Medical care of adults with spina bifida

Survival into adulthood for individuals with spina bifida has significantly improved over the last 40 years. Health services research suggests the majority of patients with spina bifida are now over 18 years old. Adults with spina bifida have ongoing increased medical needs compared to the general population, including spina bifida-specific care, age-related secondary disabilities, and general adult medical needs. Unlike pediatric-aged patients, adults may not have access to multi-disciplinary spina bifida clinics and must often coordinate their own care with adult providers less familiar with spina bifida and the associated monitoring and treatment issues. This article will provide an overview of the medical issues of adults with spina bifida, highlighting areas that are different than pediatric care.     

Development of health support services for adults with spina bifida

Purpose.?To describe, within a state-wide transition framework, the pathway to improved health support services for adults with spina bifida. Our aim was to achieve adult care that addressed adult health issues, despite significant budgetary restraints and competition for the public health dollar.

Method.?A pilot clinical service project in one of the Area Health Services and a transition workforce study were carried out in the state of NSW in Australia. The advocacy skills of clinicians were harnessed.

Results.?A regional adult spina bifida clinic was successfully created and patient resources were developed. Ongoing funding was not granted. A transition workforce study identified the poor clinic attendance of adult spina bifida patients and the high costs of hospital admissions for spina bifida related complications. Through advocacy and data provision, health funding was provided for two health support workers for a new service, the Spina Bifida Adult Resource Team.

Conclusions.?An effective transition to adult care is essential for chronic childhood illness, but young people with chronic illness often have no significant voice. Advocacy for public health spending requires compelling evidence which we were able to provide and which could be a model for other service providers.     

Urologic issues of the pediatric spina bifida patient: A review of the genitourinary concerns and urologic care during childhood and adolescence

Myelodysplasia is a complex patient problem that continues to present challenges for multidisciplinary care teams. The spectrum of bladder and bowel dysfunction created by this neurologic lesion is broad and requires long-term surveillance of these organ systems. This review outlines the diagnosis and management of urologic issues of the spina bifida patient throughout infancy, childhood and adolescence. Specifically, examining different approaches to care of these patients (proactive vs reactive), quality of life issues and medical and surgical management options and decision-making at each phase of life.     

Adolescents born with spina bifida: experiential worlds and biopsychosocial developmental challenges

The author used phenomenological (interpretive) ethnography to investigate the experience of physical disability and its attached meanings in relation to self, world, and other for adolescents born with spina bifida. Multiple in-depth interviews were conducted with 11 late-stage adolescents (aged 18-24 years). Analysis of their narrative accounts illustrated commonalities with respect to the biopsychosocial impact of living with the physical disability of spina bifida. The theme, "Experiencing self as dissimilar other" is reported upon. Findings imply that youth born with spina bifida face biological, psychological, and social challenges that might interfere with normative developmental tasks of adolescence, including identity formation. Greater emphasis needs to be directed toward humanizing and emancipating the physical and social environment for youth with physical disabilities to maximize developmental opportunities and potential while fostering positive identity.     

Risky business: Preventing skin breakdown in children with spina bifida

The purpose of this article is to provide an overview of skin issues in children with spina bifida. Included in the discussion below is a review of the etiology of pressure ulcers and the updated 2007 pressure ulcer definition and pressure ulcer staging system as defined by the National Pressure Ulcer Advisory Panel (NPUAP). Pediatric risk factors for skin breakdown are presented including risk factors unique to children with spina bifida. Pediatric pressure ulcer risk assessment scales are described. The 5 Million Lives Kids' Campaign which has a focus on preventing hospital-acquired pressure ulcers in children is also reviewed along with evidence based prevention strategies. The key to preventing skin breakdown and pressure ulcers in children with spina bifida is early identification of the child's individual risk factors so that a prevention protocol can be implemented in all settings: hospital, home and the community. Options for wound management, dressing selection and pain management are included.     

Primary care providers and medical homes for individuals with spina bifida

The contributions of primary care providers to the successful care of children with spina bifida cannot be underestimated. Overcoming systemic barriers to their integration into a comprehensive care system is essential. By providing routine and disability specific care through the structure of a Medical Home, they are often the first line resource and support for individuals and their families. The Medical Home model encourages primary care providers to facilitate discussions on topics as varied as education and employment. Knowledge of specific medical issues unique to this population allows the primary care provider to complement the efforts of other specialty clinics and providers in often neglected areas such as sexual health, obesity and latex sensitization. As individuals with spina bifida live into adulthood, and access to traditional multidisciplinary care models evolves, these skills will take on increasing importance within the scope of providing comprehensive and coordinated care.     

