Abstract
The Spina Bifida Association (SBA) is the organization that represents the needs of the population with spina bifida (SB). They are tasked with advocacy, education, optimizing care, and providing a social voice for those with spina bifida. In response to the tenet of optimizing care they were tasked with developing up to date clinical care guidelines which address health care needs for those impacted by spina bifida throughout their lifespan. This article will discuss the SB Mobility Healthcare Guidelines from the 2018 Spina Bifida Association’s Fourth Edition of the Guidelines for the Care of People with Spina Bifida.
Introduction
The Spina Bifida Association (SBA) is the national organization in the United States representing individuals of all ages with spina bifida. One of the tenets of the SBA is to be the driving force behind providing the best care possible for this population. High quality best practice guidelines are a method to achieve this goal. Guidelines not only improve quality of care but provide a framework of standardized practice [1]. The Guidelines for the Care of People with Spina Bifida, as discussed here, followed a well-defined formalized process of development [2]. These guidelines are multifaceted; however this discussion is focused on aspects of mobility and how this impacts the health and well-being of those with spina bifida.
Mobility is defined as the ability to move within a person’s environment including the home and community. Methods of mobility will be different for each individual based on multiple factors. Different approaches will need to be used based on functioning neurologic levels [3, 4]. Some children will be ambulatory without assistive interventions while others will need bracing, assistive devices or wheelchairs to navigate the home and community. Mobility is a fundamental learning experience for young children. It is well known that the childhood experience of exploring their environment leads to enhanced cognitive and psychosocial development [5]. Children with spina bifida may have significant physical limitations that can impede this natural exploration and impact developmental progress. Health care providers recognize the importance of ensuring that young people with spina bifida have opportunities to engage in meaningful activities. In addition, mobility needs may change as an individual ages; therefore it is important for health care providers to factor in the aging process. Having clear mobility guidelines, based on clinical experience and evidence-based information, can provide practitioners across the country with the best tools to care for their patients.
Guidelines goals and outcomes
The goals of the mobility guidelines were both practical and aspirational and divided into the three areas listed below.
Primary
Develop expectations for mobility based on age and neurologic
level. Understand and utilize appropriate mobility devices and therapy
interventions to optimize mobility across the age spectrum.
Secondary
Reduce the threats and effects of pain, aging, neurologic
deterioration, and obesity on mobility. Reduce risk of pressure injuries. (Integument (Skin) Guidelines) Maximize safe functional mobility and acquisition of developmental
milestones for social and environmental exploration. Maximize safe and functional mobility for Activities of Daily Living
(ADL), as well as, social, recreational, and pre-vocational/vocational goals.
Tertiary
Understand how primary and secondary outcomes affect quality of
life.
Methods
The mobility group was tasked with researching and developing guidelines of care for individuals with SB related to mobility across the age spectrum. This topic was in the previous set of guidelines and was identified by the SBA as needing an update [6]. The initial phase of this process included searching current and historical evidence-based research and recruiting clinicians/scientists with expertise in the identified topic area. The guideline development process for each topic group was well defined by SBA with timelines and working phases [7]. The mobility working group included physicians and physical therapists with a long track record of clinical and research expertise. The first task for this group was to review the previous guidelines and the International Classification of Functioning, Disability and Health (ICF) model and then develop the mobility guideline goals and objectives (noted above). Since the team was geographically separated the majority of work was done via electronic media conferencing. The team was then tasked with developing clinical questions that would direct best care for the SB population. In addition to relying on clinical expertise, questions were developed and reviewed by a panel of consumers either with SB or family members of a child/adult with SB. They were then asked to rank the clinical questions based on importance to the population. It was extremely important during this phase of guideline development to get feedback from families and individuals so as to identify what is really important in their day to day life and to understand the long-term needs of the SB population from their perspective. The group could comment on the existing questions and were also able to propose new ones.
The next phase was to do a review of scientific literature that had been identified by the SBA executive committee as being relevant to mobility issues for individuals impacted by SB. It was necessary for the mobility group to use historical articles as they provided a framework for relating neurologic level to impairment and ultimately the mobility of an individual. They also had to use data on normal development related to social and cognitive outcomes in young children.
Phase 3 methodology is well described in the article by Dicianno et al. which reviewed the group consensus process and eventually led to the guidelines being submitted to the SB oversight panel [7].
Results
The literature review resulted in the team identifying 19 journal articles that were specific to mobility. There was limited research available for many of the topics, so the group had to expand the literature search to articles related to development.
In areas where there was limited information for guidance the team had to develop an expert consensus. When members could not reach consensus, the Expert Consensus Decision Pathway was used to achieve resolution [8].
The clinical questions developed by the mobility group
The clinical questions developed by the mobility group
Current clinical guidelines for mobility
Movement is a basic human need for both survival and development. We no longer have to “run from the lions” but we still retain that need to move. Mobility, the quality or state of being physically mobile, characterizes a person’s ability to move within their environment. The quality of mobility can be compromised to varying degrees in individuals with SB. We know that movement and mobility follow a predictable pattern of development and that impaired neurologic function will impact this process. Children with spina bifida have neurologic lesions that affect both motor and sensory function and can impact mobility [9]. Using this information clinicians can predict the level of mobility a person will ultimately be able to achieve. Therefore, understanding how neurologic lesions can impact mobility provides the context for an honest and directed discussion with families regarding that common first question of “will my baby walk?”
