How are the pulmonary manifestations of spinal muscle atrophy (SMA) treated?

Updated: Nov 05, 2018
  • Author: Ashish S Ranade, MBBS, MS, MRCS; Chief Editor: Jeffrey A Goldstein, MD  more...
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Answer

Patients with SMA often have impaired cough, respiratory insufficiency, dysphagia, gastroparesis, constipation, and evolving orthopedic issues (eg, scoliosis). To address these problems, various types of equipment may be used, from respiratory support during sleep (eg, bilevel positive airway pressure [BiPAP] and mucus clearance devices) to gastrostomy tubes to wheelchairs and braces. Cognitive development typically is not affected. Usual primary care practices (especially care coordination and family support), along with routine pediatric care immunizations, developmental surveillance, and monitoring of growth, contribute to the overall well-being of the child and the family.

Care management includes optimizing breathing and coughing, addressing nutrition and feeding issues, managing mobility and activities of daily living (ADLs), and preparing for illness.

Patients with type 1 SMA have difficulty in coughing and breathing and will require respiratory care (eg, insufflator-exsufflator/cough assist, oxygen saturation monitor) and support (either noninvasive [BiPAP/ventilator] or invasive [tracheostomy]). They also lose their ability to chew and swallow food and water and will require nutrition (eg, nutritional modification or supplementation) and feeding support (eg, via a nasogastric tube, gastrostomy, or gastrojejunostomy).


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