MCAD deficiency (MCADD), also known as medium chain acyl CoA dehydrogenase deficiency disorder, is a condition caused by genetic mutations in the MCAD gene. This mutation causes deficiency in the activity of the enzymes that are responsible for the beta oxidation of the medium chain fatty acids. It is a genetically autosomal recessive disorder, which means that in order to pass on the disorder to the child, both parents must each contribute one gene to the child. If the child inherits only one gene, then the child will not suffer from MCAD deficiency, but child will be a carrier for MCAD deficiency.
What Is The Diet For MCAD Deficiency?
- The primary goal of the MCAD deficiency treatment is to prevent the problems from starting in the first place. A person affected with this disorder primarily shows symptoms if the person goes on long or extended fasting periods, or if he has high energy requirements than usual.
- The primary thing to be considered is to avoid any long periods of fasting to avoid any symptoms
- Illness often leads to a poor intake of food yet high needs for energy. It is advisable to take necessary precautions so that you do not fall ill often
Diet Needs In Infancy
- An infant with MCAD deficiency can have regular formula or breast milk as other babies
- Most of the infant formulas and the breast milk consist of about 55% of calories as fat
- Most of these amounts is used in the first year of the baby’s life for anabolic processes
- In case of breastfed infants, it is advisable to keep a watch on the quantities of breast milk produced, by ensuring a proper weight gain and feeding satisfaction of the baby
- If the baby is not feeding sufficiently, then some clinics or clinicians may use high carbs snacks, cornstarch as a source of glucose for such infants and children, especially before the nighttime fasting period
- However, if the infants are feeding properly and are generally well, there is no need of such supplements
- Infant formulas that contain MCT oil are not to be given to an infant with MCAD deficiency.
Diet Needs In Children And Adults
- If you suffer from MCAD deficiency, you must consume diets that are considered good for heart or are heart healthy
- The diet must comprise of enough kcal that are recommended according your age and weight
- With the help of a dietician, plan a proper meal and ensure that you get about 30% of your calories from the fat
- Make sure that the diet includes fresh fruits and vegetables regularly.
- The diet should consist of complex carbs
- Take care not to consume extra calories than are needed and not to overfeed yourself
- A proper weight gain will help you in the long run, however, obesity and going on long term fasting to tackle obesity are both equally dangerous for someone suffering from MCAD deficiency.
- The diets which emphasize on high protein or fats and low carbs are not good for people suffering from MCAD deficiency.
- Nutrient supplementation may not be needed as MCAD deficiency dietary guidelines do not advise taking or avoiding any nutrient or nutrients.
The general guidelines in terms of diet that are to be followed are avoiding long duration of fasting, taking frequent or properly spaced meals and snacks and taking the proper calorific intake as per your age and weight. Avoiding metabolic decompensation during an illness can be achieved by taking glucose supplements during illness.
MCAD deficiency is a genetically inherited disorder. It is a genetically autosomal recessive disorder. It occurs due to mutations in the MCAD gene, which cause an impairment in beta oxidation of medium chain fatty acids. As a result, the stored fat cannot be used as a source of energy when all stores of glucose are depleted, like in a case of fasting or an illness. The condition is characterized by a dangerous hypoglycemia and even a sudden death in some instances.