Acyl-coa dehydrogenase, very long-chain, deficiency of


Is there a treatment for Later-onset episodic myopathic Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD)?

People with later-onset episodic myopathic Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) should pay attention to their symptoms and avoid situations which causes them to break down muscle (rhabdomyolysis). People experiencing rhabdomyolysis typically have muscle pain and dark tea or cola colored urine.

They may also benefit from taking medium-chain triglyceride oil (MCT oil). MCT oil is a kind of fat that only contains medium chain fatty acids, but does not contain any very long chain fatty acids. People with VLCADD are able to burn MCT oil for energy, even when they are not able to burn other kinds of fat. Some people with later-onset episodic myopathic VLCADD may be able to increase their tolerance for exercise by supplementing with MCT oil. Speak with your metabolic physician to discuss what specific treatments are recommended.

A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website. If you are looking for a metabolic clinic for treatment in the United States, you can search for the clinic or clinics in your state.

A metabolic dietician can be found through the Genetic Metabolic Dieticians International webpage.

Behrend AM, Harding CO, Shoemaker JD, Martern D, Sahn DJ, Elliot DL, Gillingham MB. Substrate oxidation and cardiac performance during exercise in disorders of long chain fatty acid oxidation. Mol Genet Metab. 2012;105:110-5.

Genetic and Rare Disease Information Center (GARD). "VLCAD deficiency." Retrieved 1 June 2016. Available from:

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