Trimethylaminuria (TMAU), sometimes known as "Fish-Odor Syndrome," is a rare genetically transmitted metabolic disorder characterized by a distinctive, unpleasant body odor. This odor, often described as resembling rotten fish, feces, or garbage, results from the body's inability to properly break down trimethylamine (TMA), a chemical compound produced in the gut during the digestion of certain foods. While there is currently no cure for TMAU, dietary management plays a crucial role in alleviating symptoms and improving the quality of life for affected individuals.
Understanding Trimethylaminuria
In individuals with TMAU, the enzyme flavin-containing monooxygenase 3 (FMO3), which is responsible for metabolizing TMA into a non-odorous byproduct, is impaired. This impairment leads to a buildup of TMA in the body, which is then released through urine, sweat, breath, and reproductive fluids, causing the characteristic fishy odor. The severity of TMAU symptoms can vary, with some individuals experiencing a constant, strong odor, while others have a moderate smell that fluctuates in intensity.
Causes and Inheritance
Primary trimethylaminuria develops when an individual inherits abnormal FMO3 genes from both parents. In these cases, two non-functioning FMO3 genes are typically needed for a person to have symptoms. Both parents of an individual with trimethylaminuria are "carriers" of the condition, meaning they both carry one copy of an altered gene for FMO3.
Secondary TMAU can occur when something interferes with the FMO3 enzyme's ability to break down trimethylamine. This can be caused by illnesses such as liver failure or viral hepatitis, imbalances in the gut microbiome, or the use of choline or carnitine supplements.
Diagnosis
Diagnosis of trimethylaminuria involves a urine test to measure the levels of trimethylamine. Testing can be done under normal dietary conditions. However, it may help to consume a meal rich in choline (e.g., 2 eggs + 400 g "baked" [haricot] or soya beans) prior to testing. It is recommended that urine testing be performed on two separate occasions when the individual is on a non-restricted diet. Genetic testing, specifically gene sequencing, can also be used to identify mutations in the FMO3 gene.
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Dietary Management: A Personalized Approach
Dietary management is a cornerstone of TMAU symptom control. The primary goal is to reduce the intake of foods that are high in trimethylamine precursors, such as choline, trimethylamine N-oxide, and inhibitors of FMO3 enzyme activity. However, it's essential to recognize that dietary needs are highly individual, and what works for one person may not work for another.
General Dietary Recommendations
The main problem one encounters with recommending specific diet foods for TMAU is that not all TMAU+ persons do well with the TMAU protocol, implying that there might be (probably are) other concerns. Unfortunately, if we took into account what every single sufferer thinks triggers their respective odor symptoms, the diet would list one word only - WATER, and a water alone will not sustain life for very long. Therefore, the diet discussed in this post is strictly for TMA produced body/breath odor, and is based on the published TMAU protocol discussed and recommended in the article, Trimethylaminuria, written by Drs. Ian Phillips and Elizabeth Shephard.
The TMAU low-choline diet is very much a personalized development based on individual needs. It is essential to consult with a registered dietitian or licensed nutritionist experienced in TMAU to develop an individualized dietary plan.
Foods to Limit or Avoid:
- Trimethylamine-Rich Foods: Seafood (fish, cephalopods, and crustaceans) should be limited due to their high trimethylamine N-oxide content.
- Choline-Rich Foods: Eggs, liver, kidney, peas, beans, peanuts, soya products, and brassicas (Brussels sprouts, broccoli, cabbage, cauliflower) are high in choline and should be consumed in moderation or avoided.
- Lecithin and Lecithin-Containing Supplements: These should be avoided as they are precursors to trimethylamine.
- Inhibitors of FMO3 Enzyme Activity: Brassicas contain indoles, which can inhibit FMO3 enzyme activity.
Foods to Include:
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- Fruits and Vegetables: A wide variety of fruits and vegetables should form the foundation of the diet.
- Grains and High-Fiber Foods: Choose grains and high-fiber foods carefully, considering that some whole grains may be richer in choline. Pay attention to odor symptoms while adding high fiber - less processed grains, while attempting to consume as much fiber in your diet as possible.
