Question
Why are respiratory infections a significant concern for patients with Spinal Muscular Atrophy (SMA), and how are they managed?
Answer
Respiratory infections are a leading cause of morbidity and mortality in patients with SMA due to the weakness of their respiratory muscles, which impairs their ability to clear mucus and debris from the airways. This results in frequent infections, including pneumonia, that can escalate to respiratory failure if not addressed. Infants and young children with early-onset SMA are particularly vulnerable, as their already compromised breathing makes it challenging to combat the strain of infections. Proactive respiratory management is essential to minimize these risks, often involving non-invasive ventilation, airway clearance techniques, and regular monitoring to detect and address infections early.
In addition to recurrent infections, SMA patients face respiratory symptoms such as impaired cough, underdeveloped chest walls, and hypoventilation, all of which increase the risk of aspiration pneumonia. Respiratory therapists play a crucial role in managing these symptoms through techniques like pulmonary toileting, ensuring the effective clearance of airway secretions, and providing ventilatory support to prevent complications like sleep-disordered breathing.
This Ask the Expert is an edited excerpt from the course, SMA in Pediatrics (Spinal Muscular Atrophy), presented by Nadia Boussalham, BS, RRT.
