Early Intervention & Spina Bifida
How Seating, Positioning & Power Mobility for Infants Can Prevent Developmental Delays
GIRL EXAMINING FLOWER: ISTOCKPHOTO.COM/KEREMYUCEL
Children have an inherent desire to explore the world around them as they test their own strengths and learn to navigate their environments. These early experiences are formative to a child’s development, not just physically but cognitively and socially as well. Every child should have the opportunity to participate in these experiences and develop these physical, cognitive and social skills.
Yet, children with disabilities and limited mobility often do miss out on such experiences because they lack the equipment and interventions that allows them to share in developmental milestones alongside their typically developing peers. Many children are not introduced to complex rehab technology (CRT) until they enter school, but by then, many opportunities for essential development has passed.
Introducing CRT early in a child’s life can help reduce the risk of developmental delays, increase activity levels and improve physical and cognitive development. Spina bifida, as a congenital condition that is present at birth, is an example of an infant and childhood disability for which early CRT intervention is ideal.
Spina Bifida 101
Spina bifida is a complex condition that encompasses a spectrum of neural tube defects. With this condition, the neural tube does not fully close during embryogenesis, often resulting in nerve and spinal cord damage. According to the Spina Bifida Association, three common types of spina bifida include spina bifida occulta, myelomeningocele and meningocele.
Spina bifida occulta, sometimes called hidden spina bifida, is a mild form of spina bifida and causes few to no disabilities.
Myelomeningocele is the most common and most severe form. With myelomeningocele, a small membrane-covered sac forms through an opening in the baby’s spine. The sac contains cerebrospinal fluid and the protective tissues of the spinal cord, and often, nerves and parts of the spinal cord itself. Though myelomeningocele may occur anywhere along the spine, it most often manifests in the lumbar and sacral areas. Wherever the myelomeningocele occurs on the spine, nerve damage will result below it.
Hydrocephalus, an excess of cerebrospinal fluid in the brain, is commonly associated with myelomeningocele. According to the 2008 book Spina Bifida: Management and Outcome, hydrocephalus will develop in 80 to 90 percent of children with myelomeningocele.
Meningocele is similar to myelomeningocele in that both result in the formation of a sac through a spinal opening. But with meningocele, the nerves and spinal cord do not fill the sac, leaving little to no nerve damage and usually resulting in minor disabilities.
Because spina bifida can present in different ways depending on the type, impairments to mobility can vary among children. Sally Mallory, PT, ATP, CPST, education manager for Convaid|R82, explained, “There is a huge variance because it has to do with the level of the lesion on the spine, at what vertebral level that occurs. The higher the level, obviously, the more involved the client is going to be from a physical standpoint in terms of muscle function, but also possible range of motion limitations.”
For instance, a child with spina bifida occulta might not experience any mobility impairments, whereas a child with myelomeningocele could experience muscle weakness or paralysis below the chest. With the more severe forms of spina bifida, seating and mobility interventions may be necessary.
“Sit Up Straight!”
Spina bifida is a degenerative disability, and for that reason, introducing children with spina bifida to seating and positioning interventions early is necessary to reduce the severity of the disability in adolescence and adulthood. “Obviously, the disability is degenerative, so it will worsen throughout time regardless of what we do. But we can dramatically slow that down by correctly supporting a child from a very young age,” explained Ross Andrews, International Business Manager and Seating Specialist with Specialised Orthotic Services (SOS) by Drive.
Andrews recommended starting at 6 months. “Start at 6 months old, and they will generally be seated from that age upwards until they reach adulthood and full growth. The benefit at 6 months is that they are more malleable,” he said. As a result of spina bifida, children may develop scoliosis, kyphosis or lordosis, but Andrews explained that by seating these children properly starting at 6 months, such conditions are marginally correctable.
If a client does not have any current scoliosis or other spinal positioning issues, Andrews said it is still important to provide specialized seating for them starting at 6 months. “We can put them in a position that is better for them in the long run in terms of functionality and stopping anything occurring down the line.”
If spinal positioning problems are allowed to set in prior to intervention, treating and correcting them can become much more difficult. “You will see a lot of children with spina bifida, if they are seated at a later age, who will generally be sat in their seat leaned over to the right, could be the left depending on which way the scoliosis has gone, and they will be propped on the right elbow,” Andrews said. “They’re all in poor position because the client is just going to compound the issue of what he’s already got. It’s very hard once the client has been seated in that position for a couple of years to try to explain that we need to seat him in [a] new position with back support. Early intervention is absolutely key in that because at 6 months, they’ve not yet experienced anything else. If we can get clients seated, that will become the norm.”
In addition to preventing and correcting positioning issues, seating and positioning also facilitates interaction with one’s environment. In a 2005 study from the University of Ulster in Northern Ireland titled “The Fundamental Principles of Seating and Positioning in Children and Young People with Physical Disabilities,” researchers found that appropriate seating and positioning can aid in a child’s engagement with the world around them.
Andrews’ work with clients bears this out. By giving seating balance and support to strengthen muscle tone and engage the trunk, Andrews said that children are much more capable and ready to interact with their environment. “What we generally see is clients who have very low tone or a very weak trunk and poor motor control. They’ll be fighting to maintain balance. What we look to do in seating is balance and symmetry.”
