Oral intake of carnitine supplements
Most 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
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.
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,
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
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,
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
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
Some doctors will start carnitine supplements
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!
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 >