In a study of individuals (n=49) with infantile-onset (Type I) SMA, those receiving early proactive noninvasive respiratory care did better compared with those treated with supportive respiratory care4

Children with SMA demonstrate a wide range of respiratory compromise3

Respiratory compromise in children with infantile-onset (consistent with Type I) SMA may be differentiated into 3 categories:3

  1. Infants ≤5 months of age who require both continuous ventilatory support and non-oral nutritional support
  2. Infants with ineffective cough who develop acute respiratory compromise during upper respiratory tract infections and require non-oral nutritional support before 24 months of age
  3. Infants who do not develop respiratory compromise or who do not require non-oral nutritional support until after 24 months of age (approximately 10% of all children with infantile-onset SMA)

Noninvasive ventilatory support can be provided in the home2


Early proactive respiratory care was defined as:4

  • use of noninvasive bi-level positive airway pressure (BiPAP) at night and during daytime sleep, and
  • cough-assist device used at least twice-daily, initiated in the first 3 months after diagnosis.

Supportive care was defined as:4

  • other respiratory support, such as supplemental oxygen and suctioning.

Since the mid-1990s, three main areas of development have occurred in the management of restrictive lung disease in SMA:5

  • Noninvasive ventilation using new technology
  • Awareness of the importance of identifying sleep-disordered breathing
  • New multidisciplinary approach to care
  1. Wang CH et al. J Child Neurol 2007; 22: 1027–49.
  2. Spinal Muscular Atrophy Clinical Research Center. Physical/occupational therapy. Available at: Accessed November 2017.
  3. Bach JR. Paediatr Resp Rev 2008; 9: 45–50.
  4. Lemoine TJ et al. Pediatr Crit Care Med 2012; 13: e161–e165.
  5. Iannaccone ST. J Child Neurol 2007; 22: 974–8.