The child living with spinal muscular atrophy (SMA) type 2 had less fatigue and improved functional scores as time went on.
The use of a gait exoskeleton appears to lead to benefits in terms of fatigue perception and functional outcomes in spinal muscular atrophy (SMA), according to a new case report.
SMA is a genetic degenerative neuromuscular disease in which patients experience progressive muscle deterioration. Mortality, severity, and impairment are closely tied with the patient’s type of SMA, which is based on the age of symptom onset and the highest degree of functionality achieved.
The new report, published in Journal of Pediatric Nursing, tells the story of a 6-year-old boy with SMA type 2. Children with SMA type 2 are usually able to sit on their own, but are unable to walk.
The investigators explained that conventional rehabilitation and orthotic interventions have been helpful in getting children with type 2 SMA to learn to sit. However, they said the noticeable deficit of strength in patients’ lower and upper limbs and their trunks made such efforts ineffective at helping patients learn to walk. In recent years, however, the emergence of gait exoskeletons—wearable robotic devices that can assist the gaits of patients—have generated hope of improved mobility in children with SMA.
A 2018 study, for instance, found that 7 children with SMA type 2 who used a prototype pediatric exoskeleton, the ATLAS 2020, were able to safely use the gait-assistance device. A study published earlier this year involving 3 children with SMA showed that the commercialized version of that exoskeleton, ATLAS 2030, improved the strength and range of motion of children.
Yet, the study authors said exoskeleton devices have not yet been studied from a patient-care perspective that could help inform nurses.
“Doing so is necessary, because nurses need to know its effects on care aspects beforehand in order to align the use of this technology with the goal of achieving health and well-being in patients,” they wrote.
The 6-year-old boy in the case report received his SMA diagnosis at 10 months old, and he had generalizable muscle weakness and the inability to walk, but was able to sit up on his own.
“He was unable to do transfers by himself and in need of an electric wheelchair to move around,” the authors said. “He had ineffective cough and chronic respiratory failure and required mechanical ventilation during sleep.”
The patient had been taking the disease-modifying therapy nusinersen (Spinraza) every 4 months, along with the respiratory medication salbutamol (Ventolin) and undergoing physical therapy. For the study, he used the ATLAS 2025 exoskeleton model at home for 2 months. He was given a nursing assessment prior to use of the exoskeleton. Several other metrics were also assessed, including vital signs, fatigue, and functional scores, along with the conducting of nursing interviews and taking of field notes.
Over the course of the 2 months, the nurses found that the child was able to tolerate the exoskeleton well and that his use of the device appeared to lead to improvement.
“Performance using the device improved over time, vital signs did not vary significantly during the sessions, fatigue perception decreased over time, and the child reached a higher score on some functional outcomes,” they wrote.
The authors said the child’s experience suggests that exoskeletons may have a role to play in the care of children with neuromuscular diseases that impair walking, although they said further research is needed to better understand the full implications of integrating the device into standard care.
Garces E, Puyuelo G, Sánchez-Iglesias I, et al. Using a robotic exoskeleton at home: an activity tolerance case study of a child with spinal muscular atrophy. J Pediatr Nurs. Published online September 30, 2022. doi:10.1016/j.pedn.2022.09.014