Individuals 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 individuals with insufficient caloric intake or impaired oral feeding.1

Common issues affecting nutrition in individuals with spinal muscular atrophy

DESCRIPTION

HOW IT MAY AFFECT THE INDIVIDUAL

ASPIRATION

DESCRIPTION

  • Food or gastric contents enter the trachea2

HOW IT MAY AFFECT THE CHILD

  • Sudden onset of respiratory distress and pneumonia in children with Type I spinal muscular atrophy2
  • Difficulties with chewing and fatigue, as well as eating and safe swallowing1

CONSTIPATION

DESCRIPTION

  • Caused by poor tone in abdominal muscles and immobility due to weakness3
  • Constipation is common in babies with spinal muscular atrophy3

HOW IT MAY AFFECT THE CHILD

  • Chronic constipation and fecal impaction may occur3,4

DYSPHAGIA (DIFFICULTY SWALLOWING)

DESCRIPTION

  • Poor head control may affect the safety of swallowing5
  • In older children, limited range of jaw movement, decreased bite force, and fatigue in the muscles involved in chewing may contribute to swallowing difficulty6

HOW IT MAY AFFECT THE CHILD

  • Poor weight gain in patients with later-onset SMA5
  • Patients may be at risk of aspiration of food or liquids and aspiration pneumonia5

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 swallowing1,7

HOW IT MAY AFFECT THE CHILD

  • Oral feedings may result in aspiration pneumonia3
  • Feeding issues may lead to failure to thrive3
  • Feeding tubes placed by percutaneous gastrostomy may be considered before patients develop pneumonia3

GASTROINTESTINAL PROBLEMS

DESCRIPTION

  • Bloating, spitting up, vomiting after meals, and abdominal distention4

HOW IT MAY AFFECT THE CHILD

  • May lead to undernutrition4

GERD (GASTROESOPHAGEAL REFLUX DISEASE)

DESCRIPTION

  • Scoliosis may contribute to increased abdominal pressure leading to hiatal hernia and reflux gastroesophagitis8
  • Constipation can worsen gastric reflux or respiratory symptoms7

HOW IT MAY AFFECT THE INDIVIDUAL

  • Individuals may experience heartburn and pain7
  • Silent GERD may lead to increased risk of aspiration of stomach contents into the lungs4,7

OBESITY/
OVERNUTRITION

DESCRIPTION

  • Non-ambulatory individuals with spinal muscular atrophy have increased fat mass and may become overweight9
  • Excessive weight gain due to decreased activity, and a reduction in overall metabolic demand10

HOW IT MAY AFFECT THE CHILD

  • Obesity may lead to pain and increased risk of complications in the hips and back4
  • Obese individuals are at increased risk of diabetes and hypertension4

UNDERNUTRITION

DESCRIPTION

  • Weight for age lower than the 5th percentile may suggest undernutrition4
  • Weight for length less than the 50th percentile may indicate undernutrition4

HOW IT MAY AFFECT THE CHILD

  • Undernutrition may lead to growth failure2
  • May increase the risk of infection4,11
  • May lead to difficulty with wound healing4
  • May increase tendency to develop pressure sores4
  • May lead to fatigue11

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

POTENTIAL BENEFITS

NASOJEJUNAL
TUBE

POTENTIAL BENEFITS

  • Short-term solution while awaiting gastrostomy tube placement1

NASOGASTRIC
TUBE

POTENTIAL BENEFITS

  • Short-term solution while awaiting gastrostomy tube placement1

References

1. Mercuri E, Finkel RS, Muntoni F, et al; SMA Care Group. Diagnosis and management of spinal muscular atrophy: part 1: recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103-115. 2. 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. 3. Iannaccone ST. Modern management of spinal muscular atrophy. J Child Neurol. 2007;22(8):974-978. 4. 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.. 5. 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. 6. 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. 7. 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. 8. 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. 9. 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. 10. 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. 11. 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 a multidisciplinary approach.1