Andrews drew a comparison to able-bodied adults to illustrate his point. “It’s very easy for us to sit in a position and change and move to another position and to balance ourselves and perch on the edge of a desk or chair. You can maintain the ability to balance yourself. But with the clients and the children that we see, they have a very low tone. Generally, if we put them in a seat that isn’t correct for them or doesn’t give the correct amount of support, they’ll fatigue at a quicker rate throughout the day because self consciously they’re having to concentrate more on correcting the balance, the pelvis and the core, and that, in turn, will take away from them being able to use that energy to balance their head.”
Constantly focusing to keep balance diverts children’s attention and energy away from outward engagement. But when that distraction is taken away by proper seating, Andrews said that children are ready to engage. “By correctly supporting someone from the pelvis up to the shoulders with good support in the trunk, you’ve given back control over fine motor function and the ability to interact and play,” he said. “They can use the hands, the arms, and better control the head position, which in turn will usually help with speech, feeding, and them being able to just see and look around.”
“It’s really imperative that we give kids with mobility limitations access to independent mobility at the same as their peers are starting to experience independent mobility, and that happens very young,” said Amy Morgan, PT, APT, territory sales manager for Central and Southern Ohio at Permobil. “We need to start supplementing children who can’t do that on their own to at least give them the opportunity to explore their environment and to develop their brains the same way that their peers are doing.”
This is where power mobility comes in. Morgan said that infants and young toddlers have yet to develop the musculature to self propel a manual wheelchair. “While kids can do that a little bit, maybe in a small environment, sometimes it’s really more necessary at that younger age to give them power mobility, simply because their skeleton and their muscular system aren’t developed enough to operate a manual chair,” she explained.
Introducing infants to power mobility, however, is a relatively new idea. In 2009, the journal Pediatric Physical Therapy published a case report entitled “Power Mobility Training for a 7-Month-Old Infant with Spina Bifida” that explored the feasibility of implementing power training programs in infants with spina bifida as young as 7 months. Investigators found that infants could use power mobility to explore their environments in a way that could impact learning and various perceptual, cognitive and social outcomes.
If children with mobility limitations do not attain independent mobility along with their typically developing peers, they are at risk of missing critical developmental stages that could impact them in adolescence and into adulthood. For instance, Mallory said, “Many studies have shown that if a child was not given the opportunity to be mobile, and preferably independently mobile, by the age of 3, they lose the ability to learn different functions like object permanence, position in space, and they’re much less apt to interact with their environment.”
Morgan described the early developmental stages when infants and toddlers develop these skills as “the sponge phase” of the brain. “During the early years, a child’s brain is developing like crazy, and changing, and learning and adapting to their environment. And if we don’t give them that opportunity during that sponge phase, they’re going to be constantly a little bit behind.”
The visual system is essential to these different functions, and independent mobility is required to develop the visual system. Morgan explained, “If you don’t have an opportunity to touch, feel and bump into things, then you’re not going to understand ‘This is an inanimate object that will not get out of my way, and I have to go around it.’’’
When infants and toddlers are put into power chairs, they often bump into walls and other objects, and Morgan said that this is not only normal, but also essential to child development. “Sometimes I think we as clinicians expect a child that’s never moved themselves anywhere in space to start driving or using a powered wheelchair without bumping into things or without running into anything. And that’s not something we can realistically expect,” she explained.
“We don’t want to stop them from having those interactions either, because we need to make them safe in doing that, and we also need to allow them to experience that wall and feel how that stops a chair, because they have no idea. Everything’s two dimensional to someone until you have the ability to move and interact with the world. That’s when it really sets in that it is a three-dimensional world that we live in.”
Kids Will Be Kids
Independent mobility and correct seating and positioning help children with spina bifida to hone their cognitive development and increase their physical activity, reducing the physical side effects of immobility and poor posture. But more than that, these interventions offer emotional and psychosocial benefits that are essential to overall quality of life.
When children are not given the opportunity to explore the world around them and their place in it, they will start to believe that they do not have the ability to have those experiences on their own. Morgan said that independent mobility gives children with spina bifida the same confidence as their typically developing peers.
“Any child, even with no diagnosis, should be mobile in order to learn and develop,” she explained. “Learn about the world and develop their sensory systems, their visual systems, their receptive systems, start problem solving and get their confidence. Let them know that they can do things on their own and really establish their individual nature.”
If they do not have this opportunity, children will develop learned helplessness, in which they begin to believe that they need others to do everything for them.
“They learn that they can’t do it, and ultimately, they stop trying, or stop trying as hard,” Morgan noted. “It becomes harder and harder and harder to get kids to do stuff for themselves when they’ve always had everything done for them, and that’s true of every type of activity.”
In addition to building the confidence that they get with power mobility, it facilitates peer interaction and gives them the ability to do similar things that their peers are doing, Morgan said. And even beyond the child, the psychosocial benefits of mobility extend to the rest of the family. “For the parent, it’s equally beneficial,” said Morgan, “because now you’re seeing your kid do the things that you expect a child to do. They’re able to interact with other parents at the park and let their child explore and do ‘normal’ kid things.”
With early intervention in seating and mobility, children can experience independent mobility sooner, which, ultimately, allows kids to be the curious and questioning explorers that they so naturally are.
This article originally appeared in the April 2019 issue of Mobility Management.