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Fabry disease diet. Is there a diet which improves the quality of life of people with Fabry disease?

Are you aware of a diet that can improve the quality of life of people with Fabry disease? Is there a diet that is suggested to avoid when having Fabry disease? See if there is a diet that can improve the quality of life of people with Fabry disease, recommended and to avoid food when having Fabry disease

Fabry disease diet

Fabry Disease Diet: Improving Quality of Life


Fabry disease is a rare genetic disorder that affects the body's ability to break down a specific type of fat called globotriaosylceramide (GL-3). This buildup of GL-3 can lead to various symptoms, including pain, kidney problems, heart complications, and skin issues. While there is no cure for Fabry disease, adopting a healthy diet can help improve the quality of life for individuals with this condition.



The Importance of a Balanced Diet


A balanced diet plays a crucial role in managing Fabry disease. It can help minimize symptoms, support overall health, and enhance the effectiveness of other treatments. Here are some key dietary considerations for individuals with Fabry disease:



1. Low-Fat Diet


Since Fabry disease involves the accumulation of fat in the body, it is important to follow a low-fat diet. Limiting the intake of saturated and trans fats can help reduce the burden on the body and potentially slow down disease progression. Focus on consuming lean sources of protein, such as poultry, fish, legumes, and tofu. Incorporate healthy fats from sources like avocados, nuts, and seeds.



2. Low-Sodium Diet


Many individuals with Fabry disease experience kidney problems, and a low-sodium diet can help manage blood pressure and reduce fluid retention. Avoid processed and packaged foods, as they tend to be high in sodium. Instead, opt for fresh fruits, vegetables, whole grains, and herbs and spices to add flavor to your meals.



3. Adequate Hydration


Staying well-hydrated is essential for individuals with Fabry disease, as it can help prevent dehydration and support kidney function. Aim to drink at least 8 cups (64 ounces) of water per day, or more if recommended by your healthcare provider. Avoid excessive consumption of sugary beverages and alcohol, as they can contribute to weight gain and other health issues.



4. Fiber-Rich Foods


A diet rich in fiber can help regulate bowel movements and prevent constipation, which is a common symptom in Fabry disease. Include plenty of fruits, vegetables, whole grains, and legumes in your diet to ensure an adequate intake of dietary fiber. This can also help maintain a healthy weight and support overall digestive health.



5. Antioxidant-Rich Foods


Antioxidants help protect the body against oxidative stress, which is believed to play a role in Fabry disease. Include a variety of colorful fruits and vegetables in your diet, as they are rich in antioxidants. Berries, leafy greens, citrus fruits, and cruciferous vegetables like broccoli and cauliflower are excellent choices.



6. Regular Meal Patterns


Establishing regular meal patterns can help manage symptoms and maintain stable blood sugar levels. Aim to have three balanced meals and two to three snacks throughout the day. This can help prevent energy dips, stabilize metabolism, and support overall well-being.



Consulting a Healthcare Professional


While adopting a healthy diet is beneficial for individuals with Fabry disease, it is important to consult with a healthcare professional or a registered dietitian before making any significant dietary changes. They can provide personalized recommendations based on your specific needs, medical history, and current treatment plan.



Disclaimer: The information provided here is for informational purposes only and should not replace professional medical advice. Always consult with a healthcare professional before making any dietary changes or starting a new treatment plan.


Diseasemaps
2 answers
I'm a 38 year old male with erythomelalgia disease and hypohidrosis caused by Fabry Disease and I have Chronic Kidney Disease so the following diet addresses both Fabry and CKD so if you don't have CKD you don't need to be as strict with protein or minerals. I have found a reduction in both severity and frequency of burning (erythomelalgia) using various versions of this diet. A Fabry diet, in my opinion, is one where the chemical bonds in the food don't join fats and carbs because Fabry Disease means the person can't break this bond because they either don't have, don't have enough or the Alph-galactosidase enzymes they do have are improperly folded. A CKD diet means it is low in protein and minerals, particularly sodium (salt), potassium, and phosphorus.

*This diet does contain some fat-carb bonds (ex. walnuts). In order to consume this 1/3 cup of walnuts I eat 5 Beano pills (Walmart and Target have their own brand for this pill). This pill, as far as I know, is the enzyme my body needs to break this fat-carb bond.

*If I want to eat off diet I compare the fat and carbs on the nutrition label and whichever is lower I consume 1 Beano pill per gram. If the nutrition label is unavailable I eat 1 pill per bite but Warning: too much Beano (2 pills per bite) has given me an upset stomach and diarrhea while not enough can cause a continuation of nerve damage which causes my erythomelalgia disease and hypohidrosis, so clearly it is better to eat based on the nutrition label.

Example Fabry/CKD Diet: a place to start
Breakfast: Eggs with onion powder, garlic powder, cracked black pepper, and low sodium ketchup.
1st Snack: 1/3 cup walnuts with 5 Beano pills
Drink: flavored sparkling water
1st Lunch: 1 cup steamed white rice, 1 tablespoon extra virgin olive oil, 1 tablespoon balsamic vinegar, onion powder, low sodium ketchup, curry powder (for flavor)
1st Dessert: 1/2 cup pears in juice with 2 tablespoons of benefiber (or store equivalent)
2nd Snack: 1/3 cup almonds with 1 Beano for every 3 almonds
2nd Lunch: 1 cup steamed white rice, 1 tablespoon extra virgin olive oil, 1 tablespoon balsamic vinegar, onion powder, low sodium ketchup, curry powder (for flavor)
2nd Dessert: cherry pie filling (1/3 cup)
Dinner: 1/3 cup walnuts with 5 Beano pills

Vitamins:
K-2 100 mg (1 pill per day)
Vitamin A 2500 mg (1 pill per week)
ProRenal + D, Multivitamin with Omega 3 (2 pills per day)
Vitamin C 250 mg (1 pill per week)
Vitamin D 1000 IU (1 pill per day)

*I switch pie filling and fruit deserts to make the diet more interesting.
*I switch EVOO for Mayo sometimes (read the label to ensure it has no carbs and is low sodium)
*Eat Beano before eating food requiring Beano. Warning: Beano nor any equivalent have endorsed these statements nor have there been any tests, to the authors knowledge, to prove or disprove these claims.
*Results should be a direct relationship with how strictly you follow these guidelines (this diet) so the primary aspect of success to living with Fabry Disease is self control / discipline.

*References:
https://cronometer.com
https://nutritionfacts.org
https://nutritiondata.self.com

Warning: the information provided is anecdotal and has not been evaluated by a doctor. Individual results may vary. Please consult with your physician(s) prior trying any thing mentioned.

Posted Jul 1, 2020 by Fabry Father 1100

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