Abstract
Respiratory muscle weakness associated with scoliosis in type-2 spinal muscular atrophy (SMA) leads to respiratory impairment. Spinal brace, generally utilized to slow scoliosis progression and support sitting, could worsen lung function and hamper cough maneuvers. Six home-treated type-2 SMA children (aged 6-15 years, subtype 2.1-2.5) were assessed to evaluate time-dependent influence of "static-balanced brace" on pulmonary function. Lung function tests, including peak expiratory flow (PEF), peak cough flow (PCF), maximal static inspiratory pressure (MIP), maximal static expiratory pressure (MEP), forced vital capacity (FVC), were performed. PEF, MEP, FVC parameters were higher in tests after wearing braces three-hours, PCF slightly higher and MIP slightly lower compared to upon awakening values. "Static-balanced brace" did not impair lung function in our sample of six type-2 SMA children; in addition, it seemed to support cough maneuvers. Double assessment is determinant for decisions concerning use/non-use of brace differently from the usual one time evaluation procedure (base level compared to level wearing brace).
Highlights
Spinal muscular atrophy (SMA) is caused by deletion of SMN 1 gene, located on chromosome 5, responsible for production of SMN (Survival Motor Neuron) protein
In order to verify whether wearing braces can affect lung function more than the consequences of muscle fatigue induced by maintaining sitting position without a brace, we performed tests without brace upon awakening and after use/non-use of brace for 3 hours
In tests performed upon awakening, before wearing brace, peak expiratory flow (PEF), peak cough flow (PCF), maximal static inspiratory pressure (MIP) and maximal static expiratory pressure (MEP) mean and median values were higher in sitting position
Summary
Spinal muscular atrophy (SMA) is caused by deletion of SMN 1 gene, located on chromosome 5, responsible for production of SMN (Survival Motor Neuron) protein. Altered production of this protein leads to degeneration of motor neurons in spinal cord anterior horns, resulting in progressive denervation, muscle atrophy and increasing weakness [1]. Generally utilized to slow scoliosis progression and support sitting, could worsen lung function and hamper cough maneuvers. Clinical rehabilitation impact: “Static-balanced brace” did not impair lung function in our sample of type-2 SMA children; in addition, it seemed to support cough maneuvers. Double assessment is determinant for decisions concerning use/non-use of brace
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