In case of emergencies
Besides the precautions to be taken in normal circumstances also some precautions in case of emergency are necessary. Special attention is required if a patient with MCAD deficiency is ill, with vomiting, diarrhea and/or fever.
If a patient vomits and/or has diarrhea,
there is no or not an adequate intake of carbohydates from the meals,
and the body of the patient will switch to the fat metabolism to
produce energy. Because the fat metabolism does not function properly
(see also the pages on fat metabolism)
not enough energy can be produced from this, and at the same time some
toxic metabolites of the failing fat metabolism will be accumulated. In
these cases it is necessary to give the patient frequently extra
carbohydrates, also at night. This can be done for example by mixing a
powder rich in carbohydrates with water and let the patient drink it
every hour. Examples of this kind of powder are Caloreen and Fantomalt (links open in a new window).
Also fever requires special attention in patients with MCAD deficiency. A higher
body temperature requires more energy. This causes the acceleration of
the fat metabolism. In a situation where the fat metabolism is already
activated due to vomiting or diarrhea, this could make an already bad
situation even worse.
If
drinks rich in carbohydrates are refused or vomited, instant admission
to the hospital is needed for treatment with IV glucose.
Referenties
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