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Eating Tips for Fabry Disease

Reviewed by Brunilda Nazario, MD on April 19, 2021

If you have Fabry disease, changing what you eat may help you manage some of your symptoms. Good nutrition will also benefit your heart and kidneys.

There isn't a specific Fabry disease diet. You'll need to work with your doctor and a registered dietitian to find a balanced plan. They'll pick one based on your symptoms and overall health. The goal is to help you eat comfortably while getting all the nutrition you need.

Go Easy on the Sodium

Over time, untreated Fabry disease can damage your kidneys. You may not filter out sodium very well. That's a mineral in salt that affects how your body holds on to fluids. If you can't get rid of it, you can end up with other health problems, such as high blood pressure, body swelling, or shortness of breath.

Ask your doctor how much sodium is right for you. You may not need to cut back too much if your kidneys work well.

If you do need to stick to a kidney-friendly diet, aim for less than 2,000 milligrams of sodium a day. Try these tips to stay on track:

  • Buy fresh food
  • Cook for yourself
  • Use non-salt spices, like herbs or sodium-free seasoning
  • Rinse canned food with water (vegetables, beans, meats, fish)

Talk to your doctor about salt substitutes before trying them.

Check the Nutrition Facts label to see how much sodium a product has. Pick foods with a 5% daily value or less.

Get the Right Amount of Protein

Ask your health care team how much protein you need. They'll consider things like your age, body size, and how well your kidneys work. You may not need to limit your protein too much if your Fabry disease is stable.

Talk to your doctor if you're on dialysis. Your protein needs will be different. If you're not on dialysis, follow these healthy tips:

Get protein from plants. Experts used to think vegetables couldn't give you all of your essential amino acids -- the "building blocks" of protein. But that's not the case anymore. Studies show that plant-based proteins put less strain on your kidneys when compared to animal-based foods.

Some options include:

  • Tempeh and tofu
  • Beans and legumes
  • Seeds
  • Unsalted nuts or nut butters
  • Grains like quinoa or brown rice

Eat lean meats. Try skinless chicken, turkey, or 95% lean ground beef.

Choose Heart-Healthy Options

Heart-healthy foods lower your chances of stroke, heart disease, and kidney failure. That's good news if you have Fabry disease. Ask your doctor about anti-inflammatory, Mediterranean, or DASH diets. But you don't need to follow a specific plan to see benefits.

Continued

You'll want to include more of these:

  • Fruits and vegetables
  • Olive oil
  • Whole grains, like oats and brown rice
  • Beans and legumes
  • Fat-free or low-fat dairy
  • Fatty fish (such as salmon or tuna)
  • Fortified non-dairy milks

Skip or limit foods high in saturated fat and added sugar, such as:

  • Ultra-processed foods like cake, cookies, and chips
  • Sugary drinks, including juice and soda
  • Refined carbohydrates like white bread or white rice
  • Red or processed meat

Change How You Eat

Stomach problems are common with Fabry disease. Digestive issues may be the first symptoms you get. That includes belly pain and diarrhea. You may get really full after you eat a small bit of food. 

Your doctor or dietitian may give you medicine to ease some of your symptoms. But they may also suggest you try these tips:

Pay attention to your portions. Your stomach might not swell as much if you eat less food at once. Try to eat 4-6 small meals throughout the day. See how you feel when you only eat 1 to 1.5 cups of food.

Continued

Follow a routine. Once you find an eating plan that works for you, stick to it. Sudden diet changes can make your diarrhea worse. And you may get constipation if you don't eat on a regular basis.

Try a low-FODMAP diet. You may have a hard time digesting certain carbohydrates. These are called FODMAPs. They're found in foods like wheat, dairy, and some fruits and vegetables. Talk to your dietitian if you want to try a low-FODMAP diet.

Avoid your trigger foods. You'll need to figure out which ingredients make your symptoms worse. But you may want to limit:

  • High-fat food
  • Caffeine
  • Alcohol
  • Spicy food

When to Talk to Your Doctor

You may not get enough vitamins and minerals if you have diarrhea often or you can't eat much. Your doctor can check if you're low on any nutrients and suggest ways to boost your levels.

Small changes in your diet can make a big difference. But you should tell your doctor if your digestive symptoms don't get better. They may need to run some tests to find out if something else going on.

WebMD Medical Reference

Sources

SOURCES:

Pramod Mistry, MBBS, PhD, FRCP, professor of medicine and of pediatrics; professor of cellular & molecular physiology; director of Yale Lysosomal Disease Center and Gaucher Disease Treatment Center; Yale School of Medicine.

Nutrition: "Could nutritional therapy take us further in our approaches to Fabry disease?"

National Kidney Foundation: "Fabry Disease," "CKD Diet: How much protein is the right amount?" "What is a Plant-Based Diet, and Is It Good For Your Kidneys?"

UNC Kidney Center: "The Renal Diet: Sodium/Salt."

Nutrients: "Vegetable-Based Diets for Chronic Kidney Disease? It Is Time to Reconsider."

Current Opinion in Nephrology and Hypertension: "Plant-based diets for prevention and management of chronic kidney disease."

National Heart, Lung, and Blood Institute: "Heart-Healthy Living."

American Heart Association: "What is the Mediterranean Diet?"

National Institute of Neurological Disorders and Stroke: "Fabry Disease Information Page."

JAMA Internal Medicine: "Associations of Processed meat, Unprocessed Red Meat, Poultry, or Fish Intake With Incident Cardiovascular Disease and All-Cause Mortality."

Michigan Medicine: "Castroparessis Nutrition Therapy."

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