Supportive Therapy

**Supportive Therapy **

Supportive therapy is directed at providing nutrition and respiratory assistance as needed and treating or preventing complications of weakness. Recommended evaluations at baseline include assessments of nutritional and feeding requirements, respiratory function, sleep, activities of daily living, and orthopedic status. Individuals with SMA should be evaluated at least every six months, and those with more severe weakness should be evaluated more frequently.

Pulmonary: Respiratory muscle weakness often results in difficulty clearing lower respiratory secretions and hypoventilation during sleep. Important interventions include methods for mobilization and clearance of airway secretions, and respiratory support.

●Secretion mobilization and clearance techniques involve manual or mechanical chest physiotherapy with postural drainage, and manual cough assistance and/or use of a mechanical insufflation/exsufflation device.

●Noninvasive nasal ventilation is an alternative to tracheostomy and conventional ventilator support in some children with respiratory failure. Early intervention with noninvasive respiratory support can improve quality of life for infants with SMA type 1. When noninvasive ventilation becomes insufficient, decisions about initiating ventilator support should be individualized, taking into account the medical facts and the values of the family or guardians, in consultation with a palliative care team.

Nutrition and gastrointestinal: Bulbar dysfunction with impaired feeding and risk of aspiration and failure to thrive is universal in SMA type 1. Bulbar dysfunction can develop over time into a serious concern in SMA type 2. Additional common problems in SMA, particularly for those who cannot sit or stand, include gastrointestinal reflux, delayed gastric emptying, and constipation. Ambulatory patients are less prone to nutritional and gastrointestinal complications.

Management includes changing food consistency to improve food intake and protect against aspiration. Early gastrostomy in infants with SMA type 1 can help to maintain proper nutrition and reduce the risk of aspiration.

Orthopedic and musculoskeletal: Physical therapy may be helpful. Spinal bracing can be used to delay the development of progressive scoliosis that is caused by muscle weakness. However, spinal bracing applied to patients with SMA types 1 or 2 while in the sitting position significantly reduces expiratory tidal volume, and thus it should be used cautiously. Surgical repair of scoliosis may be an option but there is no consensus about efficacy.