- Poultry: Chicken and turkey are generally better choices than red meats. 1 oz of poultry is usually the best choice. Only fresh water fish, such as Tilapia, is lower in choline content.
- Dairy: Milk should be consumed as tolerated. Some people have learned to live without dairy foods, and others love their cheese on bread, and milk or cream in their coffee. We see on choline food content charts that milk and coffee are higher in choline content. Yet, there are a lot of people who feel deprived without them, and usually succumb to the temptation to consume them. Non-odorous cheeses, such as crumbly white cheeses like Greek Feta are best, depending on individual reactions.
Meal Planning and Recipe Ideas
- Breakfast: Consider options like fruit smoothies, low-choline cereals with milk alternatives, or toast with fruit spread.
- Lunch: Opt for salads with grilled chicken or turkey, vegetable soups, or sandwiches with low-choline fillings.
- Dinner: Explore recipes featuring chicken or turkey with rice or potatoes and a variety of vegetables. Some tasty protein options: Spaghetti with ground turkey and small amount of beef (optional) in lots of tomato sauce. Breaded chicken breasts, dipping the thin cut chicken breast or chicken tenders in egg whites and bread crumbs or flour and fried in Canola oil. Chicken fricassee with chopped potatoes and/or rice(white or with yellow coloring using a dash of Achote or Annato (bijol in Spanish). Can substitute chicken breast or chicken tenders for ground turkey or ground chicken. Chop potatoes into small cubes. Saute small amount of green and red peppers strips with very small amount of onion. Season chicken tenders cubed with lemon pepper and salt and cook until almost done. Add cubed potatoes until tender. Add 2 TB of tomato paste with 1/2 cup (or more as needed) of water to make sauce (not too much), or 1 to 2 can(s) of tomato sauce. If tolerated, 1/4 cup or less of red wine for flavor(optional). Cook long enough for sulfur in onion and wine to evaporate, and it's done. Cook rice on the side, can add yellow coloring to the rice (optional). Chicken tempura. Tacos with the chicken fricassee with chopped up tomatoes and lettuce.
- Snacks: Choose fruits, vegetables, or small portions of low-choline snacks. Some tasty fruits and vegetable meals and snack options: Vegetable tempura with carrots, squash, and zuccini.
Additional Considerations
- Hydration: Staying well-hydrated is essential for overall health and may help to dilute TMA in bodily fluids.
- Cooking Methods: Experiment with different cooking methods to enhance the flavor of low-choline foods.
- Food Journaling: Keeping a food journal can help identify trigger foods and patterns in symptom severity.
Beyond Diet: Additional Management Strategies
While diet is crucial, other strategies can help manage TMAU symptoms:
- Hygiene: Use acid soaps and body lotions to remove secreted trimethylamine by washing.
- Supplements: Consider activated charcoal and copper chlorophyllin to sequester trimethylamine produced in the gut. Taking riboflavin (vitamin B2) supplements to enhance any residual FMO3 enzyme activity.
- Medications: In some cases, antibiotics (metronidazole, amoxicillin, and neomycin) may be used to suppress the production of trimethylamine by reducing bacteria in the gut.
- Avoidance of Triggers: Identify and avoid circumstances that promote sweating (e.g., exercise, stress, emotional upsets).
- Genetic Counseling: Genetic counseling can provide information about the inheritance pattern of TMAU and the risk of passing it on to future generations.
Living with TMAU: Support and Resources
Living with TMAU can be challenging, both physically and emotionally. It is important to seek support from healthcare professionals, support groups, and online communities. Connecting with others who understand the condition can provide valuable emotional support and practical advice.
A Cookbook for TMAU-Friendly Recipes
As a testament to the supportive community surrounding TMAU, resources like "Cooking with Care: A Dietary Cookbook" offer TMAU-friendly recipes, encompassing a diverse range of food and flavors while adhering to a low-choline diet. These recipes are categorized into Breakfast, Lunch, Snacks & Sides, and Dinner for convenience.
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tags: #TMAU #diet #recommendations