MCAD is a genetically inherited disorder. It is an autosomal recessive disorder that is transferred to a child only if both parents contribute one mutated gene each. If only one parent contributes the mutated gene, the child will not be affected but will be a carrier of the disorder.

MCAD deficiency disorder is characterized by dangerous hypoglycemia and, also sudden death. It is recommended to not go for extended periods of fasting by those who suffer from this condition.

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Why Does MCAD Deficiency Cause Hypoglycemia?

Why Does MCAD Deficiency Cause Hypoglycemia?

MCAD deficiency is an autosomal recessive inherited disorder. It means that both the parents must contribute one mutated gene for the disorder each, for the child to be affected by the condition

  • If only one parent contributes the mutated gene, the child will not be affected with the disorder but will be a carrier for the disorder
  • The MCAD enzyme carries out the dehydrogenation of fatty acids that are medium chain fatty acids or that have a chain length between 6 to 12 carbons
  • This oxidation of the fatty acids provides us with the energy after all the stores of glucose and glycogen are absorbed by the body
  • This oxidation process uniquely takes place whenever you follow very long or extended periods of fasting or when you suffer from an illness. During such phases, the calorie intake is decreased and the demand for energy is increased
  • Thus, due to the MCAD deficiency this oxidation process cannot take place and as a result hypoglycemia can happen as all stores of glucose and glycogen will be already exhausted and, also the fats will not be oxidized to create energy further
  • This absence of energy and consumption of all the sources of glucose leads to a dangerous hypoglycemia if there are extended periods of fasting or increased energy demands
  • It can therefore, result in a sudden death

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Other symptoms of MCAD deficiency are-

  • Many people may remain a symptomatic through their lives if they are not exposed to the conditions that cause a low glucose level
  • The symptoms are usually seen in early childhood at first
  • However, for some, they may be even seen for the first time in adulthood, as a result of an exposure to surgery or an illness
  • There is hypoglycemia along with liver dysfunction, which is usually seen due to very long duration of fasting
  • Infection with vomiting can also act as a triggering factor

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Treatment of MCAD Deficiency Disorder

  • Staying away from very long or extended periods of fasting is of primary importance while considering the treatment for MCAD deficiency
  • Almost all people who are affected by a fatty acid oxidation disorder need to undertake this precautionary measure
  • Any metabolic stress is to be avoided when you are suffering from an illness
  • In order to ensure this, taking supplements of glucose or simple carbs can prevent catabolism when you are going through an illness
  • Long, extended periods of fasting are to be avoided at any costs. however, it must be kept in mind that the duration of fasting depends upon the age and is also varies from person to person affected by MCAD deficiency
  • An illness can drastically change the general fasting period that is tolerated by an MCAD affected individual otherwise and hence that should be primarily kept in mind
  • As a precautionary measure, those suffering from MCAD deficiency should always carry an emergency note in their pockets, wallets or bags which clearly has your disorder mentioned in it. This is particularly important when traveling as in case of an emergency the doctors or medical attendants might be new and unaware of your condition.

The note will help them start a proper line of treatment promptly and efficiently.

MCAD deficiency is characterized by the presence of a dangerous hypoglycemia and even sudden death. Avoid long periods of fasting and seek medical attention emergency when needed.

Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: December 19, 2018

This article does not provide medical advice. See disclaimer

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