Gastrointestinal and nutritional care in pediatric neuromuscular disorders

Table 1 Neuromuscular diseases and Neuromuscular disorder-related nutritional issues

NMD	Potential nutritional issue(s)
Spinal muscular atrophy	Dysphagia, weight lossa
Duchenne muscular dystrophy	Overweight/obesity, dysphagia, weight lossb
Myotonic dystrophy type 1	Weight loss/underweight, feeding difficulties
Congenital myopathies	Underweight/growth failure, feeding and swallowing difficulty
Charcot-Marie-Tooth disease	Unknown
Limb-Girdle muscular dystrophy	Unknown

^aEspecially spinal muscular atrophy type I and II.

^bIn later stages of Duchenne muscular dystrophy (due to progressive muscle weakness).

NMDs: Neuromuscular disorders.

Table 2 Nutritional and gastrointestinal management in children with Duchenne muscular dystrophy

Issue	Evidence
Tube feeding	Low to high probability (based on the stage of the disease)
Energy needs	Meet DRI
Formula	No special diet or formula
ONS	Possible; consider a speech-language therapist evaluation
Gastroesophageal reflux	Proton pump inhibitors; consider hydrolyzed or natural food enteral formulas
Constipation	PEG; consider hydrolyzed or natural food enteral formulas
Delayed gastric emptying	Meet fiber and fluid needs; prokinetics may be used

DMD: Duchenne muscular dystrophy; DRI: Daily recommended intake; ONS: Oral nutrition supplements; PEG: Polyethylene glycol.

Table 3 Nutritional and gastrointestinal management in children with spinal muscular atrophy

Issue	Evidence
Tube feeding	High probability (type 1 and 2)
Energy needs	Meet DRI
Formula	No special diet or formula
ONS	Possible (type 3 and 4); consider a speech-language therapist evaluation
Gastroesophageal reflux	Proton pump inhibitors; consider hydrolyzed or natural food enteral formulas
Constipation	PEG; consider hydrolyzed or natural food enteral formulas
Delayed gastric emptying	Meet fiber and fluid needs; prokinetics may be used

DRI: Daily recommended intake; ONS: Oral nutrition supplements; PEG: Polyethylene glycol.