Nutrition
Nutrition is critically important for maintaining muscle mass and strength and minimizing fatigue in SMA patients. Intermittent monitoring of dietary intake in consultation with a dietician experienced in management of patients with neuromuscular conditions or metabolic disorders can be extremely helpful.
Children may be deficient in carnitine due to decreased intake of meat related to jaw contractures interfering with chewing. Swallowing problems sometimes be difficult to detect, and in weaker children or in those with borderline nutritional status or frequent respiratory illness, swallowing studies should be performed on a regular basis to ensure that silent aspiration isn't contributing to respiratory problems.
Maintenance of appropriate nutrition is especially critical during illness. SMA subjects have diminished lean body mass and a secondary defect in fatty acid oxidation that limits their reserve in the setting of prolonged fasting. Thus, when concerns regarding the ability to safely administer oral feeds develop, alternative forms of nutrition should be considered. Options include temporary nasogastric or nasojejunal feeds, or peripheral or total parenteral nutrition (PPN or TPN).
- General Nutrition Guidelines for SMA Children
- Guidelines for Gastrostomy Tube Feeding for Infants with SMA type I
- Nutritional Care Guidelines for Children with SMA during Acute Illness or Fasting
- Nutritional Care Guidelines for Adults with SMA during Acute Illness or Fasting
- Peri-operative Nutritional Care Guidelines
- Dietary Record
See Special Considerations in SMA Type I Infants for more information.