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Very long-chain acyl-CoA dehydrogenase deficiency

(Very • Long • chain • ace-uhl • koh-AY • dee-hie-DRAH-juh-nace • di-FISH-uhn-see)

General Condition Information

Other Names

  • Very long chain acyl CoA dehydrogenase deficiency
  • Very long chain acyl-CoA dehydrogenase
  • Very long chain acyl-CoA dehydrogenase deficiency
  • Very long chain acylCoA dehydrogenase deficiency
  • Very long-chain acycl-CoA dehydrogenase deficiency
  • Very long-chain acyl-CoA deficiency
  • Very long-chain acyl-CoA dehydrogenase
  • Very-long chain acyl-CoA dehydrogenase deficiency
  • Very-long-chain acyl-CoA dehydrogenase deficiency

Condition Type

Birth Prevalence

  • It is estimated that fewer than 100 babies are born with this condition each year in the United States. 
  • Visit GeneReviews to learn more about how often this condition occurs.

Screening Finding

Elevated C14:1 +/- other long-chain acylcarnitines

What is very long-chain acyl-coa dehydrogenase deficiency

Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency is an inherited (genetic) condition that prevents the body from breaking down certain fats and turning them into energy.

VLCAD is an enzyme that processes types of fat called very long-chain fatty acids. This enzyme helps break down these fatty acids so your body can use or get rid of them.

Without enough of this enzyme, the body has trouble using fats for energy. This condition can be more or less severe depending on how much of the enzyme your baby can make.

Breaking down fat for energy allows your body to work properly, and it is especially important after a long time without food (fasting) and during illness. If your body does not make enough energy from fat, blood sugar levels can become dangerously low.

When fats are not processed, waste (including toxins) can build up and damage your liver, heart, and muscles.

If untreated, the lack of energy and increased toxins lead to the signs and symptoms of the condition. In severe cases, this condition can result in coma or death if not treated early.

Newborn Screening and Follow-Up

Condition Details

Treatment and Management

It is important to talk to your health care provider about which treatment(s) are best for your baby. The goal of treatment is to prevent the health problems caused by this condition.

Treatments may include the following:

  • Regular and frequent meals and snacks
  • Diet high in carbohydrates and low in fat
  • Medium chain triglyceride (MCT) oil to help give the body fats it can break down
  • L-carnitine supplements to help the body break down fats
  • People with VLCAD deficiency must be very careful if they get sick and have vomiting or diarrhea, or do not want to eat. They may need emergency care to prevent low blood sugar levels.

Children who receive early and ongoing treatment for VLCAD deficiency can have healthy growth and development.

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