Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD)

Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD)
A Fatty-acid Oxidation Disorder

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What is it?
Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD) is a rare autosomal recessive condition in which the body cannot oxidize fatty acids because an enzyme is either missing or not functioning correctly.

Inheritance and Frequency
The gene defect for VLCADD is an autosomal recessive genetic trait and is unknowingly passed down from generation to generation. This faulty gene usually emerges when two carriers have children together and pass it to their offspring. For each pregnancy of two such carriers, there is a 25% chance that the child will be born with the disease and a 50% chance the child will be a carrier for the gene defect.

VLCAD Deficiency is estimated to affect one in 42,500 to 125,000 newborns.

Signs & Symptoms
VLCADD's initial manifestations may include hypoketotic hypoglycemia, hepatocellular disease, and cardiomyopathy. Fatal infantile encephalopathy may be the only indication of the condition.

Long Term Effects

Treatment
Treatment of VLCADD usually consists of avoidance of fasting (by frequent meals) and use of IV glucose required when food cannot be tolerated (such as with a virus, cold, flu, or other common illness). Intake of long-chain fatty acids should be avoided. Supplemental carnitine is recommended for some affected children.

History

Screening
Visit the What Does Your State Screen page to learn about your state's newborn screening program.

Supplemental Screening
If you live in a state that does not perform screening on its newborns for all detectable disorders, there are laboratories that will provide this screening for you no matter your state of residence. For more information about supplemental screening, visit our supplemental screening page. Also visit our frequently asked questions page for more information on newborn screening.

Other Sites of Reference

Support Groups

VLCAD Support
New members welcome...click above to join!
Email: Write to Gina or Write to Lisa for information

FOD Family Support Group
2041 Tomahawk
Okemos, MI 48864
Contact Person: Deb Lee Gould
Phone, 8 AM to 8 PM EST, daily: (517) 381-1940
Email: Write to Deb

James William Lazzaro Foundation
4493 Liberty Road
South Euclid, OH 44121
Contact Person: Jamie Lazzaro
Phone: (502) 254-2209
Email: Write to JWL Foundation

United Mitochondrial Disease Foundation
P.O. Box 1151
Monroeville, PA 15146-1151
Phone: (412) 793-8077
Email: Write to UMD Foundation

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Revised 02/03/2007