From the Guidelines
Patients with Medium Chain Acyl-CoA Dehydrogenase (MCAD) deficiency should follow a diet that avoids fasting for extended periods and limits medium-chain triglycerides (MCT oil), while enriching their diet with a high protein intake. The primary goal is to prevent metabolic crises by ensuring the body has a constant supply of energy. According to the ESPEN guideline on hospital nutrition 1, enrichment with MCT (and a diet high in protein) is suggested for specific patient groups with fatty acid oxidation disorders, but for MCAD deficiency, the focus should be on avoiding MCT oil and maintaining a balanced diet with frequent meals.
- Key dietary recommendations include:
- Eating frequent meals and snacks containing carbohydrates every 3-4 hours during the day
- Limiting overnight fasting to no more than 8-12 hours, depending on age
- Avoiding dietary supplements and foods high in MCT oil, such as coconut oil
- Consuming high-carbohydrate drinks or foods during illness to prevent catabolism
- It is essential to note that a low-fat diet is generally not necessary during regular circumstances, but patients should be cautious about their fat intake, particularly regarding medium-chain triglycerides. The ESPEN guideline 1 provides insight into the management of rare metabolic disorders, emphasizing the importance of personalized dietary approaches. By following these dietary restrictions and guidelines, patients with MCAD deficiency can reduce the risk of metabolic crises and improve their overall quality of life.
From the Research
Dietary Restrictions for Medium Chain Acyl-CoA Dehydrogenase Deficiency
- Patients with medium chain acyl-CoA dehydrogenase deficiency are advised to avoid prolonged fasting and maintain a high-carbohydrate, reduced-fat diet 2
- Avoidance of fasting is considered the mainstay of treatment for medium chain acyl-CoA dehydrogenase deficiency 3, 4
- A high-carbohydrate diet is recommended to help prevent hypoglycemia and other complications associated with the disorder 2
- Reducing fat intake is also recommended, as medium chain acyl-CoA dehydrogenase deficiency affects the body's ability to metabolize medium-chain fatty acids 2
Importance of Dietary Management
- Dietary management is crucial in preventing acute attacks and long-term complications of medium chain acyl-CoA dehydrogenase deficiency 2, 5
- Proper dietary management can improve outcomes and reduce the risk of mortality and morbidity associated with the disorder 5
- Patients with medium chain acyl-CoA dehydrogenase deficiency should work with a healthcare provider or registered dietitian to develop a personalized diet plan that meets their nutritional needs and helps manage their condition 6