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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

1. Wang CH, Finkel RS, Bertini ES, et al; and Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027-1049. 2. Cure SMA. Tube feeding and SMA: recommendations and practices. http://www.curesma.org/documents/support--care-documents/2015-conference-tube-feeding.pdf. Published June 20, 2015. Accessed April 25, 2016. 3. Birnkrant DJ, Pope JF, Martin JE, et al. Treatment of type I spinal muscular atrophy with noninvasive ventilation and gastrostomy feeding. Ped Neurol. 1998;18(5):407-410. 4. Iannaccone ST. Modern management of spinal muscular atrophy. J Child Neurol. 2007;22(8):974-978. 5. Nutrition basics: fostering health and growth for spinal muscular atrophy [patient booklet]. http://www.curesma.org/documents/support--care-documents/nutrition-basics.pdf. Elk Grove Village; IL: Cure SMA; 2011. Accessed August 19, 2016. 6. Messina S, Pane M, De Rose P, et al. Feeding problems and malnutrition in spinal muscular atrophy type II. Neuromuscul Disord. 2008;18(5):389-393 7. Cha TH, Oh DW, Shim JH. Noninvasive treatment strategy for swallowing problems related to prolonged nonoral feeding in spinal muscular atrophy. Dysphagia. 2010;25(3):261-264. 8. Darras BT, Royden Jones H Jr, Ryan MM, De Vivo DC, eds. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach. 2nd ed. London, UK: Elsevier; 2015. 9. Yang JH, Kasat NS, Suh SW, Kim SY. Improvement in reflux gastroesophagitis in a patient with spinal muscular atrophy after surgical correction of kyphoscoliosis. Clin Orthop Relat Res. 2011;469(12):3501-3505. 10. Sproule DM, Montes J, Dunaway S, et al. Adiposity is increased among high-functioning, non-ambulatory patients with spinal muscular atrophy. Neuromuscul Disord. 2010;20:448-452. 11. Sproule DM, Montes J, Montgomery M, et al. Increased fat mass and high incidence of overweight despite low body mass index in patients with spinal muscular atrophy. Neuromuscul Disord. 2009;19(6):391-396. 12. Bladen CL, Thompson R, Jackson JM, et al. Mapping the differences in care for 5,000 spinal muscular atrophy patients, a survey of 24 national registries in North America, Australasia and Europe. J Neurol. 2014;261(1):152-163. 

Muscular Atrophy

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