Early research in children shows that mobility impacts not only physical parameters but cognitive and psychosocial factors. Children need to move to develop. Typically, motor development is a head to toe process [10]. The baby must overcome gravity and learn postural control. This may be impaired in children with spina bifida – therefore the guidelines around “back to sleep” and “prone to play” should be encouraged [11]. These early programs are essential for motor control and psychosocial development in all children, but may be even more critical for children with disabilities.
Functional postural strategies used in therapy are critical for early foundational elements of mobility. Developmental milestones in children with SB may be delayed or need adaptive strategies. At an age when a child should normally be sitting or standing the use of adaptive equipment is perfectly acceptable in getting them upright. Having an understanding of an individual’s potential will help guide therapists in defining strategies to facilitate movement.
Mobility can take on many faces including the use of a wheelchair as a way to navigate the environment. Based on the individual’s neurologic and functional level, early adoption of a wheelchair ]may allow a child a capacity to explore their environment that might otherwise be compromised. The timing of when a person may choose to transition to using a wheelchair for primary mobility is a very personal decision. However, individuals should be made aware that this option may enhance mobility and quality of life.
These guidelines were developed to help care providers and families optimize mobility across the age spectrum. Needs will change over time, but the benefits of mobility remain the same. Changes in mobility due to secondary complications or conditions, and the effects of aging and overuse are not well understood. Best practices that seek to understand and ameliorate threats to maintaining healthy mobility need to be developed through future research.
Below is a list of some known benefits of mobility:
Contracture management Exercise: cardiovascular-respiratory effects Strength effects and endurance Community engagement/household mobility Bone density Bowel and bladder evacuation Facilitates ability to perform self-care activities Pressure reduction/redistribution Improve underlying pain Reduce stress
The guidelines related to physical activity and wellness are additional resources related to benefits from movement [12].
There are a number of validated measures that a clinician can use to assess mobility Scales such as the Hoffer scale, Dias Functional Mobility Score, Pediatric Neuromuscular Recovery Scale (Peds NRS), and spatiotemporal gait parameters in children with myelomeningocele (MMC) are just a few employed today. With the advent of actigraphy monitoring, movement and mobility are able to be quantified and correlated with these scales. The authors feel that measures to follow neurologic changes and track improvements should be used in clinical practice. Recent data has shown that orthotics and gait aides positively impact stride and oxygen consumption [13, 14]. As we begin to understand these concepts, we can use this information to keep our patients with spina bifida moving longer and more efficiently.
Conclusion
Movement and mobility are often impaired in the SB population. The SBA has developed the current guidelines to help families and health care professionals optimize care by providing direction in approaching this population [2]. We celebrate victories and embrace challenges to advance the care and science of those with spina bifida. Further research will need to be done to improve our understanding of mobility and its physical and psychosocial benefits. Research gaps in secondary complications and medical conditions that impact mobility may provide insight on how to best manage mobility over time. Movement is fundamental to the human experience and we are entrusted to maximize a person’s potential using the above guidelines.
Footnotes
Acknowledgments
This edition of the Journal of Pediatric Rehabilitation Medicine includes manuscripts based on the most recent “Guidelines For the Care of People with Spina Bifida,” developed by the Spina Bifida Association. Thank you to the Spina Bifida Association for allowing the guidelines to be published in this forum and making them Open Access.
The Spina Bifida Association has already embarked on a systematic process for reviewing and updating the guidelines. Future guidelines updates will be made available as they are completed.
Executive Committee
Timothy J. Brei, MD, Spina Bifida Association Medical Director;
Developmental Pediatrician, Professor, Seattle Children’s Hospital Sara Struwe, MPA, Spina Bifida Association President & Chief
Executive Officer Patricia Beierwaltes, DPN, CPNP, Guideline Steering Committee
Co-Chair; Assistant Professor, Nursing, Minnesota State University, Mankato Brad E. Dicianno, MD, Guideline Steering Committee Co-Chair; Associate
Medical Director and Chair of Spina Bifida Association’s Professional Advisory
Council; Associate Professor, Department of Physical Medicine and Rehabilitation,
University of Pittsburgh School of Medicine Nienke Dosa MD, MPH, Guideline Steering Committee Co-Chair; Upstate
Foundation Professor of Child Health Policy; SUNY Upstate Medical University Lisa Raman, RN, MScANP, MEd, former Spina Bifida Association Director,
Patient and Clinical Services Jerome B. Chelliah, MD, MPH, Johns Hopkins Bloomberg School of Public
Health
Additional acknowledgements
Julie Bolen, PhD, MPH, Lead Health Scientist, Rare Disorders Health
Outcomes Team, National Center on Birth Defects and Developmental Disabilities,
Centers for Disease Control and Prevention Adrienne Herron, PhD Behavioral Scientist, Intervention Research Team,
National Center for HIV/AIDS, Viral Hepatitis, STD, and TB Prevention, Centers for
Disease Control and Prevention Judy Thibadeau, RN, MN, Spina Bifida Association Director, Research
and Services; former Health Scientist, National Spina Bifida Program, National Center
on Birth Defects and Developmental Disabilities, Centers for Disease Control and
Prevention
Funding
The development of these Guidelines was supported in part by Cooperative Agreement UO1DD001077, funded by the Centers for Disease Control and Prevention. Its contents are solely the responsibility of the authors and do not necessarily represent the official view of the Centers for Disease Control and Prevention or the Department of Health and Human Services.
Conflict of interest
The authors hereby declare no conflicts of interest.