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Respiratory support and interventions

Respiratory compromise is the major cause of morbidity and mortality in SMA. Individuals with SMA may have decreased respiratory function, underdeveloped lungs, and difficulty coughing and clearing secretions.1,2

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)
patient
patient

Noninvasive ventilatory support can be provided in the home2

table
table

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
References
  1. Wang CH et al. J Child Neurol 2007; 22: 1027–49.
  2. Spinal Muscular Atrophy Clinical Research Center. Physical/occupational therapy. Available at: http://columbiasma.org/pt-ot.html. 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.