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

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 home4

POTENTIAL BENEFITS

CONSIDERATIONS

BIPAP MACHINE

POTENTIAL BENEFITS

  • Noninvasive ventilatory support (NIV)1
  • May reduce the respiratory disturbance index, improve sleep stage distribution, and enhance quality of life1
  • In combination with airway clearance techniques, may reduce the need for intubation1

CONSIDERATIONS

  • Standardised settings not established1
  • Goal is to maintain O2 saturation ≥94% (pulse oximeter) during upper respiratory tract infections; therefore, children may receive continuous NIV and cough assist3

MECHANICALLY
ASSISTED COUGH
(Insufflator/exsufflator)

POTENTIAL BENEFITS

  • Noninvasive1
  • Effective management of secretion removal
  • May be used with an oronasal mask3
  • In combination with noninvasive ventilation, may reduce the need for intubation1

CONSIDERATIONS

  • Standardised settings not established1
  • May be intimidating for both parents/caregivers and children1
  • Full cooperation is uncommon before 2 years of age3
  • May not be effective at pressures below 35 to 40 cmH2O and -35 to -40 cmH2O3

References

1. Wang CH, et al. J Child Neurol. 2007;22(8):1027-1049. 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.