long chain fatty acid disorder

From Symptoms to Solutions

In this article, we will delve into what Long Chain Fatty Acid Disorder (LC-FAOD) is, discuss the best and worst foods for this disorder, explore its signs and symptoms and examine treatment options, including the use of DOJOLVI (triheptanoin) as the first and only FDA-approved treatment for children and adults with long-chain fatty acid oxidation disorders.

Best and Worst Foods for Long Chain Fatty Acid Disorder

One of the key aspects of managing LC-FAOD is maintaining a specific diet that includes the right foods while avoiding others.

Best Foods

Complex Carbohydrates: Complex carbohydrates like whole grains, oats and brown rice are excellent energy sources for individuals with LC-FAOD. They provide a steady release of glucose, helping to maintain blood sugar levels and prevent hypoglycemia.

Medium-Chain Triglycerides (MCTs): Unlike long-chain fatty acids, MCTs are easier for the body to break down and use for energy. Foods rich in MCTs, such as coconut oil and palm kernel oil, can be beneficial for individuals with LC-FAOD.

Lean Proteins: Lean sources of protein, such as poultry, fish and tofu, provide essential amino acids without the excess long-chain fatty acids found in fatty cuts of meat.

Fruits and Vegetables: Fresh fruits and vegetables are packed with vitamins, minerals and fiber while being low in long-chain fatty acids. They are essential for maintaining overall health and nutrition.

Nutritional Supplements: Some individuals with LC-FAOD may benefit from specialized nutritional supplements prescribed by a healthcare professional. These supplements are designed to provide essential nutrients while minimizing long-chain fatty acid intake.

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Worst Foods

High-Fat Foods: Foods that are high in long-chain fatty acids, such as fried foods, fatty cuts of meat and full-fat dairy products, should be avoided as they can exacerbate symptoms and lead to complications.

Processed Foods: Processed foods often contain hidden sources of long-chain fatty acids in the form of cooking oils, additives and preservatives. Reading food labels and avoiding highly processed items is crucial.

Fast Food: Fast food options are typically high in unhealthy fats and should be avoided by individuals with LC-FAOD.

Sugary Snacks: High-sugar snacks and drinks can lead to rapid spikes and crashes in blood sugar levels, which can worsen symptoms in individuals with LC-FAOD.

Alcohol: Alcohol consumption can disrupt the body's ability to metabolize fatty acids and should be limited or avoided.

Understanding Long Chain Fatty Acid Disorder

Long Chain Fatty Acid Disorder is a group of inherited metabolic disorders that impair the body's ability to convert long-chain fatty acids into energy effectively. Long-chain fatty acids are an essential energy source, especially during periods of fasting or strenuous activity. When someone has LC-FAOD, their body struggles to break down these long-chain fatty acids into smaller units known as acetyl-CoA, which can be used for energy production.

Signs and Symptoms of Long Chain Fatty Acid Disorder

The signs and symptoms of LC-FAOD can vary widely among individuals. Here are some common symptoms of LC-FAOD:

  • Muscle weakness or pain during physical activity.
  • Hypoglycemia (low blood sugar) leading to dizziness, fatigue and confusion.
  • Enlarged liver or liver dysfunction.
  • Cardiac issues, including arrhythmias.
  • Rhabdomyolysis: a condition in which damaged muscle tissue releases toxic substances into the bloodstream.

Treatment Options for Long Chain Fatty Acid Disorder

The primary treatment goal for LC-FAOD is to prevent the buildup of toxic fatty acid metabolites and provide a consistent source of energy. Here are some common treatment options.

Dietary Management: Maintaining a carefully balanced diet, as discussed earlier, is critical for managing LC-FAOD. Regular meals and snacks can help stabilize blood sugar levels and provide essential nutrients.

Avoiding Fasting: Individuals with LC-FAOD should avoid fasting for extended periods, as it can trigger the breakdown of muscle tissue for energy.

Medical Foods: Specialized medical foods and formula diets may be prescribed by healthcare professionals to ensure individuals receive the necessary nutrients while minimizing long-chain fatty acid intake.

Carnitine Supplements: Some individuals with LC-FAOD may benefit from carnitine supplements, which can help transport fatty acids into the mitochondria for energy production.

Triheptanoin (DOJOLVI): Triheptanoin, marketed as DOJOLVI, is the first and only FDA-approved medication for the treatment of LC-FAOD in children and adults. It is a unique fatty acid triglyceride that provides an alternative energy source for individuals with LC-FAOD, bypassing the usual fatty acid metabolism pathway. By doing so, DOJOLVI can help prevent the accumulation of toxic metabolites and provide a more reliable source of energy.

Final Notes

Long Chain Fatty Acid Disorder is a rare metabolic disorder that requires careful dietary management to prevent complications and improve the quality of life for affected individuals. Individuals with LC-FAOD should follow a personalized treatment plan developed in consultation with healthcare providers to ensure the best possible outcomes.