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Very-long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a condition in which the body is unable to break down certain fats. It is considered a fatty acid oxidation condition because people affected by VLCAD are unable to convert some of the fats they eat into energy the body needs to function.
Enzymes help start chemical reactions in the body. VLCAD happens when an enzyme called “very long chain acyl-CoA dehydrogenase” is missing or not working. This enzyme breaks down certain fats from the food we eat into energy. It also breaks down fat already stored in the body.
Newborn screening tests look for developmental, genetic, and metabolic disorders in the newborn baby. This allows steps to be taken before symptoms develop. Most of these illnesses are very rare, but can be treated if caught early. The types of newborn screening tests that are done vary from state to state.
Management of VLCAD deficiencies is primarily focused on preventing acute episodes of low blood sugar (hypoglycemia). This process includes avoiding fasting for long periods of time. A very low-fat high-carbohydrate diet, with frequent feeding is recommended. In some severe cases continuous feeding may be necessary. A doctor may recommend supplements like Carnitine, MCT oil and other supplements. Limiting exercise, avoiding cold/heat exposure, and not fasting may be sufficient to control the symptoms in mild cases.
If there is a loss of consciousness or signs of dangerously low blood sugar, medical treatment should be
sought immediately. Intravenous glucose fluids are given to address the hypoglycemia. An emergency
letter detailing the patients prescribed treatment to manage severe episodes should be carried at all
times.
The majority of fatty acid oxidation disorder patients are identified right after birth because of the newborn screening test. Signs of VLCAD deficiency can occur at any age, from birth to early adulthood. The following are symptoms: sleeping longer or more often, tiredness, behavior changes, muscle weakness (known as hypotonia), irritability, poor appetite, fever, vomiting, diarrhea, low blood sugar (called hypoglycemia).
Many of these signs may occur when your baby eats foods that his or her body cannot break down. They can be triggered by long periods of time without eating, illnesses, and infections. If your baby shows any of these signs, be sure to contact your baby’s doctor immediately.
The information on this site is not intended or implied to be a substitute for professional medical advice, diagnosis or treatment. All content, including text, graphics, images and information, contained on or available through this web site is for general information purposes only.
