Assistive Devices and Equipment in Spinal Muscular Atrophy: Braces

Assistive Devices and Equipment in Spinal Muscular Atrophy: Braces

Braces are one amongst a number of rehabilitation techniques that are used in those with neuromuscular disorders like spinal muscular atrophy (SMA).1 Their effectiveness is thought to vary depending on the individual case. Children with SMA may have spinal braces as an intervention for scoliosis,2–6 or they may use braces on their legs, ankle, or feet to aid in walking.7 

Braces for Scoliosis

Scoliosis in SMA and other neuromuscular diseases leads to respiratory impairment.8 Of the 78 patients diagnosed with SMA between 1969 and 1988, 34 of them developed scoliosis.9 The average age of scoliosis onset was 8.8 years. According to SMA experts, aggressive orthopedic management of scoliosis is important for preventing the progression of deformities associated with scoliosis and to improve patients’ comfort when sitting. Indeed, such proactive management was shown to improve orthopedic outcomes in a sample of SMA type 2 children, 29 of whom had spinal fusions between 1999 and 2009 and some of whom were subsequently braced.2

A complicating factor with spinal braces used to correct scoliosis in SMA patients is that these braces have been shown to inhibit coughing ability and thus worsen lung function.4,5,8 For instance, spinal bracing can reduce tidal expiratory volume when SMA patients are in the sitting position.4 It can also decrease mean vital capacity and forced expiratory volume.8 However, specific types of braces have been shown to actually support cough maneuvers and thus not to impair lung function.5 Further, in cases where spinal surgery followed by bracing has hampered vital capacity, this capacity has been regained over time.3

Though the spinal deformities associated with scoliosis can be difficult to correct or control with braces, other types of braces that improve walking can slow scoliosis.6

Braces for Walking

Improving SMA patients’ ability to walk is beneficial for several reasons. In addition to enhancing quality of life and potentially slowing scoliosis, walking with support is also associated with the level of acquired gross motor functions in SMA and the ability to roll by the age of five.10 The ability to roll also appears to correlate with the severity of the disease. One study on seven SMA children between the ages of 20 months and four years found that two of those who were fitted with knee-ankle-foot orthoses acquired the ability to walk on their own. Three others could walk while assisted, and the remaining two were able to stand autonomously.7

Different types of braces appear to serve distinct functions for those with SMA, with perhaps the most success in helping SMA patients to walk. The ability to walk appears to have both direct and indirect benefits for SMA patients. As new orthopedic innovations become available, it is likely that it will become easier to support SMA patients in their development and maintenance of walking ability.

References

1. Bussi L, Mangosio V. [Progressive motor disability in spinal muscular atrophy. Psychosocial aspects and rehabilitation in adolescents and adults]. Minerva Med. 1986;77(5-6):155-169.

2. Catteruccia M, Vuillerot C, Vaugier I, et al. Orthopedic management of scoliosis by Garches Brace and spinal fusion in SMA type 2 children. J Neuromuscul Dis. 2015;2(4):453-462. doi:10.3233/JND-150084

3. Granata C, Cervellati S, Ballestrazzi A, Corbascio M, Merlini L. Spine surgery in spinal muscular atrophy: long-term results. Neuromuscul Disord. 1993;3(3):207-215.

4. Tangsrud SE, Carlsen KC, Lund-Petersen I, Carlsen KH. Lung function measurements in young children with spinal muscle atrophy; a cross  sectional survey on the effect of position and bracing. Arch Dis Child. 2001;84(6):521-524.

5. Di Pede C, Salamon E, Motta M, Agosto C, Benini F, Ferrari A. Spinal bracing and lung function in type-2 spinal muscular atrophy. Eur J Phys Rehabil Med. April 2018. doi:10.23736/S1973-9087.18.05046-3

6. Rodillo E, Marini ML, Heckmatt JZ, Dubowitz V. Scoliosis in spinal muscular atrophy: review of 63 cases. J Child Neurol. 1989;4(2):118-123. doi:10.1177/088307388900400208

7. Granata C, Cornelio F, Bonfiglioli S, Mattutini P, Merlini L. Promotion of ambulation of patients with spinal muscular atrophy by early fitting of knee-ankle-foot orthoses. Dev Med Child Neurol. 1987;29(2):221-224.

8. Morillon S, Thumerelle C, Cuisset J-M, Santos C, Matran R, Deschildre A. [Effect of thoracic bracing on lung function in children with neuromuscular disease]. Ann Readapt Med Phys. 2007;50(8):645-650. doi:10.1016/j.annrmp.2007.03.018

9. Phillips DP, Roye DPJ, Farcy JP, Leet A, Shelton YA. Surgical treatment of scoliosis in a spinal muscular atrophy population. Spine (Phila Pa 1976). 1990;15(9):942-945.

10. Bono R, Inverno M, Botteon G, et al. Prospective study of gross motor development in children with SMA type II. Ital J Neurol Sci. 1995;16(4):223-230.