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

There is treatment for Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD). The treatment and management of VLCADD depends on the type of VLCADD that a child or adult has and its severity.

In general, treatment involves avoiding fasting and seeking treatment when the person is not well. These situations occur when going a long time without eating, when sick, undergoing surgery, or when doing strenuous exercise. Treatment may also include dietary management and supplements. Speak with your metabolic physician to discuss what treatments are recommended.

The next steps depend on the age of the individual and the symptoms that they have had or are having. For example, people affected by the severe forms of the condition who have symptoms of low blood sugar, should immediately be given intravenous (IV) glucose, a form of sugar that the child can burn for energy. Individuals who are not currently having symptoms should see a metabolic genetics specialist, learn about the condition, its symptoms, and treatment and should make sure that their health care providers are aware of the condition. Families should carry a letter from their doctor clearly stating the diagnosis and details of recommended treatment for low blood sugar that they can show at the emergency room if their child gets symptoms. Speak with your metabolic physician to discuss what treatments are recommended.

Individuals with the more severe forms of Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) should avoid going a long time without eating. They should be given frequent meals. If a person with VLCADD does go without eating and their blood sugar levels drop, they should be treated with intravenous (IV) glucose as an energy source. They should also be monitored for heart rhythm problems, and the breakdown of heart muscle tissue.

If heart muscle is breaking down, the child should be well hydrated (receive plenty of fluids either by mouth or intravenously). This will protect the kidneys from damage which can occur when the byproducts of the muscle breakdown reach the kidneys. Heart problems may be reversible with early, intensive supportive care and modified diet.

Children with severe early-onset cardiac and multi-organ failure form of VLCADD are usually given a low-fat diet, with additional calories given in the form of a 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. The low fat diet varies for each child but often ranges from 13%-39% of calories as total fat, and then another 15%-18% of calories supplied as MCT oil.

Families should carry a letter from their doctor clearly stating the diagnosis and details of recommended treatment for low blood sugar that they can show at the emergency room if their child gets symptoms.

Speak with your metabolic physician and metabolic dietitian 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.

Individuals with the more severe forms of Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD) should avoid going a long time without eating. They should be given frequent meals. If a person with VLCADD does go without eating and their blood sugar levels drop, they should be treated with intravenous (IV) glucose as an energy source. They should also be monitored for heart rhythm disturbance, and the breakdown of heart muscle tissue.

Children with hepatic or hypoketotic hypoglycemic form of VLCADD are usually given a low-fat formula, with additional calories given in the form of a 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 medium chain fats for energy, even when they are not able to burn other kinds of fat. The low fat diet varies for each child but often ranges from 13%-39% of calories as total fat, and then another 15%-18% of calories supplied as MCT [Solis & Singh 2002].

Families should carry a letter from their doctor clearly stating the diagnosis and details of recommended treatment for low blood sugar that they can show at the emergency room if their child gets symptoms. Speak with your metabolic physician and dietitian 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.

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.

Carnitine is a vitamin that is found in many foods. The body uses carnitine to help burn fat for energy. Carnitine is used in treating some other disorders that are related to Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency (VLCADD).

The use of carnitine is controversial and different metabolic centers have different recommendations. Talk to your metabolic physician if you have questions about whether carnitine supplements should be tried in you or your child.

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.