Needs assessment in a spina bifida program: a comparison of the perceptions by adolescents with spina bifida and their parents

The primary purpose of this study was to describe and compare medical, psychosocial, and economic needs of adolescents and young adults with a neural tube defect and their parents. A secondary aim of the study was to establish the psychometric properties of a previously published needs assessment tool. Forty-nine adolescent and 59 parents receiving services from a comprehensive spina bifida program at a large children's hospital completed a needs assessment survey designed to measure perceived needs for health and social services. The Needs Assessment Questionnaire includes 8 subscales (Accessibility/Transportation/Independence, Financial Concerns, Medical Resources, Communication/Acceptance of individuals with spina bifida, Family/Socialization, Education of others regarding spina bifida, School Services, and Vocational Training). Factors related to perceived needs and differences between adolescents and parents were explored. There was a significant difference ( <.05) between parents and adolescents in 6 of the 8 subscales. On the remaining 2 subscales, differences between parents and adolescents approached significance. In all areas of needs for services, parents were less satisfied with the delivery of services and reported that needs were not being met. A significant negative correlation between age of the adolescent and perceived needs related to Transportation/Independence ( = -0.35) and School Services ( = -0.31) was found. Parents with more than a high school education perceived significantly greater needs for Vocational Training than those with less education ( = -2.1, =.04). Identification of medical, psychosocial, and economic needs of adolescents with a neural tube defect and their families has implications for quality improvement and program development.     

Is autonomy related to the quality of performance of everyday activities in children with spina bifida?

Purpose: To investigate the relationship between the level of autonomy and the quality of performance of everyday activities in a population-based cohort of children with spina bifida and to study the agreement between the children’s and the parents’ ratings of autonomy. Methods: 50 dyads of children (aged 6–14) with spina bifida and their parents rated the children’s level of autonomy with an adapted, Swedish version of the Autonomy Scale from the Arc’s Self-Determination Scale. Each child’s quality of performance of everyday activities was assessed with the Assessment of Motor and Process Skills (AMPS). Results: The autonomy levels of the children with spina bifida were rated to be lowest in daily routines and highest in leisure activities. Binary logistic regression analyses revealed that age, motor skills and process skills were all significantly related to the autonomy level, but that process skills appeared to predominate in this respect. Concerning the perception of the autonomy level, little agreement was found between each child and his/her parent. Conclusions: It is important to understand and support the development of process skills as expressed in task performance in children with spina bifida and to pay attention to both the parent’s and the child’s opinion when setting goals and plans for interventions.

Implications for Rehabilitation

• Autonomy is vital for independence and participation in life situations and has been found to be at a low level in goal-related activities in children with spina bifida.

• This population-based study found that the level of autonomy was strongly related to the quality of performance and in particular to process skills as measured with the instrument Assessment of Motor and Process Skills (AMPS).

• It is crucial to assess and support the development of process skills, as expressed in task performance, in children with spina bifida, as well as to discuss and support issues of autonomy.

• This study showed that the children with spina bifida and their parents had different perceptions of the child’s level of autonomy, which indicates that both these opinions have to be taken into consideration when planning interventions.

     

Families of Children with Spina Bifida: A Review

Spina bifida is a congenital condition of the nervous system. It typically results in paralysis of the lower limbs, hydrocephalus, incontinence of bowel and bladder, lack of sensation below the lesion, and dislocated hips. Children with spina bifida have to cope with lifelong medical care. Despite the fact that spina bifida dates back to Stone Age, its etiology remains unknown. Families of children with spina bifida have received some research attention. Researchers have primarily examined the ways in which spina bifida impinges upon the family's finances, parents' physical/emotional health, marital relationships, social life, caretaking responsibilities, care of other children, attitude toward the disabled child, and career/social/recreational opportunities. However, because of several methodological limitations, the current research findings are inconclusive. There is need for longitudinal studies to really understand the impact of spina bifida on the family. Also, in view of the research that shows spina bifida adults are comparable to their nondisabled peers on significant psychological variables, there is need to understand strengths and positive coping strategies of families of children with spina bifida.     

Health care transition for adolescents with special healthcare needs: Where is nursing?

