The evolution and future of respiratory care for Spinal Muscular Atrophy

Carrie K Barker, Jennifer Kwon

Abstract

Spinal Muscular Atrophy (SMA) is a hereditary neuromuscular disorder characterized by progressive weakness over time. The most common cause of death in patients with SMA is respiratory failure due to weakness of the respiratory muscles. In the past, patients with the most severe forms of SMA did not typically survive more than 2 years. However, technology developed for the short-term ventilation of patients with acute respiratory failure due to poliomyelitis, beginning in the 1920s, ultimately led to advancements in long-term ventilation in patients with SMA. In addition, advancements in artificial airways and airway clearance, also developed for short-term respiratory care for patients with polio, contributed to significant improvement in life expectancy for patients with SMA, and opened the door to advancements in other areas, such as orthopedic and nutritional care.

Now that disease modifying therapies are available, the spectrum of respiratory disease in patients with SMA continues to change. However, in moving forward, it is vital to understand the natural history of SMA and the history of the respiratory care it has required, not only to provide the best possible nuanced care for current patients with SMA, but also to learn from the advances made in SMA care and apply them to other respiratory disease processes, just as the care of patients with polio created so many advancements in the care of patients with SMA.

Keywords: SMA, Mechanical Ventilation, Polio epidemic

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