Use of orthoses and orthopaedic technical devices in proximal spinal muscular atrophy. Results of survey in 194 SMA patients

Abstract

Purpose: The purpose of this study is to determine the use of orthopaedic and assistive devices for Spinal muscular atrophy (SMA) patients, following a survey of 194 patients.

Method: The use of wheelchairs, corsets and orthoses was evaluated in 194 SMA patients whose mean age was 12.6 (SD 7.2, 0.7-41.1). There were 14 patients with SMA type Ib (age range 1.7-36.9), 133 with type II (age range 0.7-37.7), 42 with type IIIa (age range 3.2-41.1) and 5 with type IIIb (age range 8.0-20.0).

Results: One hundred and sixteen patients (60%) had powered and 29 patients (15%) manual wheelchairs. Nineteen patients (10%) used long leg orthoses. Ten patients (5%) used swivel walkers and 26 (13%) had standing frames. Twenty-six patients (13%) received lower leg orthoses because of foot deformities. Eight patients (4%) used night splints for the lower limbs. One hundred and fifteen patients (59%) were fitted with corsets because of progressive scoliosis.

Conclusion: This is the first study about the provision of orthopaedic and assistive devices in a large group of SMA patients. Following the results of this survey we can optimise the strategy of providing orthoses and assistive devices for SMA patients and better adapt them to the patient's individual needs.