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  1. Care Approaches
  2. Musculoskeletal Support
  3. Nutrition
  4. Respiratory Support

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 SMA 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

Common issues affecting nutrition in children with SMA

ISSUE

DESCRIPTION

HOW IT MAY AFFECT THE CHILD

ASPIRATION

DESCRIPTION

  • Food or gastric contents enter the trachea4

HOW IT MAY AFFECT THE CHILD

  • Sudden onset of respiratory distress and pneumonia in children with Type I SMA1

CONSTIPATION

DESCRIPTION

  • Caused by poor tone in abdominal muscles and immobility due to weakness4
  • Constipation is common in babies with SMA4

HOW IT MAY AFFECT THE CHILD

  • Chronic constipation and faecal impaction may occur4,5

DYSPHAGIA (DIFFICULTY SWALLOWING)

DESCRIPTION

  • Poor head control may affect the safety of swallowing6
  • In older children, limited range of jaw movement, decreased bite force, and fatigue in the muscles involved in chewing may contribute to swallowing difficulty7

HOW IT MAY AFFECT THE CHILD

  • Poor weight gain in patients with later-onset (consistent with Type II) SMA6
  • Patients may be at risk of aspiration of food or liquids and aspiration pneumonia6

FEEDING PROBLEMS

DESCRIPTION

  • Weak infants with SMA may have difficulty feeding, causing prolonged mealtimes, fatigue with oral feedings, and choking or coughing during or after swallowing1,8

HOW IT MAY AFFECT THE CHILD

  • Oral feedings may result in aspiration pneumonia4
  • Feeding issues may lead to failure to thrive4
  • Feeding tubes placed by percutaneous gastrostomy may be considered before patients develop pneumonia4

GASTROINTESTINAL PROBLEMS

DESCRIPTION

  • Bloating, spitting up, vomiting after meals, and abdominal distention5

HOW IT MAY AFFECT THE CHILD

  • May lead to undernutrition5

GORD (GASTRO-OESOPHAGEAL REFLUX DISEASE)

DESCRIPTION

  • Scoliosis may contribute to increased abdominal pressure leading to hiatal hernia and reflux gastro-oesophagitis9
  • Constipation can worsen gastric reflux or respiratory symptoms8

HOW IT MAY AFFECT THE CHILD

  • Individuals may experience heartburn and pain8
  • Silent GORD may lead to increased risk of aspiration of stomach contents into the lungs5,8

OBESITY/
OVERNUTRITION

DESCRIPTION

  • Non-ambulatory individuals with SMA have increased fat mass and may become overweight10
  • Excessive weight gain due to decreased activity, and a reduction in overall metabolic demand11

HOW IT MAY AFFECT THE CHILD

  • Obesity may lead to pain and increased risk of complications in the hips and back5
  • Obese individuals are at increased risk of diabetes and hypertension5

UNDERNUTRITION

DESCRIPTION

  • Weight for age lower than the 5th percentile may suggest undernutrition5
  • Weight for length less than the 50th percentile may indicate undernutrition5

HOW IT MAY AFFECT THE CHILD

  • Undernutrition may lead to growth failure4
  • May increase the risk of infection5,12
  • May lead to difficulty with wound healing5
  • Increase tendency for pressure sores5
  • May lead to fatigue12

Feeding tubes may be an option for children with SMA when there is concern about insufficient caloric intake or the safety of oral feeding1,2

POTENTIAL BENEFITS

CONSIDERATIONS

NASOJEJUNAL
TUBE

POTENTIAL BENEFITS

  • Short-term solution while awaiting PEG 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 PEG tube placement1

CONSIDERATIONS

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

PERCUTANEOUS ENDOSCOPIC GASTROSTOMY TUBE (PEG TUBE)

POTENTIAL BENEFITS

  • Guidelines suggest consideration for stable and comfortable nutritional support1
  • Several approaches are used for tube placement:1
    • Percutaneous methods with endoscopic guidance
    • Placement via open or laparoscopic surgical techniques
  • Percutaneous methods with endoscopic guidance require only conscious sedation and local anaesthesia1
  • Laparoscopic surgical technique is compatible with immediate or early postoperative extubation1
  • A procedure such as Nissen fundoplication may be performed to decrease occurrence of reflux1

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
  • PEG tube does not work to minimise gastro-oesophageal reflux1

References

1. Wang CH, et al. J Child Neurol . 2007;22:1027–49. 2. Cure SMA. Tube feeding and SMA: recommendations and practices. Available at: http://www.curesma.org/documents/support--care-documents/2015-conference-tube-feeding.pdf. Accessed November 2017. 3. Birnkrant DJ, et al. Ped Neurol . 1998;18:407–10. 4. Iannaccone ST. J Child Neurol . 2007;22:974–8. 5. Cure SMA. Nutrition basics: fostering health and growth for spinal muscular atrophy [patient booklet]. Available at: http://www.curesma.org/documents/support--care-documents/nutrition-basics.pdf. Accessed November 2017. 6. Messina S, et al. Neuromuscul Disord . 2008;18:389–93. 7. Cha TH, et al. Dysphagia . 2010;25:261–4. 8. Darras BT, et al. Neuromuscular Disorders of Infancy, Childhood, and Adolescence: A Clinician’s Approach . 2nd ed. London, UK: Elsevier; 2015. 9. Yang JH, et al. Clin Orthop Relat Res . 2011;469:3501–5. 10. Sproule DM, et al. Neuromuscul Disord . 2010;20:448–52. 11. Sproule DM, et al. Neuromuscul Disord . 2009;19:391–6. 12. Bladen CL, et al. J Neurol . 2014;261:152–63.