MCAD deficiency, also known as medium chain acyl CoA dehydrogenase deficiency, is a genetically inherited disorder. It is an autosomal recessive disorder. The characteristic symptom of this disorder is a dangerous hypoglycemia and even sudden death or coma. This condition is triggered by long duration of fasting, or severe illness or vomiting episodes.
How To Prevent MCAD Deficiency?
MCAD deficiency is typically triggered by long periods of fasting. It is also triggered in conditions like a severe illness, wherein there is a metabolic decompensation and in high energy requirement. In order to prevent MCAD deficiency, it is essential to avoid long duration of fasting. It is also advisable to take precautions so that you do not fall ill very often, as during an illness, the body’s food intake typically goes down and energy requirements are very high. People affected by MCAD deficiency disorder are typically asymptomatic until they face a situation, which exposes them to extended periods of fasting or a severe illness. As a result, the people affected from MCAD deficiency can stay asymptomatic practically for their whole lives, if they are not exposed to such conditions. Consequently, a sudden death may be the first symptom for the people affected by this condition, as the first episode of exposure can lead to death in such people.
In absence of MS screening in earlier days, the only way to suspect a person for MCAD deficiency was by way of his presenting signs and symptoms. Also, in most of the cases of sudden death, MCAD deficiency was suspected and even diagnosed post-mortem. However, with the MS screening option available these days, the condition can be identified in early infancy, just within hours of the birth of the baby. Once the condition is diagnosed, the general strategy of treatment and management mostly focuses on the prevention of the symptoms arising from the MCAD deficiency as a primary goal.
Preventing MCAD deficiency through dietary modifications:
Dietary guidelines for infants-
- Infants affected by MCAD deficiency can be fed on formula or breast milk just like other children
- The formulas these days and, also the breast milk contain significant quantities of fats as a source of calories, which is utilized for anabolic processes through the first year
- The frequency of feeds for an otherwise well MCAD deficiency infant is the same as that for any other infant
- Only precaution to be taken is during the night time fasting period, which should not be for more than 4 hours between birth to 4 months and an additional hour from 5-12 months
- The sufficiency of breast milk should be monitored in breastfed infants by ensuring a proper weight gain and feeding satiety in an infant
- Formulas with MCT oil should be avoided in MCAD deficiency infants
Dietary guidelines for children and adults-
- People affected by MCAD deficiency should generally follow a heart healthy diet
- The diet should comprise of fresh fruits and vegetables everyday
- About 30% of the calorific values should come from the fats
- The diet should consist of complex carbohydrates
- Overeating and excessive consumption of calories should be avoided
- Watch your weight, gaining a healthy weight is of prime importance
- Obesity as well as extended hours of fasting can lead to negative consequences
The diet that is very popular these days advocates the use of high protein or fat and low carbs. This is highly inappropriate, especially for someone suffering from MCAD deficiency
Nutritional Supplements may not be needed generally as the dietary guidelines for MCAD deficiency do not recommend including or avoiding any nutrient or nutrients
MCAD deficiency is a genetically inherited autosomal recessive disorder. In order to prevent MCAD deficiency, it is most important to avoid long durations of fasting and take precautions to avoid severe illnesses or vomiting. It is recommended to consume recommended calories according to age, sex and weight and follow a diet according to the recommendations of a dietician, to prevent MCAD deficiency.