Oral intake of carnitine supplementsMost patients with
MCAD deficiency will at some point have a decreased amount of free
carnitine in their blood. See also the chapter on the role of
carnitine in fat metabolism for more information about why
that happens.
In theory replenishing the stocks of carnitine by intake of oral supplements will help the body to better excrete the toxic substances that are the result of a faulty beta-oxidation. These toxic substances are excreted via the urine. In theory replenishing the stocks of carnitine can make sure that in times of great metabolic stress, the long-chain fatty acids can more easily enter the matrix of the mitochondrion. There they can be oxidized by beta-oxidation and produce some amount of energy. In theory, however, replenishing the stocks of carnitine can, in times of great metabolic stress, transport more long-chain fatty acids into the mitochondria and increase the fat metabolism. This creates even more medium-chain fatty acids that can not be processed further and thus puts even more stress on the already stressed metabolism. It is also possible that the body will increase its own creation of carnitines in times of great need. (Carnitines can also be created by the body, next to the carnitines the body gets from intake of food). This would especially be the case in case of high physical activity. In practice, unfortunately, no substantial tests have been done to prove that also in practice the intake of carnitine supplements will improve the general condition of the patient with MCAD deficiency. It is also known that patients with a carnitine deficiency (as primary disorder, not as secondary consequence of MCAD deficiency) will more easily develop heart conditions. The intake of oral supplements of carnitine is not harmful for patients with MCAD deficiency. Despite the controverse around carnitine supplements, doctors will usually choose to replenish the stocks of carnitine via oral intake. It can't do any harm and it may do some good. Some doctors will start carnitine supplements as soon as the diagnosis of MCAD deficiency is made (regardless the level of carnitine in the blood). Other doctors will only start the carnitine supplements as soon as the carnitine levels in MCAD patients is too low. Finally some doctors never start carnitine supplements. Always contact your doctor if you have questions about this! Also important to know is that only carnitine supplements prescribed by your doctor are suitable! Carnitine supplements are also for sale in sports nutrition stores, but those can be capsules who desintegrate too slowly and of which only 60% of the carnitine present can also be absorbed. Those are not suitable for treating metabolic disorders! So far only precautions for normal circumstances were discussed. Read further about precautions in cases of emergency >
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This page was last modified on 1 March 2011 | |||