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  1. Musculoskeletal Support and Interventions
  2. Nutritional Support and Interventions
  3. Common Nutritional Issues
  4. Respiratory Support and Interventions

Nutritional support and interventions

Nutritional support and feeding are critical considerations in caring
for a child with spinal muscular atrophy (SMA)

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Children with spinal muscular atrophy may have difficulty eating due to weak swallowing muscles and poor head control, putting them at risk of aspiration and poor nutrition. Feeding tubes may be an option for children with insufficient caloric intake or impaired oral feeding.1,2

POTENTIAL BENEFITS

CONSIDERATIONS

NASOJEJUNAL
TUBE

POTENTIAL BENEFITS

  • Short-term solution while awaiting gastrostomy tube placement1
  • May be preferable for children with gastro-oesophageal reflux with aspiration1
  • May be preferable for children on ventilator support1

CONSIDERATIONS

  • Technical difficulty may impair function1
  • May negatively affect mask fit of noninvasive ventilation (e.g. BiPAP)1
  • Potential morbidity associated with prolonged use1

NASOGASTRIC
TUBE

POTENTIAL BENEFITS

  • Short-term solution while awaiting gastrostomy tube placement1

CONSIDERATIONS

  • May negatively affect mask fit of noninvasive ventilation (e.g. BiPAP)1
  • Potential morbidity associated with prolonged use1

GASTROSTOMY TUBE (G-TUBE)

POTENTIAL BENEFITS

  • Guidelines suggest consideration for stable and comfortable nutritional support
  • Several options for tube placement1:
    • Percutaneous methods with endoscopic guidance
    • Placement via open or laparoscopic surgical techniques
    • Antireflex procedure such as Nissen fundoplication

CONSIDERATIONS

  • Optimal timing of procedure is controversial1
  • Open surgical techniques require general anaesthesia, which could result in pulmonary complications1
  • Open surgical technique may result in postoperative complications1
  • G-tube does not work to minimise gastro-oesophageal reflux1

References

Muscular Atrophy

The clinical spectrum of SMA is highly variable and often requires comprehensive medical care involving multiple disciplines.1