Page 24 - Mobility Management, January 2018

Pediatric Series
Tech Perspectives for SMA
“Seating and positioning goals for children with SMA cannot be stated generally,” she said. “This is because the children range from being able to sit upright with head control (strong Type 2) to being only able to lie in a fully reclined position (weak Type 1).”
Getting Out & About
Cure SMA noted that SMA typically doesn’t impact cognitive function or intelligence — and anecdotally, many healthcare professionals have noted that kids with SMA seem brighter than average typically developing kids. In fact, a 2002 study called “Intelligence and cognitive function in children and adolescents with spinal muscular atrophy” and cited by the U.S. National Library of Medicine actually sought to disprove the notion that older children and teens with SMA were smarter than their able-bodied peers.
The study’s result: The kids with SMA had higher mean IQ scores than their siblings and other unrelated kids, though
not dramatically higher. Verbally, though, the kids with SMA signi cantly outscored able-bodied peers. The study’s conclusion: “It could be speculated that the development of cognitive skills and knowledge is a creative way to compensate the many restrictions due to their physical handicap.”
Regardless of whether kids with SMA are organically “smarter” than their peers or whether their skills are environmentally based, it’s obvious that inclusion — in school, family activities, outings and their communities — should be a major goal.
“To set seating and positioning goals, each child’s strength and educational/activity needs must be assessed,” Hawthorne said. “Most children with SMA do not have cognitive impairment. Therefore, how they are being educated should be considered. Are they home schooled or do they attend school? Are they using special mounts in order to use iPads, Eyegaze and other assistive devices? Can the equipment adjust to help them access these tools?
“Ideally, it is great if the seating system can be easily moved to an alternative base, so that the child can be positioned for peer group inclusion.”
On the mobility side, Hawthorne said, “Whether they are using a power wheelchair, a stroller-type wheelchair or both, their respiratory needs must be the rst priority. Many children have had a tracheotomy to assist their breathing. Others use BiPAP, so when considering mobility equipment, it must be able to transport the necessary respiratory equipment.
“In both Type 1 and 2, whether the child will be transported by adapted van or school bus and how the child can ride safely must be considered. The mobility equipment must be equipped with the correct tie-down system to ensure this.”
Transportation becomes more complicated when additional equipment, such as ventilators, must be included in addition to the wheelchair and its seating.
“It is very challenging to create vent trays large enough to carry all the respiratory equipment,” Hawthorne said. “The regulations for transporting children by bus or van require crash testing at speci c weight limits. When you consider that the respiratory
24 JANUARY 2018 | MOBILITY MANAGEMENT
equipment can weigh in excess of 20 lbs., very careful weights and measurements must be taken. It is crucial when building custom vent trays that the seating clears the equipment on the vent tray and at the same time allows the child to be fully reclined.
“Placement of the respiratory gear takes some creative strategy, as it is important not to unbalance the overall system while it is in motion.”
Something Quite Special
Hawthorne said the progressive nature of SMA makes it dif cult to determine if optimal seating and mobility equipment can improve a child’s function.
“Whether the equipment can actually improve respiration
is a very large question,” she said. “SMA is degenerative and children become weaker, with a great loss of both muscle and respiratory function over time. That said, the seating, positioning and mobility equipment can contribute a great deal to helping children maintain better function when their equipment works well for them.”
And that can make a big difference to children with SMA and their families.
“For example,” Hawthorne said, “if they can more easily access their schoolwork, have peer group and family interaction, and
be transported because their seating is comfortable, then you
are offering them something quite special: An opportunity to participate in everyday life.” m
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Nusinersen: Can SMA
Progression Be Stopped?
The late 2016 introduction of nusinersen (brand name: Spinraza) as a drug therapy for spinal muscular atrophy (SMA) in the United States poses the question: Can SMA be rendered a chronic rather than a progressive condition?
Missy Ball, MT, PT, ATP, said clinical studies of nusinersen used by children and adults have been promising.
“The ndings show support for treatment for all types of SMA (especially types 1, 2 and 3) and support early initia- tion of treatment,” she said. “Results from different studies show improvement in motor function, including ability to sit unassisted, stand or walk when [study participants] would otherwise be unexpected to do so. Also, longevity improved in a greater percentage of patients on Spinraza.”
A November 2017 study published in the New England Journal of Medicine said 51 percent of infants with SMA who were given nusinersen achieved motor-milestone responses, compared to 0 percent of the infants in the control group who didn’t receive nusinersen. The survival rate was also higher in the group that received nusinersen. Researchers suggested that early treatment might be required to fully bene t from the drug’s abilities. m