Management
Guidelines
Portal
VLCAD Nutrition Management Guidelines
First Edition
February 2019, v.1.0
Current version: v.1.1
Updated: February 2019
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Resources
Overview

A limited number of educational resources are currently available to support nutrition management of VLCAD.

Learning Objectives for Patients/Families

Learning objectives for individuals with VLCAD and their caregivers include:

  • Understand the deficiency of the VLCAD enzyme and effects on body metabolism
  • Understand the importance of adequate energy intake
  • Recognize the need to monitor signs and symptoms of metabolic decompensation
  • Recognize the need for frequent contact with appropriate healthcare providers, especially the importance of seeking medical advice during illness
  • Acquire skills needed to implement treatment recommendations
  • Realize the importance of strict adherence to treatment recommendations
  • Recognize the role of medical food in the dietary management
Resources for patients

Support and Advocacy Groups

  • Fatty Oxidation disorders (FOD): The FOD Group website and online 'Communication Network' Newsletters are intended to be used as a worldwide resource for families, friends, clinicians, researchers and others who would like to support, educate and provide a forum for the sharing of ideas and concerns for those whose lives have been touched by a Fatty acid Oxidation Disorder. This is an all-volunteer national and international support group specifically for Fatty Oxidation Disorders.

https://www.fodsupport.org

  • STAR-G Genetic Fact Sheets for VLCAD (updated February 28, 2016) in English and Spanish, concise, but accurate fact sheets that are in a downloadable format.

https://www.newbornscreening.info/Parents/fattyaciddisorders/VLCADD.html

  • Genetic Home References : (VLCAD website reviewed February 2018, published August 14, 2018) provides a brief overview VLCAD and genetic inheritance, with a comprehensive list of links to many other resources in English.

https://ghr.nlm.nih.gov/condition/very-long-chain-acyl-coa-dehydrogenase-deficiency

  • New England Genetics Collaborative: Genetics Education Materials for School Success (GEMSS) https://www.gemssforschools.org/sites/www.gemssforschools.org/files/library/pdfs_for_printing-dec2015/printable_vlcad-12.22.15.pdf
  • Social Media: VLCAD, FAOD on Facebook, Instagram, Twitter, etc.
  • National Organization for Rare Disorders (NORD): NORD is a patient advocacy organization dedicated to individuals with rare diseases and the organizations that serve them. NORD, along with its more than 280 patient organization members, is committed to the identification, treatment, and cure of rare disorders through programs of education, advocacy, research, and patient services.

https://rarediseases.org

  • CLIMB (Children Living with Inherited Metabolic Disorders): United Kingdom's provider of free metabolic disease information to young people, adults, families, professionals and other interested groups. Their mission is to provide metabolic disease-specific information, advice and support to children, young people, adults, families and professionals in the United Kingdom and to provide information and support to families worldwide, to fund educational and primary research programs and to investigate treatments and medical services.

http://www.climb.org.uk
 

Nutrition Information

  • Online smartphone Websites and Apps to track Fat and Calorie content in foods.
  • Online USDA Food Composition Database https://ndb.nal.usda.gov/ndb/
Resources for providers

($ = indicates available for purchase)

Find a Nearby VLCAD Health Care Provider

VLCAD Medical Information Online

  • American College of Medical Genetics (ACMG) ACT SHEET and algorithm for VLCAD : information to guide providers through confirmatory testing following an abnormal newborn screen for VLCAD.

    http://www.acmg.net/PDFLibrary/C14.pdf

    http://www.acmg.net/PDFLibrary/C14-1-Elevated-Algorithm.pdf

  • GeneReviews: a collection of expert-authored, peer-reviewed disease descriptions that apply genetic testing to the diagnosis, management, and genetic counseling of patients and families with specific inherited conditions. Developed under the National Center for Biotechnology Information (NCBI), a division of the National Library of Medicine (NLM) at the National Institutes of Health (NIH).

https://www.ncbi.nlm.nih.gov/books/NBK6816/

  • Screening, Technology And Research in Genetics (STAR-G) Project: a multi-state collaborative effort, led by the Hawai`i Department of Health, to obtain research data, identify strategies and develop materials for addressing the financial, ethical, legal and social issues (FELSI) surrounding the use of tandem mass spectrometry (MS/MS) for neonatal metabolic screening of culturally and ethnically diverse populations, funded by the Health Resources and Services Administration (HRSA) through a Special Projects of Regional and National Significance (SPRANS) grant. The STAR-G website includes general information on newborn screening, genetics and links to the ACMG fact sheets and algorithms, including VLCAD (last updated 2/28/16)

https://www.newbornscreening.info/information/information-for-providers/genetics-overview-for-providers/
 

Clinical Nutrition Tools

  • Metabolic Pro: nutrient analysis software that includes data for foods and beverages, including medical foods and formulas. Developed with educational grants from Nutricia North America and BioMarin. Annual user license available for a fee from Genetic Metabolic Dietitians International (GMDI). 

https://metabolicpro.org/

  • Online USDA Food Composition Database

https://ndb.nal.usda.gov/ndb/

© 2006-2015   —   SOUTHEAST REGIONAL GENETICS NETWORK   —   A HRSA SUPPORTED PARTNERSHIP   —   GRANT #UH7MC30772