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VLCAD Nutrition Management Guidelines
First Edition
February 2019, v.1.0
Current version: v.1.1
Updated: February 2019
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Benefits and Harms of Implementing the Recommendations
Overview

Nutrition management of individuals with VLCAD often includes restriction of long-chain dietary fat, supplementation with medium chain triglycerides (MCT), and eating at regular intervals. Lack of appropriate nutrition management can place a patient at nutritional risk and may lead to negative outcomes. However, intercurrent illness, stress, injury, or surgery can trigger an acute event leading to complications including hypoglycemia, cardiomyopathy, and rhabdomyolysis, the pathophysiology of which is not well-defined. Therefore, outcomes in individuals with VLCAD is multifactorial and not dependent on nutrition management alone.

Potential Harms

Potential harms include:

  • Severe long chain fat restriction without proper nutrition monitoring could result in essential fatty acid deficiency. EFA deficiency is characterized by scaly dermatitis, alopecia, thrombocytopenia, and, in children, growth failure and intellectual disability.
  • Diets severely restricted in fat may be low in fat soluble vitamins.
  • Medium chain triglyceride intake has been associated with nausea and gastric distress, especially when first introduced. This can be minimized or by starting with small doses and increasing the dose as tolerated.
  • Frequent feeding is recommended in VLCAD; however, in order to prevent excessive weight gain, energy intake and weight must be monitored
Potential Benefits

Implementing the recommendations can potentially:

  • Improve patient outcomes
  • Reduce variations in clinical practice and services across medical center
  • Enhance patient quality of life, prevent untoward consequences and complications and reduce associated medical, educational and social costs
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