolescents [36]. Camp-based psychosocial interventions promoting skills-development in goal-setting and problem-solving have shown promise in developmentally-diverse samples of children, adolescents, and adults with SB [11, 37]. Another intervention using a problem-solving model combining education and home/community practice similarly improved perceived family stress associated with SB condition management but not youth self-management [36]. Rehabilitation interventions in young and middle-aged adults have improved all aspects of self-management and independence, with moderate- to large-effect sizes including self-efficacy, management of bowel and bladder incontinence, cognitive function, and psychosocial symptoms [8, 26]. Although tested mostly in adults, technology-based interventions hold promise for expanding self-management behaviors in youth as well [20, 38, 39, 40, 41, 42].