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

Common issues affecting nutrition in children with spinal muscular atrophy

DESCRIPTION

HOW IT MAY AFFECT THE CHILD

ASPIRATION

DESCRIPTION

  • Food or gastric contents enter the trachea3

HOW IT MAY AFFECT THE CHILD

  • Sudden onset of respiratory distress and pneumonia in children with Type I spinal muscular atrophy3

CONSTIPATION

DESCRIPTION

  • Caused by poor tone in abdominal muscles and immobility due to weakness4
  • Constipation is common in babies with spinal muscular atrophy4

HOW IT MAY AFFECT THE CHILD

  • Chronic constipation and fecal 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) spinal muscular atrophy6
  • Patients may be at risk of aspiration of food or liquids and aspiration pneumonia6

FEEDING PROBLEMS

DESCRIPTION

  • Weak infants with spinal muscular atrophy 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

GERD (GASTROESOPHAGEAL REFLUX DISEASE)

DESCRIPTION

  • Scoliosis may contribute to increased abdominal pressure leading to hiatal hernia and reflux gastroesophagitis9
  • Constipation can worsen gastric reflux or respiratory symptoms8

HOW IT MAY AFFECT THE CHILD

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

OBESITY/
OVERNUTRITION

DESCRIPTION

  • Non-ambulatory individuals with spinal muscular atrophy 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 failure3
  • May increase the risk of infection5,12
  • May lead to difficulty with wound healing5
  • May increase tendency to develop pressure sores5
  • May lead to fatigue12

Feeding tubes may be an option for children with spinal muscular atrophy 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 gastrostomy tube placement1
  • May be preferable for children with gastroesophageal 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 (eg, 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 (eg, BiPAP)1
  • Potential morbidity associated with prolonged use1

GASTROSTOMY TUBE (G-TUBE)

POTENTIAL BENEFITS

  • Guidelines suggest consideration for stable and comfortable nutritional support1
  • Several approaches are used for tube placement1:
    • Percutaneous methods with endoscopic guidance
    • Placement via open or laparoscopic surgical techniques
  • Percutaneous methods with endoscopic guidance require only conscious sedation and local anesthesia1
  • 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 anesthesia, which could result in pulmonary complications1
  • Open surgical technique may result in postoperative complications1
  • G-tube does not work to minimize gastroesophageal 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