Management
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PROP Nutrition Management Guidelines
First Edition
March 2017, v.1.2
Updated: September 2017
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Nutrition Recommendations
Question
2. For individuals with PROP, what nutrition interventions are associated with positive outcomes?
Conclusion Statement
Derived from evidence and consensus based clinical practice

Individuals with PROP, at initial presentation or during illnesses, typically present with dehydration, hyperammonemia, and metabolic acidosis.  They require aggressive management that includes removing the toxic organic acids that have accumulated due to the metabolic block, minimizing protein catabolism, and promoting anabolism. Continuous hemofiltration has been used for rapid removal of toxins and allowing administration of larger volumes of fluids without risk of over hydration. Insulin has been administered to increase glucose tolerance.  Gradual rehydration with energy sources administered intravenously in amounts typically exceeding maintenance requirements, has helped to achieve an anabolic state prior to restarting oral intake.

Regular monitoring of clinical and nutritional status to determine adequacy of nutrient intake, and to guide appropriate modifications in the dietary prescription, is central to management of individuals with PROP.  Specific dietary recommendations and interventions are greatly influenced by the individual’s present clinical status, illness, failure to thrive, hypotonia, decreased mobility, and delayed feeding skills.  The main goal is to adjust nutrient intake to assure normal growth, and positive clinical and patient-reported outcomes. Challenges to achieving adequate medical and nutrition management include risk of infection, metabolic crises, vomiting, anorexia and other co-morbidities.  Despite these challenges, reports have shown that early and aggressive medical and nutrition management, typically requiring both parenteral and enteral nutritional support with use of medical foods limited in propiogenic amino acids, can result in improved survival and clinical outcomes. 

Definitions of the protein terminology used throughout this guideline are listed in Appendix B.

Recommendation 2.1

During acute illness, or at first presentation, provide aggressive nutrition management to optimize energy intake, to prevent or reverse catabolism and promote anabolism, to achieve rehydration, and to minimize propionate and ammonia accumulation.  See TABLE #6, Nutrition Management of Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 2.2

Restrict protein intake for no longer than 24-48 hours, before re-introducing complete (intact) protein (in either enteral or parenteral feedings) at approximately 0.5gm/kg/day, then increasing by increments of 0.25 gm/kg/day as tolerated. See TABLE #6, Nutrition Management of Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 2.3

Complete the total protein requirement, if not met by complete (intact) protein, with propiogenic-free amino acid medical food (enteral) or propiogenic amino acid free amino acid TPN solutions (parenteral). See TABLE #6, Nutrition Management of Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 2.4

Utilize tube feeding as necessary to supplement oral intake of nutrients and fluid, administer medications, and reduce fasting intervals. See TABLE #6, Nutrition Management of Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 2.5

Promote development and/or maintenance of oral skills for the tube-fed individual with use of adaptive / assistive feeding devices and behavior intervention and positive reinforcement and guidance techniques. See TABLE #6, Nutrition Management of Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 2.6

For the metabolically stable individual, meet the nutrient requirements listed in Question 1, with adjustments of intake made for individual tolerance, growth spurts and minor illnesses. (See also  TABLE #5, Nutrient Sources in the Nutrition Management of Well Individuals with PROP).

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
Recommendation 2.7

Provide an individualized emergency home feeding plan for mild illness (i.e., episodes without G.I. symptoms or greater than “small” urinary ketones) for: reducing total protein intake for 24-48 hours; increasing energy intake from carbohydrates/fats; supplying adequate  hydration; continuing medical food as tolerated; monitoring clinical signs and symptoms; and providing metabolic clinic contact information. See TABLE #6, Nutrition Management of Individuals with PROP.

Strength of Recommendation:
Insufficient EvidenceConsensusWeakFairStrong
Clinical Action:
ConditionalImperative
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