The population of adolescents with special healthcare needs (ASHCN) surviving into adulthood has increased dramatically over the past two decades. Approximately, nine of every 10 children diagnosed with a chronic condition are expected to reach adulthood. Experts estimate nearly 750,000 ASHCN enter into adulthood each year. Advances in medical treatments, new technologies and scientific discoveries have all contributed to the increases in ASHCN life expectancies. As a result, new demands for services have emerged to address their clear needs for long-term services and supports. Foremost among the ASHCN service needs are healthcare transition services. Healthcare transition is recognized as a needed area of practice to facilitate ASHCN transfer of care from pediatric to adult healthcare and to support the acquisition of the developmental competencies needed to successfully transition to adulthood. Yet, few evidence-based and exemplary models of care exist. Healthcare transition research is in the early stages of development. The medical community has provided the leadership with the development of healthcare transition policy, practice, and research. As a result, policymaking, practice issues, and research have a prominent medically related focus. In contrast, the influence of nursing as it pertains to these areas of professional practice is limited. Opportunities exist for pediatric and child health nursing leaders to provide direction for greater involvement in this emerging and growing field of specialty practice.     

Perspectives of school-work transitions among youth with spina bifida,their parents and health care providers

Purpose Youth with disabilities encounter many challenges in finding employment and transitioning to post-secondary education. In this article, we explore the nature of school-work transitions for youth with spina bifida, which few studies have previously addressed. Method We conducted in-depth interviews with 44 participants (nine youth and 12 young adults with spina bifida, 11 parents and 12 clinicians). We analyzed our data using a qualitative content analysis approach. Results We identified several enablers to school-work transitions, including professional support, social support and school accommodations. Lacking such support created several challenges for youth with spina bifida. We also identified several barriers to school-work transitions, including lack of education and information on finances, housing and transportation; discrimination and stigma; and challenges coping with spina bifida at school and work. Conclusions Although some supports are available, youth with spina bifida encounter many challenges in transitioning to post-secondary education and employment. With clinicians at the helm, efforts should be put in place within the health and social services system to better prepare youth, as they transition to adulthood, to become connected to appropriate information and resources to help them to succeed in their school and employment goals.

• Implications for Rehabilitation

• Youth with spina bifida encounter many challenges in transitioning to post-secondary education or work. Clinicians, educators and parents should continue to support youth with spina bifida in choosing their vocations, setting career goals and developing career plans.

• Starting from a young age, parents, clinicians and educators should help youth with spina bifida prepare for school work transitions by fostering their independence, life skills and self-management of spina bifida-related symptoms.

• Clinicians and educators should help connect youth with spina bifida to appropriate information and resources to support their school-work transitions, including information on finances, housing, transportation, school accommodations and employment supports.

     

胎儿开放性与闭合性脊柱裂的产前诊断及分类

近年来产前诊断不断发展,胎儿脊柱裂的筛查指标及方案也在不断更新,脊柱裂的检出率越来越高,但由于产前对脊柱裂的分类尚存在争议,也引发了一系列医疗纠纷。本文综合临床特征与超声表现,明确指出可供产前诊断参考的胎儿脊柱裂的定义与分类,从胚胎发育学角度阐述该畸形的发生发展,总结了一系列具有实用价值的脊柱裂产前诊断与鉴别诊断方法,旨在为产前明确诊断、开展宫内治疗提供参考依据。     

胎儿脊柱裂发生机制及超声诊断临床价值

目的探讨胎儿脊柱裂发生机制及超声表现,分析产前超声对此类畸形的诊断价值。方法回顾性分析14例产前诊断为胎儿脊柱裂的超声表现,并经引产或生产后放射影像学资料对比证实,总结其声像图特点。结果产前超声诊断脊柱裂14例,其中12例显性脊柱裂,2例隐性脊柱裂。胎儿脊柱裂的超声表现具有特征性。超声诊断与产后诊断符合率达100%。结论产前超声检查可以准确地诊断胎儿脊柱裂,具有可靠临床应用价值。     

脊柱裂胎儿小脑延髓池改变的超声研究

目的探讨超声检测胎儿小脑延髓池（CM）前后径对提示脊柱裂诊断的价值。方法胎儿脊柱裂36例,对照组为相应孕周的108例正常胎儿。经小脑横切面观察胎儿小脑形态、结构,并测量CM前后径。结果36例脊柱裂胎儿,囊性脊柱裂33例,隐性脊柱裂3例。21～36孕周胎儿34例,声像图表现为CM消失;16～20孕周胎儿2例,声像图表现为CM缩小。对照组108例胎儿,6例CM缩小,无CM消失。脊柱裂胎儿CM消失的发生率高于对照组（P〈0．05）。结论孕21周以后胎儿,CM消失是胎儿脊柱裂超声检查重要的间接征象。     

A conceptual model for the development of professional behaviours in occupational therapists

The ever-changing, dynamic practice environment coupled with increased consumer needs and awareness create an atmosphere that requires optimal professionalism from occupational therapists. Professionalism requires specific knowledge, attitudes, and values--all manifested by professional behaviours. The authors assume that professional behaviours mature through a natural developmental process; a process that requires careful nurturing on the part of educators and clinical supervisors. Based on this assumption, the authors propose this conceptual model based on Erikson's life cycle stages. The model implies that occupational therapy professional behaviours develop sequentially through stages that begin during the educational process of occupational therapists, and progress throughout their career. The purpose of this model is to provide a framework for educators and supervisors to nurture professional behaviours in students and novice clinicians, and to continue their own professional growth.     

Neurogenic bowel and continence programs for the individual with spina bifida

The neurogenic bowel in the person with spina bifida has been difficult to manage. Current literature reveals very little research conducted on the outcome of continence programs in the person with spina bifida. It has different characteristics than the neurogenic bowel seen with spinal cord injury because it occurs during fetal development. Management of the neurogenic bowel to achieve continence is based upon thirteen assessment items that include stool form, individual physiologic parameters, family patterns, diet and medications. Programs are developmentally related to appropriately support the child's involvement. Continence strategies include the use of oral medication early in the day and timed sitting, suppositories or enemas later in the day at a time convenient to the family. The timing of the medications and evacuation is very important to the success. An interdisciplinary team is important in supporting the family as the child with spina bifida grows into an independent adult. The dietician and occupational therapist have invaluable insight into diet, fluids, adaptive aids and techniques that contribute to positive outcomes of the continence program.     

Psychosocial stages and quality of life of women with breast cancer

Certain issues are universal for all women with breast cancer, irrespective of age, ethnic group, or stage of disease. Yet, along with common concerns, experiences, and anxieties, each woman may encounter a unique set of problems. Ultimately, each woman's adaptation and choices will be strongly influenced by her personal history, her psychosocial stage, and her life-cycle concerns. Changes in the criteria norms for the psychosocial stages of women's lives and their subsequent influence on quality of life are issues that have substantial implications for nursing and other healthcare professions. Younger and older women have different needs, concerns, and quality of life issues in a context of psychosocial life stages that have changed significantly across the post-World War II generations. The experience of breast cancer is perceived differently by women of distinct psychosocial life stages. At each critical life stage, the unique emerging problems require specific psychosocial supports that can reduce or avert the ensuing emotional distress. The planning and implementation of care must be tailored to address the differences demonstrated by age and psychosocial life stage, and to enhance quality of life outcomes for survivors of breast cancer, both young and old.     

A brighter future for adults who have myelomeningocele--one form of spina bifida.

Recently a new population of adults with myelomeningocele, the most severe form of spina bifida, has emerged. Because of the complex issues challenging these adults, specialized health care services provided by a multispecialty team are recommended. With the hope that similar services will be developed, the establishment of one specific teen/adult spina bifida multispecialty team is discussed in this article. An overview of myelomeningocele is presented, which includes adult focused physical/cognitive/social-emotional challenges and goals, potential medical problems, and opportunities for nurse leadership.     

Sexual functioning in adolescents and young adults with spina bifida

Cardenas DD, Topolski TD, White CJ, McLaughlin JF, Walker WO. Sexual functioning in adolescents and young adults with spina bifida.

Objective

To assess sexual education and sexual functioning in adolescents and young adults with spina bifida.

Design

Survey, inception cohort.

Setting

The community.

Participants

A cohort of adolescents and young adults (N=121; range, 15-35y; 58% women) enrolled in a longitudinal pediatric database.

Interventions

Not applicable.

Main Outcome Measures

Questions on sexual function, reproductive function, bladder and bowel continence, the Perceived Quality of Life Scale, and the Satisfaction With Life Scale.

Results

Almost all adolescents and young adults with spina bifida in our study received sexual education at school, less at home, or by physicians. Twenty-five percent of men and 68% of women were informed about reproductive function by their physicians. Participants who reported that they smoked were 10 times more likely to report being sexually active and women were 2.3 times more likely to be sexually active than men. Hydrocephalus was a significant predictor of sexual activity among women but not men. Participants with urinary incontinence were less likely to be sexually active. Women without hydrocephalus were significantly more satisfied with life than women with hydrocephalus.

Conclusions

Adolescents and young adults with spina bifida in this sample were only slightly satisfied with life and sexual activity was only associated with life satisfaction among women. Dissatisfaction with life often leads to engagement in health-risk behaviors, which may, in part, account for the association between sexual activity and smoking behavior observed in these data. Further studies of health risk behaviors among youth with spina bifida are warranted and interventions aimed at reducing health risk behaviors among adolescents and young adults should specifically include spina bifida